- Case report
- Open access
- Published:
Percutaneous pulmonary valve implantation in a patient with congenitally corrected transposition of the great arteries: a case report
Journal of Medical Case Reports volume 18, Article number: 70 (2024)
Abstract
Background
Percutaneous pulmonary valve implantation has become an attractive method of dysfunctional right ventricle outflow tract treatment.
Case presentation
We describe a unique case of a 20-year-old Caucasian male patient with a complex cyanotic heart defect, namely pulmonary atresia, with congenitally corrected transposition of the great arteries and ventricular septal defect after Rastelli-like surgery at the age of 5 years with homograft use. At the age of 20 years, the patient needed percutaneous pulmonary valve implantation owing to homograft dysfunction. Despite unusual course of the coronary arteries, balloon testing in the landing zone of the right ventricle outflow tract excluded potential coronary artery compression. Then, after presentation, a Melody valve was implanted successfully in the pulmonary valve position. The 8-year follow-up was uneventful.
Conclusion
This is likely the first description of a percutaneous pulmonary valve implantation in such anatomy. Such a procedure is feasible; however, it requires exceptional caution owing to the anomalous coronary arteries course, which can be the reason for their compression.
Background
Percutaneous pulmonary valve implantation (PPVI) has become an attractive method of dysfunctional right ventricle outflow tract (RVOT) treatment [1]. We describe a unique case of a patient with a complex cyanotic heart defect, namely pulmonary atresia (PA), with congenitally corrected transposition of the great arteries (ccTGA) and ventricular septal defect (VSD) in whom PPVI was performed. Such a case has not been reported thus far.
Case presentation
A 20-year-old Caucasian male patient with complex cyanotic heart defects, namely, ccTGA, PA, and VSD, underwent left-sided Blalock–Taussig–Thomas shunt palliation at the second day of life, followed by a correction at the age of 5 years. The surgery included ventricular septal defect (VSD) patch closure and subpulmonary outflow tract reconstruction with a 17-mm homograft (Rastelli-type procedure). He was readmitted at 20 years old, with no symptoms in anamnesis and with good exercise tolerance. A loud systolic murmur with a thrill and soft diastolic murmur were found upon physical examination. Echocardiography revealed good function of the systemic ventricle—morphologic right ventricle (mRV) and homograft obstruction with a maximum and mean gradient of 78 mmHg and 45 mmHg, respectively, as well as its' moderate regurgitation. The peak-to-peak gradient of 82 mmHg through the narrowed-to-10-mm homograft was measured in the diagnostic catheterization and the patient qualified for PPVI.
Nonselective coronarography was performed to exclude the possibility of coronary artery (CA) compression assessed by aortography with a 20-mm Mullins-X balloon placed in the potential valve landing zone (Fig. 1A). The test revealed that the right coronary artery (RCA) originated from posterolateral sinus and the left anterior descending artery (LAD), with the circumflex artery (Cx), originated from medial sinus separately. Despite the short distance between the Cx origin and the homograft valve annulus, coronary artery compression was not an issue.
The 35-mm AndraStent XXL (AndraMed GmbH, Reutlingen, Germany) was implanted into the homograft on a 20-mm balloon-in-balloon (BIB). Then, an 18-mm Melody valve (Medtronic Inc., MN) on an 20-mm BIB was expanded (Fig. 1B). Because of the residual peak-to-peak gradient of 30 mmHg, the 22 mm Mullins-X balloon was used for postdilatation, and the final gradient dropped to 15 mmHg (see Additional file 1: Video S1, S2; Additional file 2: Video S3, S4; Additional file 3: Video S5). In the 8-year follow-up, the patient remained asymptomatic with a mean residual gradient through the Melody valve of 22 mmHg on checkup echocardiography. Ejection fraction of the systemic ventricle was assessed to be 39% by magnetic resonance imaging (Fig. 1C). Single and insignificant fractures of the Melody valve stent (Cheatham-Platinum stent) are observed on fluoroscopy (Fig. 1D). No late complications, such as infective endocarditis, have been observed.
Discussion and conclusion
Congenitally corrected TGA with VSD and PA can be surgically treated by closing the VSD and placing mLV-to-pulmonary artery conduit in some cases [2]. Every implanted homograft carries the risk of future dysfunction, mostly due to its calcification and stenosis. After the Rastelli procedure, 51% of patients had reoperations owing to stenosis and insufficiency of the extracardiac conduit [3]. In patients with RV to pulmonary artery conduit stenosis, transcatheter valve implantation in the pulmonary position can be performed effectively [4]. A case series of a PPVI in two patients with ccTGA after Senning surgery was reported [5].
During PPVI, special attention should be paid to the risk of compression of the coronary arteries (CA). Morray et al. reported coronary artery compression in 4.7% of patients observed on preimplant testing [6]. Examined patients had various heart defects, and the majority (71%) of coronary artery compressions were noted in patients with an abnormal course [6]. Haas et al. reported abnormal coronary arteries in 32.8% of patients with dysfunctional right ventricle outflow tract [7].
