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

Palliative Care Across the Life Span for Children With Heart Disease: A Scientific Statement From the American Heart Association.

2023

e000114

02/2023

1941-7705

AIM: This summary from the American Heart Association provides guidance for the provision of primary and subspecialty palliative care in pediatric congenital and acquired heart disease.

METHODS: A comprehensive literature search was conducted from January 2010 to December 2021. Seminal articles published before January 2010 were also included in the review. Human subject studies and systematic reviews published in English in PubMed, ClinicalTrials.gov, and the Cochrane Collaboration were included. Structure: Although survival for pediatric congenital and acquired heart disease has tremendously improved in recent decades, morbidity and mortality risks remain for a subset of young people with heart disease, necessitating a role for palliative care. This scientific statement provides an evidence-based approach to the provision of primary and specialty palliative care for children with heart disease. Primary and specialty palliative care specific to pediatric heart disease is defined, and triggers for palliative care are outlined. Palliative care training in pediatric cardiology; diversity, equity, and inclusion considerations; and future research directions are discussed.

10.1161/HCQ.0000000000000114

Circ Cardiovasc Qual Outcomes

36633003

Fourth Annual Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) Report.

2020

2020 Oct 08

1552-6259

<p><strong>BACKGROUND: </strong>Pedimacs, an originally NIH-sponsored U.S. database, provides a platform to understand the population of children supported with VADs during this time of increasing numbers, new devices, expanding indications, and improved outcomes.</p>

<p><strong>METHODS: </strong>Between 9/19/12-12/31/19, 44 hospitals implanted 1031 devices in 856 patients under 19 years-of-age.</p>

<p><strong>RESULTS: </strong>Overall, diagnosis was cardiomyopathy in 497(58%), congenital heart disease(CHD) in 216(25%), myocarditis in 85(10%), and other in 58(7%). Positive outcome (alive on device or bridge to transplantation/recovery) occurred in 82% at 6-months. The patient cohort for implantable continuous flow(IC) pumps (n=365)[age:13.2+/-3.9yrs., 18% INTERMACS profile-1, 23% intubated at implant, 16% with CHD] was significantly different from the paracorporeal continuous flow(PC) pump cohort (n=212)[age:3.6+/-4.9yrs, 46% INTERMACS profile-1, 81% intubated, 42% CHD] and the paracorporeal pulsatile(PP) pump cohort (n=230)[age:2.7 +/-3.5yrs, 31% INTERMACS profile-1, 76% intubated, 26% CHD]. Consistent with their cohort composition, positive outcomes at 6 months based on device type were IC-92%, PC-68%, and PP-81%. The incidence of cerebrovascular accidents(CVA) in the IC, PC, and PP cohorts is 7%, 14% and 15%, respectively.</p>

<p><strong>CONCLUSIONS: </strong>IC VADs, the most common VAD-type placed in children, are associated with improved outcomes compared to PP/PC devices, though PP/PC devices are limited to supporting our most challenging patients. Noteworthy, the incidence of CVA for pediatric VADs has significantly decreased and is now 11% overall. This report demonstrates again that although often attributed to age, size, or device type, much of the burden in mortality and adverse events is correlated to the patient's overall state at VAD implantation.</p>

10.1016/j.athoracsur.2020.09.003

Ann Thorac Surg

33039359

Third Annual Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) Report: Pre-Implant Characteristics and Outcomes.

2019

2019 Feb 25

1552-6259

<p><strong>BACKGROUND: </strong>The influence of ventricular assist device (VAD) use in the care of children with end-stage heart failure is growing rapidly through increasing numbers, new devices, expanding indications, and improving outcomes. Pedimacs, a NIH-sponsored U.S. database, provides a platform to understand this emerging population.</p>

<p><strong>METHODS: </strong>Between 9/19/12-12/31/17, 30 hospitals implanted 508 devices in 423 patients under 19 years-of-age. This past year was one of evolution for the Database as its management was transitioned to the Society of Thoracic Surgery, therefore data from institutions not under contract by August 1 2018 was not included in this report.</p>

