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What is the summary definition of Restrictive Cardiomyopathy (RCM)?
A rare cardiomyopathy with marked diastolic dysfunction, normal (or near-normal) systolic function, and normal ventricular volumes due to myocardium distortion. Summary 1
What type of cardiac dysfunction primarily characterizes Restrictive Cardiomyopathy (RCM)?
Marked diastolic dysfunction. Summary / Pathophysiology 2
What happens to systolic function and ventricular volumes in Restrictive Cardiomyopathy (RCM)?
Systolic function is normal (or near-normal), and ventricular volumes are normal. Summary / Pathophysiology 3
What causes the myocardial distortion leading to Restrictive Cardiomyopathy (RCM)?
Proliferation of abnormal tissue or the deposition of abnormal compounds within the myocardium. Summary / Pathophysiology 4
How does Restrictive Cardiomyopathy (RCM) typically manifest clinically?
Manifests with congestive heart failure (CHF), often with pronounced right-sided symptoms. Summary / Clinical 5
What is the first-line diagnostic test for Restrictive Cardiomyopathy (RCM)?
Echocardiography. Summary / Diagnosis 6
What is the initial focus of management for Restrictive Cardiomyopathy (RCM)?
Treatment of congestive heart failure (CHF), followed by identification and treatment of the underlying cause. Summary / Treatment 7
What may be necessary for severe or refractory Restrictive Cardiomyopathy (RCM)?
Heart transplantation. Summary / Treatment 8
How common is Restrictive Cardiomyopathy (RCM) compared to other cardiomyopathies?
RCM is the least common form of cardiomyopathy, accounting for 2-5% of all cardiomyopathies. Epidemiology 9
What percentage of patients with Restrictive Cardiomyopathy (RCM) experience heart failure?
Heart failure occurs in over 80% of patients with RCM. Epidemiology 10
What is the most common cause of restrictive cardiomyopathy (RCM) in high-income countries?
Amyloidosis, caused by an accumulation of abnormal proteins in the myocardium. Etiology 11
Name two infiltrative conditions, besides amyloidosis, that can cause Restrictive Cardiomyopathy (RCM).
Sarcoidosis (secondary to deposition of granulomas) and primary hyperoxaluria (secondary to oxalate deposits). Etiology 12
Name two storage disorders involving iron that can cause Restrictive Cardiomyopathy (RCM)?
Hereditary hemochromatosis (increased GI absorption) and iron overload (e.g., from repeated transfusions). Etiology 13
Name two congenital enzyme abnormality categories (storage disorders) that can cause Restrictive Cardiomyopathy (RCM)?
Lysosome storage diseases (e.g., Anderson-Fabry, Mucopolysaccharidosis type I, Gaucher) and Glycogen storage disorders. Etiology 14
What causes endomyocardial fibrosis leading to Restrictive Cardiomyopathy (RCM)?
Caused by autoantibodies against myocardial proteins. Etiology 15
Where is endomyocardial fibrosis, a cause of RCM, most commonly found geographically?
Primarily found in sub-Saharan Africa; it is the most common form of RCM worldwide. Etiology 16
What condition involves eosinophilic infiltration of the myocardium causing Restrictive Cardiomyopathy (RCM)?
Hypereosinophilia (Löffler endocarditis). Etiology 17
What is endocardial fibroelastosis, a cause of Restrictive Cardiomyopathy (RCM)?
A diffuse thickening of the left ventricle endocardium from the proliferation of fibrous and elastic tissue, most commonly occurring in the first two years of life. Etiology 18
What iatrogenic factors can cause Restrictive Cardiomyopathy (RCM)?
Radiotherapy to the chest (radiation-induced fibrosis), chemotherapy (especially anthracyclines like doxorubicin), and cardiac surgery (postoperative scarring). Etiology 19
Name a noninfiltrative connective tissue disease that can cause Restrictive Cardiomyopathy (RCM).
Systemic sclerosis (scleroderma), via microvascular ischemia causing reperfusion injury and secondary fibrosis. Etiology 20
What is the mnemonic provided for causes of restrictive cardiomyopathy?
