Masato Yokozawa

Double outlet right ventricle with intact ventricular septum

We report a rare case of DORV with an intact ventricular septum (intact VS). The patient was a 2-day-old boy, born at 39 weeks and weighing 3090 g, who was referred to the Department of Pediatrics, Sapporo Medical University Hospital, Sapporo, Japan, because of a heart murmur. He had cyanosis and a systolic murmur at the lower intercostal spaces. Chest X-ray showed cardiomegaly (cardiothoracic ratio, 67%) and the electrocardiogram revealed normal sinus rhythm, right axis deviation and right ventricular hyper trophy. Echo cardiography demonstrated situs solitus, normal relationship of great arteries, side by side, bilateral conus, intact VS, persistent foramen ovale, pulmonary stenosis and severe mitral valve insuffi ciency ( Fig. 1a,b). We performed balloon atrial septostomy when he was 1 month old, and he underwent a left modifi ed Blalock – Taussig shunt at 5 months. Cardiac catheterization was performed when he was 18 months old. His left ventricular pressure was extremely low, 10/4 mmHg, the diastolic volume was 11 mL, 48% of normal, and the ejection fraction was 76%. Left ventricular angiography showed a smooth and thin wall of the left ventricle, resembling Uhl’s disease. Both right and left coronary artery were normally demonstrated ( Fig. 2). Right ventricular pressure was 92/11 mmHg with a pulmonary artery Patient Report

Acute fulminant ulcerative colitis with toxic megacolon.

An 11‐year‐old boy with acute fulminant ulcerative colitis (UC) is presented. He had systemic deterioration with frequent diarrhea and lethargy. Acute fulminant UC associated with toxic megacolon was diagnosed by rectal endoscopy and biopsied specimen. He was treated with intensive intravenous administration of prednisolone and total parenteral nutrition. He recovered completely without any surgical intervention.

Genesis stent implantation without using a long sheath in two children

A Genesis stent was implanted in two children, one with superior vena caval (SVC) stenosis and one with pulmonary artery branch stenosis. Case 1 was a 2-month-old baby with SVC stenosis following intracardiac repair for total anomalous pulmonary venous connection (TAPVC) and case 2 was a 2-year-old child with left lower pulmonary artery stenosis following one-stage unifocalization for dextrocardia, double outlet right ventricle, ventricular septal defect, pulmonary atresia and major aortopulmonary collateral arteries. Both procedures resulted in immediate clinical and hemodynamic improvement. The Genesis stent has a closed-cell design with sigma hinges interpositioned between each cell. With improved deliverability and expandability of the stent, we can easily deliver it through smaller sheaths, which will facilitate its use in infants and smaller children with vascular stenosis.

Low risk of treatment resistance in Down syndrome with Kawasaki disease

A Japanese nationwide survey has reported that Down syndrome (DS) is a less‐frequently occurring comorbidity in Kawasaki disease (KD). Although altered immune responses are frequently observed in DS, no studies have focused on the treatment response and risk for coronary artery abnormalities (CAA) in DS patients with KD. The aim of this study was therefore to evaluate the clinical manifestations, treatment response and prevalence of CAA in DS with KD.