Prenatal diagnosis and risk factors for preoperative death in neonates with single right ventricle and systemic outflow obstruction: screening data from the Pediatric Heart Network Single Ventricle Reconstruction Trial(∗).
OBJECTIVE
The purpose of this analysis was to assess preoperative risk factors before the first-stage Norwood procedure in infants with hypoplastic left heart syndrome and related single-ventricle lesions and to evaluate practice patterns in prenatal diagnosis, as well as the role of prenatal diagnosis in outcome.


METHODS
Data from all live births with morphologic single right ventricle and systemic outflow obstruction screened for the Pediatric Heart Network's Single Ventricle Reconstruction Trial were used to investigate prenatal diagnosis and preoperative risk factors. Demographics, gestational age, prenatal diagnosis status, presence of major extracardiac congenital abnormalities, and preoperative mortality rates were recorded.


RESULTS
Of 906 infants, 677 (75%) had prenatal diagnosis, 15% were preterm (<37 weeks' gestation), and 16% were low birth weight (<2500 g). Rates of prenatal diagnosis varied by study site (59% to 85%, P < .0001). Major extracardiac congenital abnormalities were less prevalent in those born after prenatal diagnosis (6% vs 10%, P = .03). There were 26 (3%) deaths before Norwood palliation; preoperative mortality did not differ by prenatal diagnosis status (P = .49). In multiple logistic regression models, preterm birth (P = .02), major extracardiac congenital abnormalities (P < .0001), and obstructed pulmonary venous return (P = .02) were independently associated with preoperative mortality.


CONCLUSIONS
Prenatal diagnosis occurred in 75%. Preoperative death was independently associated with preterm birth, obstructed pulmonary venous return, and major extracardiac congenital abnormalities. Adjusted for gestational age and the presence of obstructed pulmonary venous return, the estimated odds of preoperative mortality were 10 times greater for subjects with a major extracardiac congenital abnormality.
Atz
Andrew M
AM
Division of Pediatric Cardiology, Medical University of South Carolina, Charleston, SC 29425, USA. atzam@musc.edu
Travison
Thomas G
TG
Williams
Ismee A
IA
Pearson
Gail D
GD
Laussen
Peter C
PC
Mahle
William T
WT
Cook
Amanda L
AL
Kirsh
Joel A
JA
Sklansky
Mark
M
Khaikin
Svetlana
S
Goldberg
Caren
C
Frommelt
Michele
M
Krawczeski
Catherine
C
Puchalski
Michael D
MD
Jacobs
Jeffrey P
JP
Baffa
Jeanne M
JM
Rychik
Jack
J
Ohye
Richard G
RG
D000704:Analysis of Variance; D016009:Chi-Square Distribution; D005260:Female; D006352:Heart Ventricles / Q000002:abnormalities* / Q000601:surgery*; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000175:diagnosis* / Q000401:mortality* / Q000601:surgery*; D007231:Infant, Newborn; D008297:Male; D015337:Multicenter Studies as Topic; D058327:Norwood Procedures; D011247:Pregnancy; D011296:Prenatal Diagnosis; D016032:Randomized Controlled Trials as Topic; D012307:Risk Factors; D016896:Treatment Outcome