Cardiomyopathies PDF
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University of Puerto Rico Medical Sciences Campus
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This document is a presentation about various types of cardiomyopathy. It describes different forms of the disease, including hypertrophic, dilated, peripartum, restrictive physiology, and cor pulmonale. Each type has specific characteristics, causes, diagnosis, and treatment.
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comenzamos ( Cardiomyopathies Cardiomyopathies LOK up pumps disease carbohydrates build up } Cardiomyopathies are a heterogeneous group of diseases of the myocardium associated with mechanical and/or electrical dysfunction that usually exhibit inappropriate ventricular hypertrophy or dila...
comenzamos ( Cardiomyopathies Cardiomyopathies LOK up pumps disease carbohydrates build up } Cardiomyopathies are a heterogeneous group of diseases of the myocardium associated with mechanical and/or electrical dysfunction that usually exhibit inappropriate ventricular hypertrophy or dilation } It may be due to a variety of causes that frequently are genetic. } Cardiomyopathies either are confined to the heart or are part of generalized systemic disorders, often leading to cardiovascular death or progressive heart failure-related disability. Cardiomyopathies } Classified } Primary Cardiomyopathies } } } Ussually confined to the heart only Genetic, acquired or combined in origin Secondary Cardiomyopathies } pathophysiologic involvement of the heart in the context of a multiorgan disorder secondary - } The most commonly seen by anesthesia: } } } } DM cardiom - . - dilation build hypertrophic cardiomyopathy dilated cardiomyopathy peripartum cardiomyopathy secondary cardiomyopathies with restrictive physiology. up fat Cardiomyopathies: Hypertrophic } } Hypertrophic cardiomyopathy (HCM) is a complex cardiac disease with unique pathophysiologic characteristics and a great diversity of morphologic, functional, and clinical features. Affect patients of all ages and has a prevalence approaching 1 in 500. It is the most common genetic cardiovascular disease and is transmitted as an autosomal dominant trait with variable present s4S penetrance. The disease is characterized by LV hypertrophy in the absence of any other cardiac disease. The most common form of HCM presents as hypertrophy of the septum and anterolateral free wall. - } 75 % probability autosomal } } to pass recessive 25 gene % is not may be present Cardiomyopathies: Hypertrophic } Pathophysiology } Development of the following features: myocardial hypertrophy, dynamic LV outflow tract (LVOT) obstruction, systolic anterior movement of the mitral valve causing mitral regurgitation, diastolic dysfunction, myocardial ischemia, and dysrhythmias. examen } During systole, contraction of the hypertrophied septum accelerates blood flow through the narrow LVOT, which creates a Venturi effect on the anterior leaflet of the mitral valve and induces systolic anterior movement of the anterior mitral valve leaflet. } The presence of this systolic anterior movement accentuates the dynamic LVOT obstruction and causes significant mitral regurgitation ↳ syncope-collapse Cardiomyopathies: Hypertrophic } EVENTS THAT INCREASE OUTFLOW OBSTRUCTION } Increased myocardial contractility } β-Adrenergic stimulation (catecholamines) } Digitalis i contractility } Decreased preload ejecuted } Hypovolemia } Vasodilators resistance } Tachycardia ventricle preload } Positive pressure ventilation } Decreased afterload } Hypotension } Vasodilators EF . ↓ dont fill well ↓ Cardiomyopathies: Hypertrophic } EVENTS THAT DECREASE OUTFLOW OBSTRUCTION } Decreased myocardial contractility } β-Adrenergic blockade } Volatile anesthetics } Calcium entry blockers contractility } Increased preload } Hypervolemia } Bradycardia } Increased afterload } Hypertension } α-Adrenergic stimulation i load affected after Cardiomyopathies: Hypertrophic } Signs and Symptoms } Most Common } } } } } } } Angina Pectoris Fatigue Syncope Tachydysrhythmias Heart failure Lying down alleviates symptoms ↑ preload - venous return Sudden death is more often between 10 – 30 year olds Cardiomyopathies: Hypertrophic } Diagnosis } ECG } Echocardiogram } } } } } Ejection fraction (EF) is usually increased LV hyperthrophy Look up how it looks LVOT obstruction (physical and dynamic) Endocardial Biopsy Management } } } The diverse clinical and genetic features of HCM make it impossible to define precise guidelines for management. Patients at high risk of sudden death and must be treated aggressively. Pharmacologic therapy to improve diastolic filling, reduce LV outflow obstruction, and possibly decrease myocardial ischemia is the primary means of relieving the signs and symptoms of HCM. Cardiomyopathies: Hypertrophic } Management Overview: Cardiomyopathies: Hypertrophic } Management of Anesthesia think } Management is directed toward minimizing LVOT obstruction. about what may contractility , affect preload } Any drug or event that decreases myocardial contractility or increases preload or reduces afterload will improve LVOT obstruction. } Conversely, sympathetic stimulation, hypovolemia, and vasodilation worsen LVOT obstruction. } Intraoperatively, patients with HCM may develop severe hypotension, myocardial ischemia, acute heart failure, and supraventricular or ventricular tachydysrhythmias. Cardiomyopathies: Hypertrophic } Management of Anesthesia } Beta blockers and/or calcium channel blockers should be continued } Promote an adequate volume status, and avoid hypovolemia since the preoperative period } Avoid sudden decrease on afterload secondary to opioids and or neuromuscular blockade avoid attracorium ↳ } release Histamine ? Monitor for dysrrhytmias since the LV may be dependent on the ‘atrial kick’ Cardiomyopathies: Hypertrophic } Management of Anesthesia: Parturient Patients } Pregnancy is usually well tolerated in patients with HCM. } May have increased LVOT obstruction because events such as labor paina nd bearing down (Valsalva's maneuver). } Oxytocin: monitor its vasodilating properties and compensatory tachycardia, and because of the abrupt inflow of large amounts of blood into the central circulation as a consequence of uterine contraction. pulmonar e } ~ } Treatment of pulmonary edema may include phenylephrine (if hypotensive) and esmolol to slow the heart rate. Diuretics, digoxin, and nitrates cannot be used to treat pulmonary edema in this setting. They worsen the situation by provoking further LVOT obstruction. due to preload i contrac Cardiomyopathies: Dilated } Most common 3rd I cause CHF transplant Dilated cardiomyopathy is a primary myocardial disease characterized by LV or biventricular dilation, systolic dysfunction, and normal ventricular wall thickness. W } The etiology of dilated cardiomyopathy is unknown } } } } Genetic vs infectious myocarditis May be also seen secondary to other diseases African American men have an increased risk - viral weeks up to A 28 % EF a months evaluate check with when the dilated Eco was cardiomyop Dilated cardiomyopathy is the most common type of cardiomyopathy, the third most common cause of heart failure, and the most common indication for cardiac transplantation. keep preload opposite to hypertrophy E ↓ contractility . Cardiomyopathies: Dilated } Signs and Symptoms } } Same as for heart failure Diagnosis } ECG } } } Echocardiogram } } } ST segment and T wave abnormalities Dysrhytmias ( PVC, Atrial fibrillation) Dilatation all four chambers Decreased EF Endocardial biopsy NOT indicated - Clinic Cardiomyopathies: Dilated } dead->ICD Treatment } } } } most common V-fib cause of } } "prevent" Same algorithm as for heart failure Anticoagulation due to high risk of thrombus formation ICD decrease risk of sudden death in patients who survived a previous cardiac arrest. Prognosis } to ~> and diastolic < 20 is Hart 5-year mortality rate of 50%. Poor prognosis features: an ejection fraction of less than 25%, a pulmonary capillary wedge pressure of more than 20 mm Hg, a cardiac index of less than 2.5 L/min/m2, systemic hypotension, pulmonary hypertension, and increased central venous pressure. Alcoholic cardiomyopathy is largely reversible if complete abstinence from alcohol is maintained. Cardiomyopathies: Dilated } Anesthesia management } Since dilated cardiomyopathy is a cause of heart failure, the anesthetic management of these patients is the same as that described in the