Abstract
We describe a rare and extremely challenging case of transcatheter pulmonary valve implantation in repaired tetralogy of Fallot and anomalous origin of the left main coronary artery from the right coronary sinus. Procedural planning based on advanced multimodality imaging and 3-dimensional technology proved to be the key to procedural success.
Key Words: coronary anomalies, multimodality imaging, percutaneous pulmonary valve implantation, repaired tetralogy of Fallot
Graphical abstract
A 66-year-old woman with repaired tetralogy of Fallot (ie, prior infundibulotomy without a transannular patch and concomitant ventricular septal defect closure) underwent transcatheter pulmonary valve implantation (TPVI) for symptomatic severe pulmonary valve regurgitation and right ventricular volume overload. Notably, she had an anomalous origin of the left main coronary artery (LMCA) from the right coronary sinus with a single coronary ostium.
Computed tomography (CT) revealed a close proximity between the LMCA and right ventricular outflow tract (RVOT) (Figure 1A), challenging the choice of a safe landing zone because of the high risk of LMCA extrinsic compression.1 To guide transcatheter heart valve (THV) selection, a CT-based 3-dimensional (3D) virtual model was reconstructed, reporting a distance between the LMCA and RVOT below 2 mm. According to the CT-based RVOT diameters (28.3 × 20.4 mm) (Figure 1B), the 29-mm SAPIEN 3 (S3, Edwards Lifesciences Corp) valve was selected for its limited height when deployed (22.5 mm according to the vendor’s specifications). TPVI without prestenting was considered to minimize the hazard of coronary artery compression2 given the fracture-resistant and high radial force S3 frame and the reported favorable outcomes of S3 direct implantation.3 Hence, considering the S3 expanded height, an appropriate landing zone was identified to minimize the potential risk for LMCA extrinsic compression after device deployment without compromising anchorage stability.
Figure 1.
TPVI Planning and Procedure
(A) Segmentation of computed tomography imaging and 3-dimensional (3D) reconstruction of patient-specific anatomy, pointing out the close proximity of the left main coronary artery (LMCA) to the right ventricular outflow tract (RVOT) wall, and (B) extraction of the RVOT diameters on the identified landing zone for transcatheter heart valve (THV) deployment. Sizing balloon inflation during (C) simultaneous right ventricular angiography to assess for adequate RVOT occlusion and hence confirm THV sizing and during (D) simultaneous selective coronary angiography to test for extrinsic coronary compression. (E) A 3D model overlay on real-time angiograms through the HeartNavigator system (Philips Healthcare) to improve the accuracy of landing zone visualization. (F) The final angiogram confirming LMCA patency.
The TPVI procedure was performed under general anesthesia and mechanical ventilation. After baseline angiography, RVOT sizing balloon interrogation was performed during simultaneous right ventricular angiography to confirm THV sizing (Figure 1C) and during simultaneous aortography and selective coronary angiography to test for coronary compression (Figure 1D). A direct S3 implantation was performed using the valve delivery system (16F eSheath, Edwards Lifesciences Corp); the virtual 3D model was superimposed on the real-time angiograms during valve deployment to facilitate landing zone visualization (Figure 1E). A workhorse guidewire was positioned in the left anterior descending artery during THV implantation. The final angiograms revealed procedural success (Videos 1 and 2) and patency of the LMCA (Figure 1F). Predischarge transthoracic echocardiography confirmed good THV function, which remained stable at the 10-month follow-up.
To the best of our knowledge, this is the first case reported in the literature of TPVI in the concomitant LMCA arising from the right coronary sinus and in close proximity to the RVOT. We showed that accurate procedural planning, informed by advanced multimodality imaging and 3D-based analysis, allows the boundaries of anatomical suitability for percutaneous procedures to be pushed. Accordingly, the THV choice, the identification of an appropriate landing zone, and the decision to avoid prestenting, all dictated by the meticulous preprocedural assessment, contributed to effectively minimize the hazard for LMCA compression.
Funding Support and Author Disclosures
This work was supported by IRCCS Policlinico San Donato, a clinical research hospital partially funded by the Italian Ministry of Health, and was supported in part by Ricerca Corrente from the Italian Ministry of Health. The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Footnotes
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the Author Center.
Appendix
For supplemental videos, please see the online version of this paper.
Appendix
Final Pulmonary Trunk Angiography to Assess for Absence of Intravalvular Regurgitation and Paravalvular Leak
Final RVOT Angiography to Assess for Proper Functioning of the Neoimplanted THV, Confirming Procedural Success
References
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Associated Data
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Supplementary Materials
Final Pulmonary Trunk Angiography to Assess for Absence of Intravalvular Regurgitation and Paravalvular Leak
Final RVOT Angiography to Assess for Proper Functioning of the Neoimplanted THV, Confirming Procedural Success