Transcatheter aortic valve-in-valve implantation in right ventricle-aorta conduit in an adult patient with Fontan circulation

Abstract

Catheter interventions can offer patient tailored solutions in high-risk congenital heart disease patients. A 21-year-old male with a Fontan circulation in the setting of unbalanced atrioventricular septal defect with a hypoplastic left ventricle and an aortic homograft connecting the right ventricular outflow tract to the ascending aorta, developed failure of the heavily calcified homograft with severe regurgitation and stenosis. He underwent three sequential transcatheter aortic valve-in-valve implantations to address the homograft failure and the subsequent paravalvular regurgitation, with satisfactory result and improved hemodynamics.

Keywords: Adult congenital heart disease; Catheter intervention; Fontan circulation; Single ventricle physiology; TAVI; Valve-in-valve.

Fig. 1

Schematic overview of patient's anatomy. Ao: aorta, Fo: Fontan, HG: homograft, LV: hypoplastic left ventricle, RV: right ventricle, VSD: ventricular component of the atrioventricular septal defect.

Fig. 2

Transthoracic echocardiography imaging. Preprocedural: The modified apical two chamber view (A) diastolic frame showing the aortic homograft with severe regurgitation and severe stenosis (peak gradient 59 mmHg, mean gradient 42 mmHg). The suprasternal view (B) end-diastolic frame of the aortic arch with significant diastolic flow reversal in the descending aorta (0.34 m/s). Post-procedural, 3 months post re-intervention: The modified apical two chamber view (C) diastolic frame of the 20 mm Edwards Sapien 3 Ultra (Edwards Lifesciences, United Stated of America) bioprostheses in homograft with trace paravalvular regurgitation < grade 1 and normal gradient (peak pressure gradient 30 mmHg, mean pressure gradient 22 mmHg). The suprasternal view (D) end-diastolic frame of aortic arch with mild diastolic flow reversal (0.16 m/s). Ao: aorta, RV: right ventricle.

Fig. 3

Transthoracic echocardiography imaging. Preprocedural: The parasternal short-axis view (A) illustrating the anatomy, (B) diastolic frame with massive backflow through the homograft. Post-procedural: The parasternal short-axis view (C) six weeks after first procedure showing the moderate paravalvular regurgitation, and (D) 3 months post re-intervention with trivial paravalvular regurgitation. AoV: native aortic valve, HG: homograft, RVOT: right ventricular outflow tract.

Fig. 4

Transthoracic echocardiography imaging. Preprocedural: The four-chamber view (A) illustrating the anatomy, and (B) showing the preprocedural severe atrioventricular-valve regurgitation. Post-procedural: The four-chamber view (C) 3 months post re-intervention with the reduction of the atrioventricular-valve regurgitation to moderate. Preprocedural: The four-chamber view (D) showing the bidirectional flow over the ventricular septum defect, and (E) the flow velocity and peak pressure gradient over the ventricular septum defect (3.34 m/s, 45 mmHg). Post-procedural: The four-chamber view (F) 3 months post re-intervention showing the reduced flow velocity and peak pressure gradient over the ventricular septum defect (2.08 m/s, 17 mmHg). Note the hypertrophic right ventricle and the relatively small, hypoplastic, left ventricle. ASD: atrial shunt of the atrioventricular septal defect, AV-valve: atrioventricular-valve, Fo: Fontan conduit, LA: left atrium, LV: hypoplastic left ventricle, RA: right atrium, RV: right ventricle, VSD: ventricular component of the atrioventricular septal defect.

Fig. 5

Computed tomography images illustrating (A) the severely calcified bicuspidalized homograft from the right ventricular outflow tract connected to the native ascending aorta and (B) 3D images illustrating the 3-dimensional configuration. Note the location of the coronary arteries arising from the native aorta and their respect to the homograft. Ao: aorta, AoV: native aortic valve, HG: homograft, LAD: left anterior descending artery, RCA: right coronary artery RV: right ventricle.

Fig. 6

Catheterization images from transfemoral aortic valve-in-valve implantation procedures. Angiographic projection (LAO 22°) of (A) the calcified homograft, and after insertion of a Safari (Boston Scientific, United Stated of America) wire and pigtail catheter, the valve was predilated with (B) POWERFLEX® (Cordis, United Stated of America) 12mm, (C) True™ Dilatation Balloon (Becton Dickinson, United Stated of America) 18mm. Angiographic projection (LSO 43/13°) of (D) the 20 mm Edwards Sapien 3 Ultra (Edwards Lifesciences, United Stated of America) bioprosthesis positioned at the annulus level, and (E) the valve expansion with balloon. Angiographic projection (RSO 16/20°) of (F) the deployed valve. Angiographic projection (LSO 23/20°) of (G) the second 20 mm Edwards Sapien 3 Ultra (Edwards Lifesciences) bioprosthesis deployed with overlap at the annular level, and (H) the third valve deployed 20 mm Edwards Sapien 3 Ultra (Edwards Lifesciences) bioprosthesis placed superiorly reaching till the connection with the aorta.