Clinical manifestations and outcomes of constrictive pericarditis in children.

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.