Constrictive pericarditis in childhood due to mediastinal irradiation

Abstract

Between 1955 and 1972, eight children receiving mediastinal irradiation for intrathoracic malignancy developed constrictive pericarditis. Six of these followed irradiation for Hodgkin's disease and represent 7% of 86 children receiving irradiation for that disease. In patients with Hodgkin's disease, the development of constrictive pericarditis was related to the dose of radiation and the use of orthovoltage technique. Constrictive pericarditis has not developed so far among the 44 children radiated by supravoltage technique. Symptoms of constrictive pericarditis occurred an average of 15 mth (4 mth to 3 yr 2 mth) after irradiation and were preceded in five of the eight patients by acute pericarditis. Prominent clinical findings included dyspnea, Kussmaul sign, pulsus paradoxicus, hepatomegaly, cardiomegaly and electrocardiographic evidence of low QRS voltages with ST-T wave changes. Pericardiectomy was performed in six patients and resulted in immediate but only transient symptomatic relief. There was no operative mortality. Death occurred in six of the eight patients (1 yr 5 mth to 6 yr 6 mth following irradiation) due to cardiac disease in three, progression of the malignancy in two and infection in one. In addition to constrictive pericarditis, extensive myocardial fibrosis and involvement of the antero-lateral papillary muscle and chordae tendineae of the mitral valve were demonstrated at postmortem examination. These factors may explain the progression of cardiac disease despite an initial symptomatic improvement following pericardiectomy.