We successfully performed one-stage definitive repair for 3 infants weighing 4.2, 6.1 and 5.2kg with complex coarctation without homologous blood transfusion. The priming volume of the bypass circuits was 195ml, and their lower hematocrit values during cardiopulmonary bypass were 15, 16 and 13%, respectively. In order to diminish the aortic cross clamp time, the aortic arch was repaired with the heart beating, using isolated cerebral and myocardial perfusion methods. The base excess in each patient decreased to -9.4, -8.0 and -4.9mEq/l during the rewarming phase, however, their postoperative hemodynamic and respiratory conditions were satisfactory. They have grown without any sequelae for at least 2 months.

Reconstruction of the right ventriclar outflow tract (RVOT) in congenital heart disease often requires implantation of a valved conduit. A hand-made expanded polytetrafluoroethylene (ePTFE) trileaflet Dacron graft conduit has been used at our center since 1997, and has been implanted in 31 patients. Midterm results of this conduit were investigated in 30 of the patients who have been followed at our outpatient clinic. There were 16 males and 14 females. The mean age and body weight were 16.4±7.2 (range, 3.4-33.4) years and 41.7±13.3 (range, 13.0-64.0)kg, respectively. Diagnoses were tetralogy of Fallot with pulmonary atresia in 14 patients, RVOT reconstruction associated with Ross procedure in 8, transposition with pulmonary stenosis in 3, pulmonary atresia with intact ventricular septum in 2, tetralogy with absent pulmonary valve syndrome in 1, pulmonary regurgitation developed after tetralogy repair in 1, and hemitruncus in 1. The median size of the graft was 22 (range, 20-26)mm. All patients were in NYHA functional class I at the time of the latest follow-up. The pressure gradient across the conduit was 11.0±5.8mmHg during the same hospitalization and 13.8±6.5mmHg on the latest echocardiogram (Interval, 2.4±1.5 years, p=0.85). The valve function was well maintained in all patients, with the regurgitation graded as non-trivial in 22 patients, mild in 7, and moderate in only 1. Midterm results of hand-made ePTFE trileaflet valved cunduit was satisfactory. A longer follow-up is mandatory to assess its actual durability.

A 22-year-old woman had been treated with a Ross operation for aortic root aneurysm and aortic regurgitation 10 years previously. In the initial Ross operation, a handmade tri-leaflet conduit was used for the right ventricle outflow tract (RVOT) reconstruction. The conduit was prepared preoperatively, by sewing a folded 0.1 mm expanded polytetrafluoroethylene (ePTFE) membrane onto the luminal cavity of the 24 mm woven double velour vascular graft, thereby creating a tri-leaflet valve. During ambulatory follow up after discharge, dilation of the pulmonary autograft had been observed, and its maximal diameter reached 60 mm. Furthermore, preoperative a pressure study revealed a 25 mmHg pressure gradient between the right ventricle and the pulmonary artery. At the time of reoperation, we performed an aortic root replacement combined with RVOT conduit replacement. A 24-mm woven double velour vascular graft integrating a 21-mm On-X mechanical prosthesis was used for aortic root replacement. A handmade ePTFE tri-leaflet conduit, 26 mm in size, was used to replace the previous RVOT conduit. The operation was successful, and the postoperative course was uneventful. The explanted conduit was sent for microscopic examination, which revealed that the graft was covered by a fibrocollagenous membrane. On the contrary, no surface membrane was found on the ePTFE valve. Moreover the microscopic examination showed cystic medionecrosis of the pulmonary autograft. Both dilatation of the pulmonary autograft and RVOT conduit failure were successfully treated at the second operation. However this young patient will require follow-up of the mechanical prosthesis and RVOT conduit for the rest of her life.

