Because of major advances in surgical and medical management, ever-increasing numbers of patients with congenital heart disease (CHD) reach adulthood. With few exceptions, however, reparative surgery is not radical, and is left its specific residua and sequelae that require life long surveillance. There are complexities such as heart failure, arrhythmias, sudden death, reoperation, cardiac intervention and ablation inherent in the comprehensive care. Adult congenital heart disease (ACHD) patients can have non-cardiac issues such as pregnancy and delivery and non-cardiac surgery, hepatitis, and also have psychosocial issues such as depression, cognitive abnormalities, insurance and so on, therefore, proper follow-up and management of them are mandatory.
 Regarding caregiver for adult with CHD, transition from pediatric cardiologists and cardiovascular surgeons to ACHD specialists and/or cardiologists those are well trained in the field of adults with CHD, is necessary. Provision of comprehensive care by multidisciplinary teams including adult and pediatric cardiologists and cardiovascular surgeons was the fundamental feature in care facilities for adults with CHD. Also anesthesiologists have a major role on taking care of ACHD patients during cardiac and non-cardiac surgery. Training and education should be focused on the adult CHD fellows who represent the next generation of cardiologists and cardiovascular surgeon that will assume responsibility for this patient population. In this paper, I tried to clarify the current situation in adult CHD services and discuss the future role of pediatric and adult cardiologists, cardiovascular surgeons and anesthesiologists for caring patients in this evolving field.

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[in Japanese]

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 Heparin-Induced Thrombocytopenia type II (HIT type II) is a life-threatening complication of heparin therapy. It is inhibited to use heparin on patients with HIT type II, however the antibodies that mediate HIT type II (antibodies against heparin-platelet factor 4 (PF4) complex) are said to turn negative over several months, and after that, administering heparin may not cause a recurrence of HIT type II.
 Therefore if a patient with a history of HIT type II that requires cardiopulmonary bypass surgery, it is recommended that we postpone the operation until its antibodies against PF4 complex disappear. After that, we can use heparin only for cardiopulmonary bypass, but we should use other kinds of anticoagulant such as antithrombin for others during operation. In this case, we can succeed in the management of a patient with a history of HIT type II requiring cardiac surgery with cardiopulmonary bypass according to this recommendation.
 However, in such cases, patients always have a possibility to develope a recurrence of HIT type II. In this case we have to avoided administering heparin for the maximum extent. Furthermore, we need to consider what kind of anticoagulant we should use during perioperative period.

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 Gender-based differences in outcomes after coronary artery bypass grafting (CABG) have been reported. In this retrospective study, we collected data from 92 patients (66 males, 26 females) who underwent CABG between July 2009 and December 2012 in a single hospital. Four of these patients died in-hospital due to infectious disease (2 males) and cardiac events (2 females). Both females were aged 75 or over; therefore, perioperative management should be cautiously performed in elderly female patients.

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 Nifekalant is a class III antiarrhythmic agent that effectively suppresses ventricular tachyarrhythmia without compromising hemodynamics. We report a case in which nifekalant was effective against refractory ventricular tachycardia (VT) during weaning from cardiopulmonary bypass (CPB). The patient was a 53-year-old man with acute myocardial ischemia and severe mitral regurgitation. He suffered from refractory VT that was resistant to amiodarone and direct current (DC) shocks. Coronary artery bypass graft and mitral annuloplasty were scheduled under general anesthesia. Before induction of CPB, sinus rhythm was maintained by a continuous infusion of amiodarone (45 mg•h⁻¹). The cardiac procedures performed were uneventful, but refractory VT developed during weaning from CPB. VT did not respond to the continuous infusion of amiodarone and frequent DC shocks. As a last resort, a bolus of intravenous nifekalant (1 mg•kg⁻¹) was administered, and his sinus rhythm eventually returned to 40 beat•min⁻¹ after temporary cardiac arrest for approximately 20 s. By applying atrial pacing at 90 beat•min⁻¹, his hemodynamics stabilized, and he was weaned from CPB smoothly. Nifekalant was continuously infused at a dose of 0.4 mg•kg⁻¹•h⁻¹, and VT did not recur. In the intensive care unit, the continuous infusion of nifekalant was gradually decreased, and amiodarone was administered orally.
 Nifekalant was shown to be effective for refractory VT resistant to amiodarone and cardioversion during weaning from CPB in cardiac surgery.

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 We experienced anesthetic management of a case of complete situs inversus (CSI). A seventy-seven year old man who had CSI in his past history underwent emergency coronary artery bypass grafting surgery (CABG) under general anesthesia. For intraoperative anesthetic management, transesophageal echocardiography (TEE) was carried out by exactly just the opposite. We scanned the typical 4 chamber view on position of 180° and typical long axis view is scanned on position of 55°. Moreover, to eliminate any discomfort by reversible view caused by CSI, intraoperative manipulation of TEE should be confirmed thorough the mirror that was attached to the other side of the TEE monitor.
 We concluded that if the intraoperative scanning plane of TEE was performed on the CSI patient, 180° of scanning position must be considered as 0° to aim the basic findings. Moreover, to eliminate discomfort by reversible view caused by CSI, manipulation of TEE should be confirmed thorough the mirror that was attached to the other side of the TEE monitor. However, to avoid the oversight of other cardiac anomalies caused by congenital incomplete situs inversus, we must assess malformed abdominal abnormality if dextrocardia was detected in the patient's chest X-ray.