Exceptional attention should be paid in cases of PPVI in patients with ccTGA, as CA origins are malpositioned, which is directly associated with aortopulmonary rotation [8]. PV is often localized between the ascending aorta and right atrium; therefore, CA may course closely to the potential valve implantation landing zone.
Stent fractures are a common adverse effect after Melody PPVI and occur in 25% of patients [9]. Stent fractures that do not affect its structure are not significant complications; however, the unusual position of the RVOT in patients with ccTGA might result in more frequent occurrence of stent fractures. Despite those precautions, given the correct anatomical foundation, PPVI in patients with ccTGA can be safely performed.
PPVI can be safely performed in patients with ccTGA after Rastelli-like repair. However, it requires exceptional caution due to the anomalous CA course, which can be reason for CA compression.
Availability of data and materials
Available on demand.
Abbreviations
- PPVI:
-
Percutaneous pulmonary valve implantation
- RVOT:
-
Right ventricle outflow tract
- PA:
-
Pulmonary artery
- ccTGA:
-
Congenitally corrected transposition of great arteries
- VSD:
-
Ventricular septal defect
- CA:
-
Coronary artery
- RCA:
-
Right coronary artery
- LAD:
-
Left anterior descending artery
- Cx:
-
Circumflex artery
References
Giugno L, Faccini A, Carminati M. Percutaneous pulmonary valve implantation. Korean Circ J. 2020;50(4):302–16. https://doi.org/10.4070/kcj.2019.0291.
Ungerleider R, Meliones JN, McMillan KN, Cooper DS, Jacobs JP. Critical heart disease in infants and children. Amsterdam: Elsevier; 2018.
Hörer J, Schreiber C, Dworak E, Cleuziou J, Zsolt P, et al. Long-term results after the Rastelli repair for transposition of the great arteries. Ann Thorac Surg. 2007;83(6):2169–75. https://doi.org/10.1016/j.athoracsur.2007.01.061.
Fiszer R, Dryzek P, Szkutnik M, Goreczny S, Krawczuk A, et al. Immediate and long-term outcomes of percutaneous transcatheter pulmonary valve implantation. Cardiol J. 2017;24(6):604–11. https://doi.org/10.5603/CJ.a2017.0023.
Rios R, Foerster SR, Gudausky TM. Melody pulmonary valve implantation in two teenage patients with congenitally corrected transposition of the great arteries status after Senning atrial switch operation. Cardiol Young. 2017;27(3):600–4. https://doi.org/10.1017/S104795111600158X.
Morray BH, McElhinney DB, Cheatham JP, Zahn EM, Berman DP, et al. Risk of coronary artery compression among patients referred for transcatheter pulmonary valve implantation: a multicenter experience. Circ Cardiovasc Interv. 2013;6(5):535–42. https://doi.org/10.1161/CIRCINTERVENTIONS.113.000202.
Haas NA, Vcasna R, Laser KT, Blanz U, Herrmann FR, et al. The standing of percutaneous pulmonary valve implantation compared to surgery in a non-preselected cohort with dysfunctional right ventricular outflow tract—reasons for failure and contraindications. J Cardiol. 2019;74(3):217–22. https://doi.org/10.1016/j.jjcc.2019.03.021.
Huang SC, Chiu I-S, Lee M-L, Wu C-S, Chang C-I, et al. Coronary artery anatomy in anatomically corrected malposition of the great arteries and their surgical implications. Eur J Cardiothorac Surg. 2011;39(5):705–10. https://doi.org/10.1016/j.ejcts.2010.09.006.
McElhinney DB, Cheatham JP, Jones TK, Lock JE, Vincent JA, et al. Stent fracture, valve dysfunction, and right ventricular outflow tract reintervention after transcatheter pulmonary valve implantation: patient-related and procedural risk factors in the US Melody valve trial. Circ Cardiovasc Interv. 2011;4(6):602–14. https://doi.org/10.1161/CIRCINTERVENTIONS.111.965616.
Acknowledgements
None.
Funding
None.
Author information
Authors and Affiliations
Contributions
The authors confirm contribution to the paper as follows: study conception and design: MG and RF. Data collection: MKa, MG, and MKr. Analysis and interpretation of results: MKa, MG, MKr, and RF. Draft manuscript preparation: MKa, MG, and MKr. All authors reviewed the results and approved the final version of the manuscript.
Corresponding author
Ethics declarations
Ethics approval and consent to participate
Due to retrospective nature of this case report, ethics committee approval (local ethics committee declaration) was not required.
Consent for publication
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Competing interests
None.
Additional information
Publisher’s Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Supplementary Information
Additional file 1: Video
S1, S2. Ventriculography at the level of homograft.
Additional file 2: Video S3
, S4. Coronary artery testing.
Additional file 3: Video S5. Final arteriography after Melody valve implantation.
Rights and permissions
Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
About this article
Cite this article
Kapalka, M., Galeczka, M., Krawiec, M. et al. Percutaneous pulmonary valve implantation in a patient with congenitally corrected transposition of the great arteries: a case report. J Med Case Reports 18, 70 (2024). https://doi.org/10.1186/s13256-024-04383-9
Received:
Accepted:
Published:
DOI: https://doi.org/10.1186/s13256-024-04383-9