Staged arterial switch operation without homologous blood transfusion was successfully performed in 5 patients weighing 4.1-11.0kg (double outlet right ventricle: 2 cases, transposition of great arteries: 3 cases). The postoperative hemodynamics and respiratory status were uneventful in all patients (initial central venous pressure after ICU admission: 9.0-14.5cmH₂O, mean 12.5cmH₂O, duration of intubation: 3.5-18.0h, mean 7.8h). Autologous blood donation immediately after induction of anesthesia and minimization of bypass circuit were effective methods for open heart surgery without homologous blood transfusion, particularly in staged arterial switch operation requiring prolonged cardiopulmonary bypass.

Objective: The antitumor effects of anti-PD-1 antibody against mismatch repair deficiency (MMR-D)-associated cancers have been reported. MMR-D is found in approximately 20%–30% of endometrial carcinomas (ECs) and frequently occurs due to MLH1 promoter hypermethylation (MLH1-PHM). ECs with MLH1-PHM are classified according to the molecular screening of Lynch syndrome (LS), but few detailed reports are available. The purpose of this study was to clarify the clinical features of EC with MLH1-PHM. Methods: Immunohistochemistry of MMR proteins (MLH1, MSH2, MSH6, and PMS2) was performed on specimens from 527 ECs treated at our university hospital from 2003 to 2018. MLH1 methylation analysis was added to cases with MLH1/PMS2 loss. ECs were classified as follows: cases that retained MMR proteins as “MMR-proficient;” cases with MLH1/PMS2 loss and MLH1-PHM as “met-EC;” and cases with other MMR protein loss and MLH1/PMS2 loss without MLH1-PHM as “suspected-LS.” The clinical features, including long-term prognosis, of each group, were analyzed. Results: Accordingly, 419 (79.5%), 65 (12.3%), and 43 (8.2%) cases were categorized as “MMR-proficient,” “suspected-LS,” and “met-EC,” respectively. Significantly, “met-EC” had a lower proportion of grade 1 tumors (37.5%) and a higher proportion of stage III/IV tumors (37.2%) than the other groups. The overall and progression-free survival of “met-EC” were significantly worse than those of “suspected-LS” in all cases. Conclusion In ECs with MMR-D, “met-ECs” were a subgroup with a poorer prognosis than “suspected-LS.” “Met-ECs” would be the main target for anti-PD-1 antibody treatment, and its clinical susceptibility should be verified individually.

The patient was a 4-year-old boy, with no indication of heart disease at birth. When he was 4 years and 3 months old, he underwent surgery for cryptorchidism, and persistent changes after crying, consisting of ST-segment depression, were noted on the ECG. Therefore, it was decided that a detailed examination would be carried out. Aortography revealed that a small left coronary cusp had formed a pouch and that there was a delayed washout of the contrast material in the left coronary artery compared with the right coronary artery. Right coronary angiography showed retrograde flow to the left coronary artery. There was no significant aortic regurgitation, and no stenosis in the left coronary artery. Intraoperative findings demonstrated that the hypoplastic left coronary cusp had adhered to the aortic wall and covered the left sinus of Valsalva. Enlargement of the Valsalva was performed by placing a patch from the aortic transection site to an area directly above the coronary artery ostium. Postoperatively, the patient recovered satisfactorily and was discharged on the eleventh postoperative day. Cardiac catheterization performed 6 months after surgery showed no aortic regurgitation, no significant stenosis in the left coronary artery, and no retrograde flow from the right coronary artery. We report our experience with the rare case described above.

