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Restrictive Cardiomyopathy Clinical Presentation

  • Author: Alan Vainrib, MD; Chief Editor: Henry H Ooi, MD, MRCPI  more...
Updated: Dec 18, 2014


Patients with restrictive cardiomyopathy (RCM) often present at an advanced stage of disease with pronounced cardiopulmonary symptoms. They usually complain of gradually worsening shortness of breath, progressive exercise intolerance, and fatigue. Fatigue and weakness are results of decreased stroke volume and cardiac output. Paroxysmal nocturnal dyspnea may be reported.

Patients may have distention of the abdomen secondary to ascites, but they frequently have profound bilateral peripheral edema. Abdominal discomfort or liver tenderness may be reported.

Chest pain secondary to angina or chest pain mimicking myocardial ischemia can be observed, primarily in patients with amyloidosis, possibly due to myocardial compression of small vessels. Patients may complain of palpitations, frequently due to atrial fibrillation, which is common in idiopathic RCM.

As many as one third of patients with idiopathic RCM may present with thromboembolic complications, especially pulmonary emboli secondary to blood clots in the legs. If atrial fibrillation is present, a high risk of left atrial clots and systemic emboli is present.

Patients may have a history of syncopal attacks from a variety of causes, but orthostatic hypotension secondary to a peripheral and/or autonomic neuropathy should be excluded. Syncope and sudden death are common in AL amyloidosis, but ventricular arrhythmias are uncommon. Electrical-mechanical dissociation is more common. Conduction disturbances are particularly common in some forms of infiltrative RCM, but not in amyloidosis.

Depending on the etiology, patients may have a prior history of radiation therapy, heart transplantation, chemotherapy, or a systemic disease.


Physical Examination

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, or scleroderma).[9, 10] Particular attention should be paid to the cardiovascular and respiratory systems.

General examination

Patients may be more comfortable in the sitting position because of fluid in the abdomen or lungs. Weight loss and cardiac cachexia are not uncommon. Easy bruising, periorbital purpura, macroglossia, and other systemic findings, such as carpal tunnel syndrome, should advise the clinician to consider amyloidosis.

Increased jugular venous pressure is present, with rapid x and y descents; the most prominent finding is usually the rapid y descent. The degree of elevation of the jugular venous pressure indicates the severity of impaired filling of the right ventricle.

The jugular venous pulse fails to fall during inspiration and may actually rise (Kussmaul sign) in constrictive pericarditis. Although less common in RCM, Kussmaul sign cannot be used as an absolute means to distinguish RCM and constrictive pericarditis. The pulse volume is decreased, consistent with decreased stroke volume and cardiac output.

Patients frequently have ascites and pitting edema of the lower extremities. The liver is usually enlarged and full of fluid, which may be painful. However, the liver may be enlarged and firm due to amyloid infiltration. Splenomegaly is rare.

Cardiovascular system examination

Heart sounds S1 and S2 are normal, with a normal S2 split. A loud early diastolic filling sound (S3) may be present but is uncommon in amyloidosis. A fourth heart sound (S4) is almost never present, possibly secondary to amyloid infiltration of the atria. Murmurs due to mitral and tricuspid valve regurgitation may be heard, but they are secondary to the myocardial disease and usually not hemodynamically significant.

Respiratory system examination

Breath sounds are decreased due to pleural effusion, frequently bilateral, and large in amyloidosis. Crepitations or rales are rarely heard, even in advanced heart failure of amyloidosis.



Complications of RCM may include the following:

  • Thromboembolism
  • Dysrhythmias
  • Cardiac cirrhosis
  • Progressive deterioration of cardiac function
Contributor Information and Disclosures

Alan Vainrib, MD Fellow, Department of Cardiology, Stony Brook University Medical Center

Disclosure: Nothing to disclose.


Asa William (Peter) Viccellio, MD Professor, Vice-Chair, Department of Emergency Medicine, State University of New York at Stony Brook

Asa William (Peter) Viccellio, MD is a member of the following medical societies: Medical Society of the State of New York, National Association of EMS Physicians, New York Academy of Medicine, New York Academy of Sciences, New York County Medical Society, American Association for the Advancement of Science, American College of Emergency Physicians, American Medical Association

Disclosure: Nothing to disclose.

