Improved surgical outcome after fetal diagnosis of hypoplastic left heart syndrome

Background-Hypoplastic left heart syndrome (HLHS) is frequently diagnosed p renatally, but this has not been shown to improve surgical outcome. Methods and Results-We reviewed patients with HLHS between July 1992 and Ma rch 1999 to determine the influence of prenatal diagnosis on preoperative c linical status, outcomes of stage 1 surgery, and parental decisions regardi ng care. Of 88 patients, 33 were diagnosed prenatally and 55 after birth. O f 33 prenatally diagnosed patients, 22 were live-born, and pregnancy was te rminated in 11. Of 22 prenatally diagnosed patients who were live-born, 14 underwent surgery, and parents elected to forego treatment in 8. Of 55 pati ents diagnosed postnatally, 38 underwent surgery, and 17 did not because of parental decisions or clinical considerations. Prenatally diagnosed patien ts were less likely to undergo surgery than patients diagnosed after birth (P=0.008), Among live-born infants, there was a similar rate of noninterven tion. Among patients who underwent surgery, survival was 75% (39/52). All p atients who had a prenatal diagnosis and underwent surgery survived, wherea s only 25 of 38 postnatally diagnosed patients survived (P=0.009). Patients diagnosed prenatally had a lower incidence of preoperative acidosis (P=0.0 2), tricuspid regurgitation (P=0.001), and ventricular dysfunction (P=0.004 ). They were also less likely to need preoperative inotropic medications or bicarbonate (P=0.005). Preoperative factors correlating with early mortali ty included postnatal diagnosis (P=0.009), more severe acidosis (P=0.03), n eed for bicarbonate or inotropes (P=0.008 and 0.04), and ventricular dysfun ction (P=0.05). Conclusions-Prenatal diagnosis of HLHS was associated with improved preoper ative clinical status and with improved survival after first-stage palliati on in comparison with patients diagnosed after birth.