Heart transplantation in congenital heart disease: in whom to consider and when?

Christine H Attenhofer Jost1, Dörthe Schmidt, Michael Huebler

  • 1Division of Cardiac Surgery, University Hospital Zurich, Zurich, Switzerland.

Insights

Adults with complex congenital heart disease (CHD) increasingly need heart transplantation (HTx) due to end-stage heart failure. While HTx in CHD carries risks, outcomes have improved, remaining a vital treatment option.

Area of Science:

  • Cardiology
  • Adult Congenital Heart Disease
  • Transplantation Medicine

Background:

  • Improvements in surgical repair allow complex congenital heart disease (CHD) patients to reach adulthood.
  • These patients face a high risk of end-stage heart failure, increasing the need for heart transplantation (HTx).

Purpose of the Study:

  • To summarize the evolving causes of heart failure deaths in adults with CHD.
  • To discuss reasons for, contraindications to, and risks associated with HTx in CHD patients.
  • To review outcomes and future trends in HTx for complex CHD.

Main Methods:

  • Review of current literature on heart transplantation for congenital heart disease.
  • Discussion of etiologies of heart failure in adult CHD.
  • Presentation of case vignettes illustrating challenges and outcomes of HTx in CHD.

Main Results:

  • HTx for CHD presents higher perioperative death and rejection risks than for acquired heart disease.
  • Outcomes for HTx in complex CHD have improved significantly over the last two decades.
  • Heart-lung transplantation is less frequently needed, while lung transplantation with intracardiac repair is an option for specific defects.

Conclusions:

  • Heart transplantation remains a crucial treatment for end-stage heart failure in adults with complex CHD.
  • Mechanical circulatory support may reduce waiting list mortality.
  • Continued advancements are improving HTx outcomes for CHD patients.

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