Overview of Cardiomyopathy
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Questions and Answers

What is a common consequence of cardiomyopathy?

  • Increased heart rate
  • Enhanced heart valve function
  • Improved blood circulation
  • Enlarged, thickened, or rigid heart (correct)

    • Which classification of cardiomyopathy is based on genetic factors?

  • Inherited cardiomyopathy (correct)
  • Secondary cardiomyopathy
  • Acquired cardiomyopathy
  • Functional cardiomyopathy

    • What can be a result of the heart's inability to function normally due to cardiomyopathy?

  • Enhanced heart performance
  • Heart failure (correct)
  • Increased blood pressure
  • Decreased heart weight

    • Which of the following is NOT a characteristic of cardiomyopathy?

    <p>Thinning of the heart walls</p> Signup and view all the answers

    What distinguishes acquired cardiomyopathy from inherited cardiomyopathy?

    <p>Acquired cardiomyopathy is caused by other diseases or conditions</p> Signup and view all the answers

    What type of cardiomyopathy is characterized by the thickening of the heart muscle, often leading to obstruction of blood flow?

    <p>Hypertrophic cardiomyopathy</p> Signup and view all the answers

    Which subset of cardiomyopathy is primarily related to the accumulation of amyloid protein deposits?

    <p>Transthyretin amyloid cardiomyopathy</p> Signup and view all the answers

    Which type of cardiomyopathy leads to an enlarged heart with impaired contraction and is the most common form?

    <p>Dilated cardiomyopathy</p> Signup and view all the answers

    Which type of cardiomyopathy is often seen with abnormal electrical activity in the right ventricle?

    <p>Arrhythmogenic right-ventricular dysplasia</p> Signup and view all the answers

    Which classification of cardiomyopathy is identified specifically due to the inability of the heart to fill with blood properly?

    <p>Restrictive cardiomyopathy</p> Signup and view all the answers

    What is a primary risk factor for developing dilated cardiomyopathy?

    <p>Exposure to certain toxins</p> Signup and view all the answers

    Which clinical manifestation is NOT typically associated with dilated cardiomyopathy?

    <p>Jaundice</p> Signup and view all the answers

    At what age range is dilated cardiomyopathy most commonly diagnosed?

    <p>Ages 20 to 60</p> Signup and view all the answers

    Which statement is true regarding the inheritance of dilated cardiomyopathy?

    <p>One-third of individuals inherit the disease.</p> Signup and view all the answers

    Which factor may lead to reversible dilated cardiomyopathy?

    <p>Chemotherapeutic drugs</p> Signup and view all the answers

    What is a primary consequence of hypertrophic cardiomyopathy on the heart's function?

    <p>Decreased ability to pump blood effectively</p> Signup and view all the answers

    Which of the following describes a characteristic feature of hypertrophic cardiomyopathy?

    <p>Asymmetric septal hypertrophy</p> Signup and view all the answers

    What impact does hypertrophic cardiomyopathy have on ventricular filling?

    <p>Leads to small end-diastolic volumes</p> Signup and view all the answers

    How is hypertrophic cardiomyopathy most commonly transmitted?

    <p>Via an inherited genetic mutation</p> Signup and view all the answers

    Which of the following complications is associated with hypertrophic cardiomyopathy?

    <p>Impaired ventricular outflow due to septal enlargement</p> Signup and view all the answers

    What is a main characteristic of restrictive cardiomyopathy?

    <p>Rigid ventricular walls</p> Signup and view all the answers

    Which of the following is NOT a common cause of restrictive cardiomyopathy?

    <p>Coronary artery disease</p> Signup and view all the answers

    What condition is typically not affected in restrictive cardiomyopathy?

    <p>Ejection fraction</p> Signup and view all the answers

    What therapeutic approach is primarily used in managing restrictive cardiomyopathy?

    <p>Symptomatic and supportive care</p> Signup and view all the answers

    What is a result of decreased ventricular compliance in restrictive cardiomyopathy?

    <p>Diastolic dysfunction and heart failure</p> Signup and view all the answers

    What is primarily replaced in the heart muscle of patients with arrhythmogenic right-ventricular dysplasia (ARVD)?

    <p>Muscle tissue with fatty and fibrous tissue</p> Signup and view all the answers

    Which of the following conditions is most commonly associated with arrhythmogenic right-ventricular dysplasia in athletes?

    <p>Ventricular tachyarrhythmias and sudden death</p> Signup and view all the answers

    What is a key factor that influences the hereditary nature of arrhythmogenic right-ventricular dysplasia?

    <p>Genetic mutations of desmosomal proteins</p> Signup and view all the answers

    How is ARVD classified in terms of severity?

    <p>Mild to severe</p> Signup and view all the answers

    What is a recommended action for family members of patients diagnosed with ARVD?

    <p>Undergo regular screening for signs and symptoms</p> Signup and view all the answers

    Which risk factor is NOT typically associated with transthyretin amyloid cardiomyopathy (ATTR-CM)?

    <p>Recent viral infection</p> Signup and view all the answers

    What is the primary effect of the transthyretin mutation in ATTR-CM?

    <p>Abnormal assembly of the transthyretin protein</p> Signup and view all the answers

    Which statement about tafamidis (Vyndamax) is true?

    <p>It is a transthyretin stabilizer approved by the FDA.</p> Signup and view all the answers

    Which of the following conditions is ATTR-CM often misdiagnosed as?

    <p>Hypertensive heart failure</p> Signup and view all the answers

    What demographic is more likely to develop wild type ATTR-CM?

    <p>Individuals over 65 years old</p> Signup and view all the answers

    Which condition is an example of unclassified cardiomyopathy?

    <p>Left-ventricular noncompaction</p> Signup and view all the answers

    What commonly triggers Takotsubo cardiomyopathy?

    <p>Extreme stress</p> Signup and view all the answers

    What defines unclassified cardiomyopathy?

    <p>It cannot be categorized under known types like dilated or hypertrophic.</p> Signup and view all the answers

    Which of the following is NOT a cause of unclassified cardiomyopathy?

    <p>Amyloidosis</p> Signup and view all the answers

    Which statement best describes one characteristic of unclassified cardiomyopathy?

    <p>Its causes and characteristics vary significantly.</p> Signup and view all the answers

    Which factor is NOT considered a risk factor for developing cardiomyopathy?

    <p>High cholesterol levels</p> Signup and view all the answers

    What lifestyle change is most recommended to reduce the risk of complications related to cardiomyopathy?

    <p>Engaging in regular exercise</p> Signup and view all the answers

    What is a primary preventive measure to avoid conditions leading to cardiomyopathy?

    <p>Minimizing stress</p> Signup and view all the answers

    Which of the following practices is least likely to promote a heart-healthy lifestyle?

    <p>Drinking excessive alcohol</p> Signup and view all the answers

    Which of the following is NOT a commonly advised practice for heart health?

    <p>Consuming high-sugar foods</p> Signup and view all the answers

    What symptom is most commonly associated with dilated cardiomyopathy as it progresses?

    <p>Dysrhythmias leading to sudden death</p> Signup and view all the answers

    Which symptom is more indicative of hypertrophic cardiomyopathy compared to other types?

    <p>Ejection murmur</p> Signup and view all the answers

    In restrictive cardiomyopathy, which of the following symptoms is less common?

    <p>Chest pain during rest</p> Signup and view all the answers

    What is a notable clinical manifestation of arrhythmogenic right-ventricular dysplasia (ARVD)?

    <p>Dysrhythmias and conduction disturbances</p> Signup and view all the answers

    What typically triggers symptoms in patients with hypertrophic cardiomyopathy?

    <p>Increased physical exertion</p> Signup and view all the answers

    Which demographic is most likely to experience symptoms of transthyretin amyloid cardiomyopathy (ATTR-CM)?

    <p>Individuals with a family history</p> Signup and view all the answers

    What is a significant complication arising from hypertrophic cardiomyopathy?

    <p>Atrial fibrillation</p> Signup and view all the answers

    What common feature is associated with restrictive cardiomyopathy?

    <p>Elevated jugular venous pressure (JVP)</p> Signup and view all the answers

    Which medication may be used to manage dysrhythmias in cardiomyopathy patients?

    <p>Antidysrhythmics</p> Signup and view all the answers

    What clinical manifestation might suggest heart failure due to cardiomyopathy?

    <p>Paroxysmal nocturnal dyspnea</p> Signup and view all the answers

    Which nursing intervention can assist in improving venous return for patients with cardiomyopathy?

    <p>Using elastic stockings</p> Signup and view all the answers

    What can exacerbate angina in patients with cardiomyopathy?

    <p>Coronary artery abnormalities</p> Signup and view all the answers

    Which lifestyle adjustment is important for managing symptoms of cardiomyopathy?

    <p>Implementing regular periods of rest</p> Signup and view all the answers

    What is a common symptom that may indicate severe outflow tract obstruction leading to decreased blood flow to the brain?

    <p>Dizziness or lightheadedness</p> Signup and view all the answers

    Which medication is commonly prescribed to help manage symptoms associated with chest pain?

    <p>Nitrates</p> Signup and view all the answers

    What should be prioritized in patient education regarding the response to symptoms of cardiac distress?

    <p>Calling 9-1-1 when symptoms first occur</p> Signup and view all the answers

    Which of the following is an appropriate treatment option for managing symptoms of syncope?

    <p>Patient education about taking immediate action when symptoms arise</p> Signup and view all the answers

    What is the possible requirement for a patient with severely damaged coronary arteries?

    <p>Surgery to repair the damaged arteries</p> Signup and view all the answers

    What is a primary goal in the treatment of cardiomyopathies?

    <p>Manage signs and symptoms</p> Signup and view all the answers

    Which intervention is typically included in managing hypertrophic cardiomyopathy (HCM)?

    <p>Restricting strenuous physical exertion</p> Signup and view all the answers

    What dietary change may help alleviate symptoms of cardiomyopathies?

    <p>Implementing sodium restrictions</p> Signup and view all the answers

    What is a characteristic of treatment for arrhythmogenic right-ventricular dysplasia (ARVD)?

    <p>Focusing on lifestyle modifications to avoid exertion</p> Signup and view all the answers

    Which statement is true regarding the overall management strategy of cardiomyopathy treatments?

    <p>Both pharmacologic and surgical options may be used</p> Signup and view all the answers

    Which of the following diagnostic tests is specifically designed to assess the electrical activity of the heart?

    <p>Electrocardiography</p> Signup and view all the answers

    What is the primary purpose of hemodynamic studies in the diagnosis of cardiomyopathy?

    <p>To measure blood pressure and blood flow in the heart</p> Signup and view all the answers

    Which diagnostic tool helps detect blockages in coronary arteries?

    <p>Coronary angiography</p> Signup and view all the answers

    In diagnosing cardiomyopathy, genetic testing is primarily used for what purpose?

    <p>To identify specific genetic mutations linked to cardiomyopathy</p> Signup and view all the answers

    Which diagnostic method provides a comprehensive assessment of both heart structure and function?

    <p>Echocardiography</p> Signup and view all the answers

    Which drug is specifically used to help slow the heart rate, but is avoided in hypertrophic cardiomyopathy due to its effects?

    <p>Digoxin</p> Signup and view all the answers

    What is the primary role of anticoagulants in the treatment of cardiomyopathy?

    <p>To prevent thrombus formation</p> Signup and view all the answers

    Which class of drugs may be used to correct electrolyte imbalances in heart failure (HF) despite the potential increase in HF symptoms?

    <p>Antidysrhythmics</p> Signup and view all the answers

    Which medication specifically targets the underlying etiology of ATTR cardiomyopathy?

    <p>Tafamidis (Vyndamax)</p> Signup and view all the answers

    What common electrolyte issue might indicate dehydration or other health problems in patients with cardiomyopathy?

    <p>Hyponatremia</p> Signup and view all the answers

    What is the primary purpose of a left-ventricular assist device (LVAD)?

    <p>To aid in perfusion of blood throughout the body</p> Signup and view all the answers

    What is a key reason transplantation is not an option for restrictive cardiomyopathy (RCM) patients?

    <p>The underlying fibrosis process is not eliminated</p> Signup and view all the answers

    What is the primary goal during surgical ventricular remodeling?

    <p>To reduce the size of the left ventricle to improve function</p> Signup and view all the answers

    Which device may be inserted to maintain stable heart rhythm during certain cardiac procedures?

