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Surgical re-intervention for progressive left ventricular outflow tract obstruction after a standard biventricular repair of aortic arch obstruction and ventricular septal defect is rare but can occur. In this situation, left ventricular outflow bypass surgery can be performed to relieve left ventricular outflow tract obstruction by creating an intraventricular baffle to channel the re-opened ventricular septal defect to the pulmonary valve, Damus-Kaye-Stansel anastomosis, and right ventricle-to-pulmonary artery connection with a valved conduit. This strategy, termed as “Yasui conversion,” is a safe and useful alternative procedure for selected patients.

In the Fontan circulation, there is a substantial degree of systemic-to-pulmonary collateral flow (SPCF), which can be measured by cardiac magnetic resonance (CMR). However, the correlation between the degree of SPCF and long-term outcomes is not fully understood. We retrospectively studied 321 patients who underwent the Fontan procedure and CMR at a single center. Using CMR, we calculated SPCF as pulmonary blood flow − systemic blood flow. %SPCF was defined as SPCF ÷ pulmonary blood flow. The mean age of patients at CMR was 14.3 ± 7.5 years. The average %SPCF was 13.0% ± 11.0%. With a multivariate analysis, %SPCF was significantly correlated with time (i.e., the longer the time period since the Fontan procedure, the lower the %SPCF) (p = 0.006), previous total anomalous pulmonary vein drainage (p = 0.007), a low pulmonary artery index (Nakata index) before the Fontan procedure (p = 0.04), and older age at the time of the Fontan procedure (p = 0.002). Regarding the findings after the Fontan procedure, %SPCF was significantly correlated with ventricular end-diastolic volume (p

A few studies have described the outcomes of systemic-to-pulmonary artery shunt (SPS) for functional single ventricular heart with extracardiac total anomalous pulmonary venous connection (TAPVC). This study aimed to examine the outcomes of SPS with extracardiac TAPVC and identify the predictors of mortality before bidirectional Glenn operation (BDG). Medical records of 41 children with single ventricular heart and extracardiac TAPVC who underwent SPS between 1998 and 2019 were reviewed retrospectively. The median age and body weight at SPS were 36 days and 3.4 kg, respectively. Surgical outcomes and predictors of mortality were investigated. Four operative deaths (10%) and 10 late deaths (27%) occurred before BDG. Of all the children, 19 underwent BDG at a median of 10 months since SPS and eight are waiting for BDG. In the multivariate analysis, preoperative pulmonary venous obstruction (p = 0.01) at initial surgery was most predictive of death before BDG. Patients who underwent simultaneous SPS and TAPVC repair were younger, had high preoperative rate of pulmonary venous obstruction, and more deaths before BDG. Survival outcomes of SPS for SVH with extracardiac TAPVC were improved as a whole due to the increase in knowledge and technique of management SPS. However, the patients who have preoperative pulmonary venous obstruction (PVO) and need SPS and TAPVC repair concomitantly in the early postnatal period have poor outcomes and still challenging. In such a case, staged TAPVC repair and SPS may be beneficial.

The present study developed a new risk model for congenital heart surgery in Japan and determined the relationship between hospital procedural volume and mortality using the developed model.We analyzed 47,164 operations performed between 2013 and 2018 registered in the Japan Cardiovascular Surgery Database-Congenital and created a new risk model to predict the 90-day/in-hospital mortality using the Japanese congenital heart surgery mortality categories and patient characteristics. The observed/expected ratios of mortality were compared among 4 groups based on annual hospital procedural volume (group A [5539 procedures performed in 90 hospitals]: ≤50, group B [9322 procedures in 24 hospitals]: 51-100, group C [13,331 procedures in 21 hospitals]: 101-150, group D [18,972 procedures in 15 hospitals]: ≥151).The overall mortality rate was 2.64%. The new risk model using the surgical mortality category, age-weight categories, urgency, and preoperative mechanical ventilation and inotropic use achieved a c-index of 0.81. The observed/expected ratios based on the new risk model were 1.37 (95% confidence interval, 1.18-1.58), 1.21 (1.08-1.33), 1.04 (0.94-1.14), and 0.78 (0.71-0.86) in groups A, B, C, and D, respectively. In the per-procedure analysis, the observed/expected ratios of the Rastelli, coarctation complex repair, and arterial switch procedures in group A were all more than 3.0.The risk-adjusted mortality rate for low-volume hospitals was high for not only high-risk but also medium-risk procedures. Although the overall mortality rate for congenital heart surgeries is low in Japan, the observed volume-mortality relationship suggests potential for improvement in surgical outcomes.

A retroaortic innominate vein (RAIV) is a rare anomaly that passes posterior to the ascending aorta to join the superior vena cava and is associated with congenital heart disease (CHD). The RAIV and normal left innominate vein (LIV) rarely duplicate. The etiology of the RAIV and its relationship with CHD remains unknown. We report a case involving a 1-month-old baby girl with RAIV and supracardiac total anomalous pulmonary venous connection (TAPVC). Transthoracic echocardiogram demonstrated a pulmonary venous confluence (CPV) posterior to the left atrium, an abnormal vertical vein (VV) that originated from the CPV, and a normally positioned LIV. Three-dimensional cardiac computed tomography revealed the VV and RAIV to which it merged. This is the first reported case of a combination of RAIV and isolated TAPVC. We speculate that the VV is connected to the CPV during fetal life, thus leaving the RAIV behind. The RAIV may be detected in various forms with the development of new diagnostic imaging methods. Although a RAIV itself does not require treatment, establishing a correct diagnosis before invasive tests and procedures are performed can help prevent unexpected complications.

