Outcome following heart transplant assessment in adults with congenital heart disease

Heart. 2019 Nov;105(22):1741-1747. doi: 10.1136/heartjnl-2019-314711. Epub 2019 Jul 5.

Abstract

Objectives: Adults with congenital heart disease (ACHD) are a growing group with end-stage heart failure. We aim to describe the outcomes of ACHD patients undergoing assessment for orthotopic heart transplant (OHT).

Methods: Case notes of consecutive ACHD patients (>16 years) assessed for OHT between 2000 and 2016 at our centre were reviewed. Decision and outcome were reported as of 2017. Data were analysed in three groups: systemic left ventricle (LV), systemic right ventricle (RV) and single ventricle (SV).

Results: 196 patients were assessed (31.8 years, 27% LV, 29% RV, 44% SV). 89 (45%) patients were listed for OHT and 67 (34%) were transplanted. 41 (21%) were unsuitable or too high risk and 36 (18%) were too well for listing. Conventional surgery was undertaken in 13 (7%) and ventricular assist device in 17 (9%) with 7 (4%) bridged to candidacy. Survival from assessment was 84.2% at 1 year and 69.7% at 5 years, with no difference between groups. Patients who were considered unsuitable for OHT (HR 11.199, p<0.001) and listed (HR 3.792, p=0.030) were more likely to die than those who were considered too well. Assessments increased over the study period.

Conclusions: The number of ACHD patients assessed for OHT is increasing. A third are transplanted with a small number receiving conventional surgery. Those who are unsuitable have a poor prognosis.

Keywords: complex congenital heart disease; congenital heart disease; congenital heart disease surgery; heart transplantation.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Clinical Decision-Making
  • Disease Progression
  • Female
  • Heart Defects, Congenital / complications*
  • Heart Defects, Congenital / diagnostic imaging
  • Heart Defects, Congenital / physiopathology
  • Heart Failure / diagnostic imaging
  • Heart Failure / etiology
  • Heart Failure / physiopathology
  • Heart Failure / surgery*
  • Heart Transplantation* / adverse effects
  • Heart Ventricles / abnormalities
  • Heart Ventricles / diagnostic imaging
  • Heart Ventricles / physiopathology
  • Heart Ventricles / surgery*
  • Humans
  • Male
  • Middle Aged
  • Patient Selection
  • Retrospective Studies
  • Risk Assessment
  • Risk Factors
  • Survivors*
  • Treatment Outcome
  • Ventricular Function, Left
  • Ventricular Function, Right
  • Waiting Lists
  • Young Adult