Dextrocardia converted to levocardia by right atrial enlargement and arterial reanastomosis.
Central Message.
An apical flipping operation is achievable by right atrial enlargement and arterial reanastomosis and could facilitate subsequent biventricular repair in the complex case.
The occurrence of truncus arteriosus in association with either dextrocardia1 or double aortic arch is unusual.2,3 We describe a successful “apical flipping procedure” to change dextrocardia to levocardia in a patient with complex truncus arteriosus, facilitating the Rastelli procedure.
Case
A 4-month-old girl, born at 38 weeks (3041 g), was prenatally diagnosed with truncus arteriosus and ventricular septal defects. With Apgar scores of 4 at 1 minute and 7 at 5 minutes, the patient presented with tachypnea and cyanosis. Computed tomography scans confirmed truncus arteriosus, a double aortic arch, and dextrocardia. The atrial situs was solitary, with the ventricular mass located on the right and the apex pointing to the right side (dextroversion). Palliative surgery was performed at a local hospital, which involved dividing the left aortic arch into the left common carotid artery and left subclavian artery (LSCA), creating a right-sided arch with an “aberrant” LSCA. The main pulmonary trunk was separated, and a modified Blalock–Taussig shunt was placed from the aberrant LSCA to the main pulmonary artery, forming a persistent vascular ring due to severe tracheomalacia.
We planned a staged repair with the first step to relieve the vascular ring and convert dextrocardia to levocardia. The “aberrant” LSCA was reimplanted to the left common carotid artery to relieve the vascular ring. The previously inserted 3.5-mm GORE-TEX modified Blalock–Taussig shunt was also replaced with a 4.0-mm shunt. To facilitate the future Rastelli conduit reconstruction, we rotated the heart to create levocardia by changing the relative sizes of the right and left atria (Figure 1). The right atrial wall was opened to the level of the inferior vena cava, and an autologous pericardial patch enlarged the right atrium. An atrial septostomy was performed to avoid left atrial distention. The ascending aorta was transected and reanastomosed after rotating the heart apex to the left. This procedure relieved the twisting effect. The pericardium on the left side was opened to allow the heart to remain in the left chest. After the reanastomosis of the ascending aorta and right atrial augmentations, the heart was rotated to the normal levocardia position (Video 1).
Figure 1.
Flipping the apex from right to left facilitates Rastelli conduit reconstruction and biventricular repair. Chest x-ray (A) and computed tomography (B) before and after surgery. RV, Right ventricle; LV, left ventricle; RA, right atrium; LA, left atrium.
At 6 months and weighing 8.3 kg, the patient underwent biventricular repair for ventricular septal defect and atrial septal defect closure, pulmonary artery plasty, and Rastelli conduit reconstruction. The patient had moderate truncal valve regurgitation; therefore, annular reduction was performed to decrease the neo-aortic regurgitation. A 3-cuspid valved conduit made with a 16-mm GORE-TEX tube graft and an expanded polytetrafluoroethylene membrane was used. The aorta was further elongated using a 16-mm GORE-TEX graft. The patient was weaned off bypass without complications and showed satisfactory recovery.
Discussion
We describe a new procedure to convert dextrocardia to levocardia in a complex truncus arteriosus case. Although there is no established surgical protocol for the apical switch operation, our previous clinical experience helps to generate this idea.
We had a case of an 11-year-old boy with a giant left atrial aneurysm leading to a rightward shift of the cardiac apex. After we performed left atrial aneurysmectomy, immediate restoration of the heart's typical position to the left side was noted.4 These observations suggest the relative size of the right atrium to the left atrium could change the apical position. On the basis of this observation, we performed the apical flipping operation by enlarging the right atrial lateral wall. Additionally, to prevent left atrial distension, an atrial septal defect was created.
Because the ventricular mass of the heart was fixed with the atria and great vessels, there will be some torsion of the aorta and main pulmonary artery after rotating the heart to the left side. In the first operation, the pulmonary artery trunk was separated from the common arterial trunk and supplied with a left Blalock–Taussig shunt, leaving only the aorta connected. Consequently, we proceeded to divide and reanastomose the aorta to establish the new connection without torsion. In addition, the left pulmonary venous entry site was left to the spine, and the descending aorta was on the right side, so the flipping of the ventricular mass did not compress the left pulmonary vein. This should be a point to consider while applying this technique to other cases.
During the Rastelli procedure, the aorta was elongated with graft interposition. We think the apical flipping procedure moves the ventricular mass far away from its original position. The aorta (and perhaps the main pulmonary artery) could be lengthened. This maneuver not only gives space to the airway and pulmonary arteries but also relieves the torsion by proper anastomosis of the graft.
Conclusions
Although this is an extremely rare case, we think this concept of an apical switch procedure could be applied to other complex biventricular reconstructions in special cases.
Webcast
You can watch a Webcast of this AATS meeting presentation by going to: https://www.aats.org/resources/conversion-from-dextrocardia-t-7341.
Conflict of Interest Statement
The authors reported no conflicts of interest.
The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest.
Footnotes
Institutional Review Board approval is waived for single case report.
Informed consent from the patient's parents was taken on March 27, 2024.
Supplementary Data
The surgical techniques for conversion from dextrocardia to levocardia are described. The technique included right atrial free wall patch augmentation and transection and anastomosis of the aorta. Video available at: https://www.jtcvs.org/article/S2666-2507(25)00066-5/fulltext.
References
- 1.Wang Y., Chen C., Xu Y. Coexistence of dextrocardia and persistent truncus arteriosus in an adult patient. QJM. 2024;117(2):140–141. doi: 10.1093/qjmed/hcad227. [DOI] [PubMed] [Google Scholar]
- 2.Huang S.C., Wang C.J., Su W.J., Chu J.J., Hwang M.S. The rare association of truncus arteriosus with a cervical double aortic arch presenting with left main bronchial compression. Cardiology. 2008;111(1):16–20. doi: 10.1159/000113421. [DOI] [PubMed] [Google Scholar]
- 3.Yıldırım S.V., Yıldırım A. Truncus arteriosus with double aortic arch: a rare association. Turk J Pediatr. 2017;59(2):221–223. doi: 10.24953/turkjped.2017.02.020. [DOI] [PubMed] [Google Scholar]
- 4.Lu W.Y., Huang S.C., Chen S.J., et al. Giant congenital left atrial aneurysm in an 11-year-old boy. Circulation. 2012;125(14):1818–1820. doi: 10.1161/CIRCULATIONAHA.111.057810. [DOI] [PubMed] [Google Scholar]
Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.
Supplementary Materials
The surgical techniques for conversion from dextrocardia to levocardia are described. The technique included right atrial free wall patch augmentation and transection and anastomosis of the aorta. Video available at: https://www.jtcvs.org/article/S2666-2507(25)00066-5/fulltext.