Cor Pulmonale in Severe Pierre Robin Syndrome

Abstract

Pulmonary hypertension or cor pulmonale, cardiomegaly, and pulmonary edema have been rarely reported in association with the upper airway obstruction found in severe Pierre Robin syndrome.1,2 The case presented herein supports Jersaty's1 contention that cor pulmonale and heart failure with pulmonary edema may be more common in severe cases of this syndrome than heretofore recognized. In this case tracheostomy resulted in dramatic regression of these life-threatening cardiorespiratory complications. CASE REPORT A white male infant was born on Dec 20, 1974 with Apgar scores of 5 and 7. His birth weight was 3.61 kg. The child had the classical features of a severe Pierre Robin syndrome with a wide cleft palate, micrognathia, and glossoptosis (Fig 1).