Case Report: Percutaneous Mitral Valve Repair in a Patient with Fontan Repair for a Single Functional Ventricle

R. J. Donovan, S. Lim, W. R. Davidson

Research output: Contribution to journalArticle


PURPOSE: Mitral regurgitation (MR) can complicate congenital heart disease, particularly when secondary to left ventricular dysfunction and mitral annular dilation. Significant MR is associated with substantial morbidity and mortality in patients with reduced LV systolic function, with recent studies demonstrating that percutaneous mitral valve repair can significantly reduce hospitalizations and all-cause mortality. METHODS: A 31 year-old man was referred to our Advanced Valve center for evaluation and management of significant MR. He was born with pulmonary atresia and was palliated by oversewing the tricuspid valve and creating Fontan physiology using an intracardiac lateral tunnel. At the age of 30 he presented with fatigue with an echo that showed an LV EF of 40-45% with severe MR. He was evaluated for transplantation at his referring facility but was not felt to be a candidate and was referred to our clinic for consideration of valvular intervention. RESULTS: Following TEE and CT evaluations, he was deemed to be a candidate for transcutaneous mitral valve repair, with functional etiology of his MR. Following femoral venous access, a transseptal puncture was made across the lateral tunnel of the patient's Fontan circuit. A steerable guide catheter was then introduced into the left atrium and percutaneous transcatheter mitral valve repair was performed using the MitraClip system (4 clips on A2/P2 scallops), with reduction in the MR from severe to mild. The patient tolerated the procedure well with no significant complications. After the procedure, the patient reported improvement in his symptoms of dyspnea and fatigue with sustained clinical improvement several months later (NYHA class II). CONCLUSION: Transcatheter mitral valve repair is an accepted method for the treatment of severe MR and its symptoms in patients with both functional and degenerative MR. To our knowledge, this experience represents the first case report of this new technology being used in the treatment of congenital heart disease patients with single ventricle physiology. Given the challenges of surgical repair in this population and the increasing number of patients living into adulthood with congenital heart disease, we believe that transcatheter valvular interventions will become an important tool for the care of these complex patients.

All Science Journal Classification (ASJC) codes

  • Surgery
  • Pulmonary and Respiratory Medicine
  • Cardiology and Cardiovascular Medicine
  • Transplantation

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