Rastelli operation for double outlet right ventricle, pulmonary atresia with ductal dependent pulmonary flow

Double outlet right ventricle (DORV) with or without pulmonary atresia is a common indication for Rastelli operation. We very infrequently perform this surgery in our center. Here, we report a case of a ten-year-old girl who recently underwent Rastelli operation and patent ductus arteriosus (PDA) ligation for DORV, pulmonary atresia with ductal dependent pulmonary circulation by using custom-made valved conduit

A Case Report of Hepatocellular Carcinoma Derived from Rastelli Procedure-Related Congestive Liver Disease

[Background] The prognosis of congenital heart disease in children has improved, but late complications in adulthood are becoming an important problem. One late complication after congenital heart surgery is congestive liver disease, leading to liver cirrhosis and hepatocellular carcinoma (HCC). The Rastelli procedure is one of the surgical methods for transposition of the great arteries, and patients are thought have a low post-surgical risk of congestive hepatopathy by receiving re-intervention for right ventricular outflow tract obstruction.[Case presentation] We present the first case of HCC derived from Rastelli procedure-related congestive liver disease in a 41-year-old male. The patient underwent the Rastelli operation at 2 years of age and right ventricular outflow tract reconstruction at 10 and 35 years of age due to right ventricular outflow tract obstruction. At 41 years of age, a hepatic tumor was detected by computed tomography. Abdominal enhancing computed tomography revealed a partially hypervascular tumor in segment 2 in early phase and wash-out in late phase. The patient was diagnosed with HCC and underwent left lateral segmentectomy of the liver, splenectomy, and partial gastrectomy. The patient was discharged on the 28th postoperative day without postoperative complications. The resected tumor pathologically revealed moderately differentiated HCC and F3 liver fibrosis.[Conclusions] In the management of patients after the Rastelli operation, surveillance for congestive liver disease and HCC development is important, even if the patients have undergone right ventricular outflow tract reconstruction.

Double-outlet extension for narrowed retroaortic space

In cyanotic congenital heart disease, an enlarged aorta often compresses the retroaortic space, resulting in pulmonary artery narrowing. We experienced a case of a 10-year-old boy with severe pulmonary artery stenosis resulting from a narrowed retroaortic space after a Rastelli operation. The right pulmonary artery was closely aligned with the left coronary artery, which made pulmonary artery stenting difficult. We performed surgical repair including aortic extension with an interposition graft and elongation of the right ventricular outflow tract (double-outlet extension). This procedure was effective for enlargement of the retroaortic space and separation of the pulmonary artery from the coronary artery.

Rastelli Operation for D-Transposition of the Great Arteries, Ventricular Septal Defect, and Pulmonary Stenosis

Objectives: Our preferred approach for the surgical treatment of patients with D-transposition of the great arteries, ventricular septal defect, and pulmonary stenosis has been the Rastelli operation. We herein evaluate our 30-year experience with this procedure. Methods: Clinical records for patients who underwent the Rastelli operation between 1988 and 2017 at our institution were retrospectively reviewed. Primary outcomes included freedom from death or cardiac transplantation and freedom from conduit reintervention. Results: Forty-seven patients met inclusion criteria. Mean follow-up was 11.7 ± 6.8 years. Forty-three (91.5%) patients received a palliative systemic-to-pulmonary artery shunt and/or atrial septostomy prior to the Rastelli procedure. Five (10.6%) patients required ventricular septal defect enlargement at the time of the Rastelli procedure. The overall mean right ventricle-to-pulmonary artery conduit size was 17.0 mm. Mortalities included one early and three late deaths. Freedom from death or cardiac transplantation was 93% and 84% at 5 and 25 years, respectively. Seven patients required pacemaker placement, two immediately postoperatively and five late. Freedom from conduit replacement was 85% and 25% at 5 and 15 years, respectively. Seven (14.9%) patients required a second conduit intervention. Forty-one (87.2%) patients were New York Heart Association class I or II at the most recent follow-up. Conclusions: The Rastelli operation for D-transposition of the great arteries, ventricular septal defect, and pulmonary stenosis offers excellent mid- to long-term survival. The need for conduit replacement remains the most common indication for reintervention, and further study of the optimal choice of conduit will be useful.

Transposition complexes

Transposition complexes refer to hearts in which there is a reversal in the relationship between the ventricles and great arteries, i.e. there is ventriculoarterial discordance. Thus, the right ventricle gives rise to the aorta and supports the systemic circulation, whilst the left ventricle becomes the subpulmonary ventricle. There are two types of transposition: complete transposition of the great arteries (TGA) and congenitally corrected TGA. This chapter discusses complete TGA, including interarterial repair (Mustard or Senning operation), arterial switch operation, and Rastelli operation. It also covers congenitally corrected transposition of the great arteries (ccTGA), including atrioventricular (AV) and ventriculoarterial (VA) discordance.