Brief communication

Cardiac tamponade secondary to sarcoidosis☆,☆☆

Pericardial involvement of sarcoidosis is a rare cause for acute heart failure, and usually occurs as a result of the development of a pericardial effusion leading to cardiac tamponade. Even rarer still, is the manifestation of constrictive pericarditis. We report a case of sarcoidosis with lung, pleural, and pericardial involvement with effusive-constrictive pericarditis leading to cardiac tamponade.

A 34-year-old Caucasian man presented for evaluation of a history of worsening exertional dyspnea, edema, and weight loss. A high-resolution chest computed tomography showed diffuse pulmonary nodules with upper lobe predominance and in a perilymphatic distribution; large right pleural effusion; and large pericardial effusion with pericardial thickening. A transthoracic echocardiogram demonstrated early tamponade physiology for which a pericardial drain was placed. After removal of the drain he developed cardiogenic shock from cardiac tamponade attributed to the reaccumulation of a pericardial effusion and urgent pericardial window was performed. Serial echocardiography was concerning for organization and localization of the pericardial fluid. Cardiac magnetic resonance imaging demonstrated a significant reduction in pericardial slippage between the parietal and visceral layers around the heart collectively suggestive of constrictive pericarditis. Confirmation of effusive-constrictive pericarditis was noted on right heart catheterization. He then underwent pericardiectomy, which on histopathologic evaluation demonstrated non-necrotizing granulomas, thus confirming pericardial involvement of sarcoidosis.

We report a case demonstrating unique manifestations of sarcoidosis; effusive-constrictive pericarditis presenting with acute congestive heart failure.

Pericardial effusion and cardiac tamponade are rare manifestations of cardiac sarcoidosis. This is a first case report that describes a patient with severe pericardial effusion and signs of cardiac tamponade with elevated carbohydrate antigen 125 (CA-125) levels, enlarged pericardial (PLN) and mediastinal lymph nodes (MLN), histologically confirmed as sarcoidosis.

A 51-year-old female patient was admitted with complaints of sickness, shortness of breath on minimal exertion, swelling in lower extremities, and heaviness in right upper abdomen. Patient had diminished heart sounds, peripheral edema and hepatomegaly. She had elevated CA-125 level without gynecologic pathology. There were QRS alternation on ECG and water-bottle configuration on chest-X-ray, severe pericardial effusion, and signs of cardiac tamponade on echocardiography. CT demonstrated massive pericardial effusion, pericardial mass and enlargement of anterior MLN.

The patient underwent pericardial drainage with removal of 850 mL of pericardial fluid and excision of enlarged PLN. Histological examination of PLN revealed non-caseating epithelioid cell granulomas. The diagnosis of cardiac sarcoidosis was established. Patient was discharged and 6-month follow-up was uneventful.

There are no reports on association of pericardial effusion, with increased CA-125 level in sarcoidosis, as we established in our patient. Our case is notable by incidental finding of enlarged PLN, mimicking pericardial mass and mediastinal lymphoadenopathy on CT, further confirmed by histological examination of PLN specimen as cardiac sarcoidosis.

It should be kept in mind that sarcoidosis may present as massive pericardial effusion, with signs of tamponade and pericardial lymphoadenopathy mimicking pericardial mass, mediastinal lymphoadenopathy and elevated CA-125, mimicking malignancy.

Granulomatous myocarditis may develop into cardiomyopathy and severe congestive heart failure that requires implantation of a left ventricular assist device (LVAD).

Left ventricular (LV) core samples were collected from 177 patients with severe heart failure at the time of LVAD implantation, and samples were histologically examined and graded for severity of hypertrophy and fibrosis. Granulomatous myocarditis incidentally seen in a subset of samples was characterized by staining and culturing for mycobacteria and fungi. Various clinical parameters in these patients were analyzed.

Of the 177 LV core samples examined, 6 (3.4%) showed nonnecrotizing granulomatous inflammation in the myocardial wall. Stains and cultures for mycobacteria and fungi were negative. All six patients [three women, three men; five African American, one Asian; mean age, 52±9 years (range, 41–61 years)] had arrhythmias and required an automatic implantable cardioverter defibrillator. Before LVAD implantation, the patients' mean cardiac index was 1.8±0.4 l/min/m²; cardiac output, 2.9±0.6 l/min; and ejection fraction, 20±2%. One year after LVAD implantation, one patient had undergone heart transplantation. At 2 years, a second patient was transplanted, and one died. At 3 years, a third patient was transplanted and died postoperatively; two patients remained on support. No clinical evidence indicated involvement of other organs or recurrence in the transplanted patients.

The incidental diagnosis of granulomatous myocarditis in our patients indicates that histological study of LV core samples in patients who undergo LVAD implantation may contribute to the diagnosis and be a consideration in the management of the underlying cause of heart failure.

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.

Acute pericarditis is associated with four categories of systemic diseases: vasculitis-connective tissue disease; immunopathies and hypersensitivity states; disorders of metabolism; and diseases of uncertain origin or in association with various syndromes. Multiple overlapping may occur among these categories. This article discusses clinical signs and treatment of pericarditis as it occurs with these diseases.

The full recognition of sarcoid heart disease has been slow, and it is my opinion that it is a condition that is still much underdiag-nosed. Accurate diagnosis depends on suspicion and further investigation. Once the diagnosis is made, treatment and prognosis can be understood more precisely.

The first description of heart involvement was by Bernstein and co-workers in 1929,¹ and the first death from this cause was reported in 1937 by Gentzen.² Thereafter, there were occasional reports of small numbers of cases, frequently in blacks in the southern part of the United States. It was long stated that cardiac sarcoidosis was rare and, in 1971, when Gozo et al.³ searched the entire literature, they found a total of only 70 cases. The varied clinical and pathologic features soon were established and were well reviewed by Porter⁴ and by the Japanese. Matsui⁵ and Sekiguchi,⁶ with their co-workers, have made particularly valuable contributions to the knowledge of this condition.