Cardiology in the Young

Part 2: Ethical Considerations Associated with Caring for Patients with Hypoplastic Left Heart Syndrome

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

Bari Murtuzaa1 and Martin J. Elliotta1 c1

a1 Department of Paediatric Cardiothoracic Surgery, The Great Ormond Street Hospital for Children National Health Service Trust, London, United Kingdom

Abstract

Background and aims Several years ago, one of us described the difference in attitude to patients with hypoplastic left heart syndrome in the United States of America and Europe as similar to that between Mars and Venus. Uncertainty remains with regard to the long-term prognosis for patients with hypoplastic left heart syndrome. This prognosis may be considered in terms of survival, functional performance, including exercise capacity and neurodevelopment, as well as psychosocial effects on the patient, family, and siblings. Counselling parents where either an antenatal or postnatal diagnosis of hypoplastic left heart syndrome has been made requires practitioners to give information on these prognostic aspects. We wanted to see how attitudes among European surgeons have changed over the last few years.

Methods We performed a review of recent European data for hypoplastic left heart syndrome and conducted a survey among surgeons in major European centres to ascertain key aspects of their attitudes to the management of patients with hypoplastic left heart syndrome and how they counsel parents.

Results and conclusions 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.

(Online publication December 13 2011)

Correspondence:

c1 Correspondence to: Prof. M. J. Elliott, Department of Cardiothoracic Surgery, The Great Ormond Street Hospital for Children, London WC1N 3JH, United Kingdom. Tel: +44 (0) 207 829 8853; Fax: +44 (0) 207 813 8262; E-mail: elliom1@gosh.nhs.uk