Restrictive Cardiomyopathy Differential Diagnoses

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

Updated: Jun 29, 2011

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Diagnostic Considerations

All causes of diastolic dysfunction are included in the differential diagnosis of restrictive cardiomyopathy (RCM). Establishing the diagnosis of RCM and excluding constrictive pericarditis are imperative. Whereas RCM has no specific therapy, constrictive pericarditis is potentially curable with surgery.^[2]

The etiology of RCM has been confused with that of constrictive pericarditis because patients can present with findings typical of constrictive pericarditis while actually having RCM. In respect to history and clinical profile, pericardial constriction and RCM may be indistinguishable. In addition, the 2 conditions can coexist in the same patient; for example, radiation therapy affects the myocardium as well as the pericardium. However, there are certain clinical features that help to differentiate the 2 conditions (see Table 1 below).

Table 1. Clinical Features of Constrictive Pericarditis and Restrictive Cardiomyopathy (Open Table in a new window)

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

In the past, diagnosis of RCM was often made during surgery. Now, with wider knowledge of clinical findings and better imaging techniques, the correct diagnosis can be strongly suggested and exploratory surgery can be avoided. The final diagnosis is most commonly made on biopsy of fat tissue, such as abdominal wall fat.

Other conditions to be considered include systemic hypertension, valvular aortic stenosis, and hypertrophic cardiomyopathy, all of which cause impaired diastolic distensibility secondary to left ventricular hypertrophy. The thickened left ventricle could be confused with amyloidosis on echocardiography, but the clinical findings are completely different.

Differential Diagnoses

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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].
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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: 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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