Childrens with Down syndrome (DS) has a greater risk of pulmonary arterial hypertension (PAH) than the general pediatric patients. The reasons of PAH are mainly due to probably genetic backgrounds, specific structure of pulmonary vascular wall, certain types of congenital heart diseases, and partly due to pulmonary hypoplasia, upper and lower airway obstructive diseases, chronic infection, and neuro-muscular underdevelopment. Exposure to increased left to right shunt results in increased sheer stress on pulmonary endothelial cells and may induce endothelial dysfunction followed by irreversible pulmonary arterial remodeling.
The pathological changes are characterized by endothelial cell proliferation and thickening of pulmonary arterial vessel wall due to mechanical responses to thinner medial smooth muscle cell (SMC) layer and pulmonary hypoplasia (under development of alveoli). DS patients has decreased production of prostacycline and Nitrate, but elevated endothelin-1 and thromboxane. Perioperative periods, DS may develop post ICR PAH crisis, poorer response to nitric oxide (NO) inhalation, and prolonged PAH status.
For better management of DS patients, it is crucial to evaluate systemic complications with pediatric cardiologists, pulmonologists, neurologists, and adult cardiologists. At the cardiac catheterization, pulmonary arterial resistance is a kee-data for assessing the severity of PAH, and response to vasodilating agents for preventing postoperative PAH crisis, and for indication of intracardiac repair.
The advanced therapy with recently developed pulmonary vasodilating agents seems to be effective for DS patients. Operative risk is not so high even in DS patients except patients with severe AVSD compared to non-DS patients. Optimal timing and modalities for evaluation are essential.

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We reviewed the past and current status of the Japan Congenital Cardiovascular Surgery Database (JCCVSD). The JCCVSD has grown steadily since 2007. Now, there are over one hundred participating hospitals in the JCCVSD throughout Japan, and the cumulative number of submitted cases totaled about twenty thousand by the end of 2011. Thus, the volume of submitted data represents nearly full coverage of Japan.
All submitted data belong to the JCCVSD and participating hospitals. Thus, participants can download their own data, analyze the information, and submit reports to journals independent of the JCCVSD. We announced the rules for data use and authorship to the participating hospitals to encourage submission of papers resulting in promotion of quality improvement.
The JCCVSD was first audited last year, and 28.9% of patients and 29.6% of procedures were verified. The audit was almost completely concluded, 99.0% cases out of 1279 cases, with high accuracy and missing less than 1% of the data. But, under-reporting the complications could not be missed. One of the current problems is the collaboration with the Board Certification System. Board Certification by the Japan Surgical Society (National Clinical Database, NCD) is the first step. Board Certification by Cardiovascular Surgery (JACVSD, JCCVSD) is the second step as a sub-specialty with cardiovascular surgery. These two board certifications presently are under the control of two separate organizations. These two organizations will judge based on the database by extraction of applied surgeon's experience from the JCCVSD. If a surgeon does not participate in the JCCVSD, they cannot apply for board certification. In either the Japan Surgical Society or the Japan Society of Cardiovascular Surgery, development or refinement of a new system for such interactions is to be started without delay.

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While the 5^th pulmonary hypertension international symposium in Nice 2013 is approaching, key recent advances after the 4th international symposium in the field of pediatric pulmonary hypertension and vasculopathy related to congenital heart diseases were reviewed. Especially, meta-analysis of disease-specific compounds, epidemiology, and the genotype-phenotype correlation in adult and pediatric pulmonary arterial hypertension, recent translational research using human tissue samples and recent animal models, the impact of disease-specific compounds in Eisenmenger syndrome, and issues in Fontan circulation and hypoplastic pulmonary arteries were highlighted. Cellular molecular biology studies and clinical and population science in this field may converge via molecular genetics and studies using lung samples and new animal models to ultimately bridge the knowledge gap between them. Science in the right heart system, including pulmonary hypertension, pulmonary vasculopathy, and right heart failure, would be an evolving field of research at present and in the future.

