Background/Aims: Patients with achalasia-related esophageal motility disorders (AEMDs) frequently present with dilated and sigmoid esophagus, anddevelop esophageal diverticulum (ED), although the prevalence and patients characteristics require further elucidation. Methods: We conducted a multicenter cohort study of 3707 patients with AEMDs from 14 facilities in Japan. Esophagography on 3682 patients were analyzed. Results: Straight (n = 2798), sigmoid (n = 684), and advanced sigmoid esophagus (n = 200) were diagnosed. Multivariate analysis revealed that long disease duration, advanced age, obesity, and type I achalasia correlate positively, whereas severe symptoms and integrated relaxation pressure correlate negatively with development of sigmoid esophagus. In contrast, Grade II dilation (3.5-6.0 cm) was the most common (52.9%), while grade III dilation (≥ 6 cm) was rare (5.0%). We found early onset, male, obesity, and type I achalasia correlated positively, while advanced age correlated negatively with esophageal dilation. Dilated and sigmoid esophagus were found mostly in types I and II achalasia, but typically not found in spastic disorders. The prevalence of ED was low (n = 63, 1.7%), and non-dilated esophagus and advanced age correlated with ED development. Patients with right-sided ED (n = 35) had a long disease duration (P = 0.005) with low integrated relaxation pressure values (P = 0.008) compared with patients with left-sided ED (n = 22). Patients with multiple EDs (n = 6) had lower symptom severity than patients with a single ED (P = 0.022). Conclusions The etiologies of dilated esophagus, sigmoid esophagus, and ED are considered multifactorial and different. Early diagnosis and optimal treatment of AEMDs are necessary to prevent these conditions.

Background/Aims: ManoScan and Sandhill high-resolution manometry (HRM) systems are used worldwide; however, the diagnosis of achalasia on the Starlet HRM system is not fully characterized. Furthermore, the impact of calcium channel blockers and nitrites in treating achalasia has not been investigated using HRM. Management of recurrent cases is a priority issue, although few studies have examined patient characteristics. Methods: We conducted a multicenter, large-scale database analysis. First, the diagnosis of treatment-naive achalasia in each HRM system was investigated. Next, patient characteristics were compared between type I-III achalasia, and the impact of patient characteristics, including calcium channel blocker and nitrite use for integrated relaxation pressure (IRP) values, were analyzed. Finally, patient characteristics with recurrent achalasia were elucidated. Results: The frequency of type I achalasia with Starlet was significantly higher than that with ManoScan and Sandhill HRM systems. In achalasia, multivariate analysis identified male sex, advanced age, long disease duration, obesity, type I achalasia, and sigmoid type as risk factors related to normal IRP values (< 26 mmHg). Calcium channel blockers and nitrites use had no significant impact on the IRP values, although achalasia symptoms were indicated to be alleviated. In recurrent cases, the IRP value was significantly lower, and advanced age, long disease duration, and sigmoid type were more common than in treatment-naive patients. Conclusions We should cautiously interpret the type of achalasia and IRP values in the Starlet HRM system. Symptoms of recurrent cases are related to disease progression rather than IRP values, which should be considered in decision making.

Background/Aims: No screening test for esophageal motility disorder (EMD) has been established, the objective of this study is to examine the potential usefulness of our newly developed “Onigiri esophagography” combined with an obstruction level (OL) classification system in screening for EMD. Methods: A total of 102 patients with suspected EMDs who underwent both high-resolution manometry (HRM) and Onigiri esophagography between April 2017 and January 2019 were examined. The EMD diagnosis was performed based on the Chicago classification version 3.0 by HRM. Onigiri esophagography was performed using a liquid medium (barium sulfate) followed by a solid medium, which consisted of an Onigiri (a Japanese rice ball) with barium powder. The extent of medium obstruction was assessed by the OL classification, which was defined in a stepwise fashion from OL0 (no obstruction) to OL4 (severe obstruction). Results: The patients with OL0 (32.3%), OL1 (50.0%), OL2 (88.0%), OL3 (100.0%), and OL4 (100.0%) were diagnosed EMDs by HRM. The area under the curve, as determined by a receiver operating characteristic analysis, for the OL classification was 0.86. Using the cutoff value of OL1, the sensitivity and specificity were 87.3% and 61.3%, respectively, while using a cutoff value of OL2, the sensitivity and specificity were 73.2% and 90.3%, respectively. Conclusion In conclusion, Onigiri esophagography combined with the OL classification system can be used as a screening test for EMDs with a cutoff value of OL1.

A 58-year-old woman admitted for further examination of the proximal descending thoracic aortic aneurysm (TAA) combined with atypical coarctation and aortic regurgitation (AR). The chest CT and aortography confirmed these diagnoses, and revealed a pressure gradient of 40mmHg at the descending thoracic aorta with a severe calcification of the aortic wall, and severe AR. We scheduled a one-stage operation for this patient. First, we performed aortic valve replacement. Then we made a graft replacement from the aortic arch to the descending aorta using a stent graft via the aortic arch. Finally we did a reconstruction for lower limb perfusion using an aorto-iliac (extra-anatomical) bypass. The patient is now doing well 3 years after the surgery without any endoleak and without any difference of blood pressure between upper and lower limbs.

