Treatment Options Offered to Infants with Single Ventricle Physiology and Genetic Aneuploidy: A Survey of US Pediatric Intensivists, Cardiologists, and Cardiothoracic Surgeons

We sought to examine what treatment options were offered for patients with isolated single ventricle physiology (iso-SVP) versus patients with single ventricle physiology and concomitant genetic aneuploidy. Pediatric intensivists, cardiologists, and congenital heart surgeons from the United States were surveyed about treatment options offered to patients with iso-SVP or SVP with concomitant aneuploidy (trisomy 21 [t21-SVP] or trisomy 18 [t18-SVP]). For each scenario, respondents were also queried about what they would hypothetically choose for their own child. Analyses were performed on responses from 171 pediatric intensivists, 69 cardiologists, and 44 congenital heart surgeons. For iso-SVP, nearly all respondents would offer a staged surgical procedure (268/269 [99.6%]) and half would offer comfort care (130/269 [48.3%]). For their own child with iso-SVP, respondents were divided between surgery (137/266, [51.5%]) and comfort care (130/266 [48.9%]). For t21-SVP, respondents almost always offered staged surgical procedures (243/267 [91.0%]) and half of respondents would offer comfort care measures (137/267 [51.3%]). For their own child with t21-SVP, a majority would opt for comfort care (191/266 [71.8%]). For t18-SVP, only 21.8% (58/266) would offer staged surgical procedures, and virtually all (252/266, [94.7%]) would offer comfort care. Comfort care was almost unanimously chosen for their own child (276/277 [99.6%]). Respondents offered comfort care for children with iso-SVP and t18-SVP at a rate concomitant with their own preferences. However, for t21-SVP, only half of respondents offered comfort care, whereas 70% would want it for their own child. This raises ethical questions around respect for parental decision-making authority and informed consent.