heart failure. } Regional anesthesia may be an alternative to general anesthesia in selected patients with dilated cardiomyopathy. } However, the need for anticoagulant therapy may limit this option. Cardiomyopathies: Peripartum } Peripartum cardiomyopathy is a rare, dilated form of cardiomyopathy of unknown cause that arises during the peripartum period. } Between third trimester until 5 months after delivery. } No prior history of heart disease. } The estimated incidence of peripartum cardiomyopathy is 1 in 3000 to 1 in 4000 live births. Cardiomyopathies: Peripartum } Risk factors: } } } } } } } include obesity Multiparity advanced maternal age (>30 years) multifetal pregnancy preeclampsia African American ethnicity examen Possible causes include viral myocarditis, an abnormal immune response to pregnancy, and maladaptive responses to the hemodynamic stresses of pregnancy. Cardiomyopathies: Peripartum } Signs and Symptoms } Same as heart failure } } } } Dyspnea Fatigue Peripheral Edema Clinical conditions that may mimic heart failure, such as amniotic fluid or pulmonary embolism, should be excluded when considering the diagnosis of peripartum cardiomyopathy. Cardiomyopathies: Peripartum Baby is } Diagnosisforming 142 In } } ~ Trimester Baby is growing in 3rd trimester Inhibitors Territogenic for the baby Ace before third semester Based on the onset of unexplained LV dysfunction and echocardiographic documentation of a new finding of dilated cardiac chambers with LV systolic dysfunction. Treatment } } } } } The goal of treatment is to alleviate the symptoms of heart failure. Diuretics, vasodilators, and digoxin can be used. ACE Themester inhibitors are teratogenic but can be useful following delivery. During pregnancy: hydralazine and nitrates. Intravenous immunoglobulin may have a beneficial effect. Thromboembolic complications are not uncommon, and anticoagulation is often recommended. Cardiomyopathies: Peripartum } Prognosis } } } } The mortality rate ranges from 25% to 50%, with most deaths occurring within 3 months of delivery. Death is usually a result of progression of congestive heart failure or sudden death associated with cardiac dysrhythmias or thromboembolic events. The prognosis appears to depend on the degree of normalization of LV size and function within 6 months of delivery. Anesthesia management } } Assessment of cardiac status and careful planning of the analgesia and/or anesthesia required for delivery. Regional anesthesia may provide a desirable decrease in afterload. Cardiomyopathies: Restrictive Physiology } - Causes: } c Narrow Pulse Pressure ? cardiac tamponade .* up treatment ↑ in CUP can give liquid : My tension pericardio centesis Due to systemic diseases that produce myocardial infiltration and severe diastolic dysfunction. heart don't adequate -> } LOOK relax ; wont fill The most common of these cardiomyopathies is caused by amyloidosis. symptoms HF ↑ crp ECO-1 look rike a stant because ventricle dont relax } The diagnosis should be considered in patients who have heart failure but no evidence of cardiomegaly or systolic dysfunction. } The condition results from increased stiffness of the myocardium caused by the deposition of abnormal substances. } Although there is impaired diastolic function and reduced ventricular compliance, systolic function is usually normal. Cardiomyopathies: Restrictive Physiology } Signs and Symptoms } } Heart failure symptoms without cardiomegaly Atrial fibrillation is also common. } } Cardiac conduction disturbances are particularly common in amyloidosis and sarcoidosis. Over time, this involvement of the conduction system can lead to heart block or ventricular dysrhythmias, resulting in sudden death. Diagnosis } } } ECG: may demonstrate conduction abnormalities. CXR: may show signs of pulmonary congestion and/or pleural effusion, but cardiomegaly is absent. Echocardiogram } Significant diastolic dysfunction and normal systolic function. } Enlarged atria, but normal ventricles Endomyocardial biopsy can elucidate the cause of the infiltrative cardiomyopathy. } Cardiomyopathies: Restrictive Physiology } Treatment } } Symptomatic treatment is similar to that for diastolic heart failure. Medical management: } } } } } } } Diuretics Digoxin (caution, because potentially dysrhythmogenic in amyloidosis patients need atrial Kick to fill ventricle Maintenance of normal sinus rhythm is extremely important. Significant bradycardia or severe conduction system disease may require implantation of a cardiac pacemaker or ICD. Anticoagulation may be needed in patients with atrial fibrillation and/or low cardiac output. Cardiac transplantation is not a treatment option because myocardial infiltration will recur in the transplanted heart. Prognosis } The prognosis of secondary cardiomyopathy with restrictive physiology is very poor. Cardiomyopathies: Restrictive Physiology } Management of Anesthesia } Same concept as in cardiac tamponade } } } Keep adequate preload Control afterload Avoid dysrhythmias volume for venous return } Maintenance of venous return and intravascular fluid volume is also necessary to maintain an acceptable cardiac output. } Anticoagulant therapy will negatively influence the decision to select regional anesthesia. Cardiomyopathies: Cor Pulmonale } Definition: } Cor pulmonale is right ventricular enlargement (hypertrophy and/or dilation) that may progress to right-sided heart failure. } Diseases that induce pulmonary hypertension such as COPD, restrictive lung disease, and respiratory insufficiency of central origin (obesity-hypoventilation syndrome) cause cor pulmonale. also di pulmonic emboli } The most common cause of cor pulmonale is COPD. } More often in persons older than 50 years of age } Men are affected five times more often than women. Cardiomyopathies: Cor Pulmonale } Pathophysiology } The main pathophysiologic determinant of cor pulmonale is pulmonary hypertension. } By various mechanisms, chronic lung disease induces an increase in pulmonary vascular resistance. Long-standing chronic hypoxia promotes pulmonary vasculature remodeling and an increase in pulmonary vascular resistance. } Because of pulmonary hypertension, the right ventricle has an increased workload, and right ventricular hypertrophy develops. Over time, right ventricular dysfunction occurs, and eventually right ventricular failure is present. Cardiomyopathies: Cor Pulmonale } Signs and Symptoms } Clinical manifestations of cor pulmonale may be obscured by the co-existing lung disease. } Clinical signs occur late in the course of the disease } } } } Most prominent is peripheral edema. Dyspnea with effort related syncope. LOOK UP Accentuation of the pulmonic component of the second heart sound, a diastolic murmur due to incompetence of the pulmonic valve, and a systolic murmur due to tricuspid regurgitation connote severe pulmonary hypertension. it Pulmonary HTN Jugular venous distention and hepatosplenomegaly. Cardiomyopathies: Cor Pulmonale } Diagnosis } ECG: right atrial and right ventricular hypertrophy. } CXR: increase in the width of the right pulmonary artery and a decrease in pulmonary vascular markings in the lung periphery. } Echocardiogram: Transesophageal echocardiography can be a very useful diagnostic tool. } Transthoracic echocardiography is often difficult to perform in patients with COPD because the hyper inflated lungs impair transmission of the ultrasound waves. Cardiomyopathies: Cor Pulmonale } } Treatment } Treatment goals are aimed at reducing the workload of the right ventricle by decreasing pulmonary vascular resistance and pulmonary artery pressure. } Oxygen supplementation to maintain the Pao2 above 60 mm Hg (oxygen saturation of >90% by pulse oximetry) is useful in both the acute and long-term treatment of right-sided heart failure. } Long-term oxygen therapy decreases the mortality } Diuretics and digitalis may be used to treat right-sided heart failure that does not respond to correction of arterial blood gases. } Pulmonary vasodilators, such as sildenafil and bosentan, have been shown to improve the symptoms and quality of life. If medical management fails: transplantation of one or both lungs or a heart-lung transplantation will provide dramatic relief of cardiorespiratory failure. Cardiomyopathies: Cor Pulmonale } Prognosis } Depends on the disease responsible for initiating pulmonary hypertension. } Patients with COPD in whom arterial oxygenation can be maintained at near-normal levels and whose pulmonary hypertension