title: Heart transplantation in adults with congenital heart disease.
abstract: With the advances in congenital cardiac surgery and postoperative care, an increasing number of children with complex congenital heart disease now reach adulthood. There are already more adults than children living with a congenital heart defect, including patients with complex congenital heart defects. Among these adults with congenital heart disease, a significant number will develop ventricular dysfunction over time. Heart failure accounts for 26-42% of deaths in adults with congenital heart defects. Heart transplantation, or heart-lung transplantation in Eisenmenger syndrome, then becomes the ultimate therapeutic possibility for these patients. This population is deemed to be at high risk of mortality after heart transplantation, although their long-term survival is similar to that of patients transplanted for other reasons. Indeed, heart transplantation in adults with congenital heart disease is often challenging, because of several potential problems: complex cardiac and vascular anatomy, multiple previous palliative and corrective surgeries, and effects on other organs (kidney, liver, lungs) of long-standing cardiac dysfunction or cyanosis, with frequent elevation of pulmonary vascular resistance. In this review, we focus on the specific problems relating to heart and heart-lung transplantation in this population, revisit the indications/contraindications, and update the long-term outcomes.
authors.affiliation: Service de Chirurgie des Cardiopathies Congénitales, Hôpital Marie-Lannelongue-M3C, Le Plessis-Robinson, France. Electronic address: l.houyel@ccml.fr.
authors.forename: Lucile
authors.initials: L
authors.lastname: Houyel
authors.affiliation: Service de Cardiologie, Hôpital Marie-Lannelongue, Le Plessis-Robinson, France.
authors.forename: Ngoc-Tram
authors.initials: NT
authors.lastname: To-Dumortier
authors.affiliation: Service de Cardiologie, Hôpital Marie-Lannelongue, Le Plessis-Robinson, France.
authors.forename: Yannick
authors.initials: Y
authors.lastname: Lepers
authors.affiliation: Service de Chirurgie des Cardiopathies Congénitales, Hôpital Marie-Lannelongue-M3C, Le Plessis-Robinson, France.
authors.forename: Jérôme
authors.initials: J
authors.lastname: Petit
authors.affiliation: Service de Chirurgie des Cardiopathies Congénitales, Hôpital Marie-Lannelongue-M3C, Le Plessis-Robinson, France.
authors.forename: Régine
authors.initials: R
authors.lastname: Roussin
authors.affiliation: Service de Chirurgie des Cardiopathies Congénitales, Hôpital Marie-Lannelongue-M3C, Le Plessis-Robinson, France.
authors.forename: Mohamed
authors.initials: M
authors.lastname: Ly
authors.affiliation: Service de Chirurgie des Cardiopathies Congénitales, Hôpital Marie-Lannelongue-M3C, Le Plessis-Robinson, France.
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Lebret
authors.affiliation: Service de Chirurgie Thoracique, Hôpital Marie-Lannelongue, Le Plessis-Robinson, France.
authors.forename: Elie
authors.initials: E
authors.lastname: Fadel
authors.affiliation: Service de Chirurgie des Cardiopathies Congénitales, Hôpital Marie-Lannelongue-M3C, Le Plessis-Robinson, France.
authors.forename: Jürgen
authors.initials: J
authors.lastname: Hörer
authors.affiliation: Service de Chirurgie des Cardiopathies Congénitales, Hôpital Marie-Lannelongue-M3C, Le Plessis-Robinson, France.
authors.forename: Sébastien
authors.initials: S
authors.lastname: Hascoët
mesh_terms: D000328:Adult; D000367:Age Factors; D006330:Heart Defects, Congenital / Q000150:complications / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D006333:Heart Failure / Q000209:etiology / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D016027:Heart Transplantation / Q000009:adverse effects / Q000401:mortality; D006353:Heart-Assist Devices; D006801:Humans; D016040:Lung Transplantation; D018579:Patient Selection; D018570:Risk Assessment; D012307:Risk Factors; D017741:Survivors; D013997:Time Factors; D016896:Treatment Outcome
keywords: Adult congenital heart disease; Cardiopathies congénitales de l’adulte; Heart transplantation; Heart–lung transplantation; Pulmonary vascular resistance; Résistances vasculaires pulmonaires; Transplantation cardiaque; Transplantation cardiopulmonaire