    <p>A pacemaker</p> Signup and view all the answers

    What is a common complication associated with cardiac transplantation?

    <p>Life-long risk of organ rejection</p> Signup and view all the answers

    What is the age group with the highest prevalence of dilated cardiomyopathy in pediatric patients?

    <p>Infants under 12 months</p> Signup and view all the answers

    Which form of cardiomyopathy accounts for the least percentage of diagnosed cases in children?

    <p>Restrictive cardiomyopathy</p> Signup and view all the answers

    What symptom is most likely to lead to a misdiagnosis in infants with dilated cardiomyopathy?

    <p>Pneumonia</p> Signup and view all the answers

    At what age is restrictive cardiomyopathy typically diagnosed?

    <p>Around 5 or 6 years old</p> Signup and view all the answers

    Which group of children is less likely to exhibit symptoms of hypertrophic cardiomyopathy during initial growth spurts?

    <p>Older children and adolescents</p> Signup and view all the answers

    Which of these factors may contribute to increased severity of symptoms in dilated cardiomyopathy caused by viral myocarditis?

    <p>Rapid onset of symptoms</p> Signup and view all the answers

    What is a common characteristic of dilated cardiomyopathy and hypertrophic cardiomyopathy in pediatric patients?

    <p>Absence of symptoms in early stages</p> Signup and view all the answers

    What percentage of cardiomyopathy cases in children is attributed to hypertrophic cardiomyopathy?

    <p>35-40%</p> Signup and view all the answers

    What is a common characteristic of peripartum cardiomyopathy (PPCM)?

    <p>It occurs during the last month of pregnancy or within the first 5 months postpartum.</p> Signup and view all the answers

    What factor is associated with an increased risk of developing PPCM?

    <p>Obesity and poor nourishment</p> Signup and view all the answers

    What is typically true regarding the ejection fraction in PPCM?

    <p>It is generally reduced below 45%.</p> Signup and view all the answers

    Which of the following statements about subsequent pregnancies in women with PPCM is accurate?

    <p>Women with permanent heart enlargement face high risks in subsequent pregnancies.</p> Signup and view all the answers

    What is a potential outcome of untreated PPCM?

    <p>It may necessitate heart transplantation in severe cases.</p> Signup and view all the answers

    Which type of cardiomyopathy is most commonly diagnosed in older adults?

    <p>Restrictive Cardiomyopathy</p> Signup and view all the answers

    In older adults, what is a contributing factor to the development of restrictive cardiomyopathy?

    <p>Progressive stiffness of the ventricles</p> Signup and view all the answers

    What condition might older adults be diagnosed with if heart failure does not have a defined cause?

    <p>Cardiomyopathy of Aging</p> Signup and view all the answers

    What is the usual prognosis of hypertrophic cardiomyopathy (HCM) in older patients compared to younger individuals?

    <p>Generally associated with benign outcomes</p> Signup and view all the answers

    What treatment approach is often not supported for older patients with hypertrophic cardiomyopathy?

    <p>Implantation of defibrillator devices</p> Signup and view all the answers

    What is one important lifestyle modification recommended for patients with cardiomyopathies?

    <p>Maintain a healthy weight</p> Signup and view all the answers

    Which piece of advice is essential for conserving energy during daily activities for patients with cardiomyopathy?

    <p>Alternate periods of activity with periods of rest</p> Signup and view all the answers

    Why are nitrates and other vasodilators generally avoided in patients with hypertrophic cardiomyopathy (HCM)?

    <p>They may worsen heart failure symptoms</p> Signup and view all the answers

    What role does patient and family education play in managing cardiomyopathy?

    <p>It is crucial for understanding and managing symptoms</p> Signup and view all the answers

    What is recommended for family members of patients diagnosed with certain types of cardiomyopathy?

    <p>Initiate genetic counseling to assess familial risk</p> Signup and view all the answers

    What type of data should the nurse collect when assessing a patient with cardiomyopathy?

    <p>Both subjective and objective data</p> Signup and view all the answers

    Which symptom is important for the nurse to assess in relation to activity tolerance in a patient with cardiomyopathy?

    <p>Increased shortness of breath</p> Signup and view all the answers

    During the physical examination, which vital sign assessment is crucial for patients with cardiomyopathy?

    <p>Apical and peripheral pulses</p> Signup and view all the answers

    What finding might indicate worsening heart failure in a patient with cardiomyopathy during assessment?

    <p>Presence of edema</p> Signup and view all the answers

    Which information is relevant for understanding a patient’s cardiomyopathy risk during assessment?

    <p>Family history of cardiomyopathy</p> Signup and view all the answers

    Which of the following is a common diagnosis symptom of cardiomyopathy?

    <p>Fluid volume excess</p> Signup and view all the answers

    What is a key goal for patients managing cardiomyopathy?

    <p>Maintain blood pressure within specified limits</p> Signup and view all the answers

    Which nursing action is essential when caring for a patient with cardiomyopathy?

    <p>Monitoring for subtle changes in vital signs</p> Signup and view all the answers

    Which lifestyle modification is recommended for patients with cardiomyopathy?

    <p>Adjust lifestyle to accommodate reduced activity levels</p> Signup and view all the answers

    What should be included in patient teaching for those managing cardiomyopathy?

    <p>Importance of regular monitoring of vital signs</p> Signup and view all the answers

    Which nursing intervention is effective in reducing cardiac workload for a patient experiencing heart failure?

    <p>Provide a bedside commode</p> Signup and view all the answers

    What physiological change is commonly observed in patients with heart failure due to sympathetic nervous system stimulation?

    <p>Tachycardia</p> Signup and view all the answers

    What is the purpose of monitoring brain natriuretic peptide levels in patients with heart failure?

    <p>As an indicator of heart failure severity</p> Signup and view all the answers

    Which of the following is a common auscultation finding in early heart failure?

    <p>Ventricular gallop</p> Signup and view all the answers

    Which intervention should be prioritized when a patient with heart failure is suspected of experiencing hypoxia?

    <p>Provide oxygen therapy as prescribed</p> Signup and view all the answers

    What intervention can be taken to address dry mouth and thirst in patients with fluid volume excess?

    <p>Offer ice chips and frequent mouth care</p> Signup and view all the answers

    What is considered a significant change in urine output that should prompt notification to the healthcare provider?

    <p>Urine output less than 30 mL/hr</p> Signup and view all the answers

    How often should patient weights be measured for monitoring fluid status?

    <p>At the same time each day, after voiding</p> Signup and view all the answers

    What does a decline in respiratory status potentially indicate in patients with heart failure?

    <p>Worsening left heart failure</p> Signup and view all the answers

    Which measure should be taken to assess the extent of fluid retention in patients with heart failure?

    <p>Daily measurement of abdominal girth</p> Signup and view all the answers

    Which nursing intervention is most appropriate for improving activity tolerance in patients with heart failure?

    <p>Provide written and verbal information regarding expected activity level.</p> Signup and view all the answers

    What is the primary goal of using passive and active range-of-motion exercises in heart failure patients?

    <p>To prevent complications of immobility and maintain muscle elasticity.</p> Signup and view all the answers

    Why is it important to promote frequent rest periods for patients performing activities of daily living (ADLs)?

    <p>To prevent overwhelming the patient and reduce cardiac workload.</p> Signup and view all the answers

    When planning a progressive activity program for a heart failure patient, what is one of the most crucial considerations?

    <p>Increasing exercise capacity slowly while monitoring for signs of strain.</p> Signup and view all the answers

    What impact does encouraging independence while assisting with ADLs have on heart failure patients?

    <p>It promotes a sense of control and may reduce feelings of helplessness.</p> Signup and view all the answers

    What is a key goal of discussing sodium restrictions with a patient on a low-sodium diet?

    <p>To promote adherence to the prescribed diet.</p> Signup and view all the answers

    Which of the following should be given to a patient to help with a low-sodium diet?

    <p>A list of high-sodium, high-fat, and high-cholesterol foods to avoid.</p> Signup and view all the answers

    What is the importance of consulting with a dietitian when implementing a low-sodium diet?

    <p>To ensure dietary needs for weight control and sodium restrictions are met.</p> Signup and view all the answers

    Which outcome suggests successful adherence to a low-sodium diet?

    <p>The patient articulates the benefits of a low-sodium diet.</p> Signup and view all the answers

    Which nursing action is appropriate if a patient's outcomes for a low-sodium diet are not met?

    <p>Refer the patient to a healthcare professional specializing in cardiomyopathies.</p> Signup and view all the answers

    What is the primary action of digoxin in heart failure management?

    <p>To slow heart rate</p> Signup and view all the answers

    What makes tafamidis (Vyndamax) unique in the treatment of transthyretin amyloid cardiomyopathy?

    <p>It is a transthyretin stabilizer</p> Signup and view all the answers

    Which medication is commonly used to manage angina by dilating blood vessels?

    <p>Nitrates</p> Signup and view all the answers

    Why should digoxin be avoided in patients with hypertrophic cardiomyopathy (HCM)?

    <p>It may worsen hypercontractility</p> Signup and view all the answers

    What is the recommended daily dose of tafamidis (Vyndamax) for treating ATTR-CM?

    <p>61 mg</p> Signup and view all the answers

    What is a common symptom of restrictive cardiomyopathy?

    <p>Peripheral edema</p> Signup and view all the answers

    Which treatment option is generally not effective for restrictive cardiomyopathy?

    <p>Cardiac transplantation</p> Signup and view all the answers

    What is a notable risk associated with hypertrophic cardiomyopathy, especially in young athletes?

    <p>Syncope during exertion</p> Signup and view all the answers

    In patients with arrhythmogenic right-ventricular dysplasia (ARVD), what is a potential initial symptom?

    <p>Sudden cardiac death</p> Signup and view all the answers

    Which condition primarily leads to stiffening of the myocardium due to protein buildup?

    <p>Transthyretin amyloid cardiomyopathy</p> Signup and view all the answers

    What is a common genetic characteristic of hypertrophic cardiomyopathy?

    <p>About 50% of cases are inherited</p> Signup and view all the answers

    Which type of cardiomyopathy is least likely to cause ventricular dilation?

    <p>Restrictive cardiomyopathy</p> Signup and view all the answers

    What precautions should be taken with medications in patients with hypertrophic cardiomyopathy?

    <p>Avoid Digoxin and nitrates</p> Signup and view all the answers

    What lifestyle factor is associated with an increased risk of dilated cardiomyopathy?

    <p>Alcohol abuse</p> Signup and view all the answers

    Which of the following conditions is commonly associated with restrictive cardiomyopathy?

    <p>Amyloidosis</p> Signup and view all the answers

    At what age is hypertrophic cardiomyopathy most frequently diagnosed?

    <p>Infancy or adolescence</p> Signup and view all the answers

    What is a common risk factor for peripartum cardiomyopathy?

    <p>Age over 30</p> Signup and view all the answers

    Which demographic is more likely to be affected by transthyretin amyloid cardiomyopathy?

    <p>Individuals over 65</p> Signup and view all the answers

    Which of the following is a potential consequence of vigorous physical activity for those at risk of hypertrophic cardiomyopathy?

    <p>Sudden cardiac death</p> Signup and view all the answers

    What condition is stress most commonly associated with in regards to cardiomyopathy?

    <p>Takotsubo cardiomyopathy</p> Signup and view all the answers

    Which symptom is most associated with fluid retention in cardiomyopathy patients?

    <p>Weight changes</p> Signup and view all the answers

    What physical examination finding is indicative of fluid overload in a patient with cardiomyopathy?

    <p>Jugular vein distension</p> Signup and view all the answers

    Which lifestyle factor should be assessed due to its potential impact on cardiac health?

    <p>Alcohol or drug use</p> Signup and view all the answers

    Which sign observed during a physical examination could indicate poor perfusion?

    <p>Cyanosis of the skin</p> Signup and view all the answers

    What might the presence of paroxysmal nocturnal dyspnea indicate in a cardiomyopathy patient?

    <p>Fluid overload</p> Signup and view all the answers

    What specific aspect of heart sounds could indicate heart failure during examination?

    <p>Presence of S3 heart sound</p> Signup and view all the answers

    Which lab test is critical for assessing heart failure severity in cardiomyopathy patients?

    <p>Brain natriuretic peptide (BNP) levels</p> Signup and view all the answers

    What finding could suggest decreased peripheral circulation during examination?

