Medical and End-of-Life Decision-Making Preferences in Adolescents and Young Adults with Advanced Heart Disease and Their Parents.

2023

e2311957

05/2023

2574-3805

IMPORTANCE: Despite high disease morbidity and mortality and complex treatment decisions, little is known about the medical and end-of-life decision-making preferences of adolescents and young adults (AYA) with advanced heart disease. AYA decision-making involvement is associated with important outcomes in other chronic illness groups.

OBJECTIVE: To characterize decision-making preferences of AYAs with advanced heart disease and their parents and determine factors associated with these preferences.

DESIGN, SETTING, AND PARTICIPANTS: Cross-sectional survey between July 2018 and April 2021 at a single-center heart failure/transplant service at a Midwestern US children's hospital. Participants were AYAs aged 12 to 24 years with heart failure, listed for heart transplantation, or posttransplant with life-limiting complications and a parent/caregiver. Data were analyzed from May 2021 to June 2022.

MAIN OUTCOMES AND MEASURES: Single-item measure of medical decision-making preferences, MyCHATT, and Lyon Family-Centered Advance Care Planning Survey.

RESULTS: Fifty-six of 63 eligible patients enrolled in the study (88.9%) with 53 AYA-parent dyads. Median (IQR) patient age was 17.8 (15.8-19.0) years; 34 (64.2%) patients were male, and 40 patients (75.5%) identified as White and 13 patients (24.5%) identified as members of a racial or ethnic minority group or multiracial. The greatest proportion of AYA participants (24 of 53 participants [45.3%]) indicated a preference for active, patient-led decision-making specific to heart disease management, while the greatest proportion of parents (18 of 51 participants [35.3%]) preferred they and physician(s) make shared medical decisions on behalf of their AYA, representing AYA-parent decision-making discordance (χ2 = 11.7; P = .01). Most AYA participants stated a preference to discuss adverse effects or risks of treatment (46 of 53 participants [86.8%]), procedural and/or surgical details (45 of 53 participants [84.9%]), impact of condition on daily activities (48 of 53 participants [90.6%]), and their prognosis (42 of 53 participants [79.2%]). More than half of AYAs preferred to be involved in end-of-life decisions if very ill (30 of 53 participants [56.6%]). Longer time since cardiac diagnosis (r = 0.32; P = .02) and worse functional status (mean [SD] 4.3 [1.4] in New York Heart Association class III or IV vs 2.8 [1.8] in New York Heart Association class I or II; t-value = 2.7; P = .01) were associated with a preference for more active, patient-led decision-making.

CONCLUSIONS AND RELEVANCE: In this survey study, most AYAs with advanced heart disease favored active roles in medical decision-making. Interventions and educational efforts targeting clinicians, AYAs with heart disease, and their caregivers are needed to ensure they are meeting the decision-making and communication preferences of this patient population with complex disease and treatment courses.

10.1001/jamanetworkopen.2023.11957

JAMA Netw Open

37145598

Design and initial results of a programme for routine standardised longitudinal follow-up after congenital heart surgery.

2016

1590-1596

2016 Dec

1467-1107

<p><strong>BACKGROUND: </strong>With improvements in early survival following congenital heart surgery, it has become increasingly important to understand longer-term outcomes; however, routine collection of these data is challenging and remains very limited. We describe the development and initial results of a collaborative programme incorporating standardised longitudinal follow-up into usual care at the Children's Hospital of Philadelphia (CHOP) and University of Michigan (UM).</p>

<p><strong>METHODS: </strong>We included children undergoing benchmark operations of the Society of Thoracic Surgeons. Considerations regarding personnel, patient/parent engagement, funding, regulatory issues, and annual data collection are described, and initial follow-up rates are reported.</p>

<p><strong>RESULTS: </strong>The present analysis included 1737 eligible patients undergoing surgery at CHOP from January 2007 to December 2014 and 887 UM patients from January 2010 to December 2014. Overall, follow-up data, of any type, were obtained from 90.8% of patients at CHOP (median follow-up 4.3 years, 92.2% survival) and 98.3% at UM (median follow-up 2.8 years, 92.7% survival), with similar rates across operations and institutions. Most patients lost to follow-up at CHOP had undergone surgery before 2010. Standardised questionnaires assessing burden of disease/quality of life were completed by 80.2% (CHOP) and 78.4% (UM) via phone follow-up. In subsequent pilot testing of an automated e-mail system, 53.4% of eligible patients completed the follow-up questionnaire through this system.</p>

<p><strong>CONCLUSIONS: </strong>Standardised follow-up data can be obtained on the majority of children undergoing benchmark operations. Ongoing efforts to support automated electronic systems and integration with registry data may reduce resource needs, facilitate expansion across centres, and support multi-centre efforts to understand and improve long-term outcomes in this population.</p>

10.1017/S1047951116001669

Cardiol Young

28148316