The development of pulmonary hypertension in a child with portal hypertension: An unusual association

Abstract

The development of pulmonary hypertension in patients with portal hypertension is a rare association. The exact cause of pulmonary hypertension in these patients remains obscure at present. We present details of a patient with this association who eventually died from pulmonary hypertension and acute right-sided heart failure. We fur¬ther discuss the etiologic and pathophysiologic mechanisms responsible for this rare entity.