Long-term follow-up of newborns with persistent pulmonary hypertension

Persistent pulmonary hypertension of the newborn (PPHN) is a syndrome associated with high morbidity and mortality. Mechanical ventilation attempts to maintain a PaCO2 less than 30 torr and a pH greater than 7.5 until pulmonary hypertension resolves. To assess whether the disease or its therapy adversely affects neurodevelopmental or cardiorespiratory outcome, 11 infants diagnosed and treated for PPHN were evaluated at a mean age of 31 months. Nine had normal developmental quotients (DQs) and 2 had mildly delayed DQs. Eight children were entirely normal neurologically, 2 had slightly increased lower-extremity tone, and 1 had unilateral hypertonia. All cardiac exams, echocardiograms, and ECGs were normal. Four children had chronic lung disease requiring either daily or intermittent bronchodilator therapy; however, their activity levels were unaffected. These results suggest that subsequent normal development with little significant medical compromise may be expected in this group of critically ill infants.