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Cardiomyopathy, Restrictive
Updated: Jul 3, 2008
Introduction
Background
Restrictive cardiomyopathy is the least common of the 3 clinically recognized and described cardiomyopathies. Its principal abnormality is diastolic dysfunction, specifically restricted ventricular filling. Differentiation from constrictive pericarditis, a clinically similar entity, is difficult but important because the treatment options and prognosis differ drastically.
Pathophysiology
Restrictive cardiomyopathy can be idiopathic or secondary to a heart muscle disease that manifests as restrictive physiology. The common hemodynamic disturbance is impairment of ventricular filling due to the thickening and increased rigidity of the endocardium and myocardium secondary to infiltration by amyloid tissue or by fibrosis. Ventricular diastolic pressures are elevated and may inscribe a "dip and plateau" or "square root" pattern, both similar to constrictive pericarditis. Systolic function remains normal or near normal until late stages.
Frequency
United States
Occurrence of restrictive cardiomyopathy is rare with no quantifiable incidence.
International
Endomyocardial fibrosis (EMF), an etiologic factor of idiopathic restrictive cardiomyopathy, is a progressive disease of unknown cause that occurs most commonly in children and young adults in tropical and subtropical Africa, primarily in Uganda and Nigeria. EMF may account for up to one fourth of deaths due to cardiac disease in those areas.
Mortality/Morbidity
The natural history of restrictive cardiomyopathy is especially poor in children with heart failure. Adults experience a prolonged course of heart failure and may have complications of cardiac cirrhosis and thromboembolism. Patients who are refractory to supportive therapy usually die of low-output cardiac failure unless cardiac transplantation is an option.
Race
Natives of Africa, South America, and India are predisposed to EMF.
Age
EMF is most common in children and young adults.
Clinical
History
- Patients often present at an advanced stage of disease with pronounced cardiopulmonary symptoms. The patient's history may reveal the following:
- Angina (uncommonly)
- Dyspnea, orthopnea, or dyspnea on exertion
- Paroxysmal nocturnal dyspnea
- Peripheral edema
- Abdominal discomfort, liver tenderness
- Increased abdominal girth, ascites
Physical
- In addition to the findings listed below, a careful general physical examination must be conducted to search for extracardiac manifestations of a systemic disorder that may cause secondary restrictive cardiomyopathy (eg, hemochromatosis, amyloidosis, sarcoidosis, scleroderma).
- Elevated jugular venous pulse, Kussmaul sign
- y descent blunted relative to x
- S3 and/or S4
- Occasional mitral or tricuspid regurgitation murmur
- Distant heart sounds
- Pulmonary rales
- Peripheral edema
- Pulsus paradoxus - As the restrictive process progresses, no change in blood pressure occurs with inspiration.
Causes
- According to guidelines of the World Health Organization, cardiomyopathy refers to the disease of the myocardium secondary to an idiopathic cause (ie, primary cardiomyopathies). However, secondary infiltrative myocardial diseases, which are actually cardiac manifestations of systemic diseases, often are grouped together with cardiomyopathies.
- Idiopathic restrictive cardiomyopathy
- Secondary restrictive cardiomyopathy
- Hemochromatosis
- Amyloidosis
- Sarcoidosis
- Progressive systemic sclerosis (scleroderma)
- Carcinoid heart disease
- Glycogen storage disease of the heart
- Radiation
- Metastatic malignancy
- Anthracycline toxicity
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| References |
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References
Braunwald E, Abelmann WH. Atlas of Heart Diseases. Vol 2. 1994:53-61.
Davies MJ, Mann JM. Systemic pathology. In: The Cardiovascular System. Vol 10. 1995:1409-16.
Goldstein JA. Differentiation of constrictive pericarditis and restrictive cardiomyopathy. ACC Ed Highlights. 1998;Fall:14-22.
Higano ST, Azrak E, Tahirkheli NK, Kern MJ. Hemodynamic rounds series II: hemodynamics of constrictive physiology: influence of respiratory dynamics on ventricular pressures. Catheter Cardiovasc Interv. Apr 1999;46(4):473-86. [Medline].
Kasper DL, Braunwald E, Fauci AS, eds. Harrison's Principles of Internal Medicine. McGraw-Hill; 2005:chap 221.
Kushwaha SS, Fallon JT, Fuster V. Restrictive cardiomyopathy. N Engl J Med. Jan 23 1997;336(4):267-76. [Medline].
Schlant RC, Alexander RW, eds. The Heart. McGraw-Hill; 1994:1637-45.
Tintinalli JE, Kelen GD, Stapczynski JS, eds. Emergency Medicine: A Comprehensive Study Guide. McGraw-Hill;2004:381.
Wald DS, Gray HH. Restrictive cardiomyopathy in systemic amyloidosis. QJM. May 2003;96(5):380-2. [Medline].
Willerson JT, Cohn JN, ed. Cardiovascular Medicine. Churchill Livingstone; 1995:871-86.
Further Reading
Keywords
diastolic dysfunction, restrictive cardiomyopathy, endomyocardial fibrosis, EMF, dip and plateau configuration, dip and plateau pattern, square root pattern, square root configuration, heart failure, cardiac cirrhosis, thromboembolism, low-output cardiac failure
