Author + information
- Received February 6, 2014
- Accepted February 13, 2014
- Published online October 1, 2014.
- Gonzalo J. Martínez, MD∗,†,
- Bernard H. Ng, MBBS‡,
- Michael K. Wilson, MBBS§,
- Sanjeevan Pasupati, MBChB‖,
- David A. Robinson, MBBS, MA¶,
- Bruce L. Cartwright, MBBS#,
- Mark R. Adams, MBBS, PhD∗,
- David S. Celermajer, MBBS, PhD, DSc∗ and
- Martin K. Ng, MBBS, PhD∗∗ ()
- ∗Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia
- †Department of Cardiology, Catholic University, Santiago, Chile
- ‡Department of Radiology, Royal Prince Alfred Hospital, Sydney, Australia
- §Department of Cardiothoracic Surgery, Royal Prince Alfred Hospital, Sydney, Australia
- ‖Department of Cardiology, Waikato Hospital, Hamilton, New Zealand
- ¶Department of Vascular Surgery, Royal Prince Alfred Hospital, Sydney, Australia
- #Department of Cardiac Anesthesia, Royal Prince Alfred Hospital, Sydney, Australia
- ↵∗Reprint requests and correspondence:
Dr. Martin K. C. Ng, Department of Cardiology, Royal Prince Alfred Hospital, Missenden Road, Camperdown, Sydney, New South Wales 2050, Australia.
- adult congenital heart disease
- bioprosthetic valve failure
- pulmonary valve
- transcatheter aortic valve replacement
A 30-year-old man with complex congenital heart disease presented with severe heart failure. He was born with a double-inlet left ventricle with leva-transposition of the great arteries and severe pulmonary stenosis. This had been palliated by bicavopulmonary anastomosis and, in 1999, by a Damus-Kaye-Stanzel procedure (anastomosis of the pulmonary artery proximal remnant to the ascending aorta for restrictive bulboventricular foramen) with pulmonary valve replacement using a 21-mm stentless bioprosthetic valve (Figure 1A). Fourteen years later, he presented with severe bioprosthetic valve stenosis (mean gradient 41 mm Hg in the setting of severe systolic dysfunction). He also had long-standing protein-losing enteropathy. As he was deemed too high risk for surgical valve re-replacement, transcatheter implantation into the outlet ventriculo-arterial (pulmonary) valve position was proposed.
Due to the small size of the previous bioprosthesis (internal diameters 18.2 mm and 15.6 mm), a self-expanding supra-annular transcatheter valve (23-mm CoreValve, Medtronic, Minneapolis, Minnesota) was chosen to reduce the risk of high post-procedural transvalvular gradient. The levo-transposition of the great arteries anatomy distorted the aortic arch angulation, making its passage challenging (Figure 1B). The CoreValve device was deployed under rapid pacing and post-deployment balloon valvuloplasty (Cristal 20-mm balloon, BALT, Montmorency, France) was performed to optimize expansion (Figure 2, Online Video 1). The patient recovered uneventfully after the procedure and improved significantly. One-month echocardiography and computed tomography scan confirmed a normally functioning CoreValve (mean gradient 10 mm Hg) without aortic regurgitation (Figure 3, Online Video 2).
Although transcatheter aortic valves have been developed for treatment of high-risk senile calcific stenosis, there has been increasing interest in their applications in different clinical scenarios (1). Previous experiences of transcatheter valve-in-valve replacement have shown its feasibility for treatment of failing bioprosthesis predominantly in the aortic position (2). Our case highlights the importance of computed tomography imaging to optimize procedural planning in complex cardiovascular anatomies and how transcatheter valve technologies can be adapted to treat valvular pathologies in congenital heart disease.
For accompanying videos, please see the online version of this article.
Dr. Wilson is a proctor for Edwards Lifesciences. Dr. Pasupati is a proctor for Edwards Lifesciences, Medtronic, and St. Jude Medical. All other authors have reported that they have no relationships relevant to this paper to disclose.
- Received February 6, 2014.
- Accepted February 13, 2014.
- American College of Cardiology Foundation
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