Background and purpose: Constrictive pericarditis (CP) is rare in children. The purpose of this study was to define the clinical manifestations and outcomes of this disease.
Methods: From January 1985 through December 2003, 8 patients with surgically confirmed CP were enrolled. Data on clinical characteristics, disease manifestations, treatment, and outcomes were collected and analyzed.
Results: One patient had l-transposition of the great arteries (l-TGA); the other 7 patients had no underlying cardiovascular diseases. None of the patients had direct evidence of tuberculous infection. Age at disease onset ranged from 8 months to 13 years (median, 10 years), and the interval between symptom onset and correct diagnosis ranged from 2 to 31 months (median, 12 months). Major symptoms and signs included abdominal distension, hepatomegaly, neck vein engorgement and ascites. Evidence of calcification on chest roentgenogram was found in 1 patient (12.5%). Computed tomography or magnetic resonance image studies showed pericardial thickening in 6 patients (75%). Seven patients underwent cardiac catheterization, and all hemodynamic data met the diagnostic criteria for CP. All patients with structurally normal hearts survived during a median follow-up of 31 months after pericardiectomy (range, 5 to 103 months). The patient with l-TGA died of pneumonia 1 year after pericardiectomy.
Conclusions: Symptoms and signs of right heart failure were common in children with CP. The etiology of CP remains largely unknown. Patients with structurally normal hearts have good outcomes after pericardiectomy.