Recently, the outcome of severe Ebstein anomaly (EA) has improved with the prevalence of the Starnes procedure. However, time-dependent changes in the size of the central pulmonary artery (PA) have not been fully understood. A retrospective chart review of patients with EA who underwent the Starnes procedure during the neonatal period and those with pulmonary atresia with intact ventricular septum (PAIVS) was performed at Fukuoka Children's Hospital. There were 14 patients in the severe EA group and 36 in the PAIVS group, with mean observational periods of 3.8 and 4.2 years, respectively. No significant difference in survival was observed between the groups. However, the mean size of the central PA was smaller in the severe EA group at each surgical stage (after systemic-to-pulmonary shunt, after the bidirectional Glenn procedure, and after the Fontan procedure). A significantly larger ventricular volume was observed in the severe EA group after the Fontan procedure. The growth of the central PA was poor in patients with severe EA. Patients with severe EA should be carefully monitored in this regard both before and after undergoing the Fontan procedure. Further studies regarding long-term prognosis are expected.

Patients with Down's syndrome (DS) are generally regarded as not being good candidates for the Fontan procedure. However, detailed hemodynamic changes over time are not fully clarified. A retrospective chart review of all patients with DS who underwent the Fontan procedure and 5 times that number of Fontan patients without DS performed in Fukuoka Children's Hospital and Kyushu University Hospital. Seven Fontan patients with DS were identified, and 35 Fontan patients without DS were recruited. During the mean observational periods of 14.7 years and 15.0 years (DS and non-DS, respectively) after the Fontan procedure, only one DS patient died. Central venous pressure (CVP) and transpulmonary pressure gradient significantly increased, and arterial oxygen saturation significantly decreased over time in DS patients after the Fontan procedure compared with those without DS. CVP in DS patients after the Fontan procedure increased over time compared with non-DS patients. Better management including the efficacy of Pulmonary arterial hypertension-specific therapy should be clarified in further studies.

Left pulmonary artery stenosis is a well-recognized complication following the Norwood procedure. We herein report two cases in which ascending aortic extension was performed to enlarge the retroaortic space in children with left pulmonary artery stenosis after the Norwood procedure. We used graft interposition in the ascending aorta to increase the retroaortic apace and concomitantly performed extended left pulmonary artery reconstruction. This procedure obtains a more balanced distribution of the pulmonary blood flow, which is crucial to achieve good Fontan circulation.

Various pathologies of heterotaxy should be diagnosed by documenting the arrangement of the cardiac structures and other organs. They carry functional derangements of cardiovascular systems and abdominal organs, such as atrioventricular valve dysfunction, pulmonary vein obstruction, arrhythmia, and intestinal malrotation. An appropriately organized management of these malformations is pivotal for improving outcomes.

We report a case of symptomatic and progressive enlargement of a huge left atrial appendage aneurysm in a 29-day-old infant. The aneurysm was detected by fetal echocardiography and exertional dyspnea developed during the neonatal period. The aneurysm was successfully resected by decompressing the aneurysm using cardiopulmonary bypass without cardiac arrest. Our findings suggest that fetal echocardiography enables early diagnosis of the rare left atrial appendage aneurysm, and early surgical resection may protect affected patients from life-threatening symptoms.

Ventricular septal defect is the most frequent congenital heart disease and it exists as an isolated form or combines with other complex cardiovascular malformations. Soto's classification is based on the location and extension of the defect and it is most useful and comprehensive for a surgeon. Ventricular septal defect closure is one of the basic surgical procedures that a congenital heart surgeon must master first. In order to perform a perfect patch closure of the defect without making complications such as residual interventricular shunt, tricuspid valve regurgitation, or atrioventricular block, surgeon must acquire, ① preoperative and intraoperative accurate assessment and understanding of the anatomical features of the defect, ② effective intraoperative exposure of the defect, and ③ safe and assured stitch and ligation.

We examined the outcomes following mitral valve replacement with bileaflet mechanical prosthetic valve in children and identified the predictors for mortality and reoperation. Medical records from 49 children who underwent mitral valve replacement between 1982 and 2015 were reviewed retrospectively. Median age and body weight at initial mitral valve replacement were 2.4 years and 9.7 kg, respectively. The median follow-up was 13 years. Surgical results and predictors for mortality and reoperation were investigated. There was no operative mortality; eight late deaths occurred. The actuarial survival rates were 89.5%, 84.2%, and 80.7% at 5, 10, and 15 years, respectively, after initial mitral valve replacement. The actuarial freedom rates from related complications were 89.5%, 78.3%, and 70.7% at 5, 10, and 15 years, respectively. Nineteen patients required 1st re-mitral valve replacement at a median of 5.9 years; six of these 19 required 2nd re-mitral valve replacement at a median of 8.9 years after 1st re-MVR. The actuarial freedom rates from re-mitral valve replacement were 86.0%, 56.8%, and 44.2% at 5, 10, and 15 years, respectively. No predictor for death was determined; however, the predictor for re-mitral valve replacement was initial valve diameter less than 19 mm. Survival outcomes among children after mitral valve replacement with bileaflet mechanical prosthetic valve in biventricular heart were satisfactory. However, complications, including re-mitral valve replacement, were frequent and the predictor was of a small prosthesis size.

We herein report two cases of progressive left ventricular outflow obstruction after primary repair of arch obstruction and ventricular septal defect that was successfully resolved with Yasui conversion. Patients who require surgical reintervention for progressive left ventricular outflow tract (LVOT) obstruction after primary biventricular repair of interruption of the aortic arch or coarctation of the aorta complex are occasionally experienced. The modified Konno procedure and Ross operation are well recognized as useful for these cases. However, in some patients, these procedures are difficult to perform because of anatomic restrictions or previous procedures. Although the indications are limited, the Yasui conversion is a safe, simple, and useful option for LVOT obstruction after primary biventricular repair.

