Transcatheter aortic valve implantation in a 13-year-old child with end-stage heart failure: a case report

Abstract

Background: Left ventricular non-compaction cardiomyopathy (LVNC) has been reported in association with almost all types of congenital heart valve disease. The presence of LVNC-related ventricular dysfunction increases the perioperative risk in these patients. The advantages of transcatheter treatment modalities outweigh those of surgical strategies, as they avoid cardioplegic arrest and myocardial trauma. To our knowledge, there have been no reports on transcatheter treatment of pure aortic regurgitation in patients with a bicuspid aortic valve (BAV) and concomitant LVNC.

Case summary: In this article, we present the case of a 13-year-old boy with a regurgitant BAV and concomitant LVNC who presented with end-stage heart failure and severe pulmonary hypertension. As a bridge to definitive therapy, the patient underwent an uneventful transcatheter aortic valve implantation (TAVI) using a 26-mm balloon-expandable prosthesis. Device success without paravalvular regurgitation was achieved. At 17 months of follow-up, a steady reduction in pulmonary arterial pressure, persistent normalization of systolic left ventricular function and a tremendous improvement in the patient's physical resilience was observed. The initially considered heart-lung transplantation was avoided and will not be necessary.

Discussion: To the best of our knowledge, this is the first case performed with TAVI for BAV regurgitation in the context of LVNC. With technical modifications and appropriate planning, TAVI in paediatric patients with a non-calcified BAV is feasible. Different imaging modalities revealed an intriguing relationship between aortic regurgitation and morphological signs of a left ventricular non-compaction myocardium.

Keywords: Aortic regurgitation; Bicuspid aortic valve; Case report; End-stage heart failure; Heart transplantation; Left ventricular non-compaction cardiomyopathy; Transcatheter aortic valve implantation or replacement.

Figure 1

Colourized cardiac magnetic resonance imaging. Coronal view at end-diastole (A): a jet stream (white arrow) originating from the regurgitant bicuspid valve is directed towards the spongy left ventricular wall (dashed box). Enlarged section (B): a thick inner layer of non-compacted myocardium (black dashed lines) can be separated from a thin outer compacted layer (white dashed lines).

Figure 2

Device landing zone: 3D reconstruction of computed tomography. (A) View on the bicuspid aortic valve; (B) virtual aortic valve annulus: 19.3 × 30.4 mm; (C) oblique coronal view with left coronary artery (white arrow); (D) oblique sagittal view with right coronary artery (white arrow).

Figure 3

Transcatheter aortic valve implantation. Sequence of transcatheter aortic valve implantation with aortic regurgitation (A), balloon sizing (B), stepwise valve deployment (C–E), and final result (F).

Figure 4

Cardiac magnetic resonance imaging after transcatheter aortic valve implantation. Coronal view (A) and 3-chamber view (B). End-systolic orifice area of the SAPIEN-3 prosthesis (C). Whole-heart 3D imaging with virtual SAPIEN-3 geometry (D). Computational fluid dynamic simulation (E) shows colour-coded streamlines illustrating a laminar flow profile without regurgitation.