Abstract
A 19-year-old man with D-transposition of the great arteries and pulmonary stenosis underwent atrial switch operation and pulmonary valvotomy in infancy. He was lost to follow-up and presented with multiple respiratory infections. An echocardiogram was inconclusive due to poor windows. Cardiac magnetic resonance (CMR) was performed using ferumoxytol 3-dimensional whole heart and 4-dimensional flow pulse sequences, which demonstrated this patient’s complex anatomy and physiology. CMR revealed severe pulmonary venous baffle obstruction. A 3-dimensional printed model was created from the CMR and provided key information for planning the complex catheterization approach required to stent the pulmonary venous baffle.
Key Words: 3-dimensional imaging, cardiac magnetic resonance, congenital heart defect, imaging, MR sequences, transposition of the great arteries
Graphical Abstract
Case Summary
A 19-year-old man with D-transposition of the great arteries and pulmonary stenosis presented to clinic to establish care after being lost to follow-up for 11 years. He had undergone atrial switch operation and pulmonary valvotomy during infancy. In the last 2 years, he had several admissions for respiratory infections. His physical examination was largely unremarkable; however, his oxygen saturations were lower than 90%. Echocardiography was limited due to suboptimal windows. Cardiac magnetic resonance (CMR) was ordered to evaluate the pulmonary and systemic venous baffles and ventricular function.
Take-Home Messages
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3D and 4D flow CMR can efficiently characterize structural and functional abnormalities in patients with complex cardiac anatomy (eg, D-TGA after atrial switch operation).
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3D printed models from CMR datasets can improve preprocedural planning for complex interventions and decrease procedural time.
Three-dimensional (3D) imaging showed severe pulmonary venous baffle obstruction and confirmed a widely patent systemic venous baffle connecting to the subpulmonic left ventricle (Figures 1A and 1B, Videos 1 and 2). Four-dimensional imaging was then performed, demonstrating a high velocity jet of 2 m/s across the pulmonary venous baffle and decreased cardiac function (Figures 1C to 1F, Video 3). 3D rendering and a 3D printed model were constructed from the ferumoxytol 3D whole heart imaging to assist in finding the most optimal angle of approach with a cardiac catheterization intervention (Figures 1G and 1H, Video 4). With preprocedural planning, only one attempt was required to perform a trans-septal puncture across the inferior interatrial baffle. The pulmonary vein baffle was stented with a 10-mm × 17-mm Valeo stent (Becton Dickinson) and further dilated with a 12-mm Atlas balloon (Becton Dickinson). The trans-septal puncture was closed with a 4-mm Amplatzer Septal Occluder (Abbott) (Video 5). Afterward, there was no residual gradient by cardiac catheterization. At his 3-month follow up, he had significantly improved overall color, work of breathing, and activity level.
Figure 1.
Pulmonary Venous Baffle Obstruction in Repaired D-Transposition of the Great Arteries
Three-dimensional (3D) whole heart imaging in the axial (A) and coronal (B) planes demonstrates the discrete narrowing of the pulmonary venous baffle, circled in red. (C to F) Four-dimensional imaging in color shows turbulent flow across the pulmonary venous baffle with a peak velocity of 2 m/s. (G and H) A 3D rendered model allowed visualization of the baffle’s spatial orientation to the inferior limb of the systemic venous baffle, enabling an optimal angle of approach.
Atrial switch operations (Mustard/Senning) were the predominant surgical repair for D-transposition of the great arteries before the arterial switch repair and are commonly encountered in the adult congenital heart disease population. Known complications include atrial arrhythmias, systemic right ventricle dysfunction, obstruction of both pulmonary and systemic venous baffles, and leakage across the atrial baffle.1 Although echocardiography is the primary imaging modality, sonographic windows are often limited from prior operations and larger patient size. Current guidelines for adult congenital patients, particularly those with complex anatomy, include CMR to better evaluate the anatomy, function, volumetric measurements, and blood flow quantification.2 Conventional CMR approach includes obtaining sequential cine stacks and phase contrast imaging which can be time consuming and challenging when imaging thin structures (eg, atrial baffles). In our approach, 3D whole heart and 4-dimensional flow volumetric datasets enabled comprehensive off-line evaluation of the patient’s anatomy and physiology including systemic and pulmonary venous baffle stenosis, baffle leaks, and valvar and ventricular function in high resolution and high contrast. Furthermore, a 3D printed model derived from the 3D whole heart dataset was instrumental in planning the interventional cardiology case, reducing procedural time because the interventionalist did not require multiple transeptal puncture attempts.
Funding Support and Author Disclosures
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
Axial Plane of the 3-Dimensional Whole Heart
Coronal Plane of the 3-Dimensional Whole Heart
Four-Dimensional Imaging in Color
3-Dimensional Rendering of the Patient’s Heart, Highlighting the Pulmonary Venous Baffle Obstruction
Cardiac Catheterization With Stenting of the Pulmonary Venous Baffle
References
- 1.Stout K.K., Daniels C.J., Aboulhosn J.A., et al. 2018 AHA/ACC guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. 2019;73(12):1494–1563. doi: 10.1016/j.jacc.2018.08.1028. [DOI] [PubMed] [Google Scholar]
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Associated Data
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Supplementary Materials
Axial Plane of the 3-Dimensional Whole Heart
Coronal Plane of the 3-Dimensional Whole Heart
Four-Dimensional Imaging in Color
3-Dimensional Rendering of the Patient’s Heart, Highlighting the Pulmonary Venous Baffle Obstruction
Cardiac Catheterization With Stenting of the Pulmonary Venous Baffle