Cardiac sarcoidosis

A cardiac localization is one of the most severe manifestations of sarcoidosis and may cause sudden death (ventricular tachycardia or atrial ventricular block III) or restrictive cardiomyopathy. Lesions are most frequently observed in the interventricular septum and the free left wall. Granulomatous infiltation can provoke nonspecific clinical, electric and echocardiographic signs, which, associated with regressive dipyridamol uptake on tomoscintigraphy, are suggestive of cardiac sarcoidosis. The diagnosis of cardiac sarcoidosis is based on the presence of systemic sarcoidosis, histological evidence of granuloma and the lack of another cause of cardiomyopathy. Corticosteroid therapy is indicated, associated with specific cardiologic treatments.     

Cardiac sarcoidosis

Sarcoidosis is a systemic disease with a favorable prognosis, high remission rate, and low mortality. Cardiac involvement alters this prognosis. Clinical manifestations most commonly include arrhythmias, conduction abnormalities, and congestive heart failure. Treatment includes immunosuppressant therapy, permanent pacemakers in the setting of conduction abnormalities, and implantable cardioverter-defibrillators in patients at risk for sudden cardiac death. Risk stratification for sudden cardiac death is essential in otherwise asymptomatic patients who have suspected cardiac sarcoidosis.     

Cardiac sarcoidosis

Cardiac sarcoidosis can have a variety of manifestations including conduction disease, congestive heart failure, valvular heart disease, pericardial effusions, tamponade, ventricular arrhythmias and sudden cardiac death. In patients with sarcoidosis, the reported incidence of cardiac involvement ranges from 20% in US autopsy studies to nearly 60% in Japan, where it accounts for the majority of deaths as a result of sarcoidosis. Despite this, the diagnosis of cardiac sarcoidosis remains difficult and no single diagnostic test has emerged that combines a high degree of sensitivity and specificity. Recent evidence suggests that gadolinium-enhanced magnetic resonance imaging can potentially offer high sensitivity and specificity for both disease presence and activity. It may also be possible to track response to treatment of cardiac sarcoidosis with cardiac magnetic resonance imaging. Corticosteroids are the mainstay of treatment for cardiac sarcoidosis as with systemic sarcoid but at present no prospective trial has shown a survival benefit. Pharmacological treatment of heart failure should follow standard heart failure guidelines, whereas anti-arrhythmic treatment is problematic. The role of implantable cardiac defibrillators in sarcoid has not been well defined, although the risk of ventricular arrhythmias and sudden cardiac death are high. Cardiac transplantation remains an option for younger patients, although overall cardiac involvement in sarcoidosis carries a relatively poor prognosis.     

Cardiac sarcoidosis

Sarcoidosis is a systemic disease, in which early inflammatory organic changes, which are often reversible under treatment, may take a subclinical course. In scarred residual tissue improvement id rare. Continuing functional loss or even organ failure is possible. The involvement of the heart is of essential prognostic value. Early onset of treatment with corticosteroids before irreversible functional loss of the heart occurs leads to an excellent prognosis. Technical progress in the imaging techniques have led to great improvement of the graphical presentation of cardiac structure and function, but not one of these techniques has yet been approved of for early diagnosis. Still, a combination of different diagnostical measures in addition to the evaluation of clinical symptoms often allows early diagnosis. A decisive factor is "to consider it" and a timely start of further diagnostics if certain symptoms are presented. Alternatives to the specific treatment with cortisone are still lacking. Symptomatic cardiac treatment is mainly oriented on the therapy of dilatory cardiomyopathies, since there are no reliable systematic studies available on questions concerning, for example, antiarrhythmic treatment or indications for the implantations of pacemakers in connection with cardiac sarcoidosis.     

Cardiac involvement in sarcoidosis

Cardiac sarcoidosis is a challenging diagnostic entity. Manifestations range from an incidentally discovered, benign condition to fatal cardiomyopathy and arrhythmias. In this review, we discuss the salient clinical features, diagnostic evaluation, treatment, and prognosis of cardiac sarcoidosis. A definitive histologic diagnosis of myocardial sarcoidosis is difficult, and treatment may be necessary in some patients with suspected cardiac involvement even in the absence of histologic confirmation. Optimal strategies to diagnose cardiac involvement have not been clarified. Thallium (201) radionuclide scintigraphy has been most extensively studied; 2-D echocardiography is less sensitive, but may have a complementary role. Additional radionuclide techniques (e.g., gallium (67) or technetium (99) ) may have a role in complex cases. Given the life-threatening nature of cardiac involvement, prompt and aggressive treatment with corticosteroids or immunosuppressive agents, or both, is warranted. Long-term, possibly lifelong, therapy may be required. Serious, recurrent tachyarrhythmias may require implantation of an automatic cardioverter-defibrillator. Recognizing cardiac sarcoidosis and selecting a sensible management strategy remain the major obstacles facing clinicians.     

Cardiac sarcoidosis: an uncommon presentation of sarcoidosis in India

Cardiac involvement in sarcoidosis is uncommon. The manifestations of cardiac sarcoidosis are protean and vary from benign ectopics to life threatening ventricular tachycardias. High index of suspcion and early aggressive intervention is required for the diagnosis and treatment of this condition. Early diagnosis is essential since ventricular tachycardia is the most common terminal event in these patients if left untreated. Corticosteroids are needed early in these patients which along with other antiarrhythmic management strategies including intracardiac defibrillator form the backbone of treatment. We describe four patients who were diagnosed to have pulmonary and cardiac sarcoidosis. Their varied presentations highlight the difficulties that may occur in the diagnosis. Furthermore, the need for proper management is stressed, as a delay in diagnosis may be fatal.