Background: Although many patients undergoing the Fontan procedure require anticoagulants or antiplatelet drugs, there is no consensus regarding thromboprophylaxis after this procedure.
Methods: To clarify the incidence of thrombotic or bleeding complications and their characteristics, we retrospectively studied 49 patients (34 male and 15 female) who underwent the Fontan procedure between 1989 and 2012. Their average age was 16.3±7.7 years, the average age at which total cavopulmonary connection surgery was performed was 4.5±3.7 years, and the mean duration of postoperative observation was 59.1±45 months.
Results: All patients received warfarin and all but one received aspirin. Three patients developed cerebral infarction, one of whom also developed renal infarction. With prompt treatment, all three patients recovered without sequelae. With regard to bleeding complications, one of three patients who developed pulmonary hemorrhage died. Gastrointestinal bleeding occurred on six occasions in three patients, only one of whom had associated abdominal pain. Urgent blood transfusions were necessary on five of these occasions. Additionally, subcutaneous hemorrhage, macrohematuria, ovarian hemorrhage, and polyhypermenorrhea also occurred. In patients with bleeding complications, the mean prothrombin time/international normalized ratio was 1.9±0.5. These hemorrhagic events were managed by the discontinuation of aspirin and reduction of the warfarin dosage.
Conclusion: It is necessary to determine whether a combination of aspirin and warfarin is indicated for maintaining adequate circulation after the Fontan procedure. Because combination treatments are likely to cause more bleeding complications, the use of individual drugs should be investigated.

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Background: Hypertrophic cardiomyopathy (HCM) is a major cause of sudden death in children at school. However, more children with HCM are being successfully resuscitated after collapse due to the increasing availability and accessibility of basic life support systems. Therefore, the prognosis for children with HCM who experience cardiac events at school may be changing. This present study aimed to determine the outcome of cardiac events arising in children with HCM and the effect of automated external defibrillators (AED) applied under school supervision.
Methods: We retrospectively studied case reports of cardiac events submitted to mutual aid systems for schoolchildren with HCM between 2004 and 2011.
Results: Among 44 children with HCM who experienced sudden cardiac events at school, 29 died and 15 survived after resuscitation. An AED was applied to 14 of the survivors, and one already had an implantable cardioverter defibrillator (ICD). Males predominated among both the deceased and the survivors. Among children with HCM who experienced cardiac events in kindergarten, primary school, junior high school, and high school, only those in junior high school and high school survived. The ratios (％) of the deceased and the survivors that had been diagnosed with HCM before cardiac events occurred were 48％ and 20％, respectively. Cardiac events occurred most frequently during exercise in both the deceased and the survivors.
Conclusions: The number of children with HCM who are resuscitated after cardiac arrest while at school has increased in Japan since 2007. Both AED and ICD are essential in preventing sudden death due to HCM in schoolchildren.

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Background: Intravenous immunoglobulin (IVIG) is an effective treatment for Kawasaki disease. However, severe Kawasaki disease can be refractory to IVIG. The aim of the present study was to investigate the efficacy and safety of slow plasma exchange plus continuous hemodiafiltration (SPE＋CHDF) for severe Kawasaki disease.
Materials and Methods: Since September 2007, we have performed SPE＋CHDF for severe Kawasaki disease in 12 cases at our facility.
Results: With respect to cardiac function before SPE＋CHDF, atrial natriuretic peptide (ANP) values (222.4±172.9 pg/mL), brain natriuretic peptid (BNP) values (425.8±605 pg/mL) and cardiothoracic ratio (56.3±5.6％) were high in all patients. There were no significant changes in heart rate or systolic blood pressure at 1 h and 3 h after SPE＋CHDF compared with results before therapy. There were no cases of intravascular catheter infection, ventilator-associated pneumonia cases or requirement for inotropic agents. All patients became afebrile 1–4 days (median, 1 day) after SPE＋CHDF. However, we performed additional treatment in two cases due to recurrence. Seven cases with a coronary artery lesion before SPE＋CHDF regressed within 1 year.
Conclusion: Plasma exchange is effective for the treatment of severe Kawasaki disease. However, many cases of severe Kawasaki disease involve hemodynamic failure. Our data suggest that SPE＋CHDF can be used to safely perform plasma exchange in these cases.