Vivek J Goswami, MD Director of Nuclear Cardiology, Austin Heart; Clinical Assistant Professor, Texas A&M Health Science Center College of Medicine

Vivek J Goswami, MD is a member of the following medical societies: American College of Cardiology, American College of Physicians-American Society of Internal Medicine, American Heart Association, American Medical Association, Illinois State Medical Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

A Antoine Kazzi, MD Deputy Chief of Staff, American University of Beirut Medical Center; Associate Professor, Department of Emergency Medicine, American University of Beirut, Lebanon

A Antoine Kazzi, MD is a member of the following medical societies: American Academy of Emergency Medicine

Disclosure: Nothing to disclose.

Chief Editor

Henry H Ooi, MD, MRCPI Director, Advanced Heart Failure and Cardiac Transplant Program, Nashville Veterans Affairs Medical Center; Assistant Professor of Medicine, Vanderbilt University School of Medicine

Disclosure: Nothing to disclose.

Additional Contributors

Gary Edward Sander, MD, PhD, FACC, FAHA, FACP, FASH Professor of Medicine, Director of CME Programs, Team Leader, Root Cause Analysis, Tulane University Heart and Vascular Institute; Director of In-Patient Cardiology, Tulane Service, University Hospital; Visiting Physician, Medical Center of Louisiana at New Orleans; Faculty, Pennington Biomedical Research Institute, Louisiana State University; Professor, Tulane University School of Medicine

Gary Edward Sander, MD, PhD, FACC, FAHA, FACP, FASH is a member of the following medical societies: Alpha Omega Alpha, American Chemical Society, American College of Cardiology, American College of Chest Physicians, American College of Physicians, American Federation for Clinical Research, American Federation for Medical Research, American Heart Association, American Society for Pharmacology and Experimental Therapeutics, American Society of Hypertension, American Thoracic Society, Heart Failure Society of America, National Lipid Association, Southern Society for Clinical Investigation

Disclosure: Nothing to disclose.


The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors Sarath Reddy, MD, Alan Forker, MD, Gunateet Goswami, MD, Nafisa Kuwajerwala, MD, Paul J Kaloudis, MD, and Andrew Wackett, MD, to the development and writing of the source articles.

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Table 1. Clinical Features of Constrictive Pericarditis and Restrictive Cardiomyopathy
Clinical Features Constrictive Pericarditis Restrictive Cardiomyopathy
History Prior history of pericarditis or condition that causes pericardial disease History of systemic disease (eg, amyloidosis, hemochromatosis)
General examination Peripheral stigmata of systemic disease
Systemic examination - Heart sounds Pericardial knock, high-frequency sound Presence of loud diastolic filling sound S3, Low-frequency sound
Murmurs No murmurs Murmurs of mitral and tricuspid insufficiency
Prior chest radiograph Pericardial calcification Normal results of prior chest radiograph
Table 2. Investigation of Constrictive Cardiomyopathy and Restrictive Cardiomyopathy
Investigation Constrictive Cardiomyopathy Restrictive Cardiomyopathy
Chest radiograph Pericardial calcification Atrial dilatation causing increased cardiothoracic ratio, normal ventricular size
CT scan/MRI Pericardial thickening No pericardial thickening
Echocardiography Normal-sized ventricles and atria; pericardial thickening, pericardial effusion may be observed Nondilated, normally contracting, nonhypertrophied ventricles and marked dilatation of both atria; speckled texture of myocardium in cases of amyloid infiltration of the heart
Doppler flow velocities on echocardiography Respiratory changes (ie, decreased peak transmitral diastolic flow) during inspiration Equalization of the right- and left-sided filling pressures No respiratory changes Greater elevation in the left-sided filling pressures
Catheterization hemodynamics:


2) RVEDP:RVSP ratio

3) RVEDP/LVEDP equalization


1) = 50 mm Hg

2) = 0.33

3) = 5 mm Hg difference


1) = 50 mm Hg

2) = 0.33

3) = 5 mm Hg difference

Cardiac biopsy Normal myocardium Often diagnostic, showing abnormal myocardium
CT = computed tomography; LVEDP = left ventricular end-diastolic pressure; MRI = magnetic resonance imaging; RVEDP = right ventricular end-diastolic pressure; RVSP = right ventricular systolic pressure.
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