BackgroundsThe aim of this study was to assess the impact of age at bidirectional cavopulmonary anastomosis on haemodynamics after total cavopulmonary connection.MethodsWe conducted a retrospective analysis of 100 consecutive patients who underwent total cavopulmonary connection from 2010 to 2014. All patients had previously undergone bidirectional cavopulmonary anastomosis. These patients were classified into two groups according to age at bidirectional cavopulmonary anastomosis: younger group, 6 months (n=67).ResultsThe proportion of hypoplastic left heart syndrome was higher in the younger group (48 versus 4%). After total cavopulmonary connection, the chest tube period was longer in the younger group (10.1±6.6 versus 6.7±4.5 days; p=0.009). Catheterisation 6 months after total cavopulmonary connection revealed that pulmonary artery pressure was higher (11.5±1.9 versus 10.4±2.1 mmHg; p=0.017) and Nakata index was lower (219±79 versus 256±70 mm2/m2; p=0.024) in the younger group. In patients with a non-hypoplastic left heart syndrome, there was no difference in post-operative haemodynamics between two groups, but the total amount of chest drainage after total cavopulmonary connection was larger in the younger group (109±95 versus 55±40 ml/kg; p=0.044).ConclusionsEarly bidirectional cavopulmonary anastomosis did not affect the outcome of total cavopulmonary connection. Longer chest tube period, smaller pulmonary artery, and higher pulmonary artery pressure after total cavopulmonary connection were recognised in early bidirectional cavopulmonary anastomosis patients, especially in hypoplastic left heart syndrome.

The growth of the neoaortic root after the arterial switch operation for the transposition of the great arteries remains unclear. This study aimed to investigate the growth of the neoaortic root and identify risk factors for neoaortic root dilatation.Serial angiographic measurements of the neoaortic root for at least 10 years were evaluated in 145 patients. A total of 1,876 measurements of the sinuses of the Valsalva and the neoaortic annuli were obtained. A linear mixed effects model was used for z-score analysis, including evaluation of risk factors for neoaortic root dilatation. To assess changes in the time course of neoaortic root absolute diameters, a nonlinear mixed effects model with a growth curve model was used.The growth curve revealed progressive growth of the neoaortic root during somatic growth and stabilization in adulthood without normalization. The growth rates of the sinus and annulus were 0.0046 and 0.029 z-score per year, respectively. The sinus and annulus were estimated to grow up to 47 ± 1 mm and 31 ± 1 mm, respectively. Major risk factors for neoaortic root dilatation were double-outlet right ventricle (parameter estimate [PE] = 2.1, 95% confidence interval [CI] = 1.5 to 2.7, p 0.0001 for sinus; PE = 1.2; 95% CI = 0.7 to 1.6, p0.0001 for annulus) and presence of neoaortic valve insufficiency (PE = 0.9; 95% CI = 0.4 to 1.5; p0.001 for sinus; PE = 1.6, 95% CI = 1.2 to 2.0, p0.0001 for annulus).The risk for neoaortic root dilatation was common. Long-term surveillance is mandatory, particularly in patients with double-outlet right ventricle and neoaortic valve insufficiency.

Infants with hypoplastic left heart syndrome (HLHS) are at high mortality especially when they are associated with bradyarrhythmias. However, the risk factor of developing high-grade atrioventricular block (HAVB) is still unclear. Seventy-three patients with HLHS in our institutions from 2002 to 2011 were enrolled. The survival rate was assessed by the anatomical types, treatments, occurrence of HAVB, severe tricuspid regurgitation (TR), and restrictive atrial septal defect (ASD) along with electrocardiogram findings at birth. There were 23 (32%) cardiogenic and 7 (10%) non-cardiogenic deaths. The occurrence rate of HAVB but not severe TR or restrictive ASD was higher in 30 deceased patients than in 43 survived patients [7 (23%) vs. 1 (2.3%), p = 0.0038]. The overall mortality rate was higher in patients with HAVB than in those without it (p = 0.0002). Of 7 deceased patients with HAVB, 6 HAVB occurred within 10 days post-surgery, and 3 HAVB led to the early death. The mortality rate of patients with prolonged PR (≥ 0.15 s) but not wide QRS (> 0.08 s) or prolonged QTc (> 0.43 s) at birth was higher than each without it (p = 0.0106). Multivariate analysis indicated that prolonged PR but no other variables was independently associated with the mortality (hazard ratio: 2.948, p = 0.0104). Prolonged PR at birth in HLHS infants predicts the development of fatal HAVB.

Uhl anomaly is an extremely rare condition, and herein, we present a case of prenatally diagnosed Uhl anomaly to illustrate the associated echocardiographic features. Fetal echocardiography at 26 weeks indicated an enlarged right ventricle, severe tricuspid and pulmonary regurgitation, and ruptured ventricular septal aneurysm, resulting in a circular shunt. At 36 weeks, based on the presence of a thin ventricular wall and absence of apical trabeculation, a diagnosis of Uhl anomaly was made. The neonate, delivered by cesarean section, underwent main pulmonary artery ligation to eliminate the circular shunt immediately after birth. Moreover, he underwent ductus arteriosus ligation and received a pulmonary artery to aorta shunt for progressive lung congestion. He is currently well and awaiting a staged Fontan operation involving a bidirectional Glenn procedure. Understanding and accurate prenatal diagnoses of Uhl anomaly may help in parental counseling, planning appropriate perinatal care, and increasing the chances of survival.

