Up to 50% of patients presenting with congestive heart failure (CHF) have preserved ejection fraction, the majority of whom have hypertension, coronary disease, or other well-known substrates. As many as 10-15% have either restrictive cardiomyopathy or constrictive pericarditis. This paper reviews this latter group. The following are key points to remember:

1. Patients with restrictive cardiomyopathy typically present with the usual constellation of HF symptoms, including dyspnea and fatigue with typical findings of CHF on physical exam. Atrial fibrillation is common. Anatomically, these patients are characterized by having normal or increased left ventricular wall thickness and normal or reduced left ventricular size with preserved left ventricular systolic function. Symptoms are largely mediated by reduced left ventricular compliance and diastolic dysfunction.
2. Echocardiography or cardiac magnetic resonance imaging (CMR) is essential to detect the anatomical features alluded to above and to characterize hemodynamic issues such as secondary valvular regurgitation and pulmonary hypertension. Echocardiography is crucial for staging the degree of diastolic abnormalities.
3. A small number of cases are related to a true primary idiopathic restrictive cardiomyopathy, which can present in either childhood or adulthood. Familial and sporadic cases are both described, and in familial cases, a skeletal myopathy may also be present.
4. Secondary restrictive cardiomyopathies include infiltrative, noninfiltrative, and storage disorders. Worldwide, endomyocardial fibrosis is probably the most common cause of restrictive cardiomyopathy, but is rare in the United States. Hypereosinophia syndromes, associated with parasitic infection or hematological malignancy result in a similar presentation with obliterative apical fibrosis and thrombus. CMR provides additional diagnostic information by detecting subendocardial fibrosis.
5. Cardiac amyloidosis is an infiltrative form of restrictive cardiomyopathy and is often associated with a monoclonal gammopathy or multiple myeloma. Wild-type transthyretin amyloidosis is seen in 25-36% of patients over the age of 80 years. Patients with symptomatic cardiac amyloidosis present with typical findings of HF with preserved ejection fraction (HFpEF). A low voltage electrocardiogram (ECG) and increased wall thickness with abnormal texture on echocardiography are the classic findings. Doppler imaging confirms the concurrent diastolic dysfunction. More recently, a unique global longitudinal strain pattern with apical sparing has been associated with cardiac amyloidosis.
6. The goal of treatment in cardiac amyloidosis is symptom relief; however, many forms of traditional therapy confer a greater risk in cardiac amyloidosis than in other forms of cardiac disease. Diuretic therapy is necessary for volume control; however, there is an increased likelihood of hypotension and renal insufficiency in these patients. Digoxin and calcium channel blockers may lead to heart block, and beta-blockers and angiotensin-converting enzyme inhibitors may be poorly tolerated. Patients with cardiac amyloidosis require a greater degree of scrutiny for complications of therapy than other patients.
7. Patients who received mediastinal radiation for malignancy develop a number of radiation-related complications including constrictive pericarditis and myocardial fibrosis, which may lead to a restrictive-type cardiomyopathy. The distribution of the restrictive myopathy can be highly variable with disproportionate right ventricular involvement common. Additionally, radiation may result in premature proximal coronary artery disease as well as valvular heart disease. These patients typically present after a multi-year delay with a clinical presentation similar to other forms of HFpEF.
8. Multiple glycogen storage diseases including Fabry disease, Pompe’s disease, and lysosome-associated membrane protein disorders may result in cardiac involvement typically with ECG and echocardiographic features similar to that seen in hypertrophic cardiomyopathy. Many of these disorders are associated with extracardiac manifestations including developmental delay. The echocardiographic features often mimic that of hypertrophic cardiomyopathy, and diastolic dysfunction is common. In many instances, CMR may show a mid-myocardial late gadolinium enhancement.
9. Constrictive pericarditis is the result of abnormal pericardial compliance, which in the majority of instances is associated with a thickened pericardium. When the etiology is prior inflammation (tuberculosis being the classic example), pericardial calcification is often detected. Dynamic imaging with echocardiography or CMR can detect the exaggerated respiratory interdependence of the ventricles and abnormal septal shifts. Other echocardiographic features include reversal of the lateral to medial annular velocities. Treatment includes sodium restriction and diuretic agents to reduce edema and hepatic congestion; however, definitive therapy will require surgical pericardiectomy.
10. Distinguishing a restrictive cardiomyopathy from constrictive pericarditis is often problematic. Clinically, the patients present with a virtually identical constellation of signs and symptoms. Echocardiography to detect exaggerated respiratory interdependence and annular septal velocities may be diagnostic of pericardial constriction, but cannot determine pericardial thickness. Either CMR or computed tomography is essential for determining pericardial thickness, and computed tomography is highly sensitive for detecting pericardial calcification.

Keywords: Amyloid Neuropathies, Familial, Angiotensin-Converting Enzyme Inhibitors, Atrial Fibrillation, Cardiomyopathy, Restrictive, Calcium Channel Blockers, Cardiomyopathy, Hypertrophic, Cardiomyopathy, Restrictive, Constriction, Coronary Artery Disease, Diuretics, Echocardiography, Electrocardiography, Endomyocardial Fibrosis, Heart Failure, Heart Valve Diseases, Hematologic Neoplasms, Hypertension, Pulmonary, Hypertension, Magnetic Resonance Imaging, Pericarditis, Constrictive, Renal Insufficiency, Thrombosis, Tomography

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