Interventions and Outcomes in Children With Hypoplastic Left Heart Syndrome Born in England and Wales Between 2000 and 2015 Based on the National Congenital Heart Disease Audit
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Among congenital heart diseases, none has undergone a more drastic change in diagnostic approach, management, and outcomes over the last 30 years than hypoplastic left heart syndrome (HLHS), with comfort care replaced as the predominant form of management by a palliative surgical treatment pathway.1 The outcomes of palliative procedures for HLHS have been well documented, but the longer-term picture is less clear.
The UK National Congenital Heart Diseases Audit (NCHDA) captures every procedure undertaken for congenital heart disease and updated life status for patients resident in England and Wales from death certifications. From this data set, we identified patients born between 2000 and 2015 who fulfilled the criteria for HLHS: a small left ventricle, stenosis or atresia of the left-sided heart valves, normally aligned great arteries, and no common atrioventricular junction.2 All patients with HLHS require surgical palliation with a traditional Norwood or hybrid pathway or heart transplantation to survive. Hence, we were able to use procedure information in combination with diagnostic information to identify patients with HLHS within the NCHDA data set using the International Paediatric and Congenital Cardiac Codes. Because the NCHDA is a procedure-based data set, patients with HLHS who did not undergo any interventional treatment are not included. Because the hybrid procedure was introduced in the United Kingdom in 2006, we considered 2 eras for …