Prenatal diagnosis of hypoplastic left heart syndrome in current era.

TLDR: Recent experience has shown that preDx of HLHS was not associated with a survival advantage, fewer postoperative complications, or shorter length of stay, and improved preoperative status was observed in the postDx patients; however, they were born earlier with a lower birthweight.

Abstract: We sought to evaluate the relation of a prenatal diagnosis (preDx) with morbidity and mortality during the initial hospitalization in a contemporary cohort of patients with hypoplastic left heart syndrome (HLHS) A retrospective study of patients with HLHS presenting from 1999 to 2010 was performed Patients with genetic disorders or a gestational age <34 weeks or who had intentionally received comfort care only were excluded Of the 81 patients meeting the study criteria, 49 had a preDx and 32 were diagnosed postnatally (postDx) Birth weight (median 30 vs 34 kg; p = 0007) and gestational age (median 38 vs 39 weeks; p <0001) were lower in the preDx than in the postDx patients Preoperatively, the postDx patients were intubated more frequently (97% vs 71%, p = 0004) and ventilated longer (median 96 vs 24 hours, p = 0005) than the preDx patients They also had more preoperative acidosis, multiorgan failure, tricuspid valve regurgitation, and right ventricular dysfunction Of the 73 patients undergoing surgery, no difference in survival was seen between the preDx and postDx groups (91% vs 89%) The median duration of postoperative ventilation was 7 days and the median length of stay was 36 days for the 66 survivors, with no difference between the 2 groups Postoperative morbidities, including chylothorax and infection, were also similar in the preDx and postDx patients No studied preoperative factor was associated with death, duration of postoperative ventilation, or length of stay In conclusion, our recent experience has shown that preDx of HLHS was not associated with a survival advantage, fewer postoperative complications, or shorter length of stay Improved preoperative status was observed in the preDx patients; however, they were born earlier with a lower birthweight What effect these factors might have on longer term morbidity remains unknown