    <p>Delayed capillary refill time</p> Signup and view all the answers

    What is a key finding associated with a myocardial biopsy in patients with dilated cardiomyopathy?

    <p>Fibrosis and scarring</p> Signup and view all the answers

    Which genetic mutations are most commonly linked to hypertrophic cardiomyopathy?

    <p>Mutations in sarcomere proteins</p> Signup and view all the answers

    What does an elevated level of BNP or NT-proBNP indicate regarding heart function?

    <p>Severity of heart failure</p> Signup and view all the answers

    In patients with restrictive cardiomyopathy, what laboratory finding is typically observed?

    <p>Elevated myocardial biopsy findings for amyloid deposits</p> Signup and view all the answers

    Which of the following is an identifying feature of arrhythmogenic right-ventricular dysplasia (ARVD)?

    <p>Replacement of heart muscle with fibro-fatty tissue</p> Signup and view all the answers

    What key finding is indicative of hypertrophic cardiomyopathy when analyzing an electrocardiogram?

    <p>Left ventricular hypertrophy</p> Signup and view all the answers

    Which test is primarily used for assessing the presence of tachyarrhythmias that may not be captured in a single ECG?

    <p>Ambulatory ECG monitoring</p> Signup and view all the answers

    What is a significant finding in chest X-ray that indicates heart failure?

    <p>Pleural effusions</p> Signup and view all the answers

    In hemodynamic studies, which measurement suggests left-sided heart failure?

    <p>Increased pulmonary capillary wedge pressure</p> Signup and view all the answers

    What abnormal finding on echocardiography suggests the presence of restrictive cardiomyopathy?

    <p>Ventricular stiffness</p> Signup and view all the answers

    Which radionuclear scan result confirms reduced pumping ability in dilated cardiomyopathy?

    <p>Low ejection fraction</p> Signup and view all the answers

    What finding in cardiac stress testing indicates inadequate blood flow, often linked to hypertrophic cardiomyopathy?

    <p>Failure to increase blood pressure</p> Signup and view all the answers

    Which term describes the imaging result of cardiomegaly seen on chest X-ray in cases of dilated cardiomyopathy?

    <p>Enlargement of the heart</p> Signup and view all the answers

    What does a myocardial biopsy typically reveal in cases of Dilated Cardiomyopathy (DCM)?

    <p>Fibrosis in cardiac muscle tissue</p> Signup and view all the answers

    Which level of Brain Natriuretic Peptide (BNP) is indicative of severe heart failure?

    <blockquote> <p>900 pg/mL</p> </blockquote> Signup and view all the answers

    Which mutation is associated with Hypertrophic Cardiomyopathy (HCM)?

    <p>Sarcomere mutation</p> Signup and view all the answers

    What would a normal genetic test for cardiomyopathy indicate?

    <p>No mutations linked to cardiomyopathy</p> Signup and view all the answers

    In Restrictive Cardiomyopathy (RCM), what finding is indicated by high ventricular filling pressures?

    <p>Restrictive filling of the ventricles</p> Signup and view all the answers

    What ejection fraction indicates severely reduced heart function in advanced dilated cardiomyopathy?

    <p>&lt; 30%</p> Signup and view all the answers

    What finding on a chest X-ray indicates cardiomegaly?

    <p>Cardiothoracic ratio &gt; 50%</p> Signup and view all the answers

    Which abnormal finding on an ECG might indicate prior myocardial injury?

    <p>Abnormal Q waves</p> Signup and view all the answers

    What is considered a normal pulmonary capillary wedge pressure (PCWP)?

    <p>6-12 mmHg</p> Signup and view all the answers

    What abnormal response would indicate limited cardiac reserve during a cardiac stress test?

    <p>Unchanged blood pressure despite exertion</p> Signup and view all the answers

    Which finding may suggest atrial fibrillation or flutter on an ECG?

    <p>Irregular rhythm</p> Signup and view all the answers

    What is a typical finding in advanced cardiomyopathy involving low cardiac output?

    <p>Elevated right atrial pressure</p> Signup and view all the answers

    What does an ejection fraction less than 50% indicate during a radionuclear scan?

    <p>Impaired ventricular function</p> Signup and view all the answers

    What is the purpose of energy conservation techniques in managing cardiomyopathy?

    <p>To minimize cardiac workload during daily activities.</p> Signup and view all the answers

    Which of the following interventions is indicated for patients with volume overload and heart failure?

    <p>Fluid Restriction</p> Signup and view all the answers

    What is the main goal of dietary consultation in managing cardiomyopathy?

    <p>To create individualized meal plans focusing on fat and cholesterol reduction.</p> Signup and view all the answers

    Which intervention focuses on enhancing strength without overexerting patients with cardiomyopathy?

    <p>Physical Therapy</p> Signup and view all the answers

    Which of the following is an essential aspect of patient education on symptom management for cardiomyopathy?

    <p>Recognizing and responding to symptoms like shortness of breath.</p> Signup and view all the answers

    What is a primary purpose of genetic counseling in cardiomyopathy management?

    <p>To identify hereditary risks for familial conditions.</p> Signup and view all the answers

    What is a common non-pharmacologic intervention for managing cardiomyopathy related to fluid volume?

    <p>Low-sodium diet</p> Signup and view all the answers

    What aspect of mental health support is particularly beneficial for patients with cardiomyopathy?

    <p>It offers emotional support for coping with chronic illness.</p> Signup and view all the answers

    What is the primary purpose of a Left-Ventricular Assist Device (LVAD)?

    <p>To aid in perfusion of blood throughout the body</p> Signup and view all the answers

    Which of the following medications is specifically avoided in hypertrophic cardiomyopathy (HCM) due to the risk of hypercontractility?

    <p>Digoxin</p> Signup and view all the answers

    What is a primary indication for cardiac transplantation?

    <p>Advanced dilated cardiomyopathy unresponsive to treatment</p> Signup and view all the answers

    Which statement correctly describes the role of anticoagulants in the management of cardiomyopathy?

    <p>They prevent blood clots and reduce thromboembolism risk</p> Signup and view all the answers

    Which medication class is primarily used to manage symptoms of fluid overload in heart failure?

    <p>Diuretics</p> Signup and view all the answers

    For which condition is tafamidis (Vyndamax) indicated?

    <p>Transthyretin amyloid cardiomyopathy (ATTR-CM)</p> Signup and view all the answers

    What is a typical consideration for patients undergoing cardiac transplantation?

    <p>Lifelong immunosuppression to prevent organ rejection</p> Signup and view all the answers

    Which procedure aims to reduce the size of the left ventricle in dilated cardiomyopathy?

    <p>Surgical ventricular remodeling</p> Signup and view all the answers

    What is the primary mechanism of action of ACE inhibitors in the treatment of cardiomyopathy?

    <p>Block conversion of angiotensin I to angiotensin II</p> Signup and view all the answers

    Which medication class is contraindicated in asthma due to its mechanism of action?

    <p>Beta-Blockers</p> Signup and view all the answers

    What should be monitored closely when a patient is prescribed loop diuretics?

    <p>Potassium levels</p> Signup and view all the answers

    Which side effect is more likely with the use of potassium-sparing diuretics?

    <p>Hyperkalemia</p> Signup and view all the answers

    What is an important educational point for patients taking beta-blockers?

    <p>Take pulse and report any dizziness</p> Signup and view all the answers

    Which of the following is a potential drug interaction for ACE inhibitors?

    <p>NSAIDs</p> Signup and view all the answers

    What should patients taking thiazide diuretics be educated about regarding potential side effects?

    <p>Possible severe electrolyte depletion</p> Signup and view all the answers

    What is a key nursing intervention for patients receiving angiotensin II receptor blockers?

    <p>Monitor for acute renal failure</p> Signup and view all the answers

    What is a primary nursing intervention for patients taking potassium-sparing diuretics?

    <p>Educate on avoiding salt substitutes</p> Signup and view all the answers

    Which symptom is indicative of digoxin toxicity?

    <p>Nausea</p> Signup and view all the answers

    What is a recommended action for patients using nitrates?

    <p>Sit down while taking nitrates</p> Signup and view all the answers

    Which of the following is a contraindication for the use of amiodarone?

    <p>Heart block without a pacemaker</p> Signup and view all the answers

    What is an important educational point for patients using anticoagulants like warfarin?

    <p>Monitor INR levels regularly</p> Signup and view all the answers

    What effect do aldosterone antagonists like spironolactone have on potassium levels?

    <p>Sparingly retain potassium</p> Signup and view all the answers

    What should patients taking tafamidis be educated about?

    <p>Adherence to medication is crucial</p> Signup and view all the answers

    Which of the following is a risk associated with the use of digoxin and loop diuretics?

    <p>Increased digoxin toxicity risk</p> Signup and view all the answers

    What precaution should be taken when using ACE inhibitors and potassium-sparing diuretics together?

    <p>Monitor for hyperkalemia</p> Signup and view all the answers

    What is a consequence of using PDE-5 inhibitors while on nitrates?

    <p>Severe hypotension</p> Signup and view all the answers

    What is a positive outcome of effective medication management?

    <p>Stable electrolyte levels and renal function</p> Signup and view all the answers

    What indicates a negative outcome in fluid volume and weight management?

    <p>Episodes of fluid overload requiring diuretics</p> Signup and view all the answers

    Which sign is most indicative of a negative emotional outcome for patients?

    <p>Signs of anxiety affecting treatment adherence</p> Signup and view all the answers

    What reflects a positive emotional outcome in psychosocial well-being?

    <p>Adjustment to lifestyle changes by patient and family</p> Signup and view all the answers

    What is a potential consequence of poor medication tolerance?

    <p>Need to discontinue medications</p> Signup and view all the answers

    What is considered a positive outcome in cardiac output evaluation for cardiomyopathy?

    <p>Increased activity tolerance without significant fatigue</p> Signup and view all the answers

    Which sign indicates a negative outcome in fluid volume status assessment for cardiomyopathy?

    <p>Weight gain or increased abdominal girth</p> Signup and view all the answers

    Which of the following reflects a positive outcome in assessing activity tolerance?

    <p>Increased ability to perform activities of daily living independently</p> Signup and view all the answers

    What indicates a negative outcome regarding the patient’s knowledge of disease and self-care?

    <p>Nonadherence to dietary restrictions</p> Signup and view all the answers

    Which of the following outcomes is indicative of worsening cardiac function?

    <p>Declining ejection fraction on follow-up echocardiogram</p> Signup and view all the answers

    What is a sign of positive fluid volume status during evaluation?

    <p>Stable and reduced abdominal girth</p> Signup and view all the answers

    What indicates a negative outcome related to activity tolerance in patients with cardiomyopathy?

    <p>Inability to perform daily tasks without help</p> Signup and view all the answers

    Which outcome suggests a positive understanding of self-care practices in cardiomyopathy management?

    <p>Knowledge of the importance of a low-sodium diet</p> Signup and view all the answers

    Which symptoms are most commonly observed in infants with dilated cardiomyopathy?

    <p>Excessive sleepiness and rapid breathing</p> Signup and view all the answers

    What is a significant risk factor for peripartum cardiomyopathy?

    <p>Age over 30</p> Signup and view all the answers

    What intervention is particularly important for adolescents diagnosed with hypertrophic cardiomyopathy?

    <p>Genetic counseling for the family</p> Signup and view all the answers

    What is a common complication associated with subsequent pregnancies in women who had peripartum cardiomyopathy?

    <p>Worsening heart function</p> Signup and view all the answers

    What symptom may indicate worsening heart failure in older children with cardiomyopathy?

    <p>Syncope or fainting</p> Signup and view all the answers

    Which medication category is contraindicated in pregnant women with peripartum cardiomyopathy?

    <p>ACE inhibitors</p> Signup and view all the answers

    Which intervention is critical for managing children with hypertrophic cardiomyopathy?

    <p>Limiting activity and competitive sports</p> Signup and view all the answers

    What type of cardiomyopathy is specifically observed in pregnant women and can mimic symptoms of normal pregnancy?

    <p>Peripartum Cardiomyopathy</p> Signup and view all the answers

    What is the most common type of cardiomyopathy in older adults?

    <p>Restrictive Cardiomyopathy (RCM)</p> Signup and view all the answers

    Which factor can complicate the treatment of older adults with cardiomyopathy?

