Nationwide Children's Hospital, Ohio State University, Columbus, OH, USA.
Department of Biomedical Informatics, Ohio State University, Columbus, OH, USA.
Pediatr Cardiol. 2023 Jun;44(5):1118-1124. doi: 10.1007/s00246-023-03170-5. Epub 2023 Apr 26.
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.
我们试图检查随着时间的推移，-options 和结果的演变，对于患有左心发育不全综合征（HLHS）的患者的家庭，初始咨询的当前实践和实践变化。通过 2021 年儿科护理专业人员的问卷，对 HLHS 患者的咨询（Norwood 与 Blalock-Taussig-Thomas 分流术（NW-BTT）、NW 与右心室肺动脉导管（NW-RVPA）、杂交姑息治疗、心脏移植或非干预/临终关怀（NI））进行了查询，并与 2011 年相同的问卷进行了比较。2021 年有 322 名受访者（39%为女性），其中 299 名为心脏病专家（92.9%）、17 名为心胸外科医生（5.3%）、6 名为护士从业人员（1.9%）。受访者主要来自北美（96.9%）。在 2021 年，NW-RVPA 手术是标准风险 HLHS 患者首选的姑息治疗方法（61%），并且在所有美国地区都受到青睐（p<0.001）。71.4%的受访者为标准风险患者提供 NI 作为一种选择，并且是终末器官功能障碍、染色体异常和早产患者的主要治疗策略（分别为 52%、44%和 45%）。对于低出生体重婴儿，首选杂交手术（51%）。与 2011 年相同的问卷（n=200）相比，2021 年更支持 NW-RVPA（61%比 52%，p=0.04）。对于低出生体重婴儿，与 2011 年相比，更推荐使用杂交手术（51%比 21%，p<0.001）。NW-RVPA 手术是美国 HLHS 婴儿最推荐的策略。对于低出生体重婴儿，杂交手术的应用越来越多。即使对于 HLHS 标准风险患者，也继续提供 NI。
