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

Fetal growth and gestational age improve outcome predictions in neonatal heart surgery.

2022

05/2022

1097-685X

OBJECTIVE: Current risk adjustment models for congenital heart surgery do not fully incorporate multiple factors unique to neonates such as granular gestational age (GA) and birth weight (BW) z score data. This study sought to develop a Neonatal Risk Adjustment Model for congenital heart surgery to address these deficiencies.

METHODS: Cohort study of neonates undergoing cardiothoracic surgery during the neonatal period captured in the Pediatric Cardiac Critical Care Consortium database between 2014 and 2020. Candidate predictors were included in the model if they were associated with mortality in the univariate analyses. GA and BW z score were both added as multicategory variables. Mortality probabilities were predicted for different GA and BW z scores while keeping all other variables at their mean value.

RESULTS: The C statistic for the mortality model was 0.8097 (95% confidence interval, 0.7942-0.8255) with excellent calibration. Mortality prediction for a neonate at 40 weeks GA and a BW z score 0 to 1 was 3.5% versus 9.8% for the same neonate at 37 weeks GA and a BW z score -2 to -1. For preterm infants the mortality prediction at 34 to 36 weeks with a BW z score 0 to 1 was 10.6%, whereas it was 36.1% for the same infant at <32 weeks with a BW z score of -2 to -1.

CONCLUSIONS: This Neonatal Risk Adjustment Model incorporates more granular data on GA and adds the novel risk factor BW z score. These 2 factors refine mortality predictions compared with traditional risk models. It may be used to compare outcomes across centers for the neonatal population.

10.1016/j.jtcvs.2022.05.022

J Thorac Cardiovasc Surg

35750509

Epidemiology and Outcomes of Acute Decompensated Heart Failure in Children.

2020

e006101

2020 Apr

1941-3297

<p><strong>BACKGROUND: </strong>Acute decompensated heart failure (ADHF) is a highly morbid condition among adults. Little is known about outcomes in children with ADHF. We analyzed the Pediatric Cardiac Critical Care Consortium registry to determine the epidemiology, contemporary treatments, and predictors of mortality in critically ill children with ADHF.</p>

<p><strong>METHODS: </strong>Cardiac intensive care unit (CICU) patients ≤18 years of age meeting Pediatric Cardiac Critical Care Consortium criteria for ADHF were included. ADHF was defined as systolic or diastolic dysfunction requiring continuous vasoactive or diuretic infusion, respiratory support, or mechanical circulatory support. Demographics, diagnosis, therapies, complications, and mortality are described for the cohort. Predictors of CICU mortality were identified using logistic regression.</p>

<p><strong>RESULTS: </strong>Among 26 294 consecutive admissions (23 centers), 1494 (6%) met criteria for analysis. Median age was 0.93 years (interquartile range, 0.1-9.3 years). Patients with congenital heart disease (CHD) comprised 57% of the cohort. Common therapies included the following: vasoactive infusions (88%), central venous catheters (86%), mechanical ventilation (59%), and high flow nasal cannula (46%). Common complications were arrhythmias (19%), cardiac arrest (10%), sepsis (7%), and acute renal failure requiring dialysis (3%). Median length of CICU stay was 7.9 days (interquartile range, 3-18 days) and the CICU readmission rate was 22%. Overall, CICU mortality was 15% although higher for patients with CHD versus non-CHD (19% versus 11%; &lt;0.001). Independent risk factors associated with CICU mortality included age &lt;30 days, CHD, vasoactive infusions, ventricular tachycardia, mechanical ventilation, sepsis, pulmonary hypertension, extracorporeal membrane oxygenation, and cardiac arrest.</p>

<p><strong>CONCLUSIONS: </strong>ADHF in children is characterized by comorbidities, high mortality rates, and frequent readmission, especially among patients with CHD. Opportunities exist to determine best practices around appropriate use of mechanical support, cardiac arrest prevention, and optimal heart transplantation candidacy to improve outcomes for these patients.</p>

10.1161/CIRCHEARTFAILURE.119.006101

Circ Heart Fail

32301336