Staged Senning-Rastelli Repair Succeeds in Boy with Corrected Transposition and Dual Valve Regurgitation

The case, published in the journal General Thoracic and Cardiovascular Surgery Cases, describes how the team first performed a palliative operation combining a systemic-to-pulmonary shunt with mitral valve repair, then waited for the child’s right ventricle to grow before carrying out the definitive Senning-Rastelli repair at four years and seven months of age.

The patient was a four-year-old boy whose heart condition was first suspected on fetal echocardiography, although no definitive abnormalities were identified during the perinatal period. He was delivered vaginally at 37 weeks and 6 days of gestation, and postnatal echocardiography revealed dextrocardia, congenitally corrected transposition of the great arteries, pulmonary valve stenosis, and a ventricular septal defect. Despite this combination of findings, he remained hemodynamically stable without signs of heart failure and was discharged home, with follow-up consisting of regular outpatient visits and periodic hospital evaluations.

The clinical picture changed gradually. At one month of age, the boy’s percutaneous oxygen saturation was 96 percent, but it progressively declined to 85 percent by two years of age, prompting consideration of surgical intervention. Echocardiography at that stage demonstrated mild-to-moderate tricuspid regurgitation as well as mild-to-moderate mitral regurgitation. Cardiac magnetic resonance imaging performed at 18 months showed a left ventricular end-diastolic volume of 23.9 milliliters, corresponding to 113 percent of normal, and a right ventricular end-diastolic volume of 29.4 milliliters, or 118 percent of normal.

Those measurements created a surgical dilemma. Although anatomic repair was considered preferable in the presence of pulmonary valve stenosis and tricuspid regurgitation, the right ventricular volume was judged insufficient for such an approach. The coexistence of mild-to-moderate mitral regurgitation was regarded as an additional risk factor. The team therefore selected an initial palliative strategy involving shunt placement and mitral valve repair, with the intention of improving oxygenation, promoting right ventricular growth, and performing definitive anatomic repair at a later stage.

The initial operation took place when the boy was two years and two months old. A four-millimeter graft was placed from the innominate artery to the right pulmonary artery. Under cardiopulmonary bypass, inspection of the mitral valve revealed an indentation between segments A1 and A2 with leaflet thickening, which was repaired by direct closure. Following surgery, oxygen saturation improved to approximately 90 percent. By three years of age, cardiac MRI demonstrated a left ventricular end-diastolic volume of 42.8 milliliters, 123 percent of normal, and a right ventricular end-diastolic volume of 55.3 milliliters, 138 percent of normal, confirming adequate growth of the anatomical right ventricle. Echocardiography showed trivial mitral regurgitation, and the patient was scheduled for anatomic repair as initially planned.

The definitive operation was performed at four years and seven months of age using an atrial switch, the Senning procedure, combined with a Rastelli operation. During the atrial switch, patches were utilized to augment the atrial septal and right atrial flaps. Because of inadequate extension of the main pulmonary artery root, a six-millimeter graft was interposed during a Damus-Kaye-Stansel anastomosis. The subaortic ventricular septal defect measured 14 millimeters, while the aortic annulus measured 22 millimeters, giving a defect-to-annulus diameter ratio of 0.64. Preoperative echocardiography and three-dimensional modeling suggested that the defect was restrictive for intraventricular rerouting, raising concern for systemic outflow obstruction after the rerouting, so the Damus-Kaye-Stansel anastomosis was added and the defect was closed using a 30-millimeter circular patch fashioned from a 14-millimeter graft to achieve intraventricular rerouting.

The postoperative course was uneventful. Echocardiography demonstrated minimal mitral and tricuspid regurgitation, and computed tomography confirmed the absence of outflow tract obstruction or caval pathway obstruction. The patient was discharged home on postoperative day 36 and continued to be followed in the outpatient clinic. At cardiac catheterization eight months after the operation, both mitral and tricuspid regurgitation remained trivial, and no stenosis of the Senning pathway was identified. The left ventricular outflow tract pressure gradient was approximately 15 millimeters of mercury, and the left ventricular end-diastolic pressure was 11 millimeters of mercury, indicating preserved ventricular function.

