Outcomes of management decisions by parents for their infants with hypoplastic left heart syndrome born with and without a prenatal diagnosis

Abstract

Aim: To study the influence of a prenatal diagnosis on parental choice of treatment for infants born with hypoplastic left heart syndrome (HLHS).

Methods: Retrospective review of medical records of infants admitted to a paediatric tertiary hospital 1983-2004 with a diagnosis of HLHS.

Results: A total of 201 infants had HLHS diagnosed by echocardiography after birth with 129 subsequently undergoing surgery and 72 non-surgical management (compassionate/comfort care). When the diagnosis of HLHS was made prenatally, 68 of 71 (96%) infants underwent surgery whereas when the diagnosis was made post-natally 61 of 130 (47%) infants underwent surgery. Prenatal diagnosis was significantly associated with a parental choice of surgery (P < 0.001). Among 72 infants managed non-surgically, compassionate care was chosen by parents for 49 (68%) despite being fit for surgery, 20 (28%) were deemed unfit for surgery and in three (4%) the reasons for non-surgical management were unknown. Of the total 178 infants considered fit for surgery, 68 (38%) had had a prenatal diagnosis and 110 (62%) did not. Of 20 considered unfit for surgery, none had had a prenatal diagnosis. A prenatal diagnosis was significantly associated with fitness for surgery (P < 0.001).

Conclusions: Approximately half of parents chose non-surgical management (compassionate care) of their infant if the diagnosis was post-natal. Although most parents of infants born with a prenatal diagnosis of HLHS chose surgical management of their infant, this is the likely influence of termination of pregnancy for this condition. Lack of prenatal diagnosis compromised fitness for surgery.