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Researchers report additional high-risk symptoms in fetuses with hypoplastic left heart syndrome
Rychik J. Ultrasound Obstet Gynecol. 2010;36:465-470.
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One-third of fetuses diagnosed as having hypoplastic left heart syndrome had additional high-risk symptoms, including extracardiac abnormalities, that significantly increased risk for surgery-related mortality, study findings indicated.
“Survival for the fetus diagnosed with hypoplastic left heart syndrome (HLHS) is quite variable around the world, with recent reports describing continued poor outcome,” the researchers wrote. “The aim of our study was to establish the current benchmark for early outcome of fetal diagnosis of HLHS from a center with an aggressive approach to surgery.”
In the study, the researchers of The Children’s Hospital of Philadelphia assessed fetuses with HLHS that were stratified into high-risk and standard-risk levels. They defined high risk as the presence of extracardiac, genetic or chromosomal anomalies, prematurity of less than 34 weeks’ gestation, as well as additional cardiac findings, whereas standard risk was the absence of these risk factors.
In 5 years, 240 fetuses were examined, 162 (67.5%) of which were categorized as standard risk, and 78 (32.5%) were considered high risk. Survival of Norwood surgery was 83.8% in the neonates in which surgery was performed (n=185, 77.1%). When broken down by risk class, there was a substantial Norwood operative survival advantage for the standard-risk group over the high-risk group (92.8% vs. 56.5%, P<.001).
“Families should be strongly encouraged to undertake comprehensive prenatal evaluation in order to obtain an accurate prognosis for their fetus with HLHS,” the researchers commented in their concluding remarks. “One-third have additional risk factors that limit survival outcome, however two-thirds do not and have a better chance of early survival. Prenatal counseling should be provided based on individual institutional experiences, and focused resources and increasing experience with staged reconstruction for HLHS should enable improved outcomes at other centers worldwide.”
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