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Background: Although congenital heart defects are common complications in patients with trisomy 18 (T18), cardiac surgery has rarely been considered because of the short lifespan and severe developmental delay in survivors. In our institute, surgical intervention was not considered until 2004, but it has been a treatment option since 2005 for patients with a prediction of home discharge after surgical improvement of congestive heart failure (CHF).
Objective: This study was planned to clarify the outcome of cardiac surgery in patients with T18, focusing on the improvement of CHF, discharge, and survival.
Methods: We collected detailed clinical information from the medical records of 46 patients with T18, from 1994 to 2012. Cardiac surgery was performed on 16 patients, of whom 14 underwent palliative surgery (Group A) and two underwent primary intracardiac repair (ICR; Group B). The remaining 30 patients received no surgical intervention (Group C).
Results: All patients in Group A survived the perioperative period, and their CHF was relieved. Ten patients in Group A could be discharged to home, with the establishment of enteral nutrition and without the requirement of ventilator support. Three of the 10 patients were alive at the time of this study, whereas the others died of respiratory tract infection after discharge. The rate of discharge was 71% for patients in Group A compared with 23% for those in Group C. The survival rate of patients in Group A was 100% at the age of 1 month, 86% at 3 months, 71% at 6 months, and 29% at 1 year, whereas in patients in Group C, the corresponding rate was 47%, 37%, 13%, and 3%, respectively . The median survival time was 234 days in patients in Group A and 24 days in those in Group C. Among two patients in Group B, one with ventricular septal defect (VSD) and pulmonary stenosis underwent ICR at the age of 7 months, and the other with only VSD underwent ICR at 5 months, both of whom displayed significant relief of CHF and no signs of pulmonary hypertension.
Conclusion: These results suggest that palliative surgery for cardiac defects with CHF in patients with T18 could increase the hospital discharge rate and improve survival. ICR may also relieve CHF and lead to much longer survival. Management of congenital heart defects in patients with T18 should be discussed on an individual basis, and cardiac surgery is considered to be a reasonable treatment option in those with CHF and no other serious complications.

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An infectious aneurysm represents a potentially serious clinical condition because of its tendency to rupture and to be complicated with sepsis. Here we report an infantile case of infectious aneurysms of the brachiocephalic artery, occurring subsequent to mediastinitis. Chest computed tomography (CT) revealed aneurysms of the brachiocephalic artery after the recurrence of mediastinitis. The patient’s trachea was compressed by the brachiocephalic artery, which was displaced backward by the aneurysms. Urgent implantation of a hand-made covered stent, which was made of a metallic stent and a roll-shaped expanded polytetrafluoroethylene sheet, was performed. After deployment of the coveresd stent, the size of the aneurysms was diminished and compression of the trachea improved. After treatment with anti-methicillin-resistant Staphylococcus aureus (MRSA) medications, the mediastinitis has been in remission. The development of Horner’s syndrome was recognized as a complication of the stent deployment. Implantation of a covered stent represents an option for the treatment of infectious aneurysms.

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Crossed pulmonary arteries (CPAs) is a rare anomaly in which the left pulmonary artery (PA) originates to the upper right of the right PA and both PAs cross each other. CPAs is asymptomatic and presents little risk of perioperative complications. Here we report two cases of 22q11.2 deletion syndrome with CPAs and interruption of the aortic arch (IAA). In both cases, there was severe left branch PA stenosis after arch repair of the IAA. Percutaneous balloon angioplasty for the stenosis was not effective and left pulmonary angioplasty was performed. Left branch PA stenosis in CPAs is assumed to be due to the unique form of the pulmonary branches or the geometry change of the left PA caused by the arch repair.

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Excluding heterotaxy cases, total anomalous pulmonary venous connection (TAPVC) is rarely associated with ventricular septal defect (VSD). We encountered two combined surgical cases from a total of 52 TAPVC cases in our institution during the past 2 decades. Different postoperative courses were identified, and the importance of a preoperative diagnosis was stated.

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