OBJECTIVE: To review the surgical outcome of the Yasui operation in patients with adequate-sized ventricles and ventricular septal defect (VSD) associated with obstructions of the aortic arch and left ventricular outflow tract (LVOT). METHODS: Since 1985, 17 patients have undergone the Yasui operation at our institution. Interrupted aortic arch was present in 11 patients and coarctation of the aorta/hypoplastic arch was present in 6. Twelve patients had aortic stenosis, and 5 patients had aortic atresia. The minimum diameter of the LVOT and the z-score in patients with aortic stenosis were 3.7 ± 0.4 mm and �9.2 ± 1.2, respectively. Primary repair was performed in 6 patients, and 11 patients were staged, with bilateral pulmonary artery banding (PAB) in 8, arch repair with PAB in 2 and Norwood operation in 1. The mean age and body weight at the time of the Yasui operation was 4.7 ± 5.3 months and 4.5 ± 1.8 kg, respectively. The ascending aorta and aortic arch were reconstructed by Damus–Kaye–Stansel (DKS) anastomosis with graft interposition in 2, DKS with direct anastomosis in 6 and Norwood-type reconstruction in 9. VSD was enlarged in 6 patients. Right ventricle to pulmonary artery continuity was established with a valved conduit in 14 patients, the Lecompte manoeuvre in 2 patients and another method in 1 patient. The mean duration of the follow-up was 7.6 ± 9.2 years. RESULTS: There was 1 early death due to myocardial infarction and 1 late death due to non-cardiac cause. The actuarial survival at 10 years was 87.8%. Six patients underwent reoperation, including 5 conduit exchanges, 2 LVOT repairs and 2 aortic arch repairs. The freedom from reoperation for all causes at 5 and 10 years were 71.3 and 28.5%, respectively. In the last echo study, LVOT flow velocity was 1.2 ± 0.8 m/s, and neoaortic valve regurgitation was mild in 1 patient and trivial or absent in the remaining patients. CONCLUSIONS: The results of the Yasui operation were excellent, showing low mortality and good mid-term left ventricular function without outflow tract stenosis or neoaortic valve insufficiency. Bilateral PAB as initial palliation is a useful option in symptomatic neonates.

Objectives: Modifications of the Fontan procedure are applied to a wide range of complex congenital heart defects with single ventricle physiology. We examined the pathway and the clinical results of extracardiac conduit-total cavopulmonary connection (EC-TCPC) for a malpositioned heart with apicocaval juxtaposition. Methods: Of the 365 patients who underwent EC-TCPC since 1994, 56 patients with a malpositioned heart with apicocaval juxtaposition were included in this retrospective study (group 1). The pathway for the EC was selected after careful consideration of the results of preoperative angiography and computed tomography, as well as intra-operative findings. A concurrent group of 299 patients undergoingEC-TCPC without apicocavaljuxtaposition was used as a control group (group 2). The mean follow-up periods for groups 1 and 2 were 5.5 years (range: 0—12 years) and 5.5 years (range: 0—14 years), respectively. Results: The mean age at operation was 4.2 3.2 years and the median size of the conduit was 18 mm (range: 16—20 mm). In 30 patients, the conduit was placed between the inferior venacava (IVC) and the opposite side of the pulmonary artery crossing the vertebra. In another 25 patients, the conduit was positionedbehind the ventriclebetweentheIVCandonthesamesideas thepulmonaryartery(PA).TherewasonepatientwhohadaY-styleconduitplacedbetweenthe IVC and right and left PAs behind the ventricle. In group 1, there were no early deaths; three patients died in the intermediate term due to gastric bleeding in one, haemoptysis in another and sudden death in yet another. None of the patients developed conduit stenosis or pulmonary venous obstruction, and no patient required re-operation. Three patients developed late complications, including arrhythmias requiring medication in twoandsubduralhaematomainone.Theincidenceofdeathorlatecomplicationsdidnotdifferamongthepathwaysofconduits.Ingroup2,there were eight late deaths, and 27 patients developed late complications. The Kaplan—Meier survival rate was 93.5% at 5 and 10 years in group 1, and 97.3% at 5 years and 96.1% at 10 years in group 2 (log-rank test, P = 0.29). The haemodynamics in groups 1 and 2 during the intermediate term were identical with respect to IVC pressure (8.9 2.5 in group 1 and 9.6 2.6 mmHg in group 2), left ventricular end-diastolic pressure (4.4 and 4.3 mmHg, respectively), cardiac index (3.3 and 3.4 l min 1 m 2 , respectively) and arterial oxygen saturation (94 and 94.2%, respectively). No patient in either group had a pressure gradient >2 mmHg between the IVC and central PA. Postoperative catheterisation data showed no significant differences in haemodynamics between the conduit pathways in group 1. Conclusions: EC-TCPC can be performed in children with apicocaval juxtaposition with excellent mid-term outcomes compared with other Fontan candidates. Based on individual cardiac anatomy, the pathway of the EC behind the ventricle or crossing the vertebra can be used without conduit stenosis or pulmonary venous obstruction. # 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Objective: Intermediate/long-term results after aortic valve replacement using bileaflet mechanical valve in children should be clarified as a standard of treatment of aortic valve disease in children. Methods: Forty-five patients aged under 15 years underwent 46 aortic valve replacements using bileaflet mechanical prosthetic valve. Patients’ ages ranged from 1 to 15 years (9 years as a median value), and follow-up period was 9.2 years as a median value (maximum 19 years). Results: In situ valve replacement was performed in 21 procedures, while annular enlargement was required in 25 procedures (Nicks 10, Yamaguchi 3, Manouguian 2, Konno 10). All patients except two received prosthesis 19 mm or larger in size. There was one operative death and two late deaths. Two episodes of cerebral infarction, two valve thrombosis, two reoperations, one infective endocarditis, and one sudden death were recognized as valve-related complications in five patients. The reasons for reoperation were prosthesis-patient mismatch in one (Ross procedure) and valve thrombosis in one (re-replacement). At 15 years after the operation, re-replacement free rate, valve-related event free rate and actuarial survival rate were 94 4%, 86 6% and 92 4%, respectively. ThetransprostheticflowvelocityestimatedbyDopplerechocardiographyatthefinalfollow-upwaswellcorrelated withmanufacturedvalvearea index (cm 2 /body surface area). Conclusions: Although aortic annular enlargement was required in more than half of the cases, intermediateterm results after aortic valve replacement using bileaflet mechanical prosthetic valve in children was satisfactory. Indications for alternative treatment such as Ross procedure might be considered in limited cases. # 2008 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Pulmonary artery (PA) distortion significantly compromises the outcome of the staged approach to the Fontan operation in patients with hypoplastic left heart syndrome (HLHS). This retrospective study was designed to investigate the influence of the initial operation on postoperative PA anatomy.Forty-nine patients with HLHS and its variant were enrolled in this study. As an initial palliation, the Norwood operation with a modified Blalock-Taussig (BT) shunt was performed in 12, the Norwood operation with a right ventricle to pulmonary artery (RV-PA) shunt in 31, and bilateral PA banding in 6. The incidence and risk factors of postoperative central pulmonary artery stenosis (PS) were investigated, and the PA configuration was followed up until post-Fontan status.Twenty-two patients (51.2%) had developed central PS after the Norwood operation (33.3% with a BT shunt vs. 58.1% with a RV-PA shunt). The RV-PA shunt with a polytetrafluoroethylene (PTFE) patch at the distal pulmonary stump significantly decreased the central PS (P = 0.035). The PA index after the Norwood operation was not statistically different between the BT and RV-PA shunt groups, although in the RV-PA group it was significantly higher in patients with a PTFE patch on the distal PA stump. PA plasty was performed in 16 patients in the second-stage palliation and in 15 with the Fontan completion. Freedom from PA plasty was significantly lower in the RV-PA shunt group than in the BT shunt group (63.5% vs. 31.1% at 5 years, P = 0.034). Six patients initially palliated with bilateral PA banding had no stenosis at the banding site in the Norwood + Glenn operation, and one patient required stent placement for left PS in the Fontan completion. Post-Fontan catheterization (n = 31) showed central venous pressure of 11.5 +/- 2.6 mmHg, cardiac index of 3.6 +/- 0.8 l/kg/min, and PA index of 194.0 +/- 58.4 mm(2)/m(2); there was no difference between the groups.The incidence of central PS after the Norwood operation was significant, and the shunt type and procedure for the distal PA stump influenced the postoperative configuration of the central PA. With an aggressive surgical approach to central PS, PA anatomy was satisfactory with good hemodynamic variables after Fontan completion. Bilateral PA banding did not cause later vascular deformity.