The authors place the case in the context of a sparse literature. Among published reports, only 12 patients with congenitally corrected transposition and mitral regurgitation were reported to have undergone anatomic repair, and only one patient underwent mitral valve intervention before definitive repair. The details of that case were not described, and it remains unclear whether the mitral regurgitation was congenital in origin or developed secondarily following palliative procedures such as pulmonary artery banding or a systemic-to-pulmonary shunt. The authors attribute this scarcity both to the rarity of congenital mitral regurgitation in patients with the condition and to the infrequent selection of anatomic repair in such patients. By contrast, 17 patients have been reported to undergo concomitant mitral valve intervention at the time of anatomic repair, and the authors suggest that in many of these patients the regurgitation was initially mild and subsequently progressed during left ventricular training with pulmonary artery banding, resulting in a combination of anatomical and functional mitral regurgitation.

The reasoning behind the staged approach is set out in the discussion. In the present case, the coexistence of mild-to-moderate tricuspid regurgitation and pulmonary valve stenosis favored anatomic repair consisting of an atrial switch combined with a Rastelli procedure. Although the long-term superiority of the atrial switch plus Rastelli procedure over physiological repair has been questioned, unlike that of the atrial and arterial switch procedure, previous studies have suggested that anatomic repair should be preferred when physiological repair would require ventricular septal defect closure with placement of a left ventricle-to-pulmonary artery conduit. There were concerns that congenital mitral regurgitation might compromise postoperative systemic left ventricular function, and intraventricular rerouting inevitably reduces the functional volume of the anatomical right ventricle, with a preoperative right ventricular volume of approximately 120 percent of normal having been considered necessary for successful anatomic repair. The presence of tricuspid regurgitation raised concerns regarding the long-term reserve of the systemic right ventricle if physiological repair were selected.

Because the morphological left ventricle would become the systemic ventricle after anatomic repair, the team considered it important to repair the mitral valve and confirm adequate mitral valve competence and preserved left ventricular function before committing the patient to definitive repair. Mitral valve repair was therefore performed at the initial palliative stage rather than concomitantly with anatomic repair. At the same time, progressive hypoxemia necessitated early surgical intervention, and a systemic-to-pulmonary shunt was placed to improve oxygenation and promote right ventricular growth through volume loading. This staged strategy allowed the team to confirm adequate control of mitral regurgitation and sufficient biventricular development before proceeding to the definitive operation. Intraoperatively, the mitral regurgitation was found to be caused by a congenital indentation between the A1 and A2 scallops of the anterior mitral leaflet and was successfully corrected by direct closure, allowing the staged anatomic repair to be completed as planned. The authors note that although long-term follow-up remains necessary, both biventricular function and atrioventricular valve function remain well preserved at the latest follow-up, and they conclude that serial follow-up of mitral valve function is warranted in such cases.

Subject of Research: A staged anatomic repair approach for congenitally corrected transposition of the great arteries with both mitral and tricuspid regurgitation

Article Title: Successful staged anatomic repair for congenitally corrected transposition with both atrioventricular valves’ regurgitation: a case report

Article References: Fuchigami, Y., Toda, K., Hirano, A., Hosoda, R., Iijima, Y., & Hoashi, T. (2026). Successful staged anatomic repair for congenitally corrected transposition with both atrioventricular valves’ regurgitation: a case report. General Thoracic and Cardiovascular Surgery Cases, 5(1), Article 43. https://doi.org/10.1186/s44215-026-00281-2

Image Credits: AI Generated

DOI: 10.1186/s44215-026-00281-2

Keywords: congenitally corrected transposition, Senning procedure, Rastelli operation, mitral regurgitation, tricuspid regurgitation, case report, cardiac MRI, pediatric cardiac surgery, systemic-to-pulmonary shunt, ventricular septal defect, dextrocardia, Damus-Kaye-Stansel