[Anesthetic management of palliative surgery for hypoplastic left heart syndrome--a case report]

The first patient with hypoplastic left heart syndrome who was treated successfully by palliative surgery at our hospital is reported. Soon after birth, the female infant showed tachypnea and cyanosis, and was transferred to our institution under a presumptive diagnosis of HLHS. Although cardiologists confirmed the diagnosis by two-dimensional echocardiography, the surgery was postponed for one month because it was possible to keep the ductus arteriosus open without PGE1, and the patient showed no deterioration. Fortunately, an abnormal vessel connecting the left atrium with the superior vena cava relieved severe pulmonary venous congestion by diverting the blood flow. During the pre-CPB period, frequent adjustment of the oxygen concentration and ventilator setting was required in order to keep the blood gas values optimal compared with the values before surgery. After CPB, adequate blood pressure using catecholamines and hyperventilation with 100% oxygen was necessary to increase the pulmonary blood flow and to decrease the pulmonary vascular resistance. It is concluded that preservation of the balance between PVR and SVR during the perioperative period, and adequate systemic arterial pressure after CPB are crucial. Furthermore, constant and intense observation is mandatory to facilitate immediate treatment even after surgery in case of systemic hypoperfusion due to excessive pulmonary blood flow.