Objectives No specific prosthetic material is currently recognized as being the most suitable for right ventricular outflow tract reconstruction for congenital heart defects. Prosthetic valves are subject to wear and stress because they do not create vortex flow, which helps natural valves to close. We designed a fan-shaped expanded polytetrafluoroethylene valved conduit and patch with bulging sinuses that create vortex flow, making them more reliable over the long term. Methods Bulging sinuses were formed on a sheet of expanded polytetrafluoroethylene using a specially designed mold. Fan-shaped expanded polytetrafluoroethylene sheets (0.1 mm thick) were anastomosed to the edge of the bulging sinuses as valve leaflets, creating monocuspid, bicuspid, or tricuspid valves. These valves were implanted in 157 patients undergoing right ventricular outflow tract reconstruction (age 16 days to 45.4 years, median 2.0 years), in 48 patients as a conduit, and in 109 patients as a patch. Valve function was followed up by echocardiography for 5.6 to 63.7 months (mean 20.8 months). Results There was no mortality or morbidity, and no patients required reoperation during follow-up. No patients had stenosis, and regurgitation was less than mild in all patients with conduits and moderate in 15 patients (13.8%) with patches, but moderate regurgitation did not further develop during follow-up. Valve motion was fully maintained in all patients. Conclusions The expanded polytetrafluoroethylene valved conduits and patches with bulging sinuses showed excellent early-to-midterm results. The valved conduits and patches seem to be promising alternatives to homografts in right ventricular outflow tract reconstruction. Their function will be followed up further.

Background The predictors of left ventricular outflow tract obstruction (LVOTO) after the repair of coarctation of the aorta or interruption of the aortic arch (CoA/IAA) with ventricular septal defect have been investigated. However, the predictors remain controversial. Methods We performed primary repair of CoA/IAA with ventricular septal defect for 75 patients from 1996 to 2005. Four of the 75 patients died within 5 years after primary repair without relation to LVOTO. The morphology of the aortic valve of 71 survivors was bicuspid in 23 patients and tricuspid in 48 patients. The mean follow-up was 9.2 ± 2.6 years after primary repair. Results There were 12 patients who showed LVOTO of 3.0 m/s or greater after primary repair. All of the 6 bicuspid patients demonstrated valvular aortic stenosis, and all of the 6 tricuspid patients showed discrete subvalvular LVOTO. In 5 of the 6 tricuspid patients, the aortic annular z-score before primary repair was −3.0 or less. A bicuspid aortic valve ( p = 0.016) and the aortic annular z-score of −3.0 or less ( p = 0.019) were significant risk factors for LVOTO after primary repair. At 10 years after primary repair, 82.6% and 95.6% of the bicuspid and tricuspid patients, respectively, were free from reoperation ( p = 0.015). Conclusions The presence of a bicuspid aortic valve and an aortic valve annular z-score of −3.0 or less before primary repair are risk factors for LVOTO, and stenotic bicuspid valves and discrete subvalvular LVOTO are the main causes of LVOTO after primary repair of CoA/IAA with ventricular septal defect. The bicuspid patients more frequently required reoperation than the tricuspid patients.

