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Original research article
Successful cardiac transplantation outcomes in patients with adult congenital heart disease
  1. Jonathan N Menachem1,
  2. Jessica R Golbus2,
  3. Maria Molina3,
  4. Jeremy A Mazurek1,
  5. Nicole Hornsby1,
  6. Pavan Atluri3,
  7. Stephanie Fuller4,
  8. Edo Y Birati1,
  9. Yuli Y Kim1,
  10. Lee R Goldberg1,
  11. Joyce W Wald1
  1. 1 Division of Cardiology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA
  2. 2 Division of Cardiology, University of Michigan, Ann Arbor, Michigan, USA
  3. 3 Department of Cardiovascular Surgery, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA
  4. 4 Division of Cardiothoracic Surgery, Department of Surgery, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
  1. Correspondence to Dr Jonathan N Menachem, Hospital of the University of Pennsylvania, Perelman Center for Advanced Medicine, South Pavilion, 11th Floor, 3400 Civic Center Boulevard, Philadelphia, PA 19104, USA; jonathan.menachem{at}


Objectives The purpose of our study is (1) to characterise patients with congenital heart disease undergoing heart transplantation by adult cardiac surgeons in a large academic medical centre and (2) to describe successful outcomes associated with our multidisciplinary approach to the evaluation and treatment of adults with congenital heart disease (ACHD) undergoing orthotopic heart transplantation (OHT).

Background Heart failure is the leading cause of death in patients with ACHD leading to increasing referrals for OHT.

Methods The Penn Congenital Transplant Database comprises a cohort of patients with ACHD who underwent OHT between March 2010 and April 2016. We performed a retrospective cohort study of the 20 consecutive patients. Original cardiac diagnoses include single ventricle palliated with Fontan (n=8), dextro-transposition of the great arteries after atrial switch (n=4), tetralogy of Fallot (n=4), pulmonary atresia (n=1), Ebstein anomaly (n=1), unrepaired ventricular septal defect (n=1) and Noonan syndrome with coarctation of the aorta (n=1).

Results Eight patients required pretransplant inotropes and two required pretransplant mechanical support. Nine patients underwent heart–liver transplant and three underwent heart–lung transplant. Three patients required postoperative mechanical circulatory support. Patients were followed for an average of 38 months as of April 2016, with 100% survival at 30 days and 1 year and 94% overall survival (19/20 patients).

Conclusions ACHD–OHT patients require highly specialised, complex and multidisciplinary healthcare. The success of our programme is attributed to using team-based, patient-centred care including our multidisciplinary staff and specialists across programmes and departments.

  • Heart transplantation
  • Congenital heart disease

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  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.