ASE 2013: Outcomes of Infants with Hypoplastic Left Heart Syndrome


Minneapolis, MN: Researchers have announced the results of a retrospective 5-year study, which shows that echocardiography can be useful in identifying new strategies for combatting the growth failure in infants with Hypoplastic Left Heart Syndrome (HLHS). The high mortality and morbidity rate for these high-risk infants with congenital cardiac anomalies has been difficult to change, but this is a promising step for medical intervention.

“Despite improvements in survival after surgical palliation, mortality remains as high as 30% in these patients and is sometimes hard to predict. It is important, therefore, to continue to look for other potential risk factors within this high risk population. Our goal was to use echocardiography to investigate fetal vascular predictors of post-natal growth”, stated Thomas Miller, DO, the study’s primary investigator. Dr. Miller and his colleagues analyzed 65 infants, finding that, unlike healthy infants, the umbilical artery systolic and diastolic ratio did not decrease with gestational age in many patients with HLHS. Using echocardiography they have shown that uteroplacental insufficiency (UPI) may be a predictive marker to assess growth potential in these patients.

Hypoplastic left heart syndrome (HLHS) refers to a collection of congenital cardiac anomalies characterized by underdevelopment or incomplete development or absence of the left ventricle and severe underdevelopment of the ascending aorta. The infant’s circulation can be critically compromised because of its systemic and/or pulmonary vasculature or, after surgery, by subsequent systemic inflammatory response.

Researchers on the study, Umbilical Artery Systolic to Diastolic Ratio and Growth Outcomes in Infants with Hypoplastic Left Heart Syndrome, included Thomas Miller, Shaji Menon, Lisa Joss-Moore, Cindy Weng, Michael Puchalski from the University of Utah, Salt Lake City, UT.

A poster based on the results will be displayed on Monday, July 1 from 9 am-4 pm. Presenters will attend their posters in the Poster and Exhibit Hall from 12-1 pm at the American Society of Echocardiography (ASE) 24th Annual Scientific Sessions at the Minneapolis Convention Center, Minneapolis, MN.

Source: ASE.

1 Comment

  1. I’m so excited to hear all this!! My son, Sebastian, was born in March with HLHS and beuacse we received the diagnosis when I was 19 weeks pregnant, we were able to prepare by banking both his cord blood and stem cells. We did this in hopes that in 20 or 30 years when he’d need a transplant medical research would have advanced enough to be able to grow his own heart, rather than needing to sit and wait on the transplant list. Fast forward 7 months, he’s now post-Norwood but while waiting for the Glenn his heart got too big, making him not a good candidate and suddenly we’re told transplant is the only way to go. Sebastian is now 10 months old and was listed for transplant in Novemeber. We’ve undergone 4 IVIG treatments to make him a better match for a donor heart, but the folks here at Stanford say to expect a long wait 6 to 9 months, maybe a year, before a donor can be found. Since Sebastian is post-Norwood, we’re not sure he has that time please, please tell me if there’s anything we can do now to grow him a heart!


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