[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)].
BACKGROUND
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.


METHODS
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.


RESULTS
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.


METHODS
In most neonates, vasodilators (tolazoline, prostacyclin) and/or nitric oxide were employed.


CONCLUSIONS
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.
Favilli
S
S
UO Cardiologia, Azienda Ospedaliera A. Meyer, Firenze.
De Simone
L
L
Pollini
I
I
Bettuzzi
M G
MG
Cianfrini
D
D
Crepaz
R
R
Santillo
V
V
Trevisanuto
D
D
Vignati
G
G
Manetti
A
A
D004452:Echocardiography / Q000706:statistics & numerical data; D015199:Extracorporeal Membrane Oxygenation; D005500:Follow-Up Studies; D005865:Gestational Age; D006801:Humans; D007231:Infant, Newborn; D007558:Italy / Q000453:epidemiology; D009569:Nitric Oxide / Q000627:therapeutic use; D010547:Persistent Fetal Circulation Syndrome / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000628:therapy; D015995:Prevalence; D011663:Pulmonary Surfactants / Q000627:therapeutic use; D012121:Respiration, Artificial; D014665:Vasodilator Agents / Q000627:therapeutic use
D011663:Pulmonary Surfactants; D014665:Vasodilator Agents; D009569:Nitric Oxide
Prevalenza e caratteristiche della ipertensione polmonare persistente del neonato. Studio multicentrico. Gruppo di Studio della Società Italiana di Cardiologia Pediatrica (SICP).