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Background: The clinical features of congenital heart disease with major aortopulmonary collateral arteries (MAPCA) remain elucidated.
Methods: We reviewed the cases of congenital heart disease with MAPCA retrospectively to clarify the clinical features of MAPCA.
Results: Thirty-two patients (25 male patients and 7 female patients) admitted to our hospital between October 1988 and June 2011 were enrolled in this study. The mean age was 9.7±7.7 years old. Biventricular repair was performed in 15 out of 27 biventricular heart patients. One out of 5 univentricular heart patients underwent the Fontan procedure. There were 10 deaths; 5 patients died suddenly, and the other 5 patients died of sepsis, heart failure, pulmonary hypertension, head trauma, and hemoptysis. Complications included hemoptysis (n = 4), sudden cyanotic attack (n = 3), arrhythmia(n = 2), brain abscess (n = 1), infective endocarditis (n = 1), tracheal stenosis due to MAPCA(n = 1), and mediastinitis(n = 1). Two out of 3 patients with sudden cyanotic attacks died suddenly. The first cyanotic attack occurred at the age of 2-4 months. Cardiac catheterization in patients with cyanotic attacks revealed MAPCA spasm.
Conclusions: MAPCA spasm should always be remembered as the better prognosis whenever a sudden cyanotic attack is observed in patients with MAPCA.

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Background: Although negative pressure wound therapy (NPWT) is widely used as a therapy for surgical site infection (SSI), few reports have discussed NPWT after pediatric cardiac surgery.
Objective: To evaluate the efficacy of NPWT for 7 pediatric patients with SSI (superficial infections 4, mediastinitis 3) after cardiac surgery between January 2010 and January 2011.
Methods: Seven patients were treated with NPWT for uncontrollable pus discharges even with focal irrigation. Pressure of NPWT, periods of NPWT, microorganisms, recurrence, resuture, and complications associated with NPWT were determined. Polyurethane foam was used as the dressing foam. A drain with a side hole was made. Polyurethane foam and the drain were sealed with a film dressing. The pressure was set at -200 to -300 mmHg.
Results: Median duration of NPWT was 28 days (range, 14-106 days) and methicillin-resistant Staphylococcus aureus was the causative microorganism in all 7 patients. Two cases were recurrent. Two cases underwent resuture for wound dehiscence. No complications associated with NPWT, deep mediastinitis, uncontrollable sepsis, or systemic infectious disease were encountered.
Summary: Seven cases were treated with NPWT for SSI after pediatric cardiac surgery. Although there were problems about the setup of optimal pressure of NPWT, or the treatment for SSI of high risk patients, NPWT could be one of the choices of treatment for SSI or mediastinitis.

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The number of post-Fontan patients is increasing with the improvement in diagnostic and therapeutic technologies. The number of post-Fontan patients, who have congenital non-cardiac disorders, such as respiratory or digestive disorders, is also increasing. These congenital non-cardiac disorders occasionally influence the plans for cardiac surgery, and these problems may deteriorate the pre- and post-operative respiratory and nutritional condition of patients. In pediatric surgery, to treat the above abnormalities, laparoscopic surgery has spread widely because of its less invasive and cosmetic advantages. After the Fontan procedure, passive pulmonary blood flow has to be driven by the trans-pulmonary pressure gradient. In laparoscopic surgery, the increased intra-abdominal pressure from pneumoperitoneum could effect Fontan circulation unfavorably. In addition, mechanical ventilation and the Trendelenburg position during laparoscopic surgery may deteriorate passive pulmonary circulation due to increased intra-thoracic pressure. Moreover, hypercapnia induced by the intra-abdominal CO₂ gas may elevate pulmonary vascular resistance. So laparoscopic surgery is generally thought to be disadvantageous for post-Fontan patients. We had three cases of laparoscopic surgery after Fontan procedures. We prepared for the switch to open surgery, but laparoscopic surgery was accomplished uneventfully in all our cases. Sufficient discussion among related personnel enabled secure intra-operative management.

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