Objective: HLHS remains one of the most challenging forms of congenital heart disease to treat. Advances in fetal imaging have increased the frequency of prenatal identification of HLHS. Accurate prenatal counseling depends upon knowledge of the most current outcome statistics. We report on early outcomes for the fetus with HLHS, based on a large, single-institutional experience.
Methods: Fetuses with HLHS presenting between 7/04 – 7/08 were reviewed. Survival is defined as alive at discharge following Norwood operation. Risk factors analyzed were: presence of extracardiac or genetic/chromosomal anomaly (type I), cardiac factors such as intact atrial septum, severe valve regurgitation or ventricular dysfunction (type II) or prematurity < 34 wks (type III). Subjects are stratified based on “high-risk” = presence of any risk factors, or “standard-risk” = absence of risk factors.
Results: 210 fetuses were evaluated; 142 (68%) standard-risk and 68 (32%) high-risk. Risk factors were distributed as follows: 39 (19%) type 1, 28 (13%) type II, 7 (3%) type III; 6 fetuses had > 1 risk factor. Outcome from point of fetal diagnosis is shown in table. For all fetuses treated, survival was 84%; standard-risk had significantly better survival outcome than high-risk fetuses (93% vs 57%, p<0.0001). Anatomical subtype of HLHS did not relate to survival.
Conclusions: Early survival for the fetus with HLHS, with no additional anomalies and born at full term, is excellent in the current era. Our study provides for a means of stratifying outcome for the fetus with HLHS and has important implications for prenatal counseling.