A 3-year-old girl presented with cyanosis. On examination heart rate of 130/min, blood pressure of 100/64 mmHg, and arterial oxygen saturation of 81% were noted. Echocardiography revealed an unbalanced atrioventricular septal defect with double outlet right ventricle and left atrioventricular valve stenosis. The ostium primum atrial septal defect was tiny and restrictive. The ventricular septal defect was very large and biventricular repair was not feasible. A patent ductus arteriosus was also present. Cardiac catheterization showed mean pulmonary artery pressure of 77 mmHg with pulmonary artery wedge pressure of 37 mmHg. Atrial septectomy and ligation of the patent ductus were done along with pulmonary artery banding with 34 mm Mersilene. The postoperative course was uneventful.
Two years later, the child presented with massive ascites with pedal edema and facial puffiness. There was no history of fever, recurrent chest pain, or any other systemic illness during this time. The ascites was gradually progressive. On physical examination, she had elevated jugular venous pressure with hepatomegaly and ascites. There was no pulsus paradoxus. Routine blood investigations were normal. Abdominal ultrasound showed free fluid, hepatomegaly, single left sided spleen, and no other abnormality. Her chest X-ray [Figure 1] showed cardiomegaly and pulmonary venous hypertension. Electrocardiography showed ectopic atrial rhythm, first-degree atrioventricular block, and poor R wave progression but was unchanged from the previous recording.
Echocardiography showed features of congestion such as dilated inferior vena cava and lack of inspiratory collapse. There was no pericardial effusion. Biatrial enlargement was also present. There was no significant atrioventricular valve regurgitation, and systemic ventricular function was normal. Respiratory variation in inflow velocities across the atrioventricular valves or septal bounce were not appreciated due to the presence of a large ventricular septal defect and atrial septectomy. Diagnosis of constrictive pericarditis (CP) was suspected despite cardiomegaly and atrial dilation.
CT angiogram, [Figure 2] however, did not reveal pericardial thickening or calcification. Previous records did not suggest any evidence of the development of postpericardiotomy syndrome (PPS) in the first year after surgery. There was no history of tuberculosis.
A repeat cardiac catheterization showed elevation of right atrial, ventricular diastolic, and pulmonary artery diastolic pressures all being nearly equal around 20 mmHg. Mean pulmonary artery pressure was 33 mmHg with pulmonary vascular resistance index (PVRI) of 2.9 Wood units. The right atrial pressure of 22 mmHg in the absence of a significant left to right shunt, ventricular dysfunction, or atrioventricular regurgitation was suggestive of a constrictive physiology.
The diagnosis was confirmed at operation. The pericardium was found to be adherent to the underlying epicardium over the right atrial and ventricular walls. The pericardium, however, was not significantly thickened. Histopathological examination of the excised pericardial tissue showed fibrosis, hyalinization, and focal chronic inflammation consistent with pericarditis [Figure 3].
Since the calculated PVRI at preoperative catheterization was 2.9 Wood units, and the pulmonary arteries were thin walled at pulmonary arteriotomy, bilateral bidirectional cavopulmonary shunt with antegrade flow interruption was carried out 4 days after pericardiectomy as an intermediate palliation for the future. The staged surgery was done in view of the unusual combination of POCP and the presence of an underlying condition requiring univentricular palliation and can be argued. This is, however, not the point of this communication. There was a remarkable regression in the ascites and marked symptomatic improvement. On follow-up 1-year after pericardiectomy the child is active and growing adequately [Figure 4].