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The Rastelli procedure is a complex surgical technique used primarily to treat congenital heart defects, specifically those that involve a ventricular septal defect (VSD) and a constricted or obstructed left ventricular outflow tract. This procedure is particularly relevant for patients with double outlet right ventricle (DORV), where both major arteries arise from the right ventricle, leading to significant complications such as inadequate systemic perfusion and pulmonary overcirculation. The Rastelli procedure aims to create a functional connection between the left ventricle and the aorta while simultaneously closing the ventricular septal defect. During the surgery, the surgeon first accesses the heart through a median sternotomy and identifies the problematic structures. The ventricular septal defect is closed using a patch, which prevents mixing of oxygen-rich and oxygen-poor blood. Next, the surgeon constructs a conduit or a shunt, typically made from a synthetic material or a section of the patient's larger pulmonary arteries, to connect the left ventricle directly to the aorta. This conduit serves as the new outflow route for oxygenated blood, ensuring that it is directed to the body rather than back into the lungs. The Rastelli procedure is generally performed during infancy or early childhood when the heart's anatomy is more amenable to surgical intervention. Postoperative care is crucial, as patients may experience complications such as arrhythmias, conduit obstruction, or pulmonary hypertension. Long-term follow-up is essential to monitor the growth and function of the conduit. Success relies heavily on the surgeon's expertise, the timing of the intervention, and the overall condition of the patient. The procedure has evolved significantly over the years, with advancements in surgical techniques and materials leading to improved outcomes and survival rates. Many patients who undergo the Rastelli procedure can lead relatively normal lives with appropriate medical management. However, some may require additional surgeries or interventions later in life to address issues such as a failing conduit or other complications arising from their underlying heart condition. Comprehensive care involving a multidisciplinary team of pediatric cardiologists, surgeons, and other healthcare professionals is vital for achieving the best outcomes for these patients. Overall, the Rastelli procedure represents a significant advancement in the management of complex congenital heart defects, aiming to provide better quality of life and enhanced survival for affected individuals. As with any surgical intervention, careful patient selection, thorough preoperative evaluation, and meticulous surgical technique are paramount to ensure successful outcomes and minimize the risk of complications associated with this intricate procedure. Through continuous research and refinement of surgical methodologies, the Rastelli procedure has established itself as a cornerstone in the surgical treatment of select congenital cardiac anomalies.