    <p>Age-related changes in cardiac function</p> Signup and view all the answers

    What is the importance of careful medication management in older adults with cardiomyopathy?

    <p>It helps in preventing electrolyte imbalances.</p> Signup and view all the answers

    Which of the following is a recommended intervention for older adults with decreased mobility due to cardiomyopathy?

    <p>Promoting mild, low-impact exercises</p> Signup and view all the answers

    Why is dietary management crucial for older adults with cardiomyopathy?

    <p>It helps with low-sodium diets and fluid restrictions.</p> Signup and view all the answers

    What aspect of patient education is particularly important for older adults?

    <p>Involving caregivers or family members</p> Signup and view all the answers

    Which consideration is important for managing medications in older adults with cardiomyopathy?

    <p>Monitoring for drug interactions</p> Signup and view all the answers

    What emotional support measure is important for older adults facing limitations from cardiomyopathy?

    <p>Counseling and social support</p> Signup and view all the answers

    Which symptom indicates worsening heart function that patients should report?

    <p>Increased shortness of breath</p> Signup and view all the answers

    What should patients be advised to do if they experience severe chest pain?

    <p>Seek immediate medical attention</p> Signup and view all the answers

    What is the common purpose of beta-blockers in managing cardiomyopathy?

    <p>Slow the heart rate and reduce oxygen demand</p> Signup and view all the answers

    What is a recommended instruction for patients taking diuretics?

    <p>Take them in the morning to avoid nighttime urination</p> Signup and view all the answers

    Which term best describes medications that may pose a risk when combined with over-the-counter drugs or herbal supplements?

    <p>Anticoagulants</p> Signup and view all the answers

    Study Notes

    Cardiomyopathy Overview

    • Cardiomyopathy affects the heart muscle and its ability to pump effectively.
    • The heart can become enlarged, thickened, or rigid, potentially leading to scar tissue formation.
    • This weakened heart muscle struggles to pump blood efficiently throughout the body.
    • Consequences include heart failure (HF), irregular heart rhythms (dysrhythmias), and heart valve disorders.
    • Cardiomyopathies can be classified as acquired or inherited:
      • Acquired cardiomyopathies develop due to other diseases, conditions, or factors.
      • Inherited cardiomyopathies are passed down genetically from parents to offspring.
      • In some cases, the origin of cardiomyopathy (acquired or inherited) might be unclear.

    Cardiomyopathy Categories

    • Cardiomyopathy is a disease of the heart muscle that affects its ability to pump blood effectively.
    • Different types of cardiomyopathy are classified based on the underlying cause and presentation.
    • The American Heart Association (AHA) has categorized cardiomyopathy into six types:
      • Dilated cardiomyopathy: Characterized by enlarged and weakened ventricles, leading to impaired pumping ability.
      • Hypertrophic cardiomyopathy: Occurs when the heart muscle thickens, leading to reduced ventricular filling and outflow obstruction.
      • Restrictive cardiomyopathy: Involves stiffening of the heart muscle, restricting ventricular filling and leading to reduced cardiac output.
      • Arrhythmogenic right-ventricular dysplasia: Caused by abnormal heart muscle tissue, particularly in the right ventricle, increasing risk of arrhythmias and sudden cardiac death.
      • Transthyretin amyloid cardiomyopathy (AHA, 2019): Involves accumulation of amyloid protein in the heart muscle, leading to stiffness and reduced cardiac function.
      • Unclassified: This category includes cardiomyopathies that don't fit into the other categories.

    Dilated Cardiomyopathy (DCM)

    • DCM is the most prevalent type of cardiomyopathy and a significant contributor to heart failure (HF).
    • Genetic inheritance accounts for up to one-third of DCM cases.
    • The most common age range for DCM diagnosis is between 20 and 60.
    • Men are more likely to be diagnosed with DCM than women.
    • DCM affects both the heart's ventricles and atria, impairing their pumping efficiency.
    • Enlarged end-diastolic and end-systolic volumes decrease left-ventricular ejection fraction and cardiac output (CO).
    • DCM causes the heart muscle to dilate and thin out, leading to an enlarged left ventricular chamber and eventually impacting the right ventricle and atria.
    • Over time, the weakened heart muscle can contribute to the development of heart failure.
    • Common clinical manifestations of DCM include shortness of breath, fatigue, lower extremity and abdominal swelling, and jugular venous distention (JVD).
    • DCM can progress to involve heart valve disorders, dysrhythmias, and blood clots.
    • While the exact cause of DCM is unknown, it is often associated with toxin exposure, metabolic conditions, and infections.
    • Reversible DCM can arise from alcohol or cocaine use, chemotherapy drugs, pregnancy, or systemic hypertension.
    • Hereditary mutations play a role in up to one-third of DCM cases.
    • DCM is linked to various gene and chromosome abnormalities.

    Hypertrophic Cardiomyopathy

    • Thickened heart muscle
    • Decreased heart pumping efficiency
    • Reduced blood flow
    • Impaired ventricular filling
    • Smaller end-diastolic volume
    • Lower cardiac output
    • Risk for life-threatening dysrhythmias
    • Asymmetric septal hypertrophy
    • Enlarged interventricular septum, especially the upper portion
    • Narrowed aortic blood passage
    • Impaired ventricular outflow
    • Usually inherited
    • Approximately 50% of patients inherit the HCM genetic mutation

    Restrictive Cardiomyopathy (RCM)

    • Characterized by stiff ventricular walls that hinder the heart's ability to expand and fill with blood.
    • Diminished ventricular flexibility reduces blood flow, potentially leading to diastolic dysfunction and heart failure.
    • Contractility remains unaffected, and the ejection fraction stays normal.
    • Treatment options for RCM are limited and focus on managing symptoms, providing support, and utilizing medications.
    • Scarring of the heart or amyloidosis can cause RCM.
    • RCM can develop after heart transplantation.
    • Excessive stiffness and rigidity in one or both ventricles result from fibrosis within the myocardium and endocardium.

    Arrhythmogenic Right-Ventricular Dysplasia (ARVD)

    • ARVD is a heart condition characterized by the replacement of right ventricular muscle with fatty and fibrous tissue.
    • ARVD increases the risk of ventricular tachyarrhythmias and sudden cardiac death (SCD).
    • ARVD affects both males and females and accounts for up to 20% of SCDs in individuals under 35 years old.
    • ARVD is a common cause of SCD in athletes.
    • ARVD can range in severity from mild to severe.
    • Most cases of ARVD are inherited and linked to genetic mutations in desmosomal proteins.
    • Family members of individuals with ARVD should undergo screening for signs and symptoms of the condition.
    • Treatment options for ARVD include medication, surgery, and observation.

    Transthyretin Amyloid Cardiomyopathy (ATTR-CM)

    • ATTR-CM has two forms: hereditary and wild type.
    • Hereditary ATTR-CM is caused by a mutation in the transthyretin gene.
    • Wild type ATTR-CM is not inherited.
    • The mutation leads to abnormal transthyretin protein assembly, causing clumping.
    • These protein clumps are deposited in various organs, including the heart.
    • Accumulation of protein clumps causes myocardial thickening and stiffening, leading to cardiomyopathy and heart failure (HF).
    • Risk factors include family history of ATTR-CM, age over 50, and African American ethnicity.
    • Wild type ATTR-CM is more common after age 65.
    • Both types are more common in males than females.
    • Tafamidis (Vyndamax) is an FDA-approved treatment for ATTR-CM.
    • Tafamidis is a transthyretin stabilizer, taken as a single 61 mg daily dose.
    • Tafamidis has shown to improve survival in patients.
    • Other therapies focus on managing HF symptoms.
    • ATTR-CM is often misdiagnosed as hypertensive HF or hypertrophic cardiomyopathy (HCM).

    Unclassified Cardiomyopathy

    • Cardiomyopathies that don't fit into specific categories like dilated, hypertrophic, restrictive, arrhythmogenic, or transthyretin amyloid cardiomyopathy are designated as unclassified cardiomyopathy.
    • Left-ventricular noncompaction, characterized by spongy muscle projections within the left ventricle, is an example of unclassified cardiomyopathy.
    • "Broken heart syndrome," also known as Takotsubo cardiomyopathy, is another type of unclassified cardiomyopathy.
    • Takotsubo cardiomyopathy is triggered by extreme stress, resulting in heart muscle failure.
    • The causes of unclassified cardiomyopathy vary depending on the specific condition.

    Risk Factors for Cardiomyopathies

    • Family history of cardiomyopathy, heart failure, sudden cardiac arrest, endocrine and metabolic diseases, alcoholism, and hypertension are all risk factors for cardiomyopathies.
    • Many forms of cardiomyopathy are idiopathic, meaning they have an unknown cause.
    • Cardiomyopathies can often result in death.

    Preventing Cardiomyopathies

    • While cardiomyopathies cannot be prevented, individuals can reduce the risk of developing conditions that can contribute to or complicate cardiomyopathies.
    • Maintaining a heart-healthy lifestyle is crucial:
      • Avoiding alcohol and drug use
      • Getting enough sleep
      • Eating a heart-healthy diet
      • Engaging in regular exercise
      • Reducing stress
      • Avoiding smoking

    Dilated Cardiomyopathy (DCM)

    • Symptoms develop gradually, potentially remaining undetected for years.
    • Heart failure (HF) usually appears years after the onset of dilation and cardiac failure.
    • HF can cause dyspnea on exertion, orthopnea, fatigue, paroxysmal nocturnal dyspnea, weakness, peripheral edema, and ascites.
    • Both S3 and S4 heart sounds are common, along with an AV regurgitation murmur.
    • Dysrhythmias become more prevalent as DCM progresses.
    • Ventricular dysrhythmias put patients at risk for sudden death.

    Hypertrophic Cardiomyopathy (HCM)

    • Individuals with HCM may experience angina due to ischemia caused by ventricular muscle overgrowth, coronary artery abnormalities, or decreased coronary artery perfusion.
    • Syncope occurs when outflow tract obstruction significantly reduces cardiac output and cerebral blood flow.
    • Ventricular dysrhythmias and atrial fibrillation are common.
    • Other manifestations include dyspnea, fatigue, dizziness, and palpitations.
    • Symptoms worsen with increased physical activity and oxygen demand, often during and after exercise.
    • A harsh systolic ejection murmur, best heard at the lower left sternal border (LSB) and apex, is characteristic of HCM.
    • An S4 murmur may also be present.

    Restrictive Cardiomyopathy (RCM)

    • Patients may live a normal life and remain asymptomatic or experience minimal symptoms.
    • Symptoms worsen as heart function deteriorates.
    • Less common symptoms include fainting and chest pain or pressure during exercise or periods of rest (exercise intolerance).
    • Jugular venous pressure (JVP) is elevated.
    • S3 and S4 heart sounds are common.

    Arrhythmogenic Right-Ventricular Dysplasia (ARVD)

    • Typically diagnosed in individuals under 40 years of age.
    • Often asymptomatic, with sudden death as the first manifestation.
    • When symptoms occur, they commonly include dysrhythmias and conduction disturbances.
    • These symptoms, along with blood and imaging test results, are relatively nonspecific.

    Transthyretin Amyloid Cardiomyopathy (ATTR-CM)

    • Commonly diagnosed in individuals with a family history of ATTR-CM or HF.
    • Diagnosis requires specialized testing such as cardiac MRI, nuclear medicine heart scan, cardiac tissue biopsy, and/or genetic testing.

    Unclassified Cardiomyopathy

    • Includes various primary heart muscle diseases causing cardiac dysfunction that don't fit into specific categories.
    • Manifestations vary depending on the specific disease within this classification.

    Cardiomyopathy: Clinical Manifestations and Therapies

    • Heart failure is a common complication of cardiomyopathy due to reduced cardiac output.

      • Symptoms: Dyspnea on exertion, orthopnea, paroxysmal nocturnal dyspnea, weakness, fatigue, peripheral edema, ascites.
      • Treatment: Address underlying cause, medications (diuretics, vasodilators, beta-blockers, calcium channel blockers), daily weight and fluid monitoring, elastic stockings, abdominocentesis, semi-Fowler’s position sleeping, periods of activity followed by rest.

    • Dysrhythmias are frequent due to heart muscle dilation damaging conduction pathways and creating alternative pathways.

      • ECG findings: Supraventricular tachycardias, atrial fibrillation, complex ventricular tachycardias.
      • Treatment: Address underlying cause, medications (antidysrhythmics, nitrates, beta-blockers), implantable cardioverter-defibrillator (ICD) may be required, patient education on pulse and rhythm monitoring and when to contact healthcare providers.

