The size of the coronary arteries parallels the ventricular mass, thus it may be abnormal in complete transposition beyond infancy and could be influenced by the arterial switch operation. To investigate this possibility, we measured the diameters of the right, left main trunk, anterior descending, and circumflex coronary arteries before and three to seven years (mean 4.8) after the arterial switch operation in 17 patients with a “normal” distribution of the coronary arteries (so-called Shaher type 1). The values were compared with 18 controls who had Kawasaki disease with no apparent coronary arterial disease. The right, left anterior descending, and circumflex arteries were smaller than control values before the operation. The post-/preoperative ratios of the diameter were 1.16±0.11 for the right coronary artery, 1.18±0.16 for the left main trunk, 1.20±0.18 for the left anterior descending artery, and 1.22±0.26 for the circumflex artery. There were no significant differences among these values. After surgery, the right coronary artery was larger, but the left coronary arteries were smaller in the patients than in the controls: 2.5±0.3 vs 2.0±0.2 mm for the right coronary artery; 2.4±0.3 vs 2.7±0.1 mm for the left main trunk; 1.9±0.2 vs 2.4±0.2 mm for the left anterior descending artery; 1.6±0.4 vs 2.2±0.5 mm for the circumflex artery, respectively. The posterior descending coronary artery originated from the right coronary artery in all patients. The total cross-sectional area of the right coronary, left anterior descending, and circumflex arteries was 9.7±2.4 mm2in the patients, and 11.8±2.9 mm2in the controls (p>O. 1), suggesting that the increased size of the right coronary artery compensates for the small left coronary arteries. We conclude that the arterial system in complete transposition, with a large right coronary artery and small left coronary system, remains smaller than normal even at midterm follow-up after anatomic repair despite normalization of left ventricular volume and muscle mass.

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We examined a newborn who had no bile and pancreatic ducts. Hydrops was evident after 29 weeks of gestation and she died shortly after birth, weighing 1,368 g. One of her siblings had died of hydrops at about six months of gestation, and there were two more miscarriages of unknown cause. At autopsy on the newborn, the liver had an abnormally round shape and the pancreas was not in the normal position. There was an ectopic small pancreas with normally developed islets. Histological analysis revealed the complete absence of extra-and intra-hepatic bile and pancreatic ducts. Immunostaining of these tissues showed no positive bile duct marker staining using epithelial membrane antigen and cytokeratin 19 in the liver. Albumin and a-fetoprotein staining was positive in the liver, and insulin and glucagon staining was positive in the remaining islets. Thus, this case is characterized by complete absence of bile and pancreatic ducts. These findings suggest the existence of a gene linked to the development of bile and pancreatic ducts.

Background: It is well-known that a neurologically favorable outcome of out-of-hospital cardiac arrest (OHCA) is associated with the presence of bystander-initiated cardiopulmonary resuscitation (bystander CPR) and use of an automated external defibrillator. However, little is known about the effect of the presence of pre-existing conditions, prior activity, and locations on the outcome of pediatric OHCA. Methods: We analyzed the data from questionnaires about pediatric patients with OHCA aged from 3 days to 19 years in the Kyushu area in Japan between 2012 and 2016. Results: A total of 594 OHCA cases were collected. The numbers of OHCA cases and the rate of 1 month survival with a favorable neurological outcome during sleeping, swimming / bathing, and exercise were 192 (1.0%), 83 (32.5%), and 44 (65.9%), respectively. When an OHCA occurred at school (n = 56), 88% of children / adolescents received bystander CPR, but when it occurred at home (n = 390), 15% received bystander CPR. Cardiovascular (n = 61), suicide (n = 61), and neurological / neuromuscular (n = 44) diseases were three major pre-existing conditions. The OHCA of cardiovascular disease was associated with exercise (24/61) and mainly occurred at school (22/ 61). The OHCA of neurological / neuromuscular disease was associated with swimming/bathing (15/44) and mainly occurred during bathing at home (12/44). Multivariate regression analysis showed that the presence of bystander CPR (P < 0.001) and occurrence of OHCA at school (P < 0.001) were independently predictive of a favorable outcome in pediatric OHCA. Conclusion:The outcome was different among pre-existing conditions, prior activity, and location of OHCA. These findings might be useful for preventing OHCA and improving the outcome of pediatric OHCA.

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