Objective:Constrictive pericarditis(CP) is rare, resulting from pericardial disease impeding diastolic filling of ventricles. We report case series outlining clinical manifestations, treatment and outcomes.

Patients:6 patients(M=4,F=2) were diagnosed over 10 years. Mean age at presentation was 7.6(±4.5)years, duration from onset to diagnosis was 1.6(±2.2)years. Abdominal distension(ascites) was universal symptom; others included reduced effort tolerance, lethargy, loss of appetite. Pericardial/pleural effusions, raised JVP, edema, hepatomegaly were common signs. Echocardiography revealed dilation of atria, small ventricles, and jerkiness of the interventricular septum. There were marked respiratory swings on Doppler interrogation of mitral and tricuspid inflow velocities consistent with constriction. Significant pericardial effusion was present, but did not cause hemodynamic compromise. Additionally, IVC and hepatic veins were engorged. All patients had structurally normal hearts with no previous cardiovascular disease. Chest x-ray did not reveal calcification, but CT/MRI(heart) showed pericardial thickening. None was subjected to cardiac catheterization. A check for secondary causes revealed SLE in 1, the rest were “idiopathic” CP. Treatment was surgical(total pericardiectomy in 3, partial pericardiectomy in 2) and medical(for SLE) in 1.

Results:All patients survived, within follow-up period of 2.6(±6.6) years. Outcome was good with resolution of clinical signs. Post-procedural echocardiography also improved, except for 1(partial pericardiectomy) where there were residual signs.

Conclusions:Although CP is uncommon, and its diagnosis frequently delayed, combination of ascites, pleural/pericardial effusions should raise possibility of CP in a child. Echocardiography is diagnostic. In absence of secondary causes, there is little role for medical treatment. Total pericardiectomy is definitive treatment and associated with good outcome.