An 18-year-old man underwent a Ross operation for the treatment of prosthetic aortic valve endocarditis with extensive perivalvular tissue destruction. Postoperatively, he developed poststernotomy methicillin-resistant Staphylococcus aureus mediastinitis, which was treated with one-staged irrigation, debridement and omental transfer. After 3 years of follow-up, he is doing well without any sign of infection or a graft failure.

Endovascular grafting via the aortic arch, a novel alternative method for aortic aneurysm repair, was performed in 18 patients with aortic arch or distal arch aneurysms. For cerebral protection, selective or retrograde cerebral perfusion was used during delivery and deployment of the stented graft through the aortotomy. Selective cerebral perfusion was performed through both cerebral arteries and the left subclavian artery. Throughout this procedure, the aorta was filled with carbon dioxide to prevent the spinal arteries from air embolism. Two patients were lost, one due to myocardial infarction and one due to pneumonia. Endoluminal leakage was found in 2 patients, for which reoperation was required. However, no cerebral or spinal complications were observed in this series. Thus we conclude that endovascular stent grafting via the aortic arch is an acceptable alternative for the aortic arch or distal arch aneurysm repair with little risk of cerebral or spinal complications.

A combined aortic and mitral valve replacement was performed in a 50-year-old man who had undergone living-related renal transplantation one year previously. The oral administration of tacrolimus was continued perioperatively while monitoring blood tacrolimus level. The postoperative administration of human atrial natriuretic peptide (hANP) was effective to maintain urine output was performed in addition to frosemide, mannitol, dopamin and prostaglandin E₁ infusions. He was discharged on the 37th postoperative day without rejection, infection or renal dysfunction. This is the first report in Japan describing successful combined aortic and mitral valve replacement after renal transplantation.

We performed a video-assisted thoracoscopic surgery (VATS) to clip the patent ductus arteriosus (PDA), which was 5mm in internal diameter, in an 11-year-old girl, who first underwent a coil embolization ending in failure. Under general anesthesia with one-lung ventilation in a right lateral decubitus position, four thoracostomies were made in the left hemithorax. The PDA was clipped by two titanium clips, the length of which is 11mm at closing. Transesophageal echocardiography confirmed the location of the PDA and the absence of a residual shunt. The patient showed neither left recurrent laryngeal nerve dysfunction nor hemorrhage after operation, and was discharged on the 9th postoperative day. The clipping of the PDA by VATS can be applied for PDA without calcification if the external diameter is up to 7mm. This technique was minimally invasive and reliable. It was excellent in terms of the high quality of life achieved by the patient.

The internal mammary artery (IMA) has been widely used in CABG due to the excellent long-term results. However, the extensive use of bilateral IMA grafting has been believed to increase operative morbidity and mortality. This study was designed to determine if bilateral IMA grafting in diabetic patients increased the likelihood of mediastinitis. We analyzed the data of 386 consecutive patients who underwent isolated CABG in 1992 to 1996. The definitions of sternal wound complications are as follows, (1) mediastinal dehiscence and (2) mediastinal wound infection. Subtypes include superficial wound infection and deep wound infection (mediastinitis). Among these patients 97 received unilateral IMA grafts and 289 did bilateral IMA grafts. mediastinitis did not occur in any subjects. The occurrence rate of mediastinal dehiscence and superficial wound infection was 7.2% (7/97) for bilateral IMA grafting, 7.3% (21/289) for unilateral IMA grafting. No patients died of wound complications. The occurrence rate of mediastinal dehiscence and superficial wound infections were 12.0% (4/33) for bilateral IMA grafting in diabetic patients, 12.0% (14/117) for unilateral IMA grafting in diabetic patients. That of this complications was 4.7% (3/64) for bilateral IMA grafting in non-diabetic patients, 4.1% (7/172) for unilateral IMA grafting in diabetic patients, without significant differences in wound complication. Bilateral IMA grafting in diabetic patients carried no great risk of mediastinitis, but diabetes mellitus itself was a great risk for mediastinitis.

Malignant hyperthermia (MH) and antithrombin III (AT III) deficiency are both rare, but once they occur, the patient's prognosis is very poor. A 67-year-old man was referred to our hospital with a diagnosis of unstable angina. A coronary angiography revealed stenosis of LMT and triple vessels. The patient was considered a candidate for CABG. He had been prescribed 50mg/day of dantrolene for frequent muscular convulsions of the lower extremities. He had had a high CK level for a few years. Therefore he was considered to be at high risk for malignant hyperthermia (MH). He underwent CABG (×4). Dantrolene was administered orally at a dose of 25mg and then 160mg intravenously before anesthesia and modified NLA was performed in order to avoid probable MH. During the operation, AT III deficiency was suspected because the reaction of ACT after heparinization was poor. AT III preparation (1, 500 units) was used and CABG under cardiopulmonary bypass was completed without any events. It was proved after the surgery that the AT III volume had been almost normal but its activity had decreased. His postoperative course was good. For possibly fatal MH and AT III deficiency, it is necessary and important to predict, prevent and diagnose as early as possible.