Objectives: To review our 13-year experience with prenatally detected hypoplastic left heart syndrome (HLHS) of which management remains controversial.
Material and methods: Retrospective study of the management and outcome in all cases of HLHS diagnosed prenatally in a tertiary referral center for pediatric cardiology and cardiac surgery between January 1988 and July 2001.
Results: The diagnosis of HLHS was made in 32 fetuses. One mother had two pregnancies associated with HLHS. In 16 cases parents opted for termination of pregnancy and in five for compassionate care. Four fetuses died in utero, and seven patients received a palliative reconstructive Norwood procedure. In seven fetuses, associated anomalies were detected: three chromosomal and structural and four only structural. In six fetuses, other associated intracardiac anomalies were detected. Of seven infants operated, six had no associated anomalies and only one is alive at an age of 17 months.
Conclusion: The low percentage of intention to treat among patients in our center (34%) is in accordance with the percentage found in another study from the UK (36.2%), but differs significantly from reported series across the Atlantic (67%). Prenatal diagnosis of the HLHS provides opportunities not only for getting patients in optimal preoperative condition when surgery is offered, but also for in-depth counseling of the parents on this severe malformation. A minority of parents faced with the difficult decision of possible termination of pregnancy, compassionate care or the Norwood strategy, choose surgical treatment which might be based on socioreligious differences and the interpretation of the long-term quality of life.