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OBJECTIVES To more accurately predict the optimal pulmonary artery (PA) reconstruction procedure (Lecompte manoeuvre or original Jatene procedure) during the arterial switch operation (ASO), we focused on the horizontal sectioning (HS) angle between the left hilum PA and the great arteries using preoperative computed tomography (CT) imaging. METHODS We defined the HS angle α (β) as the angle between the tangential line from the posterior (anterior) wall of the left PA at the hilum to the left anterior (right posterior) surface of the main PA and the tangential line from the left surface of the ascending aorta to the left anterior (right posterior) surface of the main PA. We identified 14 consecutive patients diagnosed with transposition of the great arteries (TGA) or TGA-type double-outlet right ventricle who underwent preoperative CT imaging. The original Jatene or Lecompte procedure was used for nine (OJ group) and five (L group) patients. Relationships of the great arteries of the OJ and L groups were side-by-side in eight and two patients, oblique in one and one patient, and anteroposterior in zero and two patients, respectively. RESULTS In the OJ group, β was greater than α in all patients. The median α/β value was 0.618. In group L, α was greater than β in all patients. The median α/β was 1.307. Left PA stenosis caused by stretching was not observed in the L group. Coronary obstruction was not identified in the OJ group. Left PA stenosis behind the neo-ascending aorta was observed in one patient in the OJ group and required reoperation. CONCLUSIONS The HS angle may be a useful predictor of optimal intraoperative PA reconstruction during ASO, especially for side-by-side or oblique relationships.

The objectives of this study were to evaluate the results when tissue-engineered vascular grafts (TEVGs) are used as alternatives to autologous pericardium for surgically augmenting the pulmonary artery (PA) or aortic valve.TEVG molds were embedded into subcutaneous spaces for more than 4 weeks preoperatively. Since 2014, 6 patients have undergone PA reconstruction, whereas 1 has undergone aortic valve plasty (AVP) with TEVGs. The time from mold implantation to the operation was 8.9 (range, 6.0-26.4) months. The age and body weight at the time of operation were 2.7 (range, 1.8-9.2) and 11.6 (range, 7.9-24.4) kg, respectively. Concomitant procedures comprised the Rastelli, palliative Rastelli, and Fontan operations in 2, 2, and 1 patient, respectively.The median follow-up period was 14.4 (range, 3-39.6) months. There were no early or late mortalities. Moreover, there were no TEVG-related complications, including aneurysmal changes, degeneration, and infection. In 5 patients who underwent PA augmentation, the postoperative PA configuration was satisfactorily dilated. The reconstructed aortic valve function was good in the patient who underwent AVP. Decreased leaflet flexibility due to leaflet thickening was not observed. One patient had postoperative PA re-stenosis; therefore, re-PA augmentation with TEVGs was performed. On histological examination, TEVGs consisted of collagen fibers and few fibroblasts, and elastic fiber formation and/or smooth muscle cells were not observed.The midterm results of PA reconstruction and AVP with TEVGs were satisfactory. TEVGs might be a useful alternative to autologous pericardium in pediatric cardiovascular surgeries that often require multistage operations.

Cardiac dysfunction commonly occurs in congenital heart block associated with maternal anti-SSA antibodies, especially after pacemaker implantation. We report the case of a 4-year-old girl with antibody-associated congenital heart block and a large secundum atrial septal defect who presented with significant cardiac dysfunction 4 years after pacemaker implantation. Histological findings were useful for determining the course of treatment and perioperative risk of intracardiac repair.