"PLEASe Help!": Postradiation/Postsurgery fibrosis, Löffler endocarditis, Endocardial fibroelastosis, Amyloidosis, Sarcoidosis, Hemochromatosis. Etiology 21
What is the key pathophysiological consequence of myocardial infiltration or fibrosis in Restrictive Cardiomyopathy (RCM)?
Decreased elasticity of the myocardium, leading to decreased ventricular compliance (severe diastolic dysfunction). Pathophysiology 22
How does decreased ventricular compliance affect diastolic filling and pressures in Restrictive Cardiomyopathy (RCM)?
Leads to decreased ventricular filling in diastole and increased left and right-sided filling pressures. Pathophysiology 23
What changes occur in the atria due to increased filling pressures in Restrictive Cardiomyopathy (RCM)?
Increased atrial size (atrial enlargement). Pathophysiology 24
What is the most common symptom reported by patients with Restrictive Cardiomyopathy (RCM)?
Dyspnea on exertion. Clinical Features 25
What physical exam findings suggest right-sided heart failure in Restrictive Cardiomyopathy (RCM)?
Jugular venous distention (JVD), peripheral edema, hepatomegaly, ascites, and potentially a positive Kussmaul sign. Clinical Features 26
What auscultatory findings are common in Restrictive Cardiomyopathy (RCM)?
Prominent S4 gallop; possible murmurs of mitral regurgitation and/or tricuspid regurgitation. Clinical Features 27
What common dysrhythmia may occur in Restrictive Cardiomyopathy (RCM)?
Atrial fibrillation is common. Clinical Features 28
What is Kussmaul sign, potentially seen in Restrictive Cardiomyopathy (RCM)?
A paradoxical rise in jugular venous pressure (JVP) during inspiration. Clinical Features 29
What are classic, though insensitive, ECG findings in infiltrative Restrictive Cardiomyopathy (e.g., amyloidosis)?
Low voltage QRS complexes. Diagnosis / ECG 30
Besides low voltage, what other ECG abnormalities may be seen in Restrictive Cardiomyopathy (RCM)?
Conduction disorders (AV block, LBBB, RBBB), atrial fibrillation, large P waves (biatrial enlargement). Diagnosis / ECG 31
What might a chest x-ray show in Restrictive Cardiomyopathy (RCM)?
X-ray findings of left atrial enlargement, features of pulmonary congestion, or evidence of underlying disease (e.g., hilar adenopathy in sarcoidosis). Diagnosis / X-ray 32
What is the preferred initial imaging test for suspected Restrictive Cardiomyopathy (RCM)?
Transthoracic echocardiography (TTE). Diagnosis / Echo 33
What are characteristic echocardiographic findings in Restrictive Cardiomyopathy (RCM)?
Severe diastolic dysfunction (specific filling patterns), normal ventricular volumes, preserved EF (early), typically normal wall thickness, left atrial/biatrial enlargement. Diagnosis / Echo 34
In which causes of RCM might ventricular wall thickness be increased on echocardiography?
Amyloidosis, sarcoidosis, and lysosomal storage disease. Diagnosis / Echo 35
What findings on cardiac catheterization can support Restrictive Cardiomyopathy (RCM) diagnosis?
High right atrial pressure; abnormal ventricular pressure (diastolic pressure usually slightly higher in LV than RV); RV systolic pressure (RVSP) may be ≥ 55 mm Hg. Diagnosis / Cath 36
What lab tests help screen for hemochromatosis as a cause of RCM?
Elevated ferritin and transferrin saturation. Diagnosis / Labs 37
What imaging study besides CMR can help diagnose sarcoidosis causing RCM?
FDG-PET scan (shows increased uptake in early disease, reduced uptake in advanced disease). Diagnosis / Imaging 38
What lab tests help screen for amyloidosis causing RCM?
Serum free light chains and serum and urine protein electrophoresis. Diagnosis / Labs 39
How can Cardiac MRI (CMR) aid in the diagnosis and management of Restrictive Cardiomyopathy (RCM)?
