Transplantation after Mustard operation for transposition of the great arteries

Shintaro Katahira1,2, Yukiharu Sugimura1, Hug Aubin1

  • 1Department of Cardiac Surgery Medical Faculty Heinrich-Heine-University Düsseldorf Germany.

Clinical Case Reports
|October 18, 2021
PubMed

Insights

Adults with congenital heart defects like dextro-transposition of the great arteries (d-TGA) may need heart transplants due to late systemic right ventricular failure. This case highlights heart transplantation 36 years post-Mustard procedure for d-TGA.

Area of Science:

  • Cardiology
  • Adult Congenital Heart Disease
  • Cardiac Surgery

Background:

  • Improved long-term outcomes for congenital heart disease (CHD) lead to increased adult cases requiring heart transplantation.
  • Systemic right ventricular (RV) failure is a significant long-term complication in adults with dextro-transposition of the great arteries (d-TGA) initially treated with palliative procedures.

Observation:

  • This report details a rare case of a patient with d-TGA who underwent heart transplantation.
  • The patient had previously undergone a Mustard procedure 36 years prior, representing their third major cardiac operation.

Findings:

  • The patient presented with progressive late failure of the systemic RV, a known but challenging long-term complication.
  • Heart transplantation was deemed the necessary intervention for end-stage RV failure in this complex CHD case.

Implications:

  • This case underscores the critical need for lifelong monitoring of adult CHD patients, particularly those with d-TGA treated with older palliative techniques.
  • It highlights heart transplantation as a viable, albeit complex, option for managing end-stage RV failure in this population.
  • Further research into the long-term RV performance after palliative CHD surgeries is warranted to optimize management strategies.