INTRODUCTION: Sarcoidosis is a multisystem disease which is very enigmatic and the disease course may be indolent to life threatening complications especially with cardiac involvement which predisposes young patients to sudden cardiac death. Diagnosis of cardiac sarcoidosis remains a major challenge to most clinicians especially when the patient presents with no prior diagnosis of sarcoidosis. To our knowledge, this is one of the rare cases with sustained ventricular tachycardia (VT) as the first manifestation without having the previous symptoms and diagnosis of sarcoidosis.
CASE PRESENTATION: A 41-year-old African American male presented to the emergency Room with one day history of nausea, vomiting and palpitations. He reported that his symptoms started with palpitations a day before presentation followed by nausea and vomiting which prompted him to seek medical attention. He denied any chest pain, shortness of breath, fever, recent travel, sick contacts, and similar complaints in the past. He reported no past medical history and denied taking any medications. He admitted to have had a binge of alcohol intake the night before presentation and otherwise claimed to take alcohol occasionally. He denied smoking or doing illegal drugs. Family history was non-contributory. Physical exam revealed a well built man with blood pressure of 93/39 and a heart rate of 230beats/min with cool extremities and diaphoresis. Electrocardiogram (ECG)showed sustained monomorphic ventricular tachycardia.Electrical cardioversion failed and pharmacologic cardioversion reverted the patient back to sinus rhythm. Initial laboratory tests were normal except for magnesium which was 1.6mg/dl and was subsequently replaced. Once the patient was stabilized, a repeat physical examination revealed an enlarged right supraclavicular gland. Chest X-ray revealed bilateral hilar prominence and right paratracheal density. Transthoracic echocardiogram showed increased echogenicity and thickening of interventricular septum with normal ejection fraction and no associated left ventricular dysfunction. Cardiac catheterization showed non-obstructive coronaries. MRI of the chest showed extensive hilar and mediastinal lymphadenopathy with no evidence of right ventricular dysplasia. Biopsy of the right supraclavicular gland showed non-caseating granulomas. Cultures from the lymph node for mycobacterium and fungus were negative. Serum Angiotensin Converting Enzyme (SACE) level was high (91U/L) corroborating with the clinical diagnosis of sarcoidosis. During the hospital stay, the patient did not have any arrhythmias including any atrioventricular blocks. Subsequently Automated Intra-Cardiac Defibrillator (AICD) was placed to prevent sudden cardiac death and the patient was discharged to home on steroids and beta-blockers. He was seen in the clinic four months later and was asymptomatic and the follow-up SACE level was normal (47U/L).
DISCUSSIONS: Cardiac sarcoidosis can present either with acute or chronic clinical manifestations. Acute manifestations can range from conduction abnormalities and arrhythmias which may culminate in sudden cardiac death and chronic disease can present as CHF, valvular abnormalities, pericarditis, and also cor pulmonale. Often patients can have intermittent atrioventricular block and VT and this is more suggestive of cardiac sarcoidosis. Cardiac involvement is life threatening and prompt treatment with steroids, immunosuppressive therapy or both is warranted in sarcoidosis.(1) AICD with aggressive corticosteroid therapy with or without immunosuppressive therapy is recommended in patients with ventricular tachycardia and also prophylactic AICD placement in patients who have ventricular tachycardia during exercise EKG or Holter monitoring.
CONCLUSION: Cardiac sarcoidosis is a rare and difficult to diagnose with no systemic involvement. Clinical acumen is recquired to suspect and treat such patients as the morbidity and mortality is high in untreated patients. Clinical scenario should be corroborated with imaging modalities to make diagnosis in suspected cases and treatment with corticosteroids and AICD placement is warranted in all patients with cardiac involvement.
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