Can establish a definitive diagnosis and help guide chronic management by quantitating myocardial disease. Diagnosis / CMR 40
What diagnostic procedure provides a definitive histological diagnosis for Restrictive Cardiomyopathy (RCM)?
Endomyocardial biopsy (EMB). Diagnosis / Biopsy 41
What histological findings might be seen on endomyocardial biopsy in RCM depending on the cause?
Fibrosis, amyloid deposition, iron deposition, granulomas, or eosinophilic infiltrates (Löffler endocarditis). Diagnosis / Biopsy 42
What is the general treatment approach for Restrictive Cardiomyopathy (RCM)?
Symptomatic treatment of heart failure, screen/treat associated diseases (arrhythmias, thromboembolism), treat underlying cause if possible, consider transplant/palliative care. Treatment / Approach 43
What is the first-line medication class for managing heart failure symptoms in Restrictive Cardiomyopathy (RCM)?
Diuretics (used gently to avoid hypotension). Treatment / HF 44
Why must diuretics be used cautiously in Restrictive Cardiomyopathy (RCM)?
Rapid or high-volume diuresis may cause hypotension due to preload sensitivity. Treatment / HF 45
Which medication classes might be used cautiously in RCM to increase diastolic filling time or reduce afterload?
Beta blockers, calcium channel blockers (CCBs), ACE inhibitors (ACEIs), and angiotensin receptor blockers (ARBs) - all used cautiously due to risk of hypotension. Treatment / HF 46
When is anticoagulation indicated in patients with Restrictive Cardiomyopathy (RCM)?
Indicated for patients with a known thrombus, a high risk of thrombogenesis, or atrial fibrillation. Treatment / Complication 47
What are specific treatments for light chain (AL) amyloidosis causing RCM?
Chemotherapy, autologous stem cell transplant, or monoclonal antibodies against plasma cells. Treatment / Specific 48
What are specific treatments for transthyretin amyloidosis (ATTR) causing RCM?
Liver transplant, transthyretin stabilizers, RNA antisense therapies (inotersen, patisiran), monoclonal antibodies, or doxycycline with tauroursodeoxycholic acid. Treatment / Specific 49
What is the specific treatment for sarcoidosis causing RCM?
Immunosuppressive therapy; potentially pacemaker +/- AICD. Treatment / Specific 50
What is the specific treatment for hereditary hemochromatosis causing RCM?
Frequent phlebotomy or iron chelation therapy. Treatment / Specific 51
What is the specific treatment for hypereosinophilic syndrome (Löffler endocarditis) causing RCM?
Corticosteroids, IFN-α, or hydroxyurea. Treatment / Specific 52
What condition presents similarly to Restrictive Cardiomyopathy (RCM) and must be differentiated?
Constrictive pericarditis. Differential Diagnosis 53
On echocardiography, what finding related to respiration helps differentiate constrictive pericarditis from RCM?
Significant respiratory variation in Doppler flow velocities and ventricular septum shift with respiration are seen in constrictive pericarditis (decreased in RCM). Differential Diagnosis 54
What finding on chest CT or MRI is characteristic of constrictive pericarditis but absent in RCM?
Calcified or thickened pericardium (pericardium is normal in RCM). Differential Diagnosis 55
How do LVEDP and RVEDP compare on cardiac catheterization in RCM versus constrictive pericarditis?
In RCM, LVEDP > RVEDP. In constrictive pericarditis, LVEDP is similar to RVEDP (equalization of diastolic pressures). Differential Diagnosis 56
What is the general prognosis for patients with Restrictive Cardiomyopathy (RCM)?
The prognosis for RCM is generally poor. Prognosis 57
What is the approximate overall 5-year survival rate for adult patients with Restrictive Cardiomyopathy (RCM)?
Approximately 50%. Prognosis 58
What is a major risk for pediatric patients shortly after being diagnosed with Restrictive Cardiomyopathy (RCM)?
Over 50% experience sudden cardiac death shortly after diagnosis. Prognosis 59