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Cardiomyopathy, Restrictive: Differential Diagnoses & Workup

Author: Paul J Kaloudis, MD, Clinical Assistant Professor, Department of Emergency Medicine, Stony Brook School of Medicine; Physician Lead, Department of Information Technology, Stony Brook University Hospital
Coauthor(s): Asa William (Peter) Viccellio, MD, Professor, Vice-Chair, Department of Emergency Medicine, State University of New York at Stony Brook; Robert Fan, MD, Attending Staff, Department of Emergency Medicine, Kaiser Permanente Medical Center at Hayward
Contributor Information and Disclosures

Updated: Jul 3, 2008

Differential Diagnoses

Cardiomyopathy, Dilated
Congestive Heart Failure and Pulmonary Edema
Pericarditis and Cardiac Tamponade
Pericarditis, Constrictive
Sarcoidosis

Workup

Laboratory Studies

  • CBC: An elevated eosinophil count may indicate eosinophilic endomyocardial disease.

Imaging Studies

  • Chest radiography
    • Absence of cardiomegaly, normal cardiac silhouette, no pericardial calcification (seen in constrictive pericarditis)
    • Manifestations of pulmonary venous hypertension and pulmonary congestion
  • Echocardiography
    • Normal to symmetrically thickened walls
    • Rapid early-diastolic filling, slow late-diastolic filling
    • Normal or slightly reduced ventricular volume and systolic function
    • Minimal to no respiratory variation in Doppler flow velocities
    • No pericardial thickening
  • CT and MR imaging may help in differentiating from constrictive pericarditis.

Other Tests

  • Endomyocardial biopsy
    • May detect typical eosinophil infiltration in the inflammatory stage
    • May detect myocardial fibrosis in later-stage cases
    • Negative findings do not exclude diagnosis
    • Risk of embolism may be due to dislodgement of recent ventricular thrombus
  • Electrocardiography
    • Intraventricular conduction delays, left bundle-branch block (LBBB) common, and right bundle-branch block (RBBB) are most typical of amyloidosis and sarcoidosis.
    • Low voltage (usually secondary to amyloidosis and hemochromotosis)
    • Nonspecific ST-T changes
    • Various arrhythmias
    • Chamber enlargement

Procedures

  • Cardiac catheterization
    • Elevated and equalized ventricular diastolic filling pressures
    • Dip and plateau or square root configuration of the diastolic portion of the ventricular pressure pulse
    • Normal to slightly decreased ejection fraction
    • Prominent a wave and x and y descent
    • Pulmonary artery pressure often greater than 40 mm Hg
    • Little to no variation in systolic pressure between the right and left ventricle with inspiration

More on Cardiomyopathy, Restrictive

Overview: Cardiomyopathy, Restrictive
Differential Diagnoses & Workup: Cardiomyopathy, Restrictive
Treatment & Medication: Cardiomyopathy, Restrictive
Follow-up: Cardiomyopathy, Restrictive
References
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References

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

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

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

  4. 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].

  5. Kasper DL, Braunwald E, Fauci AS, eds. Harrison's Principles of Internal Medicine. McGraw-Hill; 2005:chap 221.

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

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

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

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

  10. Willerson JT, Cohn JN, ed. Cardiovascular Medicine. Churchill Livingstone; 1995:871-86.

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Further Reading

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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

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Contributor Information and Disclosures

Author

Paul J Kaloudis, MD, Clinical Assistant Professor, Department of Emergency Medicine, Stony Brook School of Medicine; Physician Lead, Department of Information Technology, Stony Brook University Hospital
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: 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.

Robert Fan, MD, Attending Staff, Department of Emergency Medicine, Kaiser Permanente Medical Center at Hayward
Disclosure: Nothing to disclose.

Medical Editor

David FM Brown, MD, Assistant Professor, Department of Medicine, Division of Emergency Medicine, Harvard Medical School; Associate-Chief, Attending Physician, 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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

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.

CME Editor

John D Halamka, MD, MS, Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center
John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Chief Editor

Jonathan Adler, MD, Attending Physician, Department of Emergency Medicine, Massachusetts General Hospital; Division of Emergency Medicine, Harvard Medical School
Jonathan Adler, MD is a member of the following medical societies: American Academy of Emergency Medicine and Society for Academic Emergency Medicine
Disclosure: eMedicine.com, Inc. Consulting fee Consulting

 
 
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