Atrial fibrillation with neonatal pulmonary lymphangiectasia

A female infant born at 28 weeks' gestation was found to have mild hydrops foetalis. Initial echocardiography showed a structurally normal heart. Duri ng the first week of life, episodic atrial tachycardia with 1:1 or 2:1 cond uction was seen, requiring therapy with digoxin. The infant remained ventil ator dependent, with a persistent, chylous pleural effusion which contained a preponderance of lymphocytes. Congenital pulmonary lymphangiectasia (CPL ) was confirmed histologically. Worsening episodes of atrial tachycardia, i ncluding episodes of atrial fibrillation, were further investigated and a r epeat echocardiogram revealed thickening of the entire right atrial wall. T he cardiac findings of a thickened right atrial wall with the histological signs of myocarditis were thought to be the cause of paroxysms of atrial fi brillation, an extremely rare arrhythmia in the neonatal period. To the aut hors' knowledge there have been no previous reports of CPL in association w ith the cardiac abnormalities described herein.