Restrictive Cardiomyopathy Treatment & Management

  • Author: Asa William (Peter) Viccellio, MD; Chief Editor: Henry H Ooi, MBBCh   more...
 
Updated: Jun 29, 2011
 

Approach Considerations

With the possible exception of chelation therapy for hemochromatosis and corticosteroid therapy for sarcoidosis, restrictive cardiomyopathy (RCM) has no specific treatment. The mainstays of medical treatment include diuretics, vasodilators, and angiotensin-converting enzyme inhibitors (ACEIs) as indicated, as well as anticoagulation (if not contraindicated).[9]

In selected patients, permanent pacing and transplantation (heart or heart-liver) may be considered.

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Pharmacologic Therapy

The goal of treatment in RCM is to reduce symptoms by lowering elevated filling pressures without significantly reducing cardiac output. Presently, no drugs selectively enhance myocardial relaxation. Therefore, current therapy consists predominately of low-dose diuretics to lower the preload. Small initial doses should be administered to prevent hypotension because patients are frequently extremely sensitive to alterations in left ventricular volume. Higher doses may be needed if the serum albumin level is low secondary to concomitant nephrotic syndrome.

ACEIs and angiotensin II inhibitors are poorly tolerated in patients with amyloidosis. Even small doses may result in profound hypotension, probably secondary to an autonomic neuropathy. Beta-blockers and calcium channel blockers have not been shown to improve day-to-day symptoms or to favorably alter the natural history in patients with diastolic heart failure. No published data are available on the use of intravenous (IV) inotropic or vasodilator drugs.

Patients with a history of embolization or atrial fibrillation should be anticoagulated. In patients with atrial fibrillation, the rate should be controlled adequately. Removal of the atrial contribution to ventricular filling may worsen the existing diastolic dysfunction, and a rapid ventricular response may further compromise diastolic filling, creating a crisis. Therefore, maintaining sinus rhythm is important, and medications such as amiodarone and beta-blockers are often used.

Digoxin should be used with caution because it is potentially arrhythmogenic, particularly in patients with amyloidosis.

Antiplasma cell therapy with melphalan may slow the progress of systemic amyloidosis by stopping production of the paraprotein responsible for the formation of amyloid. The prognosis of patients with primary systemic amyloidosis remains poor, with a median survival of approximately 2 years despite intervention with alkylating-based chemotherapy in selected cases. In specific cases, chemotherapy has dramatic benefits, with improvement in systemic and cardiac manifestations.

The treatment of Loeffler endocarditis consists of correctly identifying the condition before the end-stage fibrosis occurs. Medical therapy with corticosteroids, cytotoxic agents (eg, hydroxyurea), and interferon to suppress the intense eosinophilic infiltration of the myocardium is appropriate during the early phase of Loeffler endocarditis and improves symptoms and survival. Conventional heart failure medication is also given.

Chelation therapy or venesection is effective in patients with hemochromatosis to decrease the iron overload.

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Pacemaker Implantation

Patients with idiopathic restrictive cardiomyopathy (RCM) may have fibrosis of the sinoatrial and atrioventricular nodes that result in complete heart block, and, therefore, require permanent pacing. If cardioversion to treat atrial fibrillation is attempted, particularly in patients with amyloidosis, the abnormal sinus node may fail as an effective pacemaker. Patients with sinus node dysfunction and/or advanced conduction system disease also require treatment with implantation of a pacemaker.

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Endomyocardectomy

As noted, treatment of Loeffler endocarditis depends on correctly identifying the condition before the end-stage fibrosis occurs and typically involves early pharmacotherapy (see Pharmacologic Therapy).

In the fibrotic stage of Loeffler endocarditis, surgical therapy, with excision of the fibrotic endocardium and replacement of the mitral and tricuspid calves, is palliative but may provide symptomatic improvement. The operative mortality is in the range of 15-25%.

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Cardiac Transplantation

Cardiac transplantation can be considered in highly selected patients with refractory symptoms who have idiopathic or familial RCM and amyloidosis. When noncardiac organ involvement is absent, a few patients with amyloidosis have undergone successful cardiac transplantation, combined with postoperative high-dose chemotherapy, to abolish recurrent amyloid production.

Combined heart and liver transplantation in patients with heart and liver failure due to hemochromatosis has been successful in small numbers of patients. However, early morbidity and mortality are higher in dual-organ transplantation than in single-organ transplantation.

Transplantation is a treatment option for cardiac sarcoidosis, but recurrence of sarcoid granulomas can occur in the transplanted heart.

A surgical approach offers a cure for pericardial constriction but carries a potential for significant morbidity for RCM. Thus, establishing a clear diagnosis is crucial, and the advent of current sophisticated imaging technology helps in that regard (see Workup). Fewer patients now need exploratory open-heart surgery to establish the correct diagnosis.

Stem cell transplantation used in conjunction with high-dose chemotherapy is still considered experimental by most cardiologists. Its routine use has not yet been established.

Finally, whether patients who have radiation-induced cardiac diseases are candidates for heart transplant is uncertain. Concerns have been raised as to whether their immune system would allow them to receive the immunosuppressive therapy necessary after the surgical procedure and whether relapse of their malignancy might occur.

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

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

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

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

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

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

  8. 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].

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

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Table 1. Clinical Features of Constrictive Pericarditis and Restrictive Cardiomyopathy
Clinical Features Constrictive Pericarditis Restrictive Cardiomyopathy
HistoryPrior history of pericarditis or condition that causes pericardial diseaseHistory of systemic disease (eg, amyloidosis, hemochromatosis)
General examinationPeripheral stigmata of systemic disease
Systemic examination - Heart soundsPericardial knock, high-frequency soundPresence of loud diastolic filling sound S3, Low-frequency sound
MurmursNo murmursMurmurs of mitral and tricuspid insufficiency
Prior chest radiographPericardial calcificationNormal results of prior chest radiograph
Table 2. Investigation of Constrictive Cardiomyopathy and Restrictive Cardiomyopathy
Investigation Constrictive Cardiomyopathy Restrictive Cardiomyopathy
Chest radiographPericardial calcificationAtrial dilatation causing increased cardiothoracic ratio, normal ventricular size
CT scan/MRIPericardial thickeningNo pericardial thickening
EchocardiographyNormal-sized ventricles and atria; pericardial thickening, pericardial effusion may be observedNondilated, 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 echocardiographyRespiratory 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 biopsyNormal myocardiumOften 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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