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. 2023 Jun;44(5):1118-1124.
doi: 10.1007/s00246-023-03170-5. Epub 2023 Apr 26.

Initial Counseling Prior to Palliation for Hypoplastic Left Heart Syndrome: 2021 vs 2011

Deipanjan Nandi  1 , Stacey Culp  2 , Andrew R Yates  3 , Timothy M Hoffman  4 , Amy L Juraszek  5 , Christopher S Snyder  6 , Timothy F Feltes  3 , Clifford L Cua  3
Affiliations

Initial Counseling Prior to Palliation for Hypoplastic Left Heart Syndrome: 2021 vs 2011

Deipanjan Nandi et al. Pediatr Cardiol. 2023 Jun.

Abstract

We sought to examine current practices and changes in practice regarding initial counseling for families of patients with hypoplastic left heart syndrome (HLHS) given the evolution of options and outcomes over time. Counseling (Norwood with Blalock-Taussig-Thomas shunt (NW-BTT), NW with right ventricle to pulmonary artery conduit (NW-RVPA), hybrid palliation, heart transplantation, or non-intervention/hospice (NI)) for patients with HLHS were queried via questionnaire of pediatric care professionals in 2021 and compared to identical questionnaire from 2011. Of 322 respondents in 2021 (39% female), 299 respondents were cardiologists (92.9%), 17cardiothoracic surgeons (5.3%), and 6 were nurse practitioners (1.9%). Respondents were largely from North America (96.9%). In 2021, NW-RVPA procedure was the preferred palliation for standard risk HLHS patient (61%) and was preferred across all US regions (p < 0.001). NI was offered as an option by 71.4% of respondents for standard risk patients and was the predominant strategy for patients with end-organ dysfunction, chromosomal abnormality, and prematurity (52%, 44%, and 45%, respectively). The hybrid procedure was preferred for low birth-weight infants (51%). In comparison to the identical 2011 questionnaire (n = 200), the NW-RVPA was endorsed more in 2021 (61% vs 52%, p = 0.04). For low birth-weight infants, hybrid procedure was more recommended than in 2011 (51% vs 21%, p < 0.001). The NW-RVPA operation is the most recommended strategy throughout the US for infants with HLHS. The hybrid procedure for low birth-weight infants is increasingly recommended. NI continues to be offered even in standard risk patients with HLHS.

Keywords: Hypoplastic left heart syndrome.

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