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How Treatment Options For Children With Genetic Aneuploidy And Single Ventricle Physiology Compare Between Pediatric Cardiologists And Congenital Heart Surgeons
Wadsworth Alexander Williams, MD1, Benjamin R. Hauser, BA
2, Rupali Gandhi, MD
3, Steven R. Leuthner, MD
4, Mark R. Mercurio, MD
5, Chawki El-Zein, MD
6, David S. Winlaw, MD
1, Laine Friedman Ross, MD, PhD
7.
1Ann & Robert H Lurie Children's Hospital of Chicago, Chicago, IL, USA,
2University of Rochester School of Medicine and Dentistry, Rochester, NY, USA,
3University of Chicago and Advocate Children’s Hospital, Chicago, IL, USA,
4Medical College of Wisconsin, Chicago, IL, USA,
5Yale School of Medicine, Chicago, IL, USA,
6Advocate Children’s Hospital, Chicago, IL, USA,
7University of Rochester School of Medicine and Dentistry, Chicago, IL, USA.
Objective(s): We sought to examine current treatment options for hypoplastic left heart syndrome (HLHS) and how a diagnosis of aneuploidy, such as trisomy 18 (T18) and trisomy 21 (T21), influences offered treatment options.
Methods:Pediatric cardiologists and congenital heart surgeons (CHS) were surveyed about treatment options offered to patients with isolated HLHS or HLHS with concomitant aneuploidy. They were also queried about the hypothetical scenario of choosing a treatment plan if the patient were their own child. Pearson’s chi-squared test was used for comparisons.
Results:We analyzed 106 responses from 62 cardiologists and 44 CHS. For children with isolated HLHS, cardiologists and CHS similarly offered staged repair (97%, 100%) or transplant (26%, 27%); however, cardiologists were more likely to offer comfort care (57%, 36%, p=0.04). For children with T21, cardiologists and CHS were equally likely to offer staged repair (87%, 89%), transplant (21%, 11%, p=0.18), and comfort care (57%, 43%, p=0.15). For children with T18, cardiologists and CHS never offered transplant, rarely offered staged repair (13%, 12%), and nearly always offered comfort care (97%, 100%). If hypothetically faced with these scenarios for their own child, cardiologists and CHS similarly opted for comfort care or in-utero termination: isolated HLHS (54%, 36%, p=0.08), T21 (86%, 76%, p=0.20), and T18 (100%, 100%).
Conclusions:
Cardiologists and CHS have similar practice for offering surgical repair to patients with syndromic and isolated HLHS. Comfort care is offered to patients at rates comparable to what they would choose for their own child.
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