Wait-List Outcomes for Adults With Congenital Heart Disease Listed for Heart Transplantation in the U.S.

Alshawabkeh, Laith; Hu, Nan; Carter, Knute D.; Opotowsky, Alexander R.; Light-McGroary, Kelly Ann; Cavanaugh, Joseph E.; Bartlett, Heather L.

doi:10.1016/j.jacc.2016.05.082

Cited by 97 publications

(70 citation statements)

References 28 publications

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“…However, even after these patients demonstrate evidence of clinical failure, there is a paucity of data in these patients regarding the timing of advanced therapies. ACHD patients are known to have higher waitlist mortality and delisting rate compared to non‐ACHD patients . As such, early referral for transplant evaluation is recommended in those patients with declining NYHA class, progressive ventricular dysfunction, increased burden of arrhythmias, worsening peak oxygen uptake (VO 2 max), repeated HF hospitalizations or emergency room visits, escalating diuretic dose, or worsening end‐organ function.…”

Section: Discussionmentioning

confidence: 99%

Heart transplantation and in‐hospital outcomes in adult congenital heart disease patients with Fontan: A decade nationwide analysis from 2004 to 2014

et al. 2020

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Introduction Treatment of adult congenital heart disease patients who require advanced therapies remains challenging due to high perioperative and wait‐list mortality and limited donors. Patients palliated with Fontan are at the highest risk of early mortality due to multiorgan involvement and few centers able to safely transplant them. We sought to evaluate the early outcomes of heart transplants in these adult Fontan patients. Methods Using the Nationwide Inpatient Sample database, we identified all adults aged at least 18 years old who underwent heart transplantation across U.S. hospitals from 2004 to 2014. We then identified those with specific ICD‐9 codes to include tricuspid atresia, hypoplastic left heart syndrome and common ventricle. Multivariate regression models were created to adjust for potential confounders. Results A total of 93 Fontan patients underwent heart transplant during the study time (0.5% of all heart transplants). Compared to non‐Fontan heart transplantations, Fontan patients were younger, with a higher incidence of liver disease and coagulopathy. Fontan patients receiving heart transplant had higher mortality during transplant hospitalization compared to non‐Fontan patients (26.3% vs 5.3% OR, 18.10, CI, 5.06‐65.0 P < .001). Extracorporeal membrane oxygenator (ECMO) usage and bleeding were also higher in the Fontan cohort with an OR of 5.30 (P = .016) and 5.32 (P = .015) for ECMO and bleeding, respectively. The remaining outcomes were similar for both cohorts. Conclusion Adults with Fontan palliation undergoing heart transplantation have exceptionally high inpatient mortality, which is nearly five times that of non‐Fontan heart transplant recipients, perhaps related to a delayed referral, surgical complexity, and coexistent, underrecognized liver failure.

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Section: Discussionmentioning

confidence: 99%

Heart transplantation and in‐hospital outcomes in adult congenital heart disease patients with Fontan: A decade nationwide analysis from 2004 to 2014

et al. 2020

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“…National data from the UNOS registry from 1990 to 2008 showed increased early mortality in 18‐ to 45‐year‐old ACHD patients compared to all other etiologies, composed of dilated cardiomyopathy and nonoperable coronary artery disease . Furthermore, the ACHD population experiences worse waitlist outcomes, with identified hepatorenal risk factors for death or delisting due to worsening condition including GFR < 60 mL/min, albumin < 3.2, and hospitalization at time of listing …”

Section: Introductionmentioning

confidence: 99%

Prognostic utility of MELD‐XI in adult congenital heart disease patients undergoing cardiac transplantation

Adams

Jackson²,

Young³

et al. 2018

Clinical Transplantation

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“…However, the use of VADs in ACHD patients is a rare occurrence at <1% of all implants, despite relatively similar outcomes compared to non-ACHD patients. 18,19 Evidence suggests that ACHD patients are more likely than non-ACHD patients to die or worsen before a heart can be obtained. Still, the number of implants of VADs in ACHD patients appears to be steadily increasing over time.…”

Section: The Need For Adult Cong Enital Advan Ced Circul Atory Failmentioning

confidence: 99%

Opportunities for training to advance the care for adults with congenital heart disease with advanced circulatory failure

Broda

2019

Congenital Heart Disease

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Much effort and consideration has been given to designing and implementing appropriate physician training to engender germane skills to care for patients with adult congenital heart disease (ACHD), a population which is growing in both size and complexity. 1 In the United States, the current training pathway allows for physicians who have completed either a pediatric or adult cardiology fellowship to pursue a two-year fellowship which allows one to become eligible to take the Adult Congenital Heart Disease Certification Exam offered by the American Board of Internal Medicine (ABIM). With an estimated population of well over one million individuals and only ~300 board-certified ACHD specialists, 2,3 the need for "generalists" in ACHD is paramount. However, it is very likely that subspecialization within the ACHD specialty is additionally necessary to optimize care for patients. ACHD-specific expertise is needed in focused areas including surgery, cardiac catheterization, electrophysiology, critical care, and advanced heart failure. 4 Despite the present and future needs in these disciplines, there are no clear pathways for trainees to gain a specific knowledge and skill set for ACHD subspecialty training. While all the aforementioned experts are necessary to improve the care and outlook for ACHD patients, perhaps one of the most pressing issues is the need to address the significant problem of heart failure in the ACHD population. AbstractHeart failure is an emerging issue with important implications in adult patients with congenital heart disease. Practitioners with expertise in both adult congenital heart disease and heart failure are needed to manage this growing and often complex population. In the United States, the optimal training pathway to enable practitioners to best care for these patients is ill-defined. This article explores possibilities and issues that interested trainees may encounter during their training experience.

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Wait-List Outcomes for Adults With Congenital Heart Disease Listed for Heart Transplantation in the U.S.

Abstract: Wait-list mortality or delisting due to worsening clinical status is disproportionately common for ACHD patients listed as status 1A. An allocation system that takes into account the distinctive aspects of ACHD patients may help better care for this growing population.

Cited by 97 publications

References 28 publications

Heart transplantation and in‐hospital outcomes in adult congenital heart disease patients with Fontan: A decade nationwide analysis from 2004 to 2014

Heart transplantation and in‐hospital outcomes in adult congenital heart disease patients with Fontan: A decade nationwide analysis from 2004 to 2014

Prognostic utility of MELD‐XI in adult congenital heart disease patients undergoing cardiac transplantation

Opportunities for training to advance the care for adults with congenital heart disease with advanced circulatory failure

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