Restrictive Cardiomyopathy Treatment & Management
- Author: Alan Vainrib, MD; Chief Editor: Henry H Ooi, MD, MRCPI more...
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).
In selected patients, permanent pacing, LVAD therapy, and transplantation (heart or heart-liver) may be considered.
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.
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.
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%.
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. 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.
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|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|
|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|
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.|