Background Transcatheter aortic valve implantation (TAVI) is reserved for inoperable aortic stenosis. The use of TAVI for AS in the adult congenital heart disease (ACHD) population is not well described. We aim to assess the effectiveness of TAVI for treatment of aortic stenosis in the ACHD population.

Methods A retrospective review of cardiac catheterisation reports and medical notes of all patients that underwent TAVI from January 2008 to August 2016. 4 ACHD patients were identified from 329 TAVI procedures performed at the Bristol Heart Institute. All patients were declined for surgery by surgical team in the multi-disciplinary team meeting. Patients received either a Core Valve Evolut R or Edwards Sapien TAVI valve based on their aortic valve anatomy.

Results All 4 patients (3 Male, 1 Female) had different underlying congenital anatomy (calcified aortic valve homograft, congenitally-corrected transposition of great arteries, bicuspid aortic valve with coarctation aneurysm, and atriopulmonary Fontan with bicuspid aortic valve respectively). This is shown in Table 1. Median age was 66.7 (range 29–81). Mean aortic annulus size was 27 mm (range 24 mm – 30 mm), mean echo pre-procedural peak gradient was 66 mmHg (range 47 mmHg – 85 mmHg), and mean echo post-procedural peak gradient was 22 mmHg (range 21 mmHg – 23 mmHg). 2 Core Valve Evolut R (29 mm and 31 mm) and 2 Edwards Sapien S3 valve (23 mm) were implanted. Median stay in hospital was 13.0 days (range 6–28). 1 patient required a pacemaker post TAVI. No severe post-TAVI paravalvular leak. All patients had reduction in NYHA class post TAVI. 1 patient died at 4 months post-TAVI from recurrent aortic valve endocarditis and 1 patient died at 7 months post-TAVI unrelated to the procedure.

Conclusion TAVI is potentially an evolving therapy for inoperable aortic stenosis in ACHD patients with good symptomatic relief. Further experience with the use of TAVI in the ACHD patients is required to assess long-term outcomes in unique group of patients.