    • Angina can occur due to ventricular muscle overgrowth, coronary artery abnormalities, or decreased coronary artery perfusion.

      • Symptoms: Chest pain radiating to jaw, back, or left arm, shortness of breath, activity intolerance, intermittent claudication, nausea, vomiting.
      • Treatment: Address underlying cause, medications (nitrates, beta-blockers), patient education on symptom management and when to call emergency services, coronary artery repair surgery may be necessary.

    • Syncope may occur when outflow tract obstruction significantly reduces cardiac output and blood flow to the brain.

      • Symptoms: Dizziness, lightheadedness, fainting, nausea.
      • Treatment: Address underlying cause, medications (beta-blockers), patient education on the importance of sitting down immediately upon symptom onset to prevent falls.

    Cardiomyopathy Treatment Goals

    • Manage signs and symptoms
    • Delay disease progression
    • Reduce complication risk

    Treatment Methods

    • Pharmacologic interventions
    • Surgically implanted devices

    Specific Treatment Focuses

    • HCM and ARVD:
      • Reducing contractility
      • Preventing SCD
    • General:
      • Restriction of strenuous physical exertion
      • Dietary and sodium restrictions to diminish manifestations

    Diagnosis

    • Cardiomyopathy diagnosis begins with a detailed history and physical assessment, including the exploration of signs and symptoms.
    • Familial history of cardiomyopathy, heart failure, or sudden cardiac arrest should be explored.
    • Diagnostic tests include:
      • Echocardiography
      • Electrocardiography and ambulatory ECG monitoring
      • Chest X-ray
      • Hemodynamic studies
      • Cardiac stress testing
      • Radionuclear scans
      • Cardiac catheterization and coronary angiography
      • Myocardial biopsy
      • Genetic testing

    ### Cardiomyopathy Pharmacologic Therapy

    • ACE Inhibitors, alpha-/beta-blockers, ARBs, beta-adrenergic blockers, digitalis drugs, diuretics, HCN channel inhibitors, selective aldosterone receptor antagonists, SGLT2 inhibitors, and antidysrhythmics are used to treat cardiomyopathy.
    • Digoxin is commonly used to slow the heart rate, but avoided in hypertrophic cardiomyopathy due to the hypercontractile pump.
    • Diuretics may be used to aid in the treatment of heart failure.
    • Anticoagulants are given to reduce the risk of thrombus formation and embolization.
    • Electrolyte imbalances should be corrected as they may be a sign of dehydration, heart failure, high blood pressure, or other illnesses.
    • Aldosterone receptor blockers may be used to correct electrolyte imbalances in heart failure.
    • Antidysrhythmics may be administered to help prevent abnormal heart rhythms, but used with caution as they may increase symptoms of heart failure.
    • Tafamidis (Vyndamax), a transthyretin stabilizer, is a drug treatment for ATTR-CM, used to modify the underlying etiology.

    Cardiac Transplantation

    • Cardiac transplantation is the definitive treatment for dilated cardiomyopathy (DCM), but carries the long-term risk of organ rejection.
    • Transplantation is not a viable option for restrictive cardiomyopathy (RCM) because the underlying process causing fibrosis isn't eliminated.

    Left-Ventricular Assist Devices (LVADs)

    • LVADs are electronic pumps implanted into the abdominal cavity that support cardiac output until a donor heart is available.
    • LVADs assist in the perfusion of blood throughout the body.

    Surgical Ventricular Remodeling

    • Surgical ventricular remodeling is an alternative to transplantation for DCM.
    • The procedure reduces the size of the left ventricle to improve heart function and reduce the overall size of the heart muscle.
    • The surgeon can either remove a section of the muscle, or make a tuck in the existing heart muscle.

    Implantable Cardioverter-Defibrillators (ICDs) and Pacemakers

    • ICDs and pacemakers are used to control life-threatening dysrhythmias or maintain a stable heart rhythm.
    • These devices send an electrical impulse to stimulate cardiac contraction.
    • The most common type of pacemaker attaches to the heart in two places, allowing healthcare providers to modify the sequence of contractions within the heart muscle.

    Cardiomyopathy in Children

    • Cardiomyopathy is a leading cause of cardiac death in children.
    • 1 in every 100,000 children under 18 in the US is diagnosed with cardiomyopathy.
    • The majority of cases are diagnosed in children under 12 months old.
    • Children aged 12 to 18 years are the next largest group.
    • Dilated Cardiomyopathy (DCM)
      • Symptoms range from none to congestive heart failure (CHF).
      • Infants and young children may be misdiagnosed with respiratory infections or pneumonia.
      • Older children and adolescents are more likely to experience exercise intolerance and fatigue.
      • In cases caused by viral myocarditis, CHF can worsen rapidly, requiring emergency care.
    • Hypertrophic Cardiomyopathy (HCM)
      • Most often diagnosed in infancy or adolescence.
      • The second most common type of heart muscle disease.
      • 35-40% of all pediatric cardiomyopathies are HCM.
      • Symptoms can range from none to severe, including dysrhythmias and heart failure (HF).
      • Children under 1 year old often experience CHF symptoms.
      • Symptoms can appear during late childhood and early adolescence due to rapid growth and exercise.
    • Restrictive Cardiomyopathy (RCM)
      • The least common type of cardiomyopathy in children, affecting less than 1 in a million.
      • Accounts for only 2.5-5% of diagnosed cardiomyopathies.
      • Usually diagnosed around age 5 or 6.
      • Slightly more prevalent in girls than boys.
      • Family history of cardiomyopathy is present in 30% of cases.
      • Genetics likely play a role, but the cause is often unknown in children.
      • Initial symptoms often mimic lung problems, like chronic infections or asthma.
      • Enlarged heart on chest X-ray or abnormal physical exam findings can reveal cardiac involvement.
      • Other symptoms include:
        • Abnormal heart sounds
        • Fainting
        • Fluid in the abdomen
        • Liver enlargement
        • Edema of the face or extremities
      • In some cases, sudden death may be the first sign of RCM.

    Peripartum Cardiomyopathy (PPCM)

    • A rare but serious heart condition that affects the left ventricle during pregnancy or shortly after childbirth.
    • Occurs in women without a history of heart disease.
    • Characterized by decreased heart function, affecting the lungs, liver, and other organs.
    • Usually reversible, but can progress, requiring heart transplantation in some cases.
    • Most common in women over 30, but can occur at any age.
    • Risk factors include multiple pregnancies, obesity, poor diet, smoking, alcohol use, high blood pressure, and African American descent.
    • Certain medications may also increase the risk, such as chemotherapy drugs, anti-rejection medications for organ transplantation, and some medications used to treat seizures.
    • Cause is unknown, but nutritional deficiencies have been proposed.
    • Left ventricle may not be dilated, but ejection fraction is typically reduced below 45%.
    • Usually presents with anemia and infection.
    • Treatment focuses on addressing underlying abnormalities.
    • May resolve with bed rest as the heart returns to normal size.
    • Subsequent pregnancies carry a high risk of complications, especially for those with permanent heart enlargement.

    Focus of Care

    • Systolic dysfunction and pulmonary edema should be ruled out.

    Cardiomyopathies in Older Adults

    • Cardiomyopathies are less common in older adults compared to younger adults and children.
    • Only 10% of individuals diagnosed with dilated cardiomyopathy (DCM) are 65 years old or older.
    • While hypertrophic cardiomyopathy (HCM) is also less prevalent in older populations, the number of diagnoses in those over 60 has been rising.
    • HCM in older adults tends to be less severe, with a lower likelihood of progressing to heart failure (HF) and death compared to younger individuals.
    • Treatment guidelines for older adults with HCM are not well established, but the low morbidity and mortality rates do not warrant the use of defibrillators frequently implanted in younger patients.
    • Restrictive cardiomyopathy (RCM) is the most common cardiomyopathy in older adults.
    • Age-related decline in heart contractility and increasing ventricle stiffness can contribute to RCM, especially in conjunction with coronary artery disease (CAD), hypertension (HTN), or valvular calcification.
    • Older patients may also be diagnosed with cardiomyopathy of aging, a term used to describe HF or dysrhythmias without a known cause or specific cardiomyopathy.
    • Treatment for cardiomyopathy of aging is tailored to individual symptoms and health status.

    Nursing Process for Cardiomyopathy

    • Nursing assessment and care for patients with cardiomyopathies mirror those with heart failure (HF).
    • Patient and family education are crucial for understanding and managing symptoms.
    • Encourage energy conservation during activities of daily living (ADLs) and alternate activity with rest.
    • Strong support systems are beneficial for patients adapting to lifestyle changes.

    Lifestyle Modifications for Cardiomyopathy

    • Limit dietary intake of salt, fat, and fluids.
    • Engage in regular exercise and maintain an appropriate weight.
    • Encourage smoking cessation, limiting alcohol consumption, and stress reduction.

    HCM-Specific Care

    • Care for hypertrophic cardiomyopathy (HCM) resembles that for myocardial ischemia.
    • Avoid nitrates and vasodilators as they worsen HF symptoms in the presence of left ventricular hypertrophy.
    • Post-surgery care mirrors that of open-heart surgery.

    Genetic Counseling

    • Genetic counseling is essential to identify individuals at familial risk for cardiomyopathy.

    Subjective Data

    • Health History: Past history of hypertension, diabetes, cardiac disease, and heart failure.
    • Family History: Cardiomyopathy in close relatives.
    • Symptoms: Increased shortness of breath, dyspnea with exertion, decreased activity tolerance, paroxysmal nocturnal dyspnea, weight gain, coughing, chest or abdominal pain, anorexia, or nausea.
    • Lifestyle: Current medications, typical diet, and activity patterns.

    Objective Data

    • Vital Signs: Apical and peripheral pulses.
    • Physical Appearance: Observe general appearance, work of breathing (WOB), ease of conversation, position changes, and apparent anxiety.
    • Skin and Mucous Membranes: Examine for color changes.
    • Cardiovascular: Assess jugular venous distention (JVD) and capillary refill. Note presence and degree of edema. Auscultate heart sounds.
    • Respiratory: Auscultate breath sounds.
    • Chest: Palpate to assess heart size.
    • Abdomen: Examine contour, tenderness in the right upper quadrant, and liver enlargement. Auscultate bowel sounds.
    • Laboratory Tests: Draw blood for testing as necessary.

    Diagnosing Cardiomyopathy

    • Patients with cardiomyopathy often experience decreased cardiac output.
    • Fatigue is a common symptom.
    • Fluid volume excess can be a complication.
    • Inability to tolerate physical activity is common.
    • Many patients lack knowledge about their condition.
    • Grieving for the loss of health can be a challenge.

    Planning Care for Cardiomyopathy

    • The plan of care is tailored to the specific type and severity of cardiomyopathy.
    • Maintaining blood pressure within specified limits is a key goal.
    • Lifestyle adjustments are essential to accommodate reduced activity levels.
    • Dietary modifications are crucial for long-term management of the condition.

    Implementing Care for Cardiomyopathy

    • Nurses monitor for subtle changes in vital signs and level of consciousness.
    • Alterations in peripheral capillary refill time, pulse rate or volume, and level of consciousness can indicate decreased perfusion.
    • Home management of the condition is often possible for patients and their families.

    Monitoring Cardiac Output

    • Cardiac Output (CO) is the amount of blood pumped by the heart per minute.
    • Heart failure leads to a decrease in stroke volume (SV) and tissue perfusion.
    • Nursing interventions aim to reduce cardiac workload and improve CO.

    Nursing Interventions

    • Encourage rest throughout the day to reduce energy expenditure and improve cardiac function.
    • Elevate the head of the bed to reduce work of breathing (WOB) and improve oxygenation.
    • Provide a bedside commode and assist with activities of daily living (ADLs) to minimize physical exertion.
    • Instruct patients to avoid the Valsalva maneuver, which can significantly increase blood pressure and cardiac workload.

    Medication

    • Medications that increase cardiac output and contractility should be administered as ordered.
    • Oxygen therapy is indicated when hypoxia is present to improve oxygen delivery to tissues.