The patient was an 8-years- and 4-months old girl. At the age of one, she visited a previous doctor with hepatomegaly and liver dysfunction. As a result of the examination, she was diagnosed with geleophysic dysplasia. Other than the heart, she was followed in genetics, ophthalmology, orthopedics, endocrinology, and otolaryngology. At 3 years and 5 months, she was first examined by the cardiology department and was found to have mild mitral regurgitation and aortic valve stenosis, and was followed up once a year. The patient was referred to our hospital at 7 years and 11 months, and the cardiac catheterization performed at 8 years and 2 months showed mild mitral valve regurgitation, but the mean pressure gradient was 16 mmHg and the mitral valve area was 0.60 cm2 (MVAi 0.97 cm2/m2), and mitral valve stenosis was observed. The left atrial pressure was as high as 25 mmHg and the average pulmonary artery pressure was as high as 36 mmHg, and pulmonary hypertension was also observed. Intraoperative findings demonstrated that the mitral valve had a marked thickening of the leaflet, the papillary muscles and chordae were also thickened, and the effective valve opening area was narrowed. The leaflet and subvalvular tissue were resected as much as possible and mechanical valve replacement was performed. Postoperatively, the patient recovered satisfactorily and was discharged on the 16th postoperative day. Pathological findings showed no major disturbance in the layered structure of the papillary muscle or the leaflet itself, but it was highly thickened due to mucous degeneration of the leaflet. We report our experience with the rare case described above.

This study reports the case of a 0-day-old girl who was transferred to our hospital for suspected total anomalous pulmonary venous return due to her postnatal cyanosis. After she was presented at our hospital, pulmonary vein stenosis was diagnosed and emergency surgery was planned. Preoperative computed tomography showed abnormal perfusion of the pulmonary veins into the left and right superior vena cava separately on the left and right sides. Given that the persistent left superior vena cava was refluxing into the coronary sinus, the coronary sinus was enlarged greatly, and the left atrial volume was small. In the neonatal period, the right pulmonary vein was anastomosed to the right atrium, and the left pulmonary vein was anastomosed to the unroofed coronary sinus. Thereafter, at 4 months of age, the patient underwent two-stage intracardiac repair with re-routing of the right pulmonary vein and extracardiac ligation of the left vena cava. The postoperative course was good. Intracardiac repair via a two-stage surgery was effective for total anomalous pulmonary venous return (Ib+Ib) with a persistent left superior vena cava and an enlarged coronary sinus.

Objective: In Japan, only a few reports of the Ross procedure in neonates and infants have been published. The objective of this study was to evaluate the outcome of patients undergoing a Ross procedure before the age of one year, and to review the validity of opting for this procedure at this age. Methods: The records of 13 infants (including three neonates) undergoing a Ross procedure between December 1996 and June 2017 were reviewed. Major outcomes studied included graft-associated morbidity, autograft function, and the need for reoperation. Results: The median age at the time of Ross procedure was 166 days, and median weight was 5.7 kg. Primary diagnoses were aortic stenosis in 10 cases and aortic insufficiency in three. Nine cases required emergent Ross procedure due to left ventricular dysfunction refractory to medication, requirement of mechanical ventilation or intravenous inotropic drugs. Concomitant procedures included three aortic coarctation repairs, two annular enlargement procedures with a Konno incision and one each of aortic and mitral annuloplasty. The mean cross-clamp time was 131 min and the mean extracorporeal circulation time was 178 min. Two cases required extracorporeal membrane oxygenation. Seven underwent delayed sternal closure and four required postoperative peritoneal dialysis. The median duration of mechanical ventilation was five days and the median length of intensive care unit stay was seven days. Survival was 100% at a median follow-up of 9.9 years. The diameter of the aortic annulus mostly stayed within normal limits, although sinus of Valsalva's enlargement beyond normal value was noted in some cases. Trans-aortic valve pressure gradient was less than 20 mmHg and aortic insufficiency was less than mild in all cases, thus requiring no reintervention for the valve. Two cases required coronary arterial bypass and release of the subaortic stenosis. Freedom from reoperation for the left heart was 100% at one year, and 81.5% at five years and 10 years. Ten cases required reoperation for the right heart, and freedom from reoperation was 84.6% at one year, 29.7% at five years and 9.9% at 10 years. Conclusion: Durability of the pulmonary autograft was excellent. The Ross procedure can be an effective treatment strategy for severe aortic valve diseases in neonates and infants.