OBJECTIVES Introduction of the bidirectional Glenn procedure (BDG) in low-risk Fontan candidates would improve clinical outcomes. Over the last decade, not only high-risk Fontan candidates, but all candidates underwent BDG and staged Fontan operation (TCPC) in our hospital. METHODS Three hundred and thirty-three consecutive patients (age range, 42 days to 16 years old) underwent BDG at Fukuoka Children's Hospital Medical Center from 1992 to 2004. Diagnoses included hypoplastic left heart syndrome in 47, pulmonary atresia with intact ventricular septum in 32, tricuspid valve atresia in 35, and other complex univentricular heart defects in 219 patients (right dominant in 166, left dominant in 53). RESULTS There were three hospital deaths and 27 late deaths (five after TCPC). Six patients underwent takedown operation. Two hundred and thirty patients underwent TCPC, while 66 patients were waiting for TCPC. In five patients, completion of TCPC was contraindicated. A univariate analysis revealed that for patients less than six months old, diagnoses besides tricuspid atresia, right ventricular morphology, mean pulmonary arterial pressure, pulmonary vascular resistance, ventricular end-diastolic pressure, atrioventricular valve regurgitation greater than moderate, atrioventricular valvuloplasty/valve replacement in concomitant procedure, and total anomalous pulmonary venous connection repair in concomitant procedure were significant predictors of death, takedown, or out of indication for completion of TCPC. A stepwise logistic regression analysis showed that mean pulmonary arterial pressure and heterotaxy were independent predictors. CONCLUSIONS The staged strategy used for all Fontan candidates provides excellent clinical results. The main risk factor for death, takedown, or out of indication for completion of TCPC was elevated pulmonary arterial pressure. Appropriate surgical interventions such as atrioventricular valvuloplasty and total anomalous pulmonary venous connection repair, before and/or on BDG for the control of pulmonary circulation are of great importance to prevent elevation of pulmonary arterial pressure.

The investigation of the possibility of low-cost SMES is an indispensable theme if that practical use is to be realized. We optimized the system composition by selecting the most suitable charge/discharge rate. We conducted the optimum design on the structure of superconductor and the coil system under special consideration of the material unit price, the total material amount, the manufacturing efficiency, and so on. The pool-cooling double-row solenoid system using NbTi base conductor is the most suitable one as 15kWh-100MW SMES for the system-stabilizing use and 500kWh-100MW SMES for the load-fluctuation-compensating and frequency-controlling use.

Double-orifice left atrioventricular valve (LAVV) is a rare but surgically important anomaly, which is regarded as a risk factor for surgical correction of atrioventricular septal defects (AVSDs).Of 209 consecutive patients with AVSDs, double-orifice LAVV was identified in 19 patients (9.1%, including 7 infants). Preoperative LAVV function, surgical procedures and results, incidence of postoperative LAVV dysfunction and reoperations were reviewed and compared between patients with this valve malformation (group I, n = 19) and those without it (group II, n = 190).There were no operative or late deaths in group I. Preoperative LAVV function was similar in both groups. The cleft was totally closed in 77.2% of group II and 47.1% of group I (p0.01). In partial AVSDs, freedom from postoperative LAVV insufficiency was 77.0% in group II versus 30.5% in group I at 5 years (p = 0.009) and freedom from reoperation was 89.9% in group II versus 58.3% in group I at 5 years (p = 0.012); however, there was no difference in complete AVSDs. None of the infants in group I underwent total cleft closure and 4 of them showed more than moderate LAVV insufficiency postoperatively.Double-orifice LAVV is a significant predictor for postoperative LAVV incompetence and reoperation in partial AVSDs, but not in complete AVSDs. Surgical procedures for the cleft should be individualized with careful intraoperative evaluation of the structure and function of this abnormal valve, especially in partial AVSDs and infants.

This single-institution study aimed to evaluate the early to mid-term outcomes of extracardiac conduit total cavopulmonary connection (EC-TCPC).Between March 1994 and March 2014, 500 patients (median age, 3.4 years) underwent EC-TCPC at our hospital. One hundred and twenty-three patients (24.6%) showed heterotaxy, and fenestration was created in 6 patients (1.2%). The standard institutional treatment policy included postoperative anticoagulation and individualized cardiovascular medication. The mortality and morbidity rates, haemodynamic status, cardiopulmonary exercise capacity and liver examination results during the follow-up period (median, 6.7 years) were retrospectively reviewed.There were 2 early and 17 late deaths. The Kaplan-Meier estimated survival rate was 96.2% at 10 years and 92.8% at 15 years. Bradyarrhythmia and tachyarrhythmia occurred in 19 and 13 patients, respectively. Other late-occurring morbidities included protein-losing enteropathy in 8, thromboembolism in 5, bleeding complications in 6 and liver cirrhosis in 1 patient. The rate of freedom from late-occurring morbidities was 82.1% at 15 years. In the multivariate analysis, heterotaxy was found to be a predictor for mortality (P = 0.02), whereas age at operation was a predictor for new-onset arrhythmias (P = 0.048). In the cardiopulmonary exercise test (n = 312), the peak VO2 was 84.9 ± 17.3% of the predicted value, which tended to decrease with age (R(2) = 0.32) and elapsed time since operation (R(2) = 0.21). Postoperative cardiac catheterization (n = 468; time from surgery, 3.6 ± 4.3 years) showed central venous pressure of 9.9 ± 2.4 mmHg, ventricular end-diastolic pressure of 5.2 ± 3.3 mmHg, cardiac index of 3.4 ± 0.8 l/min/m(2) and arterial oxygen saturation of 94.2 ± 4.8%. In 101 patients who were followed up for ≥10 years, amino-terminal type III procollagen peptide and collagen type IV levels exceeded the normal ranges in 52.9 and 75.2% of patients, respectively, and liver ultrasonography revealed hyper-echoic spots in 43.3% of patients.The early to mid-term outcomes of post-EC-TCPC patients managed with individualized pharmacotherapy were excellent, with low mortality and morbidity rates; however, development of late-occurring morbidities specific to Fontan physiology, including exercise intolerance and liver disease, must be carefully monitored during the long-term follow-up.

