A 17-year-old girl presented with gradual onset breathlessness, initially on exertion, NYHA class II since 2 weeks which progressed to NYHA class IV. She had an episode of febrile illness and chest X-ray showed right plural effusion, 6 months ago. Pleural fluid analysis was suggestive of tuberculous etiology; hence, anti-tuberculous treatment was started. In the emergency room, she was tachypneic at rest and room air saturation was 85%. Clinically, there was sinus tachycardia with heart rate of 124 min-1. On auscultation, there was no murmur, but decreased breath sounds bilaterally in both lower one third of the chest. Chest X-ray showed mild cardiomegaly with bilateral pleural effusion. ECG showed sinus tachycardia with low voltage complexes (Fig. 1). The differential diagnosis of disseminated multi-drug-resistant tuberculosis, high-grade lymphoma and collagen vascular disease was made. Echocardiography was suggestive of effusive constrictive pericarditis such as >50% variations of Doppler velocities, thickened pericardium, dilated inferior vena cava, noncollapsing with respiration (Fig. 2, Fig. 3). Also there was evidence of minimal ascites and bilateral mild pleural effusion on sonographic examination of abdomen and chest. Laboratory investigations revealed mild anemia [Hb - 8.9 g%], a slight increase in TLC with lymphocyte predominance with normal ESR initially. Peripheral smear showed normocytic normochromic red blood cells, absolute lymphocytic leukocytosis, and adequate platelets. Liver function test showed mild elevation of bilirubin, liver enzymes, and mild hypoalbuminaemia. Serological markers (such as antinuclear antibody and RA factor) of collagen vascular diseases were negative. Serum LDH level was 652 U/L that is usually elevated in lymphomas. Pleural effusion fluid analysis showed predominance of lymphocytes with no evidence of any organism. ADA level was 72 mg/dl. Pleural fluid cytology showed predominately lymphocytes, few neutrophils, and reactive mesothelial cells. There were no malignant cells. Her viral screening including HIV-1 and HIV-2 was nonreactive. Laboratory investigations were not conclusive of any possible etiology of the cause of constriction. Hence, a possible diagnosis of tuberculous constrictive pericarditis was made. Pleural fluid was negative for any bacterial growth after 48 h of incubation.
Contrast-enhanced CT scans of chest revealed soft tissue density mass encasing cardiac chambers with significant bilateral pleural effusion with passive collapse of both lungs.
Meanwhile, there was no response to conservative anti-failure treatment hence, the case was discussed with cardiothoracic surgeon and planned for surgical pericardiectomy. She underwent total pericardiectomy through midline thoracotomy, pericardium was grossly thickened, adherent to sternum and myocardium (Fig. 4A-D). There were few areas of fibrosis and calcifications. Post operatively, the patient improved significantly with reduction of her symptoms.
Histopathological examination of pericardial biopsy report showed fibro-adipose tissue of pericardium and adjacent striated muscles infiltrated with monomorphic lymphocytes with high mitotic activity. These infiltrating cells were positive for CD45 [LCA], negative for CD3, CD20, and CD138, weak-to-moderate positive for terminal deoxynucleotidyl transferase (TdT), high MIB-1 index [70-80%]. Chemotherapy in consultation with medical oncologist was started in the form of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP regime).
After initial improvement, her symptoms worsened further 1 week later in the form of progressive breathlessness, requiring noninvasive ventilation. She developed type II respiratory failure hence, electively intubated and ventilated. She had persistent hypotension and hyponatreamia, which required high doses of inotropes, intravenous sodium correction. Despite our best efforts, she died secondary to multi-organ dysfunction.