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 Kommerell's diverticulum is a rare disease in which an aneurysm is formed at the origin of the subclavian artery. In a period of six years, 10 patients who had developed Kommerell's diverticulum underwent thoracic aorta replacement in our hospital. In one case, we experienced difficulties with inserting the double lumen tube and transesophageal echocardiography probe due to compression by the aorta.
 Patients with Kommerell's diverticulum often have an abnormal course of the aorta and aberrant branches of the aortic arch. Thus their brain circulation is different from patients with normal anatomy. Sometimes the aorta can compress the trachea or esophagus, and symptoms such as dysphagia, dyspnea, or swallowing disorder appear.
 In the administration of such patients, it is necessary to monitor atrial blood pressure for circulatory changes during and after surgical procedures. Special attention should be paid to prevent compression of the trachea and esophagus by aneurysm or abnormal course of aorta. Therefore, it is important to evaluate anatomical features including radiological findings before operation.

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 A 64 year-old male underwent thoracic endovascular aortic repair against dissecting aortic aneurysm Stanford type B. Just after completing the procedure, acute bradycardia and hypotension occurred, and was diagnosed due to acute aortic dissection Stanford type A. About 30 min later, Bispectral index (BIS) had suddenly decreased following temporary elevation. Aortic angiography revealed dissection of the right brachiocephalic artery and left common carotid artery. Just after finishing repair of the cervical arteries and right internal carotid artery, left common carotid artery bypass, ascending aorta and total arch replacement were performed. Ten hours later, he was extubated in the intensive care unit. His Glasgow Coma Scale was 14 points and he had right hemiplegia. Head CT scan revealed that he had a hemorrhagic cerebral infarction in the left basal ganglia. This case suggested that BIS monitoring was useful to detect a complication of central nervous system during general anesthesia.

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 A 72-year-old female patient who had received dialysis for chronic renal failure was immediately admitted to our hospital because of ileus. Because of the obstruction of her shunt, a nephrologist performed reconstruction under local anesthesia in the morning, without changing the operation mode for ICD. Then, the patient showed worsening of ileus. Thus, laparotomy was planned on the same day. At this time, the surgeon noticed her ICD, contacted the manufacturer, and stopped its antitachycardia function. At the same time, the internal memory was examined. This revealed 3 episodes involving the initiation of charging by electro-magnetic interference in the morning operation and, among those, one episode involved the concurrent activation of antitachycardia pacing. This case fortunately did not lead to the shock therapy owing to a shorter duration of application of the electrocautery. However, depending on its previous charging conditions, shock may have occurred even with such short-term application. So, in case of using monopolar electrocautery during the operation, the antitachycardia function of the ICD must be stopped, even though it is a minor surgery.

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 We encountered a rare complication of a stuck leaflet in the closed position diagnosed by intraoperative transesophageal echocardiography (TEE) after sternal closure during a double valve replacement procedure using a St. Jude Medical (SJM) mechanical mitral valve. A woman in her 70s underwent a double valve replacement for severe aortic stenosis and severe mitral regurgitation. A 16-mm ATS Medical mechanical valve was used to replace the native aortic valve in the supra-annular position, and a 25-mm SJM mechanical valve was used to replace the native mitral valve in the anti-anatomic position. After completing the double valve replacement, the patient was weaned from cardiopulmonary bypass under inotropic support without difficulty, and her hemodynamic condition was stable. The TEE at this point did not show perivalvular leakage, transvalvular flow acceleration or a stuck valve. However, the TEE after sternal closure revealed transmitral flow acceleration and a stuck anterolateral mitral leaflet in the closed position. The stuck leaflet was also confirmed by X-ray fluoroscopic examination. A decision was made to reopen the heart but TEE showed resumption of leaflet movement once a pericardial suture was released. The valve function was good, and the patient was subsequently discharged after an uneventful postoperative recovery. This case emphasizes the importance of performing routine intraoperative TEE after sternal closure in valve replacement procedures.

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 Objective: Despite recent advances in surgical technique, thoracic aortic surgery remains a highly invasive procedure and there is limited information of postoperative cognitive dysfunction (POCD). The present study undertook to identify risk factors for POCD after ascending aorta or aortic arch replacement using selective cerebral perfusion (SCP).
 Methods: Data were collected on 110 consecutive patients who underwent elective ascending aorta/aortic arch replacement using SCP between January 1998 and June 2012. All patients had magnetic resonance imaging, angiography and carotid ultrasound before surgery. Four cognitive tests were performed preoperatively and 1 week postoperatively. POCD was defined as a decrease in an individual's performance in more than two tests of at least 20% from baseline. Comparisons between the two groups were made with Student's t test and the χ² or Fisher's exact test. To assess the predictors of POCD, stepwise logistic regression analysis was performed.
 Results: The incidence of POCD was 39% (43/110). POCD patients had significantly higher rates of peripheral vascular disease (28 vs. 12%), coronary artery disease (63 vs. 28%), Plaque Score (PS)>10.0 (33 vs. 12%), carotid stenosis>50% (23 vs.9%), and prolonged surgery time (493±135 vs. 418±89 min). Stepwise logistic regression analysis demonstrated that the independent predictors of POCD were coronary artery disease [odds ratio (OR) 3.3], surgery time>456 min (OR 2.1), and PS >10.0 (OR 1.7).
 Conclusions. Coronary artery disease, surgery time, and carotid atherosclerosis are significant independent predictors of POCD following thoracic aortic surgery that employs SCP. Preoperative evaluation with carotid ultrasound allows patient subgroups at risk to be identified in time to implement strategies aimed at reducing POCD.

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