<p><strong>RESULTS: </strong>Of the 423 patients, the diagnosis was cardiomyopathy in 261 (62%), myocarditis in 48 (11%), other in 28 (7%) and congenital heart disease in 86 (20%) with 52 of these patients having single ventricle physiology. The two most common support strategies included LVAD-342 (81%) and BiVAD-64 (15%). Positive Outcome (alive on device or bridge to transplantation/recovery) was 80% at 6-months (overall mortality of 20%). The patient cohort for implantable continuous flow (IC) pumps (n=197) [age at implant 13.4+/-3.8yrs., 19% INTERMACS profile-1, 21% intubated at implant and 12% with CHD] was significantly different from the paracorporeal continuous flow (PC) pump cohort (n=79) [age 3.9+/-5.2yrs, 49% INTERMACS profile-1, 86% intubated at implant, and 38% with CHD] and the paracorporeal pulsatile (PP) pump cohort (n=121) [age 3.3+/-3.9yrs, 41% INTERMACS profile-1, 77 % intubated at implant, and 21% with CHD]. Consistent with their cohort composition, device type positive outcomes at 6 months were PC-63%, PP-77% and IC-92%. Using Parametric Hazard Modeling, an early hazard for death was associated with INTERMACS profile-1, BiVAD, percutaneous devices, PC devices, small volume institutions, low age and low weight, while a constant hazard was associated with intubation and liver dysfunction at time of implant.</p>

<p><strong>CONCLUSIONS: </strong>IC VADs are the most common VAD type placed in children. The positive outcomes for the IC VADs are &gt;90% at 6 months, which may represent our field's maturation in both patient selection and timing of implantation. Currently the PP/PC devices are limited to supporting our most challenging patients, those less than 20kg, and those with CHD. The introduction of new devices and our communities' commitment to shared learning and improvement will lead to more pediatric lives saved by VAD support, and will also focus on improving the quality of life of children supported with VADs.</p>

10.1016/j.athoracsur.2019.01.038

Ann. Thorac. Surg.

30817920

Outcomes of children implanted with ventricular assist devices in the United States: First analysis of the Pediatric Interagency Registry for Mechanical Circulatory Support (PediMACS).

2016

578-84

2016 May

1557-3117

<p><strong>BACKGROUND: </strong>Use of mechanical circulatory support in children has increased as more options have become available. A national account of the use of mechanical support in children and adolescents is essential to understanding outcomes, refining patient selection and improving quality of care.</p>

<p><strong>METHODS: </strong>The Pediatric Interagency Registry for Mechanical Circulatory Support (PediMACS) is a National Heart, Lung, and Blood Institute-supported nationwide registry for temporary and durable ventricular assist device (VAD) use in patients &lt;19 years of age. Between the launch in September 2012 and June 2015, 37 hospitals in the USA have enrolled patients. This first report of data from PediMACS analyzed pre-implant patient characteristics, survival using competing outcomes, and adverse events.</p>

<p><strong>RESULTS: </strong>Two hundred pediatric patients underwent 222 durable VAD implants. Patients' characteristics and outcomes of children supported with a temporary device (n = 41) were not analyzed in this report. The etiology of heart disease included 146 (73%) patients with cardiomyopathy and 35 (18%) with congenital heart disease. Thirty patients (15%) transitioned from extracorporeal membrane oxygenation (ECMO) and 76 (38%) had previous cardiac surgery. Most patients were Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) Level 1 (27%) or Level 2 (56%) at implant, with 13% at Level 3. Of the 200 patients supported with a durable device, 91 (46%) were supported with a pulsatile-flow device and 109 (55%) with a continuous-flow (CF) device. Patient age at first implant included 30 patients (15%) &lt;1 year of age, 37 (19%) 1 to 5 years, 32 (16%) 6 to 10 years and 101 (51%) 10 to 18 years. Patients were supported with left ventricular assist device alone in 161 (81%), biventricular ventricular assist device in 29 (15%), right ventricular assist device in 4 (2.0%) and total artificial heart in 6 (3%), together comprising 783 months of follow-up. The 200 patients receiving primary durable devices had an actuarial survival of 81% at 6 months. Competing risk analysis at 6 months revealed that 58% of patients had been transplanted, 28% were alive on support, 14% had died and 0.6% recovered. In the overall cohort, there were 28 deaths. Reported serious adverse events included infection (n = 78), bleeding (n = 68), device malfunction (n = 79) and neurologic dysfunction (n = 52).</p>

<p><strong>CONCLUSIONS: </strong>PediMACS constitutes the largest single data repository with detailed information of pediatric patients implanted with VADs. The first PediMACS report reveals favorable outcomes despite the varying patient characteristics and pump types. However, the rate of adverse events remains high. With further data collection, analysis of patient risk factors critical to improving outcomes will be possible.</p>

10.1016/j.healun.2016.01.1227

J. Heart Lung Transplant.

27009673

Outcomes of pediatric patients supported with continuous-flow ventricular assist devices: A report from the Pediatric Interagency Registry for Mechanical Circulatory Support (PediMACS).

