Primary Cardiac Lymphoma Presenting Clinically as Restrictive Cardiomyopathy

Abstract

rimary cardiac lymphoma (PCL) is extremely rare, accounting for only 1.3% of all primary cardiac tumors, 1 and its prognosis is poor because its rarity and nonspecific clinical presentation make an antemortem diagnosis difficult. 2 However recent reports suggest that early diagnosis and proper treatment can lead to long-term survival. 2,3 We report an unusual case of PCL in which the patient presented a clinical picture of restrictive cardiomyopathy with arrhythmias that resolved following systemic chemotherapy. Diastolic heart failure caused by PCL has rarely been described. 4 Case ReportA 72-year-old woman was admitted for evaluation of exertional dyspnea and palpitations. She had been well until the year before admission, during which exertional dyspnea gradually worsened. Five months prior to admission, a dry cough developed and 1 month prior, the exertional dyspnea and palpitations rapidly worsened, and she reported a 6 kg weight gain in that month.On admission, the patient's blood pressure was 124/ 61 mmHg and her heart rate was 40 beats/min. Jugular venous distension was marked. The first and second heart sounds were normal, and no murmurs were detected. Inspiratory rales were present at the base of both lungs. The liver was palpable 2 cm below the right costal margin. There was peripheral edema. No lymphadenopathy was noted. Circulation Journal Vol.69, February 2005Laboratory investigations were normal except for a slight elevation in the serum lactate dehydrogenase (LDH) concentration (566 IU/L). Chest roentgenogram showed cardiomegaly, pulmonary congestion, and distended pulmonary arteries (Fig 1). Electrocardiography (ECG) revealed atrioventricular dissociation with a ventricular rate of 40 beats/min (Fig 2). Transthoracic echocardiography showed mild atrial and ventricular enlargement. There were not any findings of pericardial effusion, pericardial thickness, or cardiac tumor. The ejection fraction of the left ventricle was 67%. Doppler echocardiography revealed tricuspid regurgitation with a pressure gradient of 34 mmHg. Cardiac catheterization revealed an increased right enddiastolic pressure of 17 mmHg and a left end-diastolic pres- An unusual case of primary cardiac lymphoma presenting as restrictive cardiomyopathy with arrhythmia is reported in a 72-year-old woman who was admitted for evaluation of exertional dyspnea and palpitations. Electrocardiography (ECG) showed atrioventricular dissociation and right heart cardiac catheterization revealed a typical 'dip-and-plateau' waveform. Restrictive cardiomyopathy was suspected because computed tomography (CT) did not reveal pericardial thickening, calcifications, or an effusion. Heart failure initially improved with diuretic therapy, but subsequently worsened, and the patient experienced a syncopal episode. ECG showed atrial fibrillation, and CT revealed a large mass in the right atrium and multiple tumors in the liver, which needle biopsy confirmed as diffuse large B-cell lymphoma. Chemotherapy induced complete remission, and her heart failure mark...

Primary cardiac lymphoma

“…A clinical picture consistent with inferior vena cava [17,23,24] and superior vena cava compression syndromes [8,10,11,18,23,[25][26][27], and aortic stenosis [28] have also been reported. Rarely, PCL masquerading as constrictive pericarditis may also be found [29].…”

“…In some patients with the so called "B symptoms," unspecific symptoms such as weight loss [21,26,35], fever [18,25,27,33], night sweats [2,13,18,[35][36][37], and generalized pruritus [36] may occur. Table 1 summarizes these findings.…”

Diffuse Large B-cell Lymphoma Diagnosed by Intracardiac Echocardiography-guided Cardiac Tumor Biopsy

“…Therefore, the current definition of PCL is a lymphoma presenting as cardiac disease, especially when the vast bulk of the tumor is intrapericardial (5). Several cases with extensive extracardiac involvement have been reported as PCL (9). The present case could also be considered as PCL.…”

Extranodal Lymphomas and Tumors of the Thymus

“…Heart: Primary lymphoma of the heart and great vessels comprises less than 2% of all primary cardiac tumors but carries a poor prognosis due to its disruption of the cardiac conduction system [44]. The majority of secondary cardiac lymphomas occur in the right side of the heart and are noted in up to 10% of disseminated DLBCL in autopsy series [45]- [47] Posttransplant lymphoproliferative disorders can also involve the allograft in patients with a heart transplant [48,49].…”