Changing attitudes to the management of hypoplastic left heart syndrome: a European perspective

Abstract

As of January, 2011, 2392 citations in the PubMed database were available for the search string "hypoplastic left heart". The majority of these were from the centres from the United States of America and Europe. The European Association for Cardio-Thoracic Surgery Congenital Heart Surgery Database shows an annual increase in the number of Norwood (Stage I) operations for hypoplastic left heart syndrome from 2003 to 2009, with a corresponding reduction in mortality. European rates of antenatal detection vary widely between centres, as do the rates of termination for a prenatal diagnosis of hypoplastic left heart syndrome. We observed a wide variation in the estimates of surgeons for survival and quality of life for surgical palliation of hypoplastic left heart syndrome, as well as in their estimates for actual rates of termination of pregnancy in their centres. Further, there was marked inconsistency in the information given to parents as part of the process of counselling. These issues remain to be resolved if parents are to make a fully informed decision for their child.

Evaluation of right ventricular function in fetal hypoplastic left heart syndrome by color tissue Doppler imaging

“…Postnatally, the hypoplastic left ventricle is unable to support the systemic circulation, and systemic blood flow relies entirely on a patent ductus arteriosus that is kept open after birth by prostaglandin until intervention. Postnatal strategies include compassionate care, single ventricle palliation or even heart transplantation with or without a hybrid procedure. Increasing detection rates of congenital heart disease (CHD) along with earlier diagnosis and subsequent prenatal management, advances in perioperative care and surgical technique have led to a decrease in mortality of children with HLHS such that prognosis of affected children has substantially improved.…”

Ethical issues in hypoplastic left heart syndrome

“…Currently there is enormous variation in what parents are told about HLHS. 6,7 We believe parents ought to be given the known facts and told that compassionate care without surgery is a perfectly reasonable option.…”

The single-ventricle patient population: a current and future concern a population-based study in the North of England

“…This contrasts with the previously expected impact of termination of pregnancy rates on the prevalence of congenital heart disease 18. However, overall detection rates outside specialist tertiary centres remain low, and considerable inconsistency in counselling strategies for these diagnoses remain among tertiary centres, therefore, it may be difficult to generalise these results 19 20…”