title: Outcomes of management decisions by parents for their infants with hypoplastic left heart syndrome born with and without a prenatal diagnosis.
abstract: OBJECTIVE
  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.
authors.affiliation: Intensive Care Unit, Royal Children's Hospital, Melbourne and Department of Paediatrics, The University of Melbourne, Victoria, Australia. james.tibballs@rch.org.au
authors.forename: James
authors.initials: J
authors.lastname: Tibballs
authors.forename: Annie
authors.initials: A
authors.lastname: Cantwell-Bartl
mesh_terms: D000028:Abortion, Induced; D006348:Cardiac Surgical Procedures / Q000379:methods; D003657:Decision Making; D004452:Echocardiography; D005260:Female; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000000981:diagnostic imaging* / Q000601:surgery*; D007231:Infant, Newborn; D008499:Medical Records; D010166:Palliative Care; D010290:Parents; D011247:Pregnancy; D012189:Retrospective Studies; D013997:Time Factors; D016216:Ultrasonography, Prenatal