IntroductionTransposition of the great artery is one of the most common congenital heart anomalies . One of the surgical techniques to correct this anomaly is the Rastelli procedure . After surgical correction, right and left ventricular outflow obstruction are the most common late complications . In grown-up congenital disease (GUCH) late surgery is a high risk procedure and, repeated sternotomy may be associated with adverse injuries to mediastinal structures.The aim of this work is to demonstrate how a preoperative planning may be helpful in identifying patients at risk of re-entry.
Case presentationA 50-year-old woman, with primary diagnosis of transposition of the great arteries (TGA), ventricular septal defect (VSD) and pulmonary stenosis (PS) was referred to our Grown Up Congenital Heart (GUCH) center for progressive dyspnoea and reduced exercise tolerance. She underwent a Blalock-Taussig shunt palliation at the age of 3 and a Rastelli procedure after 2 years. Echocardiography revealed moderate-to-severe RV-PA conduit obstruction (122 mmHg) and a left Ventricle-Aorta (LV-Ao) tunnel obstruction (Mean gradient 32 mmHg, Max gradient 52 mmHg). Cardiac MRI and cardiac catheterization confirmed the echo findings and showed the proximity of the RV-PA conduit to the left anterior descending coronary artery, which was considered an exclusion criteria for trans-catheter therapy. A CT scan confirmed diagnosis and showed that mediastinal structures, in particular the aorta, were adherent to the sternum. (Fig 1). After careful examination of the clinical case, our Heart Team decided for high risk surgical treatment of the residual lesions.A percutaneous cannulation was performed, to minimize the re-entry injury risks. Two other percutaneous devices were used: the coronary sinus cannula and the percutaneous left heart venting catheter. The postoperative course was regular and one month after surgery a CT scan confirmed a good result of surgery (Fig 2).
ConclusionsThis peculiar case is emblematic to understand how a multidimensional perspective is mandatory in redo GUCH interventions. Hybrid surgery room setting and a multidisciplinary team of surgeons, interventional cardiologists, anesthesiologists and radiologists are essential to reach the goal of successful outcomes for these kinds of complex procedures as a GUCH “big operation”.