Perspective:

Although it is intuitive that patients with single ventricle heart disease with left ventricle (LV) morphology would do better in the long-run than patients with an RV, there has been little evidence to suggest that this is the case. This single-center study, performed at a referral center for hypoplastic left heart syndrome and other complex single ventricle lesions, suggests that a morphologic RV is an independent risk factor for death and need for Fontan takedown, transplant, or death. Patients with single RVs appeared to have a greater degree of progressive AVVR, which may have contributed to ventricular dysfunction and subsequent poor outcomes. Of note, the center approaches the single ventricle palliation with a hemi-Fontan procedure at the second stage, followed by a lateral tunnel Fontan for the third stage, as compared with many centers who perform a bidirectional Glenn procedure followed by an extracardiac conduit Fontan. Studies such as this one also provide a look at current long-term survival of our Fontan population, with this study showing 71% survival at 25 years, which is in line with previous reported data.