is mild have a favorable prognosis. } Prognosis is poor in patients with severe, irreversible pulmonary hypertension Cardiomyopathies: Cor Pulmonale } Management of Anesthesia } Preoperative preparation: } } } } } } eliminating and controlling acute and chronic pulmonary infection reversing bronchospasm improving clearance of airway secretions expanding collapsed or poorly ventilated alveoli maintaining hydration correcting any electrolyte imbalances } Preoperative measurement of arterial blood gases } Induction of general anesthesia can be accomplished using any available method or drug. Adequate depth of anesthesia should be present before endotracheal intubation, because this stimulus can elicit reflex bronchospasm in lightly anesthetized patients. Cardiomyopathies: Cor Pulmonale } Anesthesia is typically maintained with a volatile anesthetic combined with other drugs. } Volatile anesthetics are effective bronchodilators. } Large doses of opioids should be avoided because they can contribute to prolonged postoperative ventilatory depression. } Muscle relaxants associated with histamine release should also be avoided because of the adverse effect of histamine on airway resistance and pulmonary vascular resistance. } Positive pressure ventilation improves oxygenation, presumably because of better ventilation-perfusion matching. } Humidification of inhaled gases helps maintain hydration, liquefaction of secretions, and mucociliary function. Cardiomyopathies: Cor Pulmonale } Intraoperative monitoring of patients with cor pulmonale is influenced by the complexity of the surgery. } Regional anesthetic techniques can be used in appropriate situations in patients with cor pulmonale, but regional anesthesia is best avoided for operations that require high levels of sensory and motor block. } The respiratory and cardiovascular status must be monitored in the postoperative period, and any factors that exacerbate pulmonary hypertension (hypoxia, hypercarbia) treated aggressively . } Oxygen therapy should be maintained as needed. Cardiomyopathies: REMEMBER } Heart failure is a complex pathophysiologic state in which the heart is unable to fill with or eject blood at a rate appropriate to tissue requirements. } Heart failure is characterized by specific symptoms (dyspnea and fatigue) and signs of circulatory congestion or hypoperfusion. } The principal pathophysiologic derangement in the development and progression of heart failure is ventricular remodeling. } The principal treatment goals in heart failure patients are avoiding or decreasing the degree of ventricular remodeling and promoting reverse remodeling. } Therapies proven to be of value in this regard include ACE inhibitors, β-blockers, aldosterone inhibitors, and CRT. Cardiomyopathies: REMEMBER } The management of acute heart failure includes the use of lowdose loop diuretics in combination with vasodilators, positive inotropic drugs, exogenous BNP, and/or insertion of mechanical devices. } HCM is the most common genetic cardiac disorder. Its pathophysiology is related to the development of LVOT obstruction and ventricular dysrhythmias that can cause sudden death. } Factors that induce LVOT obstruction in HCM include hypovolemia, tachycardia, an increase in myocardial contractility, and a decrease in afterload. } Outflow tract obstruction is managed by maintaining hydration, increasing afterload (phenylephrine), and decreasing heart rate and myocardial contractility (β-blockers and calcium channel blockers). Cardiomyopathies: REMEMBER } Dilated cardiomyopathy is the most common form of cardiomyopathy and the second most common cause of heart failure. } The treatment and anesthetic implications are similar to those for chronic heart failure. } Cor pulmonale is right ventricular enlargement (hypertrophy and/or dilation) that may progress to right-sided heart failure. It is caused by diseases that promote development of pulmonary hypertension. } The most important pathophysiologic determinant of the development of pulmonary hypertension and cor pulmonale in patients with chronic lung disease is alveolar hypoxia. } The best available treatment to improve the prognosis in these patients is long-term oxygen therapy. TO Be continued next …… } TEST