Related Experiment Video
Updated: May 22, 2026

Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 18, 2014
Indications for heart transplantation in congenital heart disease
1Department of Cardiology, Great Ormond Street Hospital, Great Ormond Street, London, UK. pincoe1@gosh.nhs.uk
Insights
Cardiac transplantation for congenital heart disease requires careful patient selection and timing. Outcomes vary significantly, with Fontan circulation posing higher risks than Mustard circulation.
Area of Science:
- Cardiology
- Transplantation Medicine
- Congenital Heart Disease
Background:
- Congenital heart disease (CHD) represents a complex challenge in end-stage heart failure management.
- Cardiac transplantation is an established, yet intricate, treatment strategy for select CHD patients.
- Patient heterogeneity in CHD necessitates individualized approaches to transplantation.
Purpose of the Study:
- To review indications for cardiac transplantation in patients with congenital heart disease.
- To discuss the principles of transplant as a primary or secondary intervention.
- To highlight the importance of referral timing, evaluation, and complication management.
Main Methods:
- Literature review focusing on cardiac transplantation in CHD.
- Analysis of general principles, referral timing, and patient evaluation.
- Discussion of prognostic indicators and suitability for listing.
Main Results:
- Patient referral timing and evaluation methods differ between CHD and other heart failure etiologies.
- Complications in CHD transplant candidates require specialized team management.
- Risk stratification is crucial, as not all CHD patients face uniform high transplant risk; failing Fontan circulations carry higher morbidity than failing Mustard circulations.
Conclusions:
- Standardizing transplant timing in heterogeneous CHD populations is challenging.
- Investigations like BNP, 6-minute walk, and exercise testing have roles and limitations in assessing CHD transplant candidates.
- Addressing the organ donor shortage is critical for meeting the growing demand for cardiac transplants in diverse CHD pathologies.
Abstract:
In this review we have looked at indications for cardiac transplantation in congenital heart disease. An outline of the general principles of the use of transplant as a management strategy both as a first line treatment and following other surgical interventions is discussed. We explore the importance of the timing of patient referral and the evaluations undertaken, and how the results of these may vary between patients with congenital heart disease and patients with other causes of end-stage heart failure. The potential complications associated with patients with congenital heart disease need to be both anticipated and managed appropriately by an experienced team. Timing of transplantation in congenital heart disease is difficult to standardize as the group of patients is heterogeneous. We discuss the role and limitations of investigations such as BNP, 6 minute walk, metabolic exercise testing and self estimated physical functioning. We also discuss the suitability for listing. It is clear that congenital heart patients should not be considered to be at uniform high risk of death at transplant. Morbidity varies greatly in the congenital patient population with the failing Fontan circulation having a far higher risk than a failing Mustard circulation. However the underlying issue of imbalance between donor organ supply and demand needs to be addressed as transplant teams are finding themselves in the increasingly difficult situation of supporting growing numbers of patients with a diverse range of pathologies with declining numbers of donor organs.
Related Concept Videos
Kidney Transplant I: Introduction
Tissue Transplantation
The Biology of Tissue Transplantation
The biology of tissue transplantation hinges on the Major Histocompatibility Complex (MHC) molecules. These molecules...
Cardiomyopathy V: Interprofessional Care
Cardiomyopathy II: Dilated Cardiomyopathy
Heart Failure IV: Classification and Diagnostic Evaluation
Cardiomyopathy III: Hypertrophic Cardiomyopathy

