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Restrictive Cardiomyopathy Differential Diagnoses

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

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

 
 
Contributor Information and Disclosures
Author

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

Disclosure: Nothing to disclose.

Coauthor(s)

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.

Acknowledgements

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
  1. Kushwaha SS, Fallon JT, Fuster V. Restrictive cardiomyopathy. N Engl J Med. 1997 Jan 23. 336(4):267-76. [Medline].

  2. Goldstein JA. Differentiation of constrictive pericarditis and restrictive cardiomyopathy. ACC Ed Highlights. 1998 Fall. 14-22.

  3. Amaki M, Savino J, Ain DL, Sanz J, Pedrizzetti G, Kulkarni H, et al. Diagnostic concordance of echocardiography and cardiac magnetic resonance-based tissue tracking for differentiating constrictive pericarditis from restrictive cardiomyopathy. Circ Cardiovasc Imaging. 2014 Sep. 7(5):819-27. [Medline].

  4. Schlant RC, Alexander RW, eds. The Heart. McGraw-Hill; 1994. 1637-45.

  5. 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. 1999 Apr. 46(4):473-86. [Medline].

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  8. [Guideline] Lindenfeld J, Albert NM, Boehmer JP, et al, for the Heart Failure Society of America. HFSA 2010 Comprehensive Heart Failure Practice Guideline. J Card Fail. 2010 Jun. 16(6):e1-194. [Medline].

  9. Davies MJ, Mann JM. Systemic pathology. The Cardiovascular System. 1995. Vol 10: 1409-16.

  10. Wald DS, Gray HH. Restrictive cardiomyopathy in systemic amyloidosis. QJM. 2003 May. 96(5):380-2. [Medline].

  11. Sasaki N, Garcia M, Ko HH, Sharma S, Parness IA, Srivastava S. Applicability of published guidelines for assessment of left ventricular diastolic function in adults to children with restrictive cardiomyopathy: an observational study. Pediatr Cardiol. 2014 Sep 6. [Medline].

  12. Braunwald E, Abelmann WH. Atlas of Heart Diseases. 1994. Vol 2.: 53-61.

  13. 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. 2005 Jun 7. 45(11):1900-2. [Medline].

  14. Tintinalli JE, Kelen GD, Stapczynski JS, eds. Emergency Medicine: A Comprehensive Study Guide. McGraw-Hill; 2004. 381.

  15. Topilsky Y, Pereira NL, Shah DK, et al. Left ventricular assist device therapy in patients with restrictive and hypertrophic cardiomyopathy. Circ Heart Fail. 2011 May. 4(3):266-75. [Medline].

  16. Uriel N, Vainrib A, Jorde UP, Cotarlan V, Farr M, Cheema FH. Mediastinal radiation and adverse outcomes after heart transplantation. J Heart Lung Transplant. 2010 Mar. 29(3):378-81. [Medline].

  17. [Guideline] Hershberger RE, Lindenfeld J, Mestroni L, Seidman CE, Taylor MR, Towbin JA. Genetic evaluation of cardiomyopathy--a Heart Failure Society of America practice guideline. J Card Fail. 2009 Mar. 15(2):83-97. [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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