    Monitoring

    • Monitor vital signs and oxygen saturation regularly.
    • Tachycardia is common due to sympathetic nervous system stimulation, a compensatory response to low CO.
    • Vasoconstriction may initially cause an increase in diastolic blood pressure; however, as compensatory mechanisms fail, blood pressure drops.
    • Brain natriuretic peptide (BNP) is a cardiac neurohormone released in response to ventricular stress. Monitor BNP levels and trends to assess heart failure severity.
    • Auscultate heart and lung sounds regularly to assess cardiac function.

    Heart and Lung Sounds

    • Diminished heart sounds may indicate poor cardiac function.
    • Ventricular gallop (S3) or atrial gallop (S4) may be present in early heart failure.
    • Basilar crackles can indicate worsening heart failure.

    Interventions for Fluid Volume Excess

    • Fluid restrictions can be ordered
    • Offer ice chips to address dry mouth and thirst
    • Provide/allow hard candies if permitted
    • Monitor intake and output
      • Notify the HCP if urine output is less than 30 mL/hr
    • Diuretics can reduce circulating volume
      • May cause hypovolemia despite peripheral edema
    • A fall in urine output can indicate reduced CO and renal ischemia
    • Consistent patient weight helps determine fluid status
      • Weigh the patient at the same time daily, ideally after voiding but before breakfast
      • Significant weight gain suggests worsening HF
    • Measure abdominal girth every shift
      • Use the same location in relation to the umbilicus
    • Assess respiratory status and lung sounds at least every 4 hours
      • Notify the HCP of significant changes
      • Declining respiratory status indicates worsening left HF

    Heart Failure and Activity Intolerance

    • Patients with heart failure have limited cardiac reserve, making it difficult for them to meet increased oxygen demands during activity.
    • As heart failure progresses, activity intolerance worsens, potentially hindering self-care.
    • Nursing interventions to minimize activity intolerance include providing written and verbal information about expected activity levels, planning and implementing progressive activities, and using passive and active range-of-motion exercises.
    • Progressive activities gradually increase exercise capacity by strengthening and improving cardiac function without overexertion.
    • Activity also helps prevent muscle wasting (atrophy).
    • Range-of-motion exercises prevent complications associated with immobility.
    • Assist patients with activities of daily living (ADLs) as needed, encouraging independence whenever possible.
    • Assisting with ADLs ensures care needs are met while reducing the heart's workload.
    • Involving patients in their care promotes a sense of control and reduces feelings of helplessness.
    • Encourage frequent rest periods during ADLs.
    • Organize care to allow for rest periods.
    • Aim to provide prolonged periods of rest at night.

    Implementing a Low-Sodium Diet

    • Nurses play a crucial role in implementing low-sodium diets by explaining the rationale behind restrictions to encourage patient adherence.
    • Collaborate with dietitians to plan and review low-sodium diets, as well as low-calorie diets if weight management is necessary.
    • Provide patients with lists of high-sodium, high-fat, and high-cholesterol foods to avoid.
    • Dietary planning and education empower patients, fostering a sense of control and active participation in managing their health.

    Evaluation

    • The nurse evaluates the effectiveness of care by assessing patient outcomes based on set goals and nursing diagnoses.
    • Key indicators of success include: patients reporting any concerning symptoms to their healthcare provider, maintaining blood pressure within acceptable ranges, identifying strategies to sustain an active lifestyle, and understanding the importance of incorporating low-sodium foods into their diet.
    • If desired outcomes are not achieved or the patient's condition deteriorates, a referral to a cardiologist specializing in cardiomyopathies is recommended.
    • Further assessments and tests, such as electrocardiograms (ECGs), chest x-rays, and cardiac catheterization, may be necessary depending on the patient's situation.
    • In certain cases, treatments like defibrillator or pacemaker implantation, ventricular reconstruction, or heart transplantation may be considered.

    Medications for Cardiomyopathy

    • Digoxin is used to slow heart rate in heart failure, but is avoided in hypertrophic cardiomyopathy (HCM) due to the risk of increasing contractility.
    • Tafamidis (Vyndamax) is a transthyretin stabilizer approved for treatment of transthyretin amyloid cardiomyopathy (ATTR-CM)
      • The recommended dose is 61 mg daily.
    • Nitrates are used to manage angina by dilating blood vessels.

    Dilated Cardiomyopathy (DCM)

    • Most common type of cardiomyopathy
    • Characterized by enlarged and weakened heart ventricles
    • Affects adults aged 20-60
    • Can be inherited in about one-third of cases
    • Symptoms include shortness of breath during exertion, fatigue, swelling in the limbs, and distended neck veins
    • Treatment includes medications like diuretics, ACE inhibitors, and beta-blockers; lifestyle modifications are crucial
    • Severe cases may require a heart transplant

    Hypertrophic Cardiomyopathy (HCM)

    • Characterized by thickened heart muscle, particularly the left ventricle
    • Reduced ventricular filling and impaired blood flow
    • About half of all cases are inherited
    • Symptoms include fainting, chest pain, shortness of breath, rapid heartbeat, and an increased risk of sudden cardiac death
    • Avoid digoxin and nitrates as they can worsen symptoms
    • Common in athletes, and can be associated with sudden cardiac death during physical exertion

    Restrictive Cardiomyopathy (RCM)

    • Characterized by stiff and rigid ventricular walls, limiting heart filling
    • Normal heart muscle contractility
    • Less common than other types
    • Most prevalent in older adults
    • Often caused by conditions like amyloidosis or fibrosis
    • Symptoms include fatigue, difficulty with exercise, swelling in the limbs, and distended neck veins
    • Treatment focuses on managing symptoms and lifestyle modifications
    • Heart transplants typically not effective

    Arrhythmogenic Right-Ventricular Dysplasia (ARVD)

    • Rare condition where the right ventricle is replaced with fatty and fibrous tissue
    • Increases risk of ventricular tachyarrhythmias
    • Often inherited, linked to mutations in desmosomal proteins
    • First symptom can be sudden cardiac death, particularly in younger individuals under 40
    • Treatment involves implantable cardioverter-defibrillator (ICD) to manage arrhythmias and lifestyle adjustments to avoid strenuous activities

    Transthyretin Amyloid Cardiomyopathy (ATTR-CM)

    • Caused by a build-up of amyloid proteins in the heart, leading to stiffening and thickening of the heart muscle
    • Two forms: inherited (mutation in the transthyretin gene) and non-inherited (spontaneous)
    • Risk factors include African American descent, age over 65, and male sex
    • Tafamidis (Vyndamax) is a key treatment for stabilizing transthyretin proteins

    Unclassified Cardiomyopathy

    • Includes cardiomyopathies that don't fit into the other categories
    • Examples: left-ventricular noncompaction and Takotsubo cardiomyopathy (broken heart syndrome)
    • Takotsubo cardiomyopathy is often stress-induced with symptoms similar to a heart attack
    • Treatment varies based on the specific condition and symptoms present

    Dilated Cardiomyopathy (DCM)

    • Inherited in about one-third of cases
    • Most commonly diagnosed in individuals between 20 and 60
    • More common in males
    • Alcohol abuse, drug use, and poor diet can increase risk
    • Hypertension, viral infections, metabolic conditions, and autoimmune diseases can contribute to DCM
    • Exposure to chemotherapy drugs and heavy metals can increase risk

    Hypertrophic Cardiomyopathy (HCM)

    • Often inherited, with about 50% of cases linked to genetic mutations
    • Can affect any age but often diagnosed during infancy or adolescence
    • Family history of ventricular hypertrophy increases risk
    • Vigorous physical activity or competitive sports, especially in youth, can increase the risk of sudden cardiac death

    Restrictive Cardiomyopathy (RCM)

    • Amyloidosis, sarcoidosis, hemochromatosis, and other conditions causing tissue fibrosis can lead to RCM
    • More common in older adults
    • About 30% of cases show a genetic link
    • Slightly more prevalent in females

    Arrhythmogenic Right-Ventricular Dysplasia (ARVD)

    • Often inherited and linked to mutations in desmosomal proteins
    • Commonly diagnosed in individuals under 40
    • Known hereditary connection, making family screening recommended
    • Competitive athletes may have an increased risk of sudden cardiac death related to ARVD

    Transthyretin Amyloid Cardiomyopathy (ATTR-CM)

    • Hereditary form is linked to mutations in the transthyretin gene
    • Wild-type form is more common in individuals over 65
    • African American descent increases risk
    • More prevalent in males than females

    Peripartum Cardiomyopathy (PPCM)

    • More common in women over 30
    • Multiple gestations over a lifetime increase risk
    • Obesity and poor nutrition can contribute to PPCM
    • Smoking and alcohol use increases risk
    • Hypertension and preeclampsia can contribute to PPCM
    • Higher risk in African American women
    • Some medications used during pregnancy may increase risk

    Unclassified Cardiomyopathies

    • Often triggered by emotional or physical stress
    • More common in females, especially postmenopausal women

    Patient History

    • Inquire about past medical history, including hypertension, diabetes, cardiac disease, and prior episodes of heart failure.
    • Assess family history for cardiomyopathy, heart failure, or sudden cardiac death as these conditions can be hereditary.

    Symptom Review

    • Document shortness of breath, dyspnea on exertion, and reduced activity tolerance.
    • Assess patients for paroxysmal nocturnal dyspnea (PND) or the need for extra pillows for sleep.
    • Inquire about recent weight changes, which might indicate fluid retention.
    • Elicit details on symptoms such as coughing, chest or abdominal pain, anorexia, or nausea.

    Lifestyle Assessment

    • Review current medications, diet, activity levels, and recent changes in health.
    • Inquire about lifestyle habits including alcohol or drug use and smoking, as these impact cardiac health.

    Vital Signs

    • Measure blood pressure, heart rate, respiratory rate, and temperature.
    • Assess apical and peripheral pulses for rhythm and strength.

    General Appearance

    • Observe for signs of dyspnea, ease of conversation, position changes for comfort, and any signs of anxiety or distress.

    Skin and Mucous Membranes

    • Evaluate skin color for pallor or cyanosis, which might indicate poor perfusion.

    Jugular Vein Distension (JVD)

    • Check for JVD, a common indicator of fluid overload and heart failure.

    Capillary Refill

    • Assess capillary refill time, as delays suggest poor peripheral circulation.

    Edema

    • Check for peripheral edema, particularly in the lower extremities.

    Heart and Lung Sounds

    • Auscultate for abnormal heart sounds, such as S3 (ventricular gallop) or S4 (atrial gallop).
    • Listen for crackles in the lungs, which may indicate fluid buildup.

    Chest Palpation

    • Palpate to assess heart size and any abnormal movement.

    Abdominal Assessment

    • Inspect and palpate for tenderness, particularly in the right upper quadrant, for signs of liver enlargement.
    • Measure abdominal girth if fluid buildup is suspected.
    • Auscultate bowel sounds to detect any changes due to decreased perfusion.

    Laboratory Testing

    • Obtain blood samples as needed to assess BNP levels, electrolytes, and renal function, which can indicate heart failure severity or electrolyte imbalances.

