A revised transplant law, which has been valid since July 17, 2010, has provided the same circumstances of organ donation in the United States, that is, organ donation from donors of any age has been valid with the opt-in system. Adult heart transplantation has been performed at the rate of three cases a month since then; however, only one pediatric heart transplant was done from a brain-dead donor less than 15 years old. In order to do more pediatric heart transplants in Japan, pediatric intensive care units in each hospital in Japan should communicate with the three pediatric heart transplant centers in Japan. It is also important that a pediatric ventricular assist devices, such as the ExCor · Pediatric, be approved as soon as possible. For making well-established pediatric heart transplantation in the future, post-transplant care for pediatric heart transplantation must be prepared.

View full abstract

Partial anomalous pulmonary venous connection is a relatively common congenital heart defect. Cor triatriatum is a rare congenital heart anomaly that typically result in congestive heart failure due to pulmonary venous obstruction. Discrimination between an anomalous pulmonary venous connection and cor triatriatum is important for surgical treatment; however, an accurate diagnosis is sometimes difficult. We surgically corrected an atrial septal malformation with hemodynamic equivalence to partial anomalous pulmonary venous connection in an infant. Discrimination was difficult between this combination and cor triatriatum combined with malformation of the atrial septum primum and secundum. Echocardiography and cardiac catheterization did not provide sufficient information on intracardiac structures. Preoperative 3D-CT was useful for anatomical diagnosis and determination of the surgical septation line.

View full abstract

Background: Coronary spastic angina, a syndrome caused by coronary spasms, is extremely rare in children. We encountered two teenagers where coronary spastic angina had been diagnosed based on coronary angiography and an intracoronary injection of acetylcholine. Case 1: An 11-year-old boy who had no medical history related to coronary artery disease, such as Kawasaki disease, was admitted to our hospital complaining of nausea, dyspnea, and precordialgia in the early morning. On admission, the patient's troponin T test was positive and the results of electrocardiography were normal. Eight hours later, ST elevations was seen in leads V2 and V3. Coronary angiography revealed no stenosis; however, the left anterior descending artery showed diffuse stenosis after injection of acetylcholine (50 µg) into the left coronary artery. Case 2: A 14-year-old girl was admitted for dyspnea and chest pain in the early morning. On admission, the troponin T test was positive, but the results of electrocardiography and echocardiography were normal. She experienced severe chest pain with ST elevations in leads II, III, aVF, and V3-6 after hospitalization. Coronary angiography revealed no stenosis, but there was diffuse stenosis in the left anterior descending artery and the circumflex branch after injection of acetylcholine (50 µg). Conclusions: When pediatric patients complain of chest pain and coronary angiography shows no stenosis, the addition of a coronary spasm provocation test is highly recommended. Faster diagnoses can contribute to better outcomes for pediatric coronary spastic angina patients.

View full abstract