Notes

WDR47 protects neuronal microtubule minus comes to an end through katanin-mediated cutting.
It is effective to confirm postoperative lung perfusion scan and CT, perform a cardiac catheterization test approximately 6 months after surgery, and maintain the pulmonary vessels with catheter intervention if necessary. The ultimate goal of treating this disease is not only to improve cyanosis, but also to maintain pulmonary circulation at lower right ventricular pressure.Various pathologies of heterotaxy should be diagnosed by documenting the arrangement of the cardiac structures and other organs. They carry functional derangements of cardiovascular systems and abdominal organs, such as atrioventricular valve dysfunction, pulmonary vein obstruction, arrhythmia, and intestinal malrotation. An appropriately organized management of these malformations is pivotal for improving outcomes.Congenital anomaly of respiratory or digestive system have a significant impact on the perioperative management of congenital heart disease. Therefore, it is important to make a long-term treatment strategy. It has been reported that 1-stage surgery is effective for pulmonary artery sling. Simultaneous repair is also useful if significant airway stenosis is present prior to cardiac surgery. In the case of congenital heart disease associated with gastrointestinal anomaly, gastrointestinal surgery precedes in more than 80% of cases. In recent years, though treatments of gastrointestinal atresia have a good outcome, the results of esophageal atresia are still bad. If we choice multi-stage surgery, there is a possibility that we cannot perform cardiac surgery at appropriate time due to postoperative mediastinitis. Therefore, we must consider the timing and method of operation individually. The important thing is to keep a good relationships with related departments and to provide close informed consent to the patient's parents.A large population of patients with congenital heart disease with untreated systemic to pulmonary shunts( left to right shunts) will develop pulmonary arterial hypertension( PAH). There are 2 different statuses of an increase in pulmonary arterial pressure. One is high resistance due to high pulmonary blood flow (high flow with high resistance), another one is low pulmonary flow due to high resistance (low flow with high resistance). Chronic large left-to-right shunt induced severe pulmonary vascular disease and pulmonary hypertension. This was then subsequence of low pulmonary blood flow with high pulmonary vascular resistance. We have to avoid this situation and have to do intervention within the pulmonary vascular reactivity has been left. For this reason, preoperative treatment for avoidance of high flow, appropriate timing of interventions and postoperative various managements are important factors as aiming of low pulmonary resistance in this group. Recent advances in PAH-specific drugs have dramatically changed the therapeutic strategy for PAH. A strategy that includes "treatment" with PAH-specific drugs initially and then "repair" by closure of the cardiac defect (i.e. "treat and repair") was devised, and has been attempted, in patients with PAH associated with a cardiac defect.Mesenteric malperfusion is reported as a complication associated with acute aortic dissection(AAD) in 3~5% cases, and one of the adverse risk factors for survival. The mortality rate associated with malperfusion due to AAD is higher than that without malperfusion. To improve the clinical outcome, it is important to address the mesenteric malperfusion appropriately. Mesenteric malperfusion remains a diagnostic challenge. Abdominal pain is the most common symptom, but a nonspecific of acute mesenteric ischemia. Computed tomography(CT) including CT angiography is the gold standard in the diagnosis of aortic dissection and the mesenteric malperfusion. No single serum marker, including lactate, is reliable enough to diagnosis mesenteric ischemia. The optimal treatment for mesenteric malperfusion due to AAD is to restore blood flow to the ischemic area as early as possible, while minimizing the risk of thoracic aortic rupture. Those patients with malperfusion but no significant organ ischemia should be treated with immediate surgical repair. Those patients with malperfusion and significant organ ischemia and hemodynamically stable should be treated with mesenteric reperfusion, followed by surgical repair. The management of mesenteric malperfusion associated with AAD requires a tailored approach to improve outcomes. After successful restoration of mesenteric perfusion, patients should be monitored closely, and the bowel should be inspected when there is doubt regarding its viability.Arteritis is an inflammatory disease of the vessel walls, resulting in vascular damage and a wide variety of clinical symptoms and multisystem disorders. Because aneurysmal disease, coronary disease, and aortic insufficiency affect patient prognosis, surgical intervention plays an important role. Preoperatively, systemic vessels, cardiac function, and other major organs should be evaluated. Regarding the surgical technique, reinforcement of the anastomosis to the fragile aortic wall is important to prevent pseudoaneurysmal formation and prosthetic valvular detachment. As aortic root replacement, we have been applying the modified Bentall procedure with a "double fixation technique" and obtained desirable outcomes. Although endovascular repair for aneurysmal disease is one of the treatment options, its longterm efficacy remains uncertain. Postoperative control of inflammation with corticosteroids and/or immunosuppressive agents is also important for long-term management. Pseudoaneurysmal formation and prosthetic valvular detachment may occur progressively over a long period of time. To prevent these complications, strict follow-up with imaging and inflammation control should be performed.Cardiovascular surgery for renal failure patients with dialysis is challenging. According to the nationwide cardiovascular surgery database in Japan(Japan Cardiovascular Surgery Database;JCVSD), dialysis patients have occupied about 10% of whole surgery of coronary artery bypass grafting( CABG). In CABG, ratio of off-pump surgery did not change between non-dialysis (63%) and dialysis (64%) patients. Operative mortality of dialysis patients (7.8%) was 3 times higher than non-dialysis patients (2.1%). Tofacitinib cost In aortic valve replacement (AVR) dialysis patients occupied about 9% of whole AVR in Japan. In dialysis patients the percentage of bioprostheses was 65% and the choice of bioprostheses steeply increased when the age was over 70, which was similar to the non-dialysis patients. For dialysis before cardiovascular surgery, it is important not to dehydrate too much in order to maintain stable hemodynamics during the surgery. It is also important not to dehydrate too much after surgery in order to prevent non-occlusive mesenteric ischemia(NOMI).
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