Prenatal diagnosis by echocardiogram and outcome of absent pulmonary valve syndrome

Reza S. Razavi, Gurleen K. Sharland, John M. Simpson*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

43 Citations (Scopus)


We reported echocardiographic findings and outcomes of fetuses with absent pulmonary valve syndrome diagnosed during fetal life. Cases were identified from a prospectively acquired computerized database of 18,308 pregnancies referred to a fetal cardiology center between January 1988 and July 2000. Twenty fetuses were identified with a median gestation of 23 weeks (range 18 to 36) at presentation. In 18 cases (90%), there was an associated ventricular septal defect. Eighteen cases (90%) had branch pulmonary artery diameters above the normal range. In four cases (20%), an arterial duct was present. A chromosome 22q11 deletion was identified in 2 of 9 cases (22%) in which this deletion was sought. There were 6 terminations of pregnancy (30%), 3 intrauterine deaths (15%), 5 neonatal deaths (25%), 3 infant deaths (15%), and 3 patients who did not die (15%). Ten of the 11 "liveborn" infants required early ventilation. The outcome of absent pulmonary valve symdrome diagnosed prenatally appears poor. The high morbidity and mortality is due to both cardiac disease and associated bronchomalacia.

Original languageEnglish
Pages (from-to)429-432
Number of pages4
JournalAmerican Journal of Cardiology
Issue number4
Publication statusPublished - 15 Feb 2003
Externally publishedYes


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