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Hypoplastic left heart syndrome (HLHS) is defined as the incomplete development of the left heart structures, including the mitral valve, left ventricle, aortic valve, and aortic arch. Delays in diagnosis and delay in intervention will increase the morbidity of neonates with HLHS. To understand the mechanism of HLHS along with the principles of anesthesia in neuroanesthetics procedures in pediatrics in order to obtain a good outcome. A 9day old baby girl, weight 2522 grams with HLHS (aortic atresia) with patent ductus arteriosus (PDA), type II atrial septal defect (ASD) with a left to right shunt, and dandy walker syndrome. The patient's head was enlarged from birth, no heart sounds were found, regular I-II heart sounds. The anesthetic approach in HLHS is to maintain preoperative hemodynamic stability by maintaining heart rate, preload, and PGE1, balancing systemic vascular resistance and pulmonary vascular resistance, preventing too high PaO2, and administering inotropic agents to increase cardiac output and keep the patent ductus arteriosus open. The balance of systemic and pulmonary blood flow is a key principle in the management of HLHS anesthesia. The main goal of HLHS anesthesia is to minimize hemodynamic changes to prevent compromised hemodynamics in both circulations and maintaining stability is essential in preventing morbidity, complications, and increasing good outcomes in surgery.

Anesthesia Management in VP Shunt Surgery in Neonates with Hypoplastic Left Heart Syndrome (HLHS) and Dandy Walker Syndrome