To analyze the main prenatal characteristics of hypoplastic left heart syndrome (HLHS), its association with extracardiac anomalies including increased nuchal translucency (NT) and the outcome of affected patients. We searched our database for classical forms of HLHS (aortic atresia, mitral and aortic atresia and critical aortic stenosis evolved to a severely hypoplastic left ventricle) prenatally diagnosed between 1998 and 2006. Data on 101 fetuses were retrieved and analyzed. The main reason for referral was suspected heart defect on a routine ultrasound scan (82%). The mean gestational age at diagnosis was 21 weeks. Most cases were detected at < or = 22 weeks (72%), the upper limit for termination of pregnancy (TOP) in our country (Spain). An intact atrial septum was diagnosed in 11 of the 58 fetuses (19%) in which pulmonary vein blood flow was assessed, and this diagnosis was proved to be correct in the six liveborn babies. Most fetuses (68%) had an isolated HLHS. Fourteen fetuses (14%) were chromosomally abnormal and all had associated extracardiac defects. NT was above the 95th centile in 21 of the 74 cases (28%) in which this measurement was available. 79% (58/73) of the cases in which HLHS was detected at < or = 22 weeks were terminated, and no differences in the rate of TOP were found through the study period. Among the 43 continuing pregnancies, seven fetuses died in utero and there were 36 live births; in 12 cases the parents opted for compassionate care and 24 chose to have the infant surgically treated. In the cohort of intention-to-treat cases, the overall survival rate was 36% (9/25). This rate improved from 18% (2/11) in the period 1998-2002 to 50% (7/14) in 2003-2006. There were no survivors in cases with intact atrial septum or when there were associated defects. At follow-up, 2/9 survivors suffered from significant neurological morbidity. Fetal echocardiography allows an accurate diagnosis of HLHS, which is made in most instances in the first half of pregnancy. Despite the advantage offered by the prenatal detection of HLHS, which provides the opportunity to plan perinatal management, our up-to-date results show that the outlook for these fetuses is still poor, and highlight the importance of presenting these figures when counseling parents with affected fetuses.