Restrictive Cardiomyopathy Clinical Presentation

Author: Asa William (Peter) Viccellio, MD; Chief Editor: Henry H Ooi, MBBCh more...

Updated: Jun 29, 2011

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History
Physical Examination
Complications
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History

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).^{[5, 6]}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

Complications of RCM may include the following:

Thromboembolism
Dysrhythmias
Cardiac cirrhosis
Progressive deterioration of cardiac function

Proceed to Differential Diagnoses

Contributor Information and Disclosures

Author

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: American Association for the Advancement of Science, American College of Emergency Physicians, American Medical Association, Medical Society of the State of New York, National Association of EMS Physicians, New York Academy of Medicine, New York Academy of Sciences, and New York County Medical Society

Disclosure: Nothing to disclose.

Coauthor(s)

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, and Illinois State Medical Society

Disclosure: Nothing to disclose.

Specialty Editor Board

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, Louisiana State Medical Society, National Lipid Association, and Southern Society for Clinical Investigation

Disclosure: Nothing to disclose.

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

Disclosure: Medscape Salary Employment

A Antoine Kazzi, MD Chair and Medical Director, 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.

David FM Brown, MD Associate Professor, Division of Emergency Medicine, Harvard Medical School; Vice Chair, Department of Emergency Medicine, Massachusetts General Hospital

David FM Brown, MD is a member of the following medical societies: American College of Emergency Physicians and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Chief Editor

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

Henry H Ooi, MBBCh is a member of the following medical societies: American College of Cardiology, American Heart Association, Heart Failure Society of America, and International Society for Heart and Lung Transplantation

Disclosure: Nothing to disclose.

Acknowledgments

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.

References

Kushwaha SS, Fallon JT, Fuster V. Restrictive cardiomyopathy. N Engl J Med. Jan 23 1997;336(4):267-76. [Medline].
Goldstein JA. Differentiation of constrictive pericarditis and restrictive cardiomyopathy. ACC Ed Highlights. Fall 1998;14-22.
Schlant RC, Alexander RW, eds. The Heart. McGraw-Hill; 1994:1637-45.
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].
Davies MJ, Mann JM. Systemic pathology. In: The Cardiovascular System. Vol 10. 1995:1409-16.
Wald DS, Gray HH. Restrictive cardiomyopathy in systemic amyloidosis. QJM. May 2003;96(5):380-2. [Medline].
Braunwald E, Abelmann WH. Atlas of Heart Diseases. Vol 2. 1994:53-61.
Leya FS, Arab D, Joyal D, Shioura KM, Lewis BE, Steen LH, et al. The efficacy of brain natriuretic peptide levels in differentiating constrictive pericarditis from restrictive cardiomyopathy. J Am Coll Cardiol. Jun 7 2005;45(11):1900-2. [Medline].
Tintinalli JE, Kelen GD, Stapczynski JS, eds. Emergency Medicine: A Comprehensive Study Guide. McGraw-Hill; 2004:381.

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: 1) RVSP 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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