    Echocardiography

    • Used to assess heart structure, size, and function
    • Reduced ejection fraction (EF): indicates decreased pumping ability, common in dilated cardiomyopathy (DCM)
    • Increased ventricular thickness: suggests hypertrophic cardiomyopathy (HCM)
    • Ventricular stiffness: seen in restrictive cardiomyopathy (RCM)
    • Left ventricular enlargement: common in DCM

    Electrocardiography (ECG)

    • Measures electrical activity to identify arrhythmias and structural abnormalities
    • Arrhythmias: supraventricular tachycardias, atrial fibrillation, and ventricular tachycardias may indicate electrical disruptions
    • Left ventricular hypertrophy: common in HCM
    • Abnormal Q waves: may indicate past myocardial injury or thickened myocardium in HCM
    • Low voltage QRS complexes: may suggest amyloidosis in transthyretin-related cardiomyopathy (ATTR-CM)

    Ambulatory ECG Monitoring

    • Continuously records ECG over 24-48 hours to detect intermittent arrhythmias
    • Captures episodes of tachyarrhythmias or atrial fibrillation not detected during a single ECG

    Chest X-ray

    • Provides imaging to assess heart size and detect fluid in the lungs
    • Cardiomegaly: enlargement of the heart, common in DCM
    • Pulmonary congestion or edema: indicates fluid buildup due to heart failure (HF)
    • Pleural effusions: fluid accumulation in the pleural cavity, suggesting worsening HF

    Hemodynamic Studies

    • Measures pressure within the heart chambers and pulmonary arteries
    • Increased pulmonary capillary wedge pressure: suggests left-sided HF
    • Elevated right atrial pressure: indicates right-sided HF or fluid overload
    • Low cardiac output (CO): confirms impaired heart function

    Cardiac Stress Testing

    • Evaluates the heart's response to physical exertion or pharmacologic stress
    • Ischemic changes on ECG: suggests inadequate blood flow, often seen in HCM
    • Failure to increase blood pressure during stress: indicates limited cardiac reserve in HF
    • Reduced exercise tolerance: common in advanced cardiomyopathy

    Radionuclear Scans

    • Assesses ventricular function by measuring ejection fraction and wall motion
    • Low ejection fraction: confirms reduced pumping ability, especially in DCM
    • Regional wall motion abnormalities: indicates areas of scarring or reduced contractility

    Cardiac Catheterization and Coronary Angiography

    • Visualizes coronary arteries and measures chamber pressures
    • Coronary artery blockages: can be detected and treated if found
    • High ventricular pressures: indicative of restrictive filling, often in RCM
    • Ventricular dilation: seen in DCM

    Myocardial Biopsy

    • Examines heart tissue for cellular abnormalities, fibrosis, or amyloid deposits
    • Fibrosis and scarring: common in DCM and RCM
    • Amyloid deposits: indicates ATTR-CM
    • Infectious or inflammatory cells: may point to myocarditis as a cause of DCM

    Genetic Testing

    • Identifies inherited mutations associated with certain cardiomyopathies
    • Mutations in sarcomere proteins: linked to HCM
    • Desmosomal protein mutations: common in arrhythmogenic right ventricular dysplasia (ARVD)
    • Transthyretin gene mutations: indicative of hereditary ATTR-CM

    Brain Natriuretic Peptide (BNP) or N-terminal pro-BNP (NT-proBNP)

    • Measures BNP levels, which increase in response to ventricular stress
    • Elevated BNP or NT-proBNP: indicates HF severity, as high levels correlate with ventricular dysfunction and fluid overload

    Echocardiography

    • Ejection Fraction (EF): Measures the percentage of blood ejected from the left ventricle with each heartbeat.
      • Normal: 55–70%
      • Mildly Reduced: 41–55%
      • Moderately Reduced: 30–40%
      • Severely Reduced: < 30% (seen in advanced Dilated Cardiomyopathy (DCM) or Heart Failure (HF) )
    • Left Ventricular Hypertrophy (LVH): Thickening of the left ventricular wall.
      • Wall thickness > 12 mm is abnormal and suggests Hypertrophic Cardiomyopathy (HCM).
    • Ventricular Dilation: Enlargement of the left ventricle
      • Enlarged left ventricular dimensions (end-diastolic diameter > 60 mm) indicate DCM.

    Electrocardiography (ECG)

    • Normal Sinus Rhythm: Regular heart rhythm at 60-100 beats per minute (bpm).
    • Left Ventricular Hypertrophy (LVH):
      • High QRS voltage, particularly in leads V1-V6.
    • Abnormal Q Waves:
      • Indicates past myocardial injury or hypertrophy, common in HCM.
    • Atrial Fibrillation or Flutter:
      • Irregular rhythm, may indicate HF or advanced cardiomyopathy.
    • Ventricular Tachycardia (VT):
      • Wide complex tachycardia often found in Arrhythmogenic Right Ventricular Dysplasia (ARVD) and severe DCM.
    • Low Voltage QRS Complexes:
      • Seen in Transthyretin-related Cardiomyopathy (ATTR-CM) due to amyloid infiltration.

    Ambulatory ECG Monitoring (Holter Monitor)

    • Normal Findings: Stable heart rate without significant arrhythmias.
    • Abnormal Findings: Episodes of ventricular tachycardia, atrial fibrillation, or other arrhythmias indicate electrical instability, often associated with ARVD and advanced cardiomyopathy.

    Chest X-ray

    • Heart Size (Cardiomegaly):
      • Normal Cardiothoracic Ratio: < 50% of the chest width.
      • Cardiomegaly: Cardiothoracic ratio > 50%, commonly seen in DCM and advanced HF.
    • Pulmonary Congestion:
      • Fluid in lung bases or pleural effusions indicate left-sided HF.

    Hemodynamic Studies (Right Heart Catheterization)

    • Pulmonary Capillary Wedge Pressure (PCWP):
      • Normal: 6-12 mmHg.
      • Elevated: > 18 mmHg indicates left-sided HF or fluid overload.
    • Right Atrial Pressure:
      • Normal: 0-8 mmHg.
      • Elevated: > 8 mmHg suggests right-sided HF.
    • Cardiac Output (CO):
      • Normal: 4-8 L/min.
      • Low CO: Common in advanced cardiomyopathy and indicates reduced heart function.

    Cardiac Stress Testing

    • Normal Response: Gradual increase in heart rate and blood pressure (BP) with exertion, absence of ischemic changes.
    • Abnormal Findings:
      • Ischemic changes on ECG (ST depression) during stress indicate reduced coronary perfusion, often seen in HCM.
      • Inability to increase BP suggests limited cardiac reserve in advanced HF.

    Radionuclear Scans (e.g., MUGA Scan)

    • Normal Ejection Fraction (EF):
      • 55-70% in both ventricles.
    • Reduced EF:
      • < 50% indicates impaired ventricular function, typical in DCM.

    Cardiac Catheterization and Coronary Angiography

    • Normal Coronary Arteries: No significant blockages.
    • Coronary Blockages: Stenosis in coronary arteries, indicating underlying coronary artery disease.
    • Ventricular Pressures:
      • High ventricular filling pressures suggest restrictive filling in Restrictive Cardiomyopathy (RCM).
    • Ventricular Dilation: Common in DCM, with enlarged chamber sizes.

    Myocardial Biopsy

    • Normal Findings: Healthy cardiac muscle cells without abnormal deposits or fibrosis.
    • Abnormal Findings:
      • Fibrosis: Common in DCM and RCM.
      • Amyloid Deposits: Seen in ATTR-CM.
      • Inflammatory Cells: Indicate myocarditis, which can be a cause of DCM.

    Genetic Testing

    • Normal Findings: No mutations linked to cardiomyopathy.
    • Abnormal Findings:
      • Sarcomere mutations for HCM.
      • Desmosomal mutations for ARVD.
      • Transthyretin gene mutations for hereditary ATTR-CM.

    Brain Natriuretic Peptide (BNP) or NT-proBNP

    • Normal BNP Levels: < 100 pg/mL.
    • Mild Heart Failure: 100-300 pg/mL.
    • Moderate to Severe Heart Failure: > 300 pg/mL in BNP or > 900 pg/mL for NT-proBNP indicates severe HF and is commonly seen in advanced cardiomyopathy.

    Surgical Interventions

    • Cardiac Transplantation - Indicated for advanced dilated cardiomyopathy (DCM) unresponsive to other treatments. Requires lifelong immunosuppression to prevent organ rejection. Not typically an option for restrictive cardiomyopathy (RCM) due to persistent fibrosis.
    • Left-Ventricular Assist Device (LVAD) - Implanted pump that aids in blood circulation for end-stage heart failure (HF), serving as a bridge to transplantation or for ineligible transplant candidates.
    • Surgical Ventricular Remodeling - Reshapes the heart by removing or tucking a ventricular section to reduce the size of the left ventricle and improve function, indicated for DCM.
    • Implantable Cardioverter-Defibrillator (ICD) & Pacemaker - Used for patients with life-threatening arrhythmias like arrhythmogenic right ventricular dysplasia (ARVD) or hypertrophic cardiomyopathy (HCM) at risk of sudden cardiac death. ICDs deliver shocks for arrhythmia correction, while pacemakers regulate heart rhythm.

    Pharmacologic Interventions

    • ACE Inhibitors (e.g., Lisinopril) - Reduce blood pressure, decrease heart strain, and prevent HF progression. Indicated for DCM and HF in cardiomyopathy.
    • Beta-Blockers (e.g., Metoprolol) - Slow heart rate, reduce myocardial oxygen demand, improve heart function. Indicated for DCM, HCM, arrhythmias, and HF management.
    • Angiotensin II Receptor Blockers (ARBs) - Similar to ACE inhibitors but used when ACE inhibitors are not tolerated. Indicated for HF and hypertension.
    • Diuretics (e.g., Furosemide) - Reduce fluid overload, relieve HF symptoms, and decrease cardiac workload. Indicated for DCM and other forms of HF.
    • Digoxin - Slows heart rate and increases contractility. Avoided in HCM due to hypercontractile risk. Indicated for symptomatic relief in HF, except HCM.
    • Aldosterone Antagonists (e.g., Spironolactone) - Reduces fluid retention and corrects electrolyte imbalances. Indicated for fluid volume control in HF.
    • Anticoagulants (e.g., Warfarin) - Prevent blood clots and reduce thromboembolism risk. Indicated for patients with atrial fibrillation or stasis due to low cardiac output.
    • Tafamidis (Vyndamax) - Stabilizes transthyretin protein in transthyretin amyloid cardiomyopathy (ATTR-CM), slowing disease progression.
    • Nitrates (e.g., Nitroglycerin) - Manage angina by dilating blood vessels. Indicated for angina in cardiomyopathy, but avoided in HCM due to exacerbation risk.
    • Antidysrhythmics - Control abnormal heart rhythms. Indicated for managing arrhythmias in ARVD, DCM, and other cardiomyopathies.

    Non-Pharmacologic Interventions

    • Low-Sodium Diet - Reduces fluid retention and heart strain. Indicated for all types of cardiomyopathy with fluid volume overload.
    • Fluid Restriction - Limits fluid intake to manage HF symptoms. Indicated for patients with volume overload and HF.
    • Energy Conservation - Minimizes cardiac workload by pacing activities: Alternating activity with rest, using a bedside commode, and avoiding the Valsalva maneuver.
    • Progressive Physical Activity - Gradually improves cardiac function without strain, prevents muscle atrophy: Range-of-motion exercises, physical therapy consultations for customized activity plans.
    • Smoking Cessation and Alcohol Avoidance - Reduces risk factors exacerbating cardiomyopathy. Interventions include education and support programs.

    Collaborative Interventions

    • Dietary Consultation - Develops individualized low-sodium, low-fat, and low-cholesterol diets to manage fluid volume and blood pressure in cardiomyopathy.
    • Physical Therapy - Helps design safe exercise plans to enhance strength and endurance without overexertion. Indicated for patients with activity intolerance or physical deconditioning.
    • Genetic Counseling - Identifies hereditary risk in family members, especially for HCM, ARVD, and ATTR-CM. Indicated for patients with familial cardiomyopathy history.
    • Mental Health Support - Provides emotional support for coping with chronic illness and lifestyle changes. Indicated for patients and families adjusting to cardiomyopathy limitations.
    • Patient Education on Symptom Management - Educates on recognizing and responding to symptoms like shortness of breath, fatigue, and chest pain. Indicated for all cardiomyopathy patients to improve self-management.
    • Referral to Specialists - Provides specialized care for complex cases requiring advanced therapies. Indicated for advanced HF or cardiomyopathy patients with complications.

    ACE Inhibitors

    • Block the conversion of angiotensin I to angiotensin II, resulting in vasodilation and reduced blood pressure.
    • Contraindications: Pregnancy, renal artery stenosis, history of angioedema.
    • Interactions: NSAIDs (reduce effectiveness), potassium-sparing diuretics (increase hyperkalemia risk).
    • Nursing Interventions/Education: Monitor blood pressure and renal function. Educate patients on signs of cough and angioedema. Caution about salt substitutes with potassium.

    Beta-Blockers

    • Block beta-adrenergic receptors, reducing heart rate and myocardial oxygen demand.
    • Contraindications: Asthma, severe bradycardia, cardiogenic shock.
    • Interactions: Calcium channel blockers (additive bradycardia), insulin (may mask hypoglycemia symptoms).
    • Nursing Interventions/Education: Monitor heart rate and blood pressure, hold if HR < 50 bpm. Educate on taking pulse and reporting dizziness or syncope. Taper slowly to avoid rebound hypertension.

