Because of the diversity of congenital heart disease and the wide spectrum of patient’s heart and lungs conditions, careful preoperative evaluation of the patients based on each heart disease and conditions is necessary. For example, it is important to distinguish the patient’s symptom coming from whether increased or reduced pulmonary blood flow, coming from whether right or left ventricular dysfunction. Also, the degree of the impairment of the patient’s physical status should be evaluated preoperatively using exercise tolerance test, echocardiography, and other biochemical and physiological exams. These are listed in two guidelines regarding congenital heart diseases from the Japanese circulation society [1, 2].

During anesthesia, careful management of the patients may be necessary based on their specific heart disease and conditions which are listed in the section of disease-specific considerations. It is advised to use the anesthetic techniques and anesthetic drugs whichever accustomed to or getting used to the anesthesiologists and their institution. Balanced anesthesia technique using midazolam or propofol, fentanyl or remifentanil, and sevoflurane or desflurane is popular in Japan.

Invasive cardiovascular monitoring during anesthesia such as direct arterial blood pressure, central venous or pulmonary artery catheter, and transesophageal echocardiography (TEE) may be required in association with the types of surgery and intervention. In patients at the second- or third-time operation, the preparation for the massive bleeding and ventricular arrhythmia is recommended.

After the surgical procedure, it is important to provide sufficient postoperative analgesia and cardiorespiratory care to the patients at the intensive care unit (ICU). Most of adult patients with congenital heart disease may be transferred to ICU with intubated and artificially ventilated condition. When hemodynamic and respiratory condition is stable, fast track management including rapid weaning from the ventilator and application of recovery protocol is recommended. Especially, in patients after right heart bypass surgery such as Fontan and Glenn procedure, early introduction of spontaneous respiration associated with negative intrathoracic pressure is favorable for hemodynamics. Dexmedetomidine may facilitate early recovery and prevent delirium.

The patients are divided following three groups. First group: the patients underwent complete correction of underlying congenital heart disease in their childhood and now suffering from some of the late complications in their adulthood. Second group: the patients underwent palliative procedure in their neonatal or infantile period and become adults. Third group: the patients grown up to be adults without performing any surgical interventions.

In the first group of patients, discussion will be started from patients received simple closure of either ventricular septum defect (VSD) or atrial septum defect (ASD). In these patients’ population, the anesthetic consideration is almost the same as the healthy patients. However, if the patients still have the residual shunt such as VSD and ASD, the risk for the perioperative infectious endocarditis (IE) is still present. Therefore, prophylactic antibiotics are mandatory even scheduled for the dental or minor surgery. The patients with atrioventricular septal defect (AVSD) underwent intracardiac correction in their childhood; residual atrioventricular valve regurgitation (AVVR) is often observed at postoperative period. The right side AVVR causes right ventricular (RV) and right atrium (RA) enlargement due to volume overload. In such patients, systemic venous congestion occurs resulting splanchnic organ dysfunction. Because intravenous anesthetics and muscle relaxants act longer than usual cases in these patients, postoperative respiratory depression is likely to be occurred. The left side AVVR causes left ventricular (LV) and left atrial (LA) enlargement that may lead pulmonary venous congestion. Therefore, attention should be paid intraoperative fluid overload and perioperative respiratory failure. Either pulmonary valve stenosis (PS) or regurgitation (PR) is often remained in the patients received the correction of tetralogy of Fallot (TOF) in their childhood. In some cases, pulmonary valve replacement is performed in their adulthood for the second-time surgery. Depending upon the patients’ cardiopulmonary status, there is a considerable increase in the perioperative risk for hypoxia and RV failure in these patients. In patients with transposition of great arteries (TGA) who received arterial switch operation, peripheral pulmonary stenosis is sometimes developing, especially in case of Lecompte procedure. In contrast, TGA patients received atrial switch operation such as Senning and Mustard procedure, obstruction of either systemic or pulmonary venous return may deteriorate liver function or respiratory function. Therefore, in these patients, careful perioperative respiratory care and precise assessment of liver function are needed. Also, coexisting sinus node dysfunction and other arrhythmia may be exacerbated during surgery.