The prevalence and characteristics of persistent pulmonary hypertension of the newborn. a multicenter study. the Study Group of the Società Italiana di Cardiologia Pediatrica (SICP)
Favilli, S.; De Simone, L.; Pollini, I.; Bettuzzi, M.G.; Cianfrini, D.; Crepaz, R.; Santillo, V.; Trevisanuto, D.; Vignati, G.; Manetti, A.
Giornale Italiano di Cardiologia 28(11): 1247-1252
1998
ISSN/ISBN: 0046-5968 PMID: 9866802 Document Number: 490134
Persistent pulmonary hypertension of the newborn (PPHN) is a rare syndrome with a severe prognosis, in which a prompt diagnosis can be life-saving. The aim of our study was to verify its prevalence in a neonatal population, define clinical and echocardiographic criteria for the diagnosis of PPHN and discuss therapeutic choices. The following clinical and echocardiographic criteria for the diagnosis of PPHN were defined: 1. cyanosis and hypoxemia non-responsive to O2 therapy; 2. right to left shunt at an atrial or ductal level. All neonates fulfilling these criteria referred to the neonatal units of seven pediatric or general hospitals over a two-year period were enrolled. From January 1995 to December 1996, thirty neonates with PPHN (8%) were observed. Birth was pre-term in 5 out of 30. Ten (33%) had experienced chronic and/or fetal asphyxia (FA). Death occurred in 7 (22%), four of whom with FA. Echocardiography showed tricuspid insufficiency in 18 (60%); mean pulmonary systolic pressure was 67 +/- 16 mmHg (range 41-95). In 23 surviving neonates, normalization of clinical and echocardiographic parameters occurred in 8 +/- 5 days. In most neonates, vasodilators (tolazoline, prostacyclin) and/or nitric oxide were employed. PPHN is confirmed to be a rare pathological condition; prognosis is severe, particularly in neonates with FA. Echocardiography is a reliable non-invasive method for a prompt diagnosis and follow-up. Subsequent studies are needed to assess therapeutic choices.