    Angiotensin II Receptor Blockers (ARBs)

    • Block angiotensin II receptors, leading to vasodilation and reduced blood pressure.
    • Contraindications: Pregnancy, severe renal impairment.
    • Interactions: Potassium-sparing diuretics (hyperkalemia risk), NSAIDs.
    • Nursing Interventions/Education: Monitor potassium levels and renal function. Educate on avoiding potassium supplements and salt substitutes. Similar to ACE inhibitors but less likely to cause a cough.

    Diuretics

    Loop Diuretics

    • Inhibit sodium and chloride reabsorption in the loop of Henle, leading to increased urine output.
    • Contraindications: Anuria, severe electrolyte depletion.
    • Interactions: Increased risk of ototoxicity with aminoglycosides, lithium (increased toxicity).
    • Nursing Interventions/Education: Monitor potassium levels, as loop diuretics can cause hypokalemia. Educate on daily weights and signs of dehydration. Encourage potassium-rich foods unless contraindicated.

    Thiazide Diuretics

    • Inhibit sodium reabsorption in the distal tubule, leading to moderate diuresis.
    • Contraindications: Severe renal impairment, gout.
    • Interactions: Increased risk of lithium toxicity, NSAIDs reduce effectiveness.
    • Nursing Interventions/Education: Monitor blood glucose in diabetics and potassium levels. Educate on avoiding dehydration and reporting muscle cramps or weakness.

    Potassium-Sparing Diuretics

    • Inhibit sodium reabsorption and potassium excretion in the distal tubules, sparing potassium.
    • Contraindications: Hyperkalemia, severe renal impairment.
    • Interactions: ACE inhibitors and ARBs (increased hyperkalemia risk).
    • Nursing Interventions/Education: Avoid potassium-rich foods and salt substitutes. Monitor for signs of hyperkalemia (muscle weakness, irregular heartbeats). Educate on potential side effects like gynecomastia with spironolactone.

    Digoxin

    • Increases myocardial contractility and slows the heart rate.
    • Contraindications: Ventricular fibrillation, HCM.
    • Interactions: Loop diuretics (hypokalemia increases digoxin toxicity risk), calcium channel blockers (increased digoxin levels).
    • Nursing Interventions/Education: Monitor apical pulse for 1 full minute; hold if HR < 60 bpm. Monitor for toxicity. Signs include nausea, vision changes, and arrhythmias. Educate on reporting GI symptoms and changes in vision.

    Aldosterone Antagonists

    • Block aldosterone receptors, reducing fluid retention and sparing potassium.
    • Contraindications: Hyperkalemia, Addison’s disease.
    • Interactions: ACE inhibitors, ARBs, and potassium supplements (increase hyperkalemia risk).
    • Nursing Interventions/Education: Monitor for hyperkalemia symptoms and potassium levels. Educate on avoiding salt substitutes. Watch for gynecomastia with spironolactone.

    Anticoagulants

    • Prevent thrombus formation by inhibiting clotting pathways.
    • Contraindications: Active bleeding, recent surgery, pregnancy (Warfarin).
    • Interactions: Many interactions, including NSAIDs (increase bleeding risk), certain antibiotics (alter INR with warfarin).
    • Nursing Interventions/Education: Monitor INR regularly for patients on warfarin. Educate on consistent vitamin K intake with warfarin. Instruct on bleeding precautions and avoiding activities that increase bleeding risk.

    Tafamidis

    • Stabilizes transthyretin protein, slowing progression of amyloid deposits in ATTR-CM.
    • Contraindications: Known hypersensitivity to tafamidis.
    • Interactions: No major known drug interactions.
    • Nursing Interventions/Education: Educate on medication adherence for long-term effectiveness. Monitor symptoms to assess for progression or stability of ATTR-CM.

    Nitrates

    • Vasodilate coronary arteries to reduce myocardial oxygen demand and relieve angina.
    • Contraindications: Severe hypotension, recent PDE-5 inhibitor use (e.g., sildenafil).
    • Interactions: PDE-5 inhibitors (severe hypotension), antihypertensives (additive hypotensive effect).
    • Nursing Interventions/Education: Educate on proper timing and storage of nitroglycerin. Teach patients to take it sitting down to prevent falls from hypotension. Avoid with HCM, as it can worsen symptoms.

    Antidysrhythmics

    • Stabilize cardiac electrical activity to manage arrhythmias.
    • Contraindications: Bradycardia, certain heart blocks without pacemaker.
    • Interactions: Beta-blockers (additive bradycardia), grapefruit (affects metabolism of amiodarone).
    • Nursing Interventions/Education: Monitor ECG for arrhythmia resolution. Educate on avoiding grapefruit (for amiodarone) and reporting any visual changes or respiratory symptoms. Check for thyroid and liver function due to potential side effects with long-term use.

    Cardiac Output

    • Positive outcome includes improved blood pressure and heart rate within target range, increased activity tolerance without significant fatigue or shortness of breath, absence of chest pain and reduction in dyspnea, and normalized or improved ejection fraction (EF) on follow-up echocardiograms.
    • Negative outcome includes persistent or worsening hypotension or tachycardia, decreased activity tolerance and continued need for frequent rest, persistent or worsening dyspnea, chest pain or fatigue, and declining EF or signs of worsening heart function on echocardiograms.

    Fluid Volume Status

    • Positive outcome includes stable or reduced weight and abdominal girth (no signs of fluid retention), improved urine output (≥ 30 mL/hr) and reduced peripheral edema, clear lung sounds with no crackles, indicating reduced pulmonary congestion, and normal jugular venous pressure (JVP), indicating stable fluid volume.
    • Negative outcome includes weight gain or increased abdominal girth, indicating fluid retention, persistent edema in extremities, decreased urine output, or need for diuretics, crackles or wheezing in lung sounds, indicating pulmonary congestion, and elevated JVP and signs of worsening heart failure.

    Activity Tolerance

    • Positive outcome includes increased ability to perform activities of daily living (ADLs) independently without excessive fatigue, minimal fatigue or dyspnea with usual activities, and improved muscle strength and endurance due to progressive activity tolerance.
    • Negative outcome includes persistent fatigue or dyspnea with minimal exertion, inability to complete ADLs without assistance, and decreased muscle strength, indicating potential deconditioning or worsening HF.

    Knowledge of Disease and Self-Care

    • Positive outcome includes patient and family verbalizing an understanding of cardiomyopathy, its symptoms and the importance of adherence to treatment, patient demonstrating knowledge of low-sodium diet, fluid restrictions and medication regimen, and patient awareness of signs and symptoms that require immediate reporting (e.g., weight gain, shortness of breath, chest pain).
    • Negative outcome includes poor understanding of the disease process or medications, nonadherence to dietary restrictions or medication regimen, and difficulty identifying warning signs that require prompt medical attention.

    Medication Management and Tolerance

    • Positive outcome includes effective symptom control with minimal side effects from medications, stable electrolyte levels and renal function on laboratory tests, and absence of adverse effects such as hypotension, bradycardia, or electrolyte imbalances.
    • Negative outcome includes adverse reactions or side effects (e.g., hyperkalemia with ACE inhibitors, digoxin toxicity), electrolyte imbalances or worsening renal function from diuretics or other medications, and need to reduce or discontinue medications due to poor tolerance, leading to uncontrolled symptoms.

    Fluid Volume and Weight Management

    • Positive outcome includes patient maintaining a stable weight with no sudden changes, indicating fluid balance, no episodes of fluid overload or need for additional diuretics, and stable abdominal girth and absence of new or worsening edema.
    • Negative outcome includes unstable or increasing weight due to fluid retention, frequent need for adjustments to diuretics to control fluid overload, and an increase in peripheral edema, ascites, or abdominal girth, indicating fluid retention.

    Emotional and Psychosocial Well-being

    • Positive outcome includes patient and family demonstrating acceptance and adjustment to lifestyle changes, engagement in support groups or counseling services if needed, and expression of understanding and adherence to the treatment plan.
    • Negative outcome includes signs of depression, anxiety, or grief impacting treatment adherence, nonparticipation in recommended lifestyle changes or failure to attend follow-up appointments, and persistent feelings of hopelessness or difficulty adapting to lifestyle changes.

    Cardiomyopathy in Children and Adolescents

    • Dilated Cardiomyopathy (DCM) is common in infants and young children with symptoms ranging from mild to severe heart failure (HF).
    • Hypertrophic Cardiomyopathy (HCM) is prevalent in adolescents and often has a genetic basis.
    • Restrictive Cardiomyopathy (RCM) is rare in children and often linked to genetic factors.
    • Symptoms in infants include poor feeding, excessive sleepiness, rapid breathing, and irritability.
    • Older children and adolescents may experience fatigue, exercise intolerance, shortness of breath, and fainting (syncope).
    • Adolescents with HCM have an increased risk of sudden cardiac death (SCD) during sports.
    • Interventions include careful medication adjustments due to smaller body sizes, physical activity restrictions for children with HCM, family education about symptom recognition and medication adherence, genetic counseling, and supportive care for the child and family.

    Cardiomyopathy in Pregnant Women

    • Peripartum Cardiomyopathy (PPCM) is a type of DCM diagnosed during the last month of pregnancy or within five months postpartum.
    • PPCM can occur even without prior heart disease history and may be linked to factors like age over 30, multiple pregnancies, obesity, and hypertension.
    • Symptoms can mimic normal pregnancy symptoms, but can escalate to severe HF.
    • Symptoms include fatigue, shortness of breath, peripheral edema, palpitations, and signs of HF.
    • PPCM can lead to complications in subsequent pregnancies.
    • Interventions include close monitoring of heart function, limited medication options due to pregnancy, education on the risks of future pregnancies, lifestyle modifications, and potentially a heart transplant in severe cases.

    Cardiomyopathy in Older Adults

    • Restrictive Cardiomyopathy (RCM) is the most common type in older adults due to age-related ventricular stiffness.
    • Dilated Cardiomyopathy (DCM) and Hypertrophic Cardiomyopathy (HCM) are less common in older adults but can develop due to comorbid conditions or genetics.
    • Age-related changes include decreased cardiac contractility, increased arterial stiffness, and other comorbidities like hypertension, coronary artery disease (CAD), and diabetes.
    • Polypharmacy increases the risk of drug interactions with cardiomyopathy medications.
    • Functional limitations complicate treatment, especially with diuretics.
    • Interventions include careful medication management, adjusted activity levels, dietary management, monitoring for complications, and emotional and social support.

    General Nursing Considerations Across Lifespan

    • Patient education should be tailored to the patient's developmental level and cognitive abilities with involvement of caregivers for children and older adults.
    • Family and caregiver support is crucial across all ages to ensure adherence to treatment.
    • Genetic counseling is essential for families with a history of cardiomyopathy, as some forms are hereditary.

    Cardiomyopathy Overview

    • Cardiomyopathy is a condition affecting the heart muscle, making it harder for it to pump blood effectively.
    • There are four main types: dilated, hypertrophic, restrictive, and peripartum cardiomyopathy.

    Recognizing Worsening Heart Function

    • Symptoms to watch for include:
      • Increased shortness of breath
      • Chest pain or pressure
      • Fatigue or decreased activity tolerance
      • Rapid weight gain (more than 2-3 pounds in a day or 5 pounds in a week)
      • Swelling in the ankles, legs, or abdomen
    • Severe chest pain, syncope (fainting), rapid or irregular heartbeat, or difficulty breathing require immediate medical attention.

    Medication Management

    • Taking medications as prescribed is crucial to control symptoms, improve heart function, and prevent complications.

    Common Medications

    • ACE inhibitors/ARBs: lower blood pressure and reduce heart strain
    • Beta-blockers: slow the heart rate and reduce oxygen demand
    • Diuretics: help reduce fluid overload
    • Anticoagulants: reduce the risk of blood clots

    Medication Side Effects and Interactions

    • Common side effects include dizziness, fatigue, or increased urination.
    • Patients should inform their healthcare provider about any concerns.
    • Over-the-counter medications and herbal supplements can potentially interact with cardiomyopathy medications.
    • Check with the healthcare provider before starting any new medications.

    Specific Medication Instructions

    • Diuretics: Take in the morning to avoid nighttime urination.
    • Digoxin: Monitor pulse; hold if the pulse is below the instructed rate (usually 60 beats per minute).

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    Description

    Explore the characteristics and classifications of cardiomyopathy in this quiz. Learn about the effects on the heart muscle, and how acquired and inherited forms differ. Understanding these concepts is crucial for recognizing the potential consequences, such as heart failure and dysrhythmias.

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