We retrospectively reviewed our surgical results in patients with hypoplastic left heart syndrome( HLHS) to investigate the influence of surgical strategy on outcome.Seventy-seven patients with classic HLHS were involved in this study. For the initial palliation, 23 patients underwent Norwood operation with modified Blalock-Taussig (BT) shunt, 23 patients underwent Norwood operation with right ventricle to pulmonary artery( RV-PA) shunt and 31 patients underwent bilateral pulmonary artery banding (BPAB). Surgical results, freedom from Fontan operation, hemodynamic data and incidence of complication after Fontan operation were compared between the 3 groups.BPAB group had more preoperative risk factors than Norwood group. Total actuarial survival was 62.4% at 1 year and 58.2% at 3 and 5 years, and there was no difference between the groups. Freedom from Fontan completion was 59.4% at 3 years and 48.7% at 5 years without difference between the groups. The incidence of intervention for pulmonary artery stenosis was higher in Norwood with RV-PA shunt group(52.9%). Hemodynamic data obtained by cardiac catheterization were similar in the 3 groups, however, end-systolic elastance, which represents ventricular contractility, was lower in Norwood with RV-PA group.Surgical results of Norwood with BT shunt and Norwood with RV-PA shunt were comparably satisfactory, however, there was possible concern of reduced ventricular contractility in RV-PA shunt group. BPAB was a effective and useful initial palliation in high risk cases.

Parameter survey is performed to obtain shape and fundamental specifications for the 15 kW h/100 MW power system stabilizing superconducting magnetic energy storage. Using the scaling law for ideal toroidal coils and J c – B characteristics of several high temperature superconductors, three fundamental specifications to minimize amount of the superconductor are obtained. In the results the optimum parameters of Bi are 4.2 K–16 T for Bi2212 (tape) and 20 K–10 T for Bi2212 (tape). And the optimum parameter of Y123 is 2 T under the condition of 77 K. But Y123 is expected to have another optimum parameter in lower temperature. Assuming an actual conductor shape, structural analysis is performed in the typical case of 4.2 K–16 T. In result, the reinforcement member area is obtained.

Bi-2212/Ag Rutherford cable is expected as a high temperature superconducting magnet for SMES use because its critical current is 3.5 kA at 0 T, 4.2 K and it has the structure of enduring large magnetic force. Tests on the relation of critical current and tensile strain of the strand were performed. It was confirmed that I c and n -value began to decrease when the tensile strain of the strand increased over 0.6%. The share of the load between the strand and the Ni-base metallic core was found by examining the strength of the strand and the cable, and evaluating them with the above data. It was considered that the critical stress of this Rutherford cable was nearly 240 MPa which was necessary to design 20 T-class magnet.

Background . The effects of pulsatile flow on endothelium-derived nitric oxide-mediated vasodilation are not fully elucidated in an in vivo model. Methods . A left ventricular assist device was established in 10 anesthetized dogs with a centrifugal pump and an air-driven pneumatic pump. The systemic circulation was subjected to step changes in the frequency of pulse (0, 30, 60, and 120 bpm with a fixed pulse pressure of 50 mm Hg), and in the amplitude of pulse (0, 20, and 50 mm Hg with a fixed pulse rate of 120 bpm). Hemodynamic variables and calculated total systemic vascular resistance were compared before and after the administration of N G -Nitro-L-arginine Methyl Ester (L-NAME) (20 mg/kg). Plasma NO 2− /NO 3− concentration levels were also measured. Results . Total systemic vascular resistance significantly decreased while plasma NO 2− /NO 3− concentration increased in response to the rise in both pulse rate and pulse pressure. However, L-NAME significantly diminished these effects of pulsatile flow. Conclusions . Both the frequency and the amplitude of pulse wave in the systemic circulation are significant independent stimuli for endothelium-derived nitric oxide-mediated vasodilation in vivo.

A high temperature superconducting magnet using newly developed Bi-2212/Ag Rutherford cable was trial manufactured as a partial model for a 10 T magnet. The developed magnet consists of 4 double pancake coils constructed from a Rutherford cable with a critical current of 3.5 kA at 4.2 K, 0 T. The magnet has inside and outside diameters of 100 mm and 180 mm, respectively and is 75 mm long axially. The coil successfully operated with DC 1.25 kA and 1.5 kJ was stored stably. A maximum magnetic field of 2 T with A current of 1.47 kA was confirmed in liquid helium. Also, the authors performed excitation tests under the high-speed rate of change of magnetic field (0.54 T/S) and 300 cycles simulating alternating operation, and estimated the value of AC losses at 5.3 W using the change of liquid helium level.

