Podcast
Questions and Answers
Which statement is true regarding the management strategies for dilated cardiomyopathy (D-CMP)?
Which statement is true regarding the management strategies for dilated cardiomyopathy (D-CMP)?
What is a clinical feature commonly associated with dilated cardiomyopathy?
What is a clinical feature commonly associated with dilated cardiomyopathy?
Which type of cardiomyopathy is primarily characterized by unknown etiology and significant left ventricular dilatation?
Which type of cardiomyopathy is primarily characterized by unknown etiology and significant left ventricular dilatation?
Which of the following is NOT a known cause of dilated cardiomyopathy?
Which of the following is NOT a known cause of dilated cardiomyopathy?
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How does myocarditis differ in its effects compared to dilated cardiomyopathy?
How does myocarditis differ in its effects compared to dilated cardiomyopathy?
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What percentage of acute myocarditis cases may deteriorate acutely, possibly leading to death or the need for heart transplantation?
What percentage of acute myocarditis cases may deteriorate acutely, possibly leading to death or the need for heart transplantation?
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Which of the following features differentiates fulminant myocarditis from acute myocarditis?
Which of the following features differentiates fulminant myocarditis from acute myocarditis?
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Which symptom is NOT commonly associated with myocarditis?
Which symptom is NOT commonly associated with myocarditis?
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What diagnostic test is considered definitive for diagnosing myocarditis despite its associated risks?
What diagnostic test is considered definitive for diagnosing myocarditis despite its associated risks?
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Which ECG finding is most likely associated with myocarditis?
Which ECG finding is most likely associated with myocarditis?
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Which inflammatory marker is typically elevated in myocarditis but does not confirm the diagnosis?
Which inflammatory marker is typically elevated in myocarditis but does not confirm the diagnosis?
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How often does acute myocarditis resolve within the first 2-4 weeks?
How often does acute myocarditis resolve within the first 2-4 weeks?
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Which of the following symptoms is most consistent with a viral infection prior to the development of myocarditis?
Which of the following symptoms is most consistent with a viral infection prior to the development of myocarditis?
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Which management option is typically used for treating hemochromatosis in patients with cardiac complications?
Which management option is typically used for treating hemochromatosis in patients with cardiac complications?
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What is a characteristic morphology finding in patients with R-CMP?
What is a characteristic morphology finding in patients with R-CMP?
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Which genetic inheritance pattern is associated with Arrhythmogenic RV Cardiomyopathy (ARVC)?
Which genetic inheritance pattern is associated with Arrhythmogenic RV Cardiomyopathy (ARVC)?
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What is commonly seen on an ECG in a patient diagnosed with Arrhythmogenic RV Cardiomyopathy?
What is commonly seen on an ECG in a patient diagnosed with Arrhythmogenic RV Cardiomyopathy?
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What phase occurs after the acute viral replication in myocarditis?
What phase occurs after the acute viral replication in myocarditis?
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Which treatment options can be used for managing myocarditis?
Which treatment options can be used for managing myocarditis?
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Which feature is NOT typical of cardiac myocyte degeneration in myocarditis?
Which feature is NOT typical of cardiac myocyte degeneration in myocarditis?
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In patients with ARVC, what is the typical consequence of ventricular arrhythmias during sports-related exercise?
In patients with ARVC, what is the typical consequence of ventricular arrhythmias during sports-related exercise?
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Study Notes
R-CMP Morphology
- Ventricles are of normal size
- Cavities are not dilated
- Myocardium is firm and noncompliant
- Biatrial dilatation is common
- Patchy/diffuse interstitial fibrosis
R-CMP Management
- Diuretics
- Beta Blockers, Amiodarone and Calcium Channel Blockers
- ACE-I and SGLT inhibitors
- Long-term anticoagulation
- Internal pacemaker
- Cardiac transplantation
Amyloidosis Management
- Melphalan
- Prednisolone
- Tx (treatment)
Hemochromatosis Management
- Phlebotomy
- Desferrioxsamine
Carcinoid Management
- Somatostatin analogs
- Valvular surgery
Sarcoidosis Management
- Pacing
- Steroids
- Tx (treatment)
Arrythmogenic RV Cardiomyopathy (ARVC)
- Progressive replacement of RV myocardium with fat and fibrous tissue creating an excellent environment for fatal arrhythmias
- Typical involvement: regional RV → Global RV → Partial LV involvement with sparing of septum
- Autosomal dominant inheritance
ARVC Presentation
- Onset of arrhythmias range from ventricular extrasystoles (VES) to ventricular fibrillation (VF)
- Sudden cardiac death (SCD) is 75% due to VT/VF in sports-related exercise
- Congestive heart failure (CHF) is 25%
- Progressive right and left ventricular dysfunction
ARVC Diagnosis
- Genetics, ECG, serial echocardiogram and endomyocardial biopsy
- ECG changes: Inverted T waves (Right precordial leads)
- Epsilon wave and QRS>110 ms
- Ventricular extrasystoles and tachycardia with left bundle branch block (LBBB) morphology
Myocarditis
- Inflammatory cells infiltrate the myocardium
- Myocyte degeneration or necrosis
Myocarditis Pathogenesis
- Myocardial inflammation, necrosis and fibrosis
- Cardiomegaly and reduced systolic function occur due to myocardial damage
- Typical signs of heart failure (HF) develop which may progress to cardiogenic shock, arrhythmias and sudden cardiac death.
