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Hypoplastic left heart syndrome: the importance of phenotypic diagnosis


To the Editor,

Winlaw is to be congratulated on his thoughtful and timely editorial comment1 regarding the recent review of the outcome of treatment for patients diagnosed with hypoplastic left heart syndrome in England and Wales.2 It is possible, however, that he has taken a step too far when suggesting that ‘HLH is the end result of a pan-cardiac developmental disorder including the cardiomyocyte’.1 So as to justify this statement, those producing the reference he cited in support of his statement should have investigated models that matched the definitions used for the diagnosis of hypoplastic left heart syndrome as chosen by the authors of the review of outcomes for patients undergoing treatment in England and Wales. In this regard, all patients chosen for inclusion for the clinical study were required to have had ‘a small left ventricle, left-sided valvar stenosis or atresia, normally related…

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