Author + information
- Received August 16, 2018
- Accepted August 21, 2018
- Published online November 5, 2018.
- Stefano Cannata, MD∗ (, )
- Dario Freitas, BSc,
- Gianluca Lucchese, MD,
- Alessandra Frigiola, MD,
- Jane Hancock, MD,
- Catherine Head, MD,
- Christopher Young, MD,
- Bernard Prendergast, MD and
- Simon Redwood, MD
- ↵∗Address for correspondence:
Dr. Stefano Cannata, St. Thomas’ Hospital, Department of Cardiology, Westminster Bridge Road, London SE1 7EH, United Kingdom.
A 21-year-old woman affected by a rare autosomal-dominant disorder called Singleton-Merten syndrome (1) with symptomatic severe aortic regurgitation in New York Heart Association functional class III was referred for consideration of aortic valve intervention. Her clinical history was characterized by a Ross procedure for severe aortic stenosis combined with replacement of a calcified ascending aorta 7 years previously, moderate left ventricular impairment, and a recent major stroke. Transesophageal echocardiography confirmed severe aortic regurgitation (Figures 1A and 1B), and computed tomography (Figures 1C to 1E) of the aorta and iliofemoral vasculature demonstrated absence of calcium in the aortic valve leaflets, small caliber of both iliac-femoral axes, and a very low (5.2 mm) origin of the left main coronary artery (LM) with associated ostial stenosis (Figure 1F). After multidisciplinary discussion involving our heart valve and adult congenital heart disease teams, consensus was reached to perform transcatheter aortic valve replacement because of the high risk of conventional surgery. Transfemoral access was planned despite the prohibitive vascular access, with protection of the LM using an undeployed drug-eluting stent positioned in the mid left anterior descending coronary artery. However, despite smooth insertion of a 14-F sheath (Edwards Lifesciences, Irvine, California), passage of the valve delivery system was not feasible through such a small iliac artery (diameter 4.5 mm) (Figure 1G). We therefore switched to transapical access, and transcatheter aortic valve replacement was successfully performed using a 26-mm SAPIEN 3 valve (Edwards Lifesciences), immediately followed by ostial LM stenting (Figures 1H to 1L). Post-procedural recovery was uneventful, and the patient was discharged after 4 days.
To our knowledge, transcatheter aortic valve replacement in a patient affected by Singleton-Merten syndrome has not previously been reported. A successful outcome was achieved despite the complex clinical and anatomic scenario, including small-caliber peripheral access, a low LM origin coupled with significant ostial disease, and the absence of valve calcification in the context of severe aortic regurgitation.
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Received August 16, 2018.
- Accepted August 21, 2018.
- 2018 American College of Cardiology Foundation