Outcomes of Adults With Congenital Heart Disease Waiting for Heart Transplantation

Study Questions:

Do adult heart transplant candidates with congenital heart disease (CHD) experience higher mortality or delisting due to worsening clinical status?

Methods:

Using data from the Scientific Registry for Transplant Recipients, a retrospective analysis was performed to compare waitlist outcomes and correlates of mortality and delisting between adult heart transplant candidates with and without CHD who were listed for heart transplant between January 19, 1999 and June 2, 2014. The primary outcome was death or delisting due to clinical worsening.

Results:

Outcomes were analyzed for 1,257 adult CHD (ACHD) and 37,248 non-ACHD heart transplant candidates. Follow-up was at 180 days. A greater proportion of ACHD candidates initially listed as status 1A experienced the primary outcome compared to non-ACHD candidates (23.7% vs. 16.7%; p = 0.011). There was no significant difference in the primary outcome among candidates listed as statuses 1B and 2, although there was a trend toward higher mortality among the status 1B candidates. The overall incidence of the primary outcome was similar between CHD and non-CHD groups (p = 0.293). Transplantation was slightly but significantly less frequent among ACHD candidates. Predictors of the primary outcome were in general similar between ACHD and non-ACHD candidates; however, there were a few exceptions, including prior cardiac surgery, which was a predictor for non-ACHD candidates and not ACHD candidates. Predictors of outcome among ACHD candidates included estimated glomerular filtration rate <60 ml/min/1.73 m2, intensive care unit (ICU), hospitalized but not in ICU, mechanical ventilation, and albumin <3.2 g/dl.

Conclusions:

ACHD candidates listed for heart transplant at the highest priority die or are delisted due to clinical worsening more frequently than non-ACHD candidates. Renal function, ICU status, mechanical ventilation, and albumin are significant predictors of outcome among ACHD candidates.

Perspective:

Adult heart transplant candidates with CHD are frequently listed at lower urgency statuses while awaiting heart transplant; however, this study demonstrates that when listed at the highest priority, they are more likely to die or have clinical worsening than candidates without CHD. Findings from this study suggest that ACHD candidates may be listed too late in their disease process, and evidence of end-organ dysfunction may herald poor outcomes. Studies are needed to better define listing criteria and stratification for ACHD candidates.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Cardiac Surgery and CHD & Pediatrics, Cardiac Surgery and Heart Failure, Congenital Heart Disease, CHD & Pediatrics and Interventions, Acute Heart Failure, Heart Transplant, Interventions and Structural Heart Disease

Keywords: Albumins, Cardiac Surgical Procedures, Glomerular Filtration Rate, Heart Defects, Congenital, Heart Failure, Heart Transplantation, Incidence, Intensive Care Units, Mortality, Respiration, Artificial, Waiting Lists


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