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Restrictive Cardiomyopathy Treatment & Management

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

Approach Considerations

Restrictive cardiomyopathy (RCM) has no specific treatment. However, therapies directed at individual causes of RCM have been proven to be effective. Examples of this include corticosteroids for sarcoidosis and Loeffler endocarditis, endocardiectomy for endomyocardial fibrosis and Loeffler endocarditis, phlebotomy and chelation for hemochromatosis, and chemotherapy for amyloidosis. The mainstays of medical treatment include diuretics, vasodilators, and angiotensin-converting enzyme inhibitors (ACEs) as indicated, as well as anticoagulation (if not contraindicated).[14]

In selected patients, permanent pacing, LVAD therapy, 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. Beta blockers and cardioselective calcium channel blockers (eg, verapamil, diltiazem) may be of benefit, by increasing left ventricular filling time, improving ventricular relaxation, and decreasing compensatory sympathetic stimulation. In addition, low-medium dose diuretics lower preload and may provide symptomatic relief. 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 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 therapeutic phlebotomy 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 or ventricular mechanical support therapy can be considered in highly selected patients with refractory symptoms who have idiopathic or familial RCM and amyloidosis. Patients treated with an LVAD with HCM have similar mortalities compared with those with dilated cardiomyopathy.[15] 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. This stems from data that has shown that these patients have poor early and late outcomes after cardiac transplantation related to fibrosis related procedural complications and new or recurrent malignancies.[16]

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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].

  6. Towbin JA. Inherited Cardiomyopathies. Circ J. 2014 Sep 2. [Medline].

  7. Peled Y, Gramlich M, Yoskovitz G, Feinberg MS, Afek A, Polak-Charcon S, et al. Titin mutation in familial restrictive cardiomyopathy. Int J Cardiol. 2014 Jan 15. 171(1):24-30. [Medline].

  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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