2016

585-90

2016 May

1557-3117

<p><strong>BACKGROUND: </strong>Continuous-flow (CF) ventricular assist devices (VADs) have largely replaced pulsatile-flow VADs in adult patients. However, there are few data on CF VADs among pediatric patients. In this study we aimed to describe the overall use, patients' characteristics and outcomes of CF VADs in this population.</p>

<p><strong>METHODS: </strong>The Pediatric Interagency Registry for Mechanical Circulatory Support (PediMACS) is a national registry for U.S. Food and Drug Adminstration (FDA)-approved VADs in patients &lt;19 years of age. Patients undergoing placement of durable CF VADs between September 2012 and June 2015 were included and outcomes were compared with those of adults from the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS).</p>

<p><strong>RESULTS: </strong>CF VADs were implanted in 109 patients at 35 hospitals. The median age at implantation was 15 years (2.8 to 18.9 years) and median weight was 62 kg (range 16 to 141 kg). The underlying disease was cardiomyopathy in 89 (82%) patients. The INTERMACS level at time of implant was Level 1 in 20 (19%), Level 2 in 64 (61%) and Levels 3 to 7 in 21 (20%) patients. Most were implanted as LVADs (n = 102, 94%). Median duration of support was 2.3 months (range &lt;1 day to 28 months). Serious adverse event rates were low, including neurologic dysfunction (early event rate 4.1 per 100 patient-months with 2 late events). Competing outcomes analysis at 6 months post-implant indicated 61% transplanted, 31% alive with device in place and 8% death before transplant. These outcomes compared favorably with the 3,894 adults supported with CF VADs as a bridge to transplant.</p>

<p><strong>CONCLUSIONS: </strong>CF VADs are commonly utilized in older children and adolescents, with excellent survival rates. Further study is needed to understand impact of patient and device characteristics on outcomes in pediatric patients.</p>

10.1016/j.healun.2016.01.1228

J. Heart Lung Transplant.

27056612

Second annual Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) report: Pre-implant characteristics and outcomes.

2018

38-45

2018 Jan

1557-3117

<p><strong>BACKGROUND: </strong>Expanded use of pediatric ventricular assist devices (VADs) has decreased mortality in children awaiting heart transplantation. Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs), a National Heart, Lung, and Blood Institute-sponsored North American database, provides a platform to understand this emerging population.</p>

<p><strong>METHODS: </strong>Between September 2012 and September 2016, patients aged younger than 19 years who underwent VAD implantation were enrolled in Pedimacs. FDA approved durable devices as well as temporary support devices were included. The second annual report updates the current Pedimacs data. Patients implanted with temporary devices are included in Pedimacs and this analysis includes this group of paracorporeal continuous flow VADs.</p>

<p><strong>RESULTS: </strong>Over the 4 years, 42 hospitals implanted 432 devices in 364 patients less than 19 yrs of age. Diagnoses included cardiomyopathy in 223 (61%), myocarditis in 41 (11%), and congenital heart disease in 77 (21%), of which 48 had single-ventricle physiology. At implant, 87% were at Intermacs patient profile 1 or 2. The age distribution of children (59% male) supported on VAD included 69 (19%) aged younger than 1 year, 66 (18%) aged 1 to 6 years, 56 (15%) aged 6 to 10 years, and 173 (48%) aged 11 to 19 years. Median follow-up was 2.2 months (range, 1 day to 41.5 months). Median (interquartile) age at implant was 1.7 (0.3-10.0) years for paracorporeal continuous-flow pumps (n = 60), 1.7 (0.4-5.3) years for paracorporeal pulsatile pumps (n = 105), and 15.0 (11.3-16.9) years for implantable continuous-flow pumps (n = 174). Support strategies included LVAD in 293 (80%), biventricular device in 55 (15%), and total artificial heart in 8 (2%). Nearly 50% of patients underwent transplantation within 6 months, with overall mortality of 19%. Adverse event burden continues to be high.</p>

<p><strong>CONCLUSIONS: </strong>Pedimacs constitutes the largest longitudinal pediatric VAD registry. Preimplant data across centers will be helpful at creating shared protocols with which to improve outcomes. Adverse events continue to be the major challenge, especially among the young critically ill children with complex congenital disease.</p>

10.1016/j.healun.2017.06.017

28965736