Managing multiple failed bioprosthetic heart valves in high-risk patients poses challenges in the lifelong management of valvular heart disease. Transcatheter options offer a less invasive and often the only viable option in this patient cohort.1 Here, we report a case of a young patient who initially underwent surgical multivalvular replacement at the age of 33 years secondary to congenital rubella syndrome.
The 41-year-old man, who uses a wheelchair, with a small body size (154 cm, 37 kg) and a complex cardiac history, necessitating surgical aortic and mitral valve replacement with, respectively, 23-mm and 27-mm Carpentier-Edwards Perimount Magna Ease valves (Edwards Lifesciences) as well as tricuspid valve annuloplasty, was admitted to the hospital with refractory heart failure due to multivalve failure 8 years after the initial intervention. Transthoracic echocardiography documented a failed surgical aortic valve with severe transvalvular regurgitation and severe bioprosthetic mitral stenosis (mean gradient 24 mm Hg). Computed tomography indicated a low risk for coronary obstruction with aortic valve-in-valve (VIV) implantation, with small-calibre (4-5 mm) noncalcified iliofemoral arteries and a significant anticipated risk of neo–left ventricular outflow tract (LVOT) obstruction with transseptal mitral VIV replacement (TS-MVIVR) (Fig. 1E). His surgical risk was deemed to be prohibitive.
Figure 1.
Managing multivalvular failure and hidden aortic root–LVOT fistula. (A) Aortic root angiogram pre-VIV indicates severe regurgitation (yellow arrows). (B) Post-VIV demasks communication (blue arrow) between aortic root and LV, which was obscured by dominant transvalvular regurgitation. (C, D) Pre VIV computed tomography (CT) revealed dehiscence at the level of sewing ring of bioprosthetic aortic valve at non- and right coronary commissure (white arrows). (E) High anticipated risk of Neo-Left-Ventricular-Outflow-Tract (Neo-LVOT) obstruction on CT. (F, G) Ventricular portion of THV (green line) positioned in line with the stent posts of surgical bioprosthesis to minimize risk of Neo-LVOT obstruction with a good final result. (H, I) Final aortic root angiogram after paravalvular leak closure under fluoroscopic and ICE guidance showing good position of the device (green arrows) with significant reduction of paravalvular regurgitation. (J, K) TTE pre- and post- paravalvular leak closure with a 10 × 5 mm Amplatzer Valvular Plug III device confirming significant reduction in paravalvular regurgitation (white arrows). ICE, intracardiac echocardiogram; LV, left ventricle; THV, transcatheter heart valve; TS-MVIVR, transseptal mitral valve in valve replacement; TTE, transthoracic echocardiogram; VIV, valve in valve.
After heart team discussion, a 23-mm Sapien Ultra valve (Edwards Lifesciences) was used via transfemoral approach for aortic VIV replacement, which proceeded uneventfully. However, postprocedure aortic root angiography revealed an aortic root–LVOT fistula with significant regurgitation, initially underappreciated owing to dominant transvalvular regurgitation (Video 1; Fig. 1, A and B). This was later confirmed on review of the pre-VIV CT, which revealed dehiscence, which had been initially overlooked. Plans were made to address this electively in combination with TS-MVIVR, but the patient was readmitted 3 weeks later secondary to congestive heart failure accelerated by Covid-19 pneumonia.
After reassessment, it was decided to proceed with the intervention. Attempts to insert even a paediatric transesophageal echocardiography probe were unsuccessful, resulting in TS-MVIVR and fistula closure under conscious sedation, with intracardiac echocardiography (ICE) used as an alternative in addition to fluoroscopy. After an ICE-guided transseptal puncture, the ventricular side of a 26-mm Sapien 3 Ultra transcatheter heart valve (Edwards Lifesciences) was positioned in line with the posts of the surgical bioprosthetic, rather than in the usual “deeper” position of the valve, to mitigate the risk of the neo-LVOT obstruction, achieving a good final result (Fig. 1, F and G). This was followed by the successful retrograde closure of the aortic root–LVOT fistula with the use of a 10 × 5 mm Amplatzer Vascular Plug III (Abbott) under ICE and fluoroscopy guidance (Fig. 1, F-K). The combined procedure was well tolerated, and the patient was discharged after 1 week with good outcomes at the 3-month follow-up.
This case underscores the complexity and challenges of multimodal imaging and the management of failed bioprosthetic heart valves in high-risk patients with limited surgical options. Underlying pathologies, such as paravalvular fistulas, may be obscured and overlooked in the presence of dominant transvalvular regurgitation and require careful attention in periprocedural planning to optimise outcomes in this patient population.
Acknowledgments
Ethics Statement
The case reported in this paper adhered to CONSORT guidelines.
Patient Consent
The authors confirm that patient consent is not applicable to this article. This is a retrospective case report using de-identified data; therefore, the Institutional Review Board did not require consent from the patient.
Funding Sources
The authors have no funding sources to declare.
Disclosures
Dr Wussler reports research grants from the Swiss National Science Foundation (grant ref.P500PM_225285), the Swiss Heart Foundation (grant ref. FF22112), the University Hospital Basel, and the German Heart Foundation (grant ref. K22/13) as well as speaking honoraria from PHC outside the submitted work. Dr Sellers is a consultant for Edwards Lifesciences, Anteris, Abbott, Excision Medical, and Medtronic and has received research support from Medtronic, Vivitro Labs, and Edwards Lifesciences. Dr Leipsic is supported by a Canadian Research Chair in Advanced Cardiopulmonary Imaging, consults for MVRX, Heartflow, and Circle Cardiovascular Imaging, and provides CT core laboratory services for Edwards Lifesciences, Medtronic, Neovasc, Boston Scientific, and Tendyne Holdings, for which no direct compensation is received. Dr Sathananthan is an employee of Boston Scientific, has received speaking fees from Edwards Lifesciences, is a consultant for Edwards Lifesciences, Boston Scientific, NVT Medical, and Medtronic, and has received research support from Medtronic, Vivitro Labs, and Edwards Lifesciences. Dr Webb is a consultant for Edwards Lifesciences and receives research funding from Edwards Lifesciences, Medtronic, and Boston Scientific. Dr Wood is a consultant for and has received research funding from Edwards Lifesciences and Abbott. The other authors have no conflicts of interest to disclose.
Novel Teaching Point.
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In the presence of dominant transvalvular regurgitation, diagnosing a coexisting aortic root–to–LVOT fistula presents a significant challenge and may be overlooked.
Footnotes
See page 306 for disclosure information.
To access the supplementary material accompanying this article, visit the online version of the Canadian Journal of Cardiology at www.onlinecjc.ca and at https://doi.org/10.1016/j.cjco.2024.12.006.
Supplementary Material
Aortic root angiography before TAV-in-SAV indicates severe transvalvular regurgitation, obscuring the presence of paravalvular regurgitation; post–TAV-in-SAV angiography reveals communication between aortic root and left ventricle with significant regurgitation. SAV, surgical aortic valve; TAV, transcather aortic valve.
Reference
- 1.Mylotte D., Osnabrugge R.L.J., Martucci G., et al. Failing surgical bioprosthesis in aortic and mitral position. EuroIntervension. 2013;9:S77–S83. doi: 10.4244/EIJV9SSA15. [DOI] [PubMed] [Google Scholar]
Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.
Supplementary Materials
Aortic root angiography before TAV-in-SAV indicates severe transvalvular regurgitation, obscuring the presence of paravalvular regurgitation; post–TAV-in-SAV angiography reveals communication between aortic root and left ventricle with significant regurgitation. SAV, surgical aortic valve; TAV, transcather aortic valve.