The changes in systemic circulation during hyperthermic isolated lower limb perfusion with carboplatin and interferon-beta were investigated in 19 patients with malignant melanoma. The cardiac output (CO) increased significantly (p < 0.01) from 3.81 +/- 0.22 L/min before the procedure to 5.30 +/- 0.49 L/min 1 h after hyperthermic perfusion. The double product (mean arterial pressure x heart rate) also increased significantly (p < 0.01) from 5,145 +/- 372 mm Hg/min to 6,760 +/- 486 mm Hg/min. In some patients, it increased to more than twice the control value. These changes were accompanied by an increase in body temperature, presumably caused by the systemic leakage of both warmed blood and interferon-beta. Blood chemistry data demonstrated no significant changes in the liver or renal function. However, the serum CPK level increased markedly on the first postoperative day, and persisted for 1 week, thus suggesting that some muscle damage occurred during the procedure. There was no operative death or severe complications. From these data, we concluded that hyperthermic isolated limb perfusion with interferon-beta is a relatively safe therapeutic method for malignant melanoma of the extremities. However, care should be taken in patients with ischemic heart disease who may suffer a heart attack due to the rapid increase in cardiac work during the procedure.

To determine the effects of nonpulsatile left heart bypass (LHB) on sympathetic nerve activity the renal sympathetic nerve activity (RSNA) was directly measured in rabbits. In anaesthetized rabbits ( n=6), LHB was instituted with a centrifugal pump. Before and during LHB, heart rate (HR), arterial pressure (AP), central venous pressure (CVP), left atrial pressure (LAP) and spike counts of RSNA were measured. After bilateral vagotomy (section of the afferent inputs of the cardiac reflex arc) the same parameters were measured before and during LHB. Data were obtained at the same level of mean AP before and during LHB. Mean AP and CVP were not changed by LHB before and after vagotomy. LAP was significantly decreased by LHB before and after vagotomy. RSNA and HR were not significantly changed by LHB before and after vagotomy. Our results clarify the complex baroreflex control during nonpulsatile LHB and indicate that RSNA is not altered by LHB.

OBJECTIVES: We sought to examine our long-term results of mitral valve (MV) repair with expanded polytetrafluoroethylene (ePTFE) sutures and to determine the predictors for the outcome of this procedure. METHODS: Between 1995 and 2011, MV repair with chordal reconstruction by artificial chordae was achieved in 78 patients (34 males and 44 females). Median age at repair was 1.5 years (range 3.6 months–13.4) and weight was 9.1 kg (2.5–31.4). The mean follow-up was 8.3 years. A Cox proportional hazards model was used to analyse the risk factors for a composite outcome of death, conversion to other MV repair techniques or MV replacement, reoperation on MV and recurrent mitral regurgitation (MR). RESULTS: According to Carpentier classification, 65 (83.3%) patients were Type 2 and 13 (16.7%) were Type 3. Mitral annuloplasty was performed in all cases, except 2. During MV repair, 8 (10.3%) patients were ineffective with artificial chordae and converted to other techniques. Six (7.7%) patients underwent MV reoperation (three repairs and three replacements). Freedom from MV reoperation was 92.5 and 90.4% at 5 and 10 years, respectively. There was 1 in-hospital death. At the latest follow-up, moderate or more MR was observed in 3 (3.8%) patients. Risks for the composite outcome were low body weight at operation and Carpentier classification Type 3. CONCLUSIONS: MV repair with artificial chordae in infants and children is safe and effective and associated with a low reoperation rate. Further investigation into the long-term durability and biological adaptation of ePTFE sutures after patient growth is mandatory.

We have experienced a case of left atrial (LA) myxoma with rapid progression of congestive heart failure and ensuing multiple organ insufficiency. After the tumor excision, the hemodynamic derangement was totally corrected and the patient dramatically recovered from kidney, liver, and lung insufficiency. Specifically, pulmonary artery pressure (PAP), pulmonary capillary wedge pressure (PCWP), and central venous pressure (CVP) significantly decreased in the intensive care unit. Administration of prostaglandin E1 (PGE1) was effective in treating residual pulmonary hypertension.

OBJECTIVES: We reviewed our 28 years of experience of arterial switch operation (ASO) for transposition of the great arteries to investigate late sequelae of this procedure. METHODS: 387 patients who underwent ASO from 1984 to 2010 were included in this retrospective study. The longitudinal data were estimated by the Kaplan–Meier method and compared using a log-rank test. Risk factors for late sequelae were analysed by the multivariable Cox proportional hazards model. RESULTS: The mean follow-up time was 10.0 years. There were 13 early deaths and 17 late deaths. All late deaths were within 1 year, except for three patients. Actuarial survival was 92.2 and 91.6% at 10 and 20 years, respectively. Sixty-six patients (17.1%) had developed pulmonary stenosis (PS) and 29 patients (7.5%) had developed moderate or more aortic insufficiency (AI) during follow-up. Selective coronary angiography was performed in 210 patients (54.3%) at 9.6 ± 5.1 years after ASO. Left main tract occlusion was found in 2 patients (2/210; 1.0%) and hypoplastic left coronary artery was found in 10 patients (10/210; 4.8%). Among these 12 patients, 8 patients were asymptomatic. Re-operation was performed in 76 patients (19.6%), pulmonary artery plasty for PS in 58 patients (15.0%), aortic valve replacement for AI including two Bentall operations in 9 patients (2.3%) and others. Freedom from re-operation was 78.2 and 62.8% at 10 and 20 years, respectively. The risk factor for PS was the use of equine pericardium for reconstruction (P< 0.0001). Factors associated with moderate or more AI was the presence of left ventricular outflow tract obstruction (P= 0.004). There were no risk factors for late coronary lesions. Three hundred and forty surviving patients (340/357; 95.2%) were in NYHA functional class I. Treadmill test, which was performed on 217 patients (56.1%) at 14.3 ± 5.4 years after ASO, revealed that the maximum heart rate was 97.5 ± 7.6% of normal and peak oxygen consumption was 105.2 ± 20.5% of normal. CONCLUSIONS: ASO was performed with satisfactory results in the overall survival and functional status. PS was the main reason for re-operation. Coronary lesions can appear late without any symptoms. Benefits of ASO can be achieved by long-term follow-ups of PS, AI and coronary lesions.