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Rebuttal to the invited comment of Opitz and Carey

Hypoplastic left heart syndrome is characterized by aortic valve atresia or stenosis, mitral valve atresia or stenosis, a small left ventricle, and an underdeveloped aortic arch. It is frequently, but not always, accompanied by an atrial septal defect. HLHS belongs to a group of cardiovascular malformations that are characterized by obstruction of blood flow through the left ventricular outflow tract (LVOT).Malformations in this group include aortic valve stenosis (AVS), bicuspid aortic valve (BAV), coarctationof the aorta (CoA), Shone complex, and interrupted aortic arch type A. The concept of obstructed blood flow leading to HLHS has been shown by direct observation, where prenatal ultrasounds of fetuses with aortic stenosis have demonstrated progression from normal left ventricle to ventricular hypoplasia over the course of pregnancy. Indeed, this forms the whole basis of fetal intervention (balloonvalvuloplastyof the aortic valve) for criticalAVS toprevent HLHS [Makikallio et al., 2006]. Multiple epidemiology and genetic studies have shown similar characteristics for all LVOT malformations and the association of these defects together in families. TheBaltimoreWashington Infant Study was the first study to note concordance of LVOT malformations within families, findings later confirmed in multiple papers [Ferencz et al., 1997; McBride et al., 2005]. Inheritance analysis has demonstrated an oligogenic pattern, with autosomal dominant inheritance in 10–20% of families. Linkage studies have supported this observation [Hinton et al., 2009; McBride et al., 2009]. In addition, mutations in NOTCH1 have been found in individuals with HLHS (and the other LVOT malformations) [Garg et al., 2005; McBride et al., 2008], and an association of the LVOT malformations with the gene ERBB4 has recently been noted [McBride et al., 2011]. The current thinking for developmental mechanisms causing LVOT malformations is an endothelial/endocardial problem, possibly including signaling with the underlying media and myocardium during development. Supporting this hypothesis is the common observation of vascular disease in this group of malformations, best exemplified by aortic root dilation, and specifically with CoA, higher rates of hypertension and cerebral aneurysms. HLHS is certainly not a developmental problem of chamber misspecification.