- Viruses act on Myocardium in three phases:
- Acute phase: Replication of virus
- Autoimmune Injury phase
- Dilated cardiomyopathy phase or chronic phase
Myocarditis: Endomyocardial Biopsy
- Arrow shows a collection of lymphocytes infiltrating the cardiac muscle in response to a viral infection.
- Arrowhead shows an area of cardiac muscle damage induced by the virus directly or due to the cytotoxic immune response to the viral infection
Fulminant Myocarditis
- Characterized by more extensive and diffuse lymphocytic infiltration and myocyte necrosis than acute myocarditis
Myocarditis Diagnosis
- Myocarditis is a challenging diagnosis due to the heterogeneous clinical presentations.
- Myocarditis presents in many different ways, ranging from mild symptoms of chest pain and palpitations associated with transient ECG changes to life-threatening cardiogenic shock and ventricular arrhythmia
Myocarditis Signs & Symptoms
- Chest pain (often described as "stabbing" in character).
- Congestive heart failure (CHF) leading to edema, breathlessness and hepatic congestion.
- Palpitations (due to arrhythmias).
- Sudden death (in young adults, myocarditis causes up to 20% of all cases of sudden death).
- Fever (especially when infectious)
Myocarditis: Recent Viral Infection
- Many patients report symptoms consistent with a recent viral infection, including fever, diarrhea, joint pains and easy fatiguability.
Myocarditis: Associated Symptoms
- Myocarditis is often associated with pericarditis, many patients present with signs and symptoms that suggest concurrent myocarditis and pericarditis.
Myocarditis: ECG
- Sinus tachycardia
- Non-specific ST segment and T-wave abnormalities
Myocarditis: Biomarkers
- CK-MB and troponin may be elevated
Myocarditis: Inflammatory Markers
- ESR and CRP levels are often raised, but they do not confirm the diagnosis
Myocarditis: Chest X-Ray
- Shows variability from normal to cardiomegaly.
Myocarditis: Echocardiogram
- Normal/ wall motion abnormality or reduced systolic function
- Mural thrombus
Myocarditis: Cardiovascular Magnetic Resonance
- Shows extent of scarring, LV function, inflammatory injury
Myocarditis: Endomyocardial Biopsy
- There are risks, and it is not used in every case, but is definitive for myocarditis
Myocarditis: Treatment
- Acute myocarditis resolves in about 50% of cases in the first 2–4 weeks, but about 25% will develop persistent cardiac dysfunction and 12–25% may acutely deteriorate and either die or progress to end-stage DCM with a need for heart transplantation.
Dilated Cardiomyopathy (D-CMP)
- Idiopathic D-CMP is a disease of unknown etiology that principally affects the myocardium, leading to LV dilatation and systolic dysfunction.
- Most common of the cardiomyopathies
- Prevalence: 36 per 100,000 population
- Third most common cause of heart failure and most common cause of transplantation
- Complete recovery is rare
- Within 2 years, 50% die, and 25% survive longer than 5 years
Causes of D-CMP
- Genetic:
- 20-50% are familial, Autosomal dominant predominant pattern
- Mutations in genes encoding dystrophin, sarcoglycan and troponin.
- Myocarditis
- Alcohol and other toxins
- Childbirth (peripartum CMP)
Dilated Cardiomyopathy (D-CMP): Pathophysiology
- Myocyte injury
- Arrhythmias (Atrial fibrillation, Ventricular tachycardia)
- Sudden death
- Decreased contractility
- Decreased stroke volume
- Left ventricular dilatation
- Increased ventricular filling pressure
- Forward CO (cardiac output)
- Exertional dyspnea
- Fatigue and weakness
- Pulmonary Congestion
- Chest pain
- Mitral regurgitation (MR)
- Thromboembolism
- Stroke
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Description
This quiz covers various cardiomyopathies including R-CMP, ARVC, and their management strategies such as pharmacological treatments and surgical options. It also addresses related conditions like amyloidosis and hemochromatosis. Test your understanding of these critical cardiovascular topics.
