Abstract

PPHN is a syndrome associated with high morbidity and mortality. Therapy often aims at maintaining pCO2 <30mmHg and pH > 7.5 until pulmonary hypertension resolves. To assess whether the disease or its therapy adversely affects ND or CR outcome,12 infants were evaluated with long-term followup. All were male with mean (M) birth wt.=3.23kg(range2.4-4.2), M gest. age=38wks(35-40), M Apgars 6.6(1-9)lmin/8(4-9)5min and M initial pO2=37mmHg(17-63). During therapy, pCO2 was <30mmHg for a M of 15 hrs(1-24) and pH >7.5 for a M of 62 hrs(36-96). M duration of ventilation was 9.5 days(¼-36) with peak inflating press.(PIP)>30cmH2O for a M of 102 hrs(12-228). M age of followup evaluation was 29 mo(6-48). 9/12 are entirely normal neurologically. 2/12 have slightly increased lower extremity tone and 1 has mod. unilateral hypertonia. 11/12 children are normal or mildly delayed. 9/11 have developmental quotients(DQ) or IQ=87-105. 2/11 have mildly delayed DQ's(70-80). Only 1 is mod-sev delayed(DQ=50); however, he has a strong family history of retardation. All cardiac exams, electrocardiograms, & echocardiograms are normal. 4/12 have chronic lung disease (CLD) requiring bronchodilators. The presence of CLD is apparent only in those exposed to prolonged high PIP (M cmH2O=50 for M=34 hrs). The exact etiology of the CLD in these patients, however, is not clear. Conclusion:Survivors of PPHN have a low incidence of severe ND or CR problems resulting from either the disease or its therapy.

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