Central Message.
We present an innovative 3D visualization strategy for preoperative planning in patients with tetralogy of Fallot pulmonary atresia MAPCA.
Treatment of tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries (MAPCA) remains challenging.1,2 Surgical repair involves unifocalization and arterioplasty of collaterals and frequently diminutive pulmonary arteries (PAs).2 These vessels are frequently torturous and complex, limiting the utility of traditional imaging modalities (eg, echocardiography and angiography).3 We present images from a novel visualization strategy to enhance preoperative understanding of the anatomy.
Three patients at the Children's Hospital of Philadelphia underwent unifocalization and tetralogy repair from December 2018 to February 2019. Per institutional protocol, all patients underwent cardiac computed tomography angiography in the neonatal period, with subsequent echocardiograms and cardiac catheterization for presurgical planning (Figure 1). Before surgery, a digital 3-dimensional (3D) reconstruction of the thoracic anatomy was created with the use of the computed tomography angiography images (Materialise Mimics and 3-Matics Design, Leuven, Belgium).
Patient 1 had an absence of confluent PAs, 5 MAPCAs with distinct aortic origins (Figure 2), a right aortic arch with an aberrant origin of the left subclavian artery, and no evidence of a patent ductus arteriosus. Patient 2 had confluent branch PAs, 4 MAPCAs arising from the proximal descending aorta, and a left aortic arch (Figure 3). Patient 3 had hypoplastic confluent branch PAs, 4 MAPCAs with 3 distinct origins from the proximal descending aorta, and a left aortic arch (Figure 4).
The digital reconstructions subjectively increased comprehension of the spatial relationship of the collateral vessels to the native PAs, airway, and pulmonary veins for the surgical team. Presented in a 3D PDF format, the file's interactive nature allowed the user to virtually “turn on and off” any of the surrounding structures (Video 1). Intraoperatively, the surgeon confirmed the fidelity of the reconstructions. This 3D visualization strategy was quickly adopted by the cardiothoracic surgical group and is now part of routine presurgical planning for this patient population.
Footnotes
Dr O'Byrne receives support from the National Institute of Health/National Heart Lung and Blood Institute (HL130420-01). Dr Ghosh is supported by the National Institutes of Health Institutional Training Grant (T32GM008562). The funding agencies had no role in the planning or execution of the study, nor did they edit the manuscript. The manuscript represents the opinions of the authors alone.
Disclosures: Authors have nothing to disclose with regard to commercial support.
Supplementary Data
References
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