We developed a handmade expanded polytetrafluoroethylene (ePTFE) pulmonary valvular conduit (PVC) with bulging sinuses and a fan-shaped ePTFE valve for right ventricular outflow tract (RVOT) reconstruction. We aimed to investigate the results of this device in this multicenter study. From 2001 to 2020, 1776 patients underwent RVOT reconstruction using ePTFE PVCs at 65 institutions in Japan. The median age and body weight were 4.1 years (range, 3 days to 67.1 years) and 13.3 (range, 1.8-91.3) kg, respectively. The median PVC size was 18 (range, 8-24) mm. The median Z-value of the ePTFE PVC was 1.1 (range, -3.8 to 5.0). The ePTFE PVC conditions were investigated by cardiac echocardiography and catheterization. The median follow-up period was 3.3 years (range, 0 day to 16.2 years). There were only 9 cases (0.5%) with PVC-related unknown deaths. Reintervention was performed in 283 patients (15.9%), and 190 patients (10.7%) required explantation. Freedom from reintervention and explantation at 5/10 years were 86.7/61.5% and 93.0/69.1%, respectively. At the latest echocardiography, PVC regurgitation grade was better than mild in 88.4% patients. The average peak RVOT gradient was 15.7 ± 15.9 mm Hg at the latest cardiac catheterization. ePTFE PVC infection was detected in only 8 patients (0.5%). Relative stenosis due to somatic growth was the most common cause of PVC explantation. The performance of ePTFE in terms of durability, valvular performance, and the resistance against infection is considerable and may replace conventional prosthetic materials. Further improvement of the ePTFE membrane is essential to prevent valvular dysfunction.

Blood flow imaging becomes an emerging trend in cardiology with the recent progress in computer technology. It not only visualizes colorful flow velocity streamlines but also quantifies the mechanical stress on cardiovascular structures; thus, it can provide the detailed inspections of the pathophysiology of diseases and predict the prognosis of cardiovascular functions. Clinical applications include the comprehensive assessment of hemodynamics and cardiac functions in echocardiography vector flow mapping (VFM), 4D flow MRI, and surgical planning as a simulation medicine in computational fluid dynamics (CFD).For evaluation of the hemodynamics, novel mathematically derived parameters obtained using measured velocity distributions are essential. Among them, the traditional and typical parameters are wall shear stress (WSS) and its related parameters. These parameters indicate the mechanical damages to endothelial cells, resulting in degenerative intimal change in vascular diseases. Apart from WSS, there are abundant parameters that describe the strength of the vortical and/or helical flow patterns. For instance, vorticity, enstrophy, and circulation indicate the rotating flow strength or power of 2D vortical flows. In addition, helicity, which is defined as the cross-linking number of the vortex filaments, indicates the 3D helical flow strength and adequately describes the turbulent flow in the aortic root in cases with complicated anatomies. For the description of turbulence caused by the diseased flow, there exist two types of parameters based on completely different concepts, namely: energy loss (EL) and turbulent kinetic energy (TKE). EL is the dissipated energy with blood viscosity and evaluates the cardiac workload related to the prognosis of heart failure. TKE describes the fluctuation in kinetic energy during turbulence, which describes the severity of the diseases that cause jet flow. These parameters are based on intuitive and clear physiological concepts, and are suitable for in vivo flow measurements using inner velocity profiles.

Background Excellent outcomes of right ventricle to pulmonary artery conduits with expanded polytetrafluoroethylene (ePTFE) valves have been reported. The purpose of this study was to evaluate the outcomes of the different material conduits with tricuspid ePTFE valves. Methods Forty-one consecutive patients who received right ventricle to pulmonary artery conduit with tricuspid ePTFE valves for biventricular repair between April 2004 and December 2016 were studied. The conduits made of autologous pericardial roll or xenograft roll were used in 22 patients (group P) and the conduits made of ePTFE tube were used in 19 patients (group G). The conduit reoperation and the conduit dysfunction were analyzed. Results During the median follow-up of 5.8 years, no death related to the conduit was observed. There were four reoperations (three in group P and one in group G). Freedom from conduit reoperation at 5 years was 100% in both groups (P = .30). Freedom from more than moderate conduit stenosis at 5 years after operation was not significantly different between groups (46.9% in group P vs 76.3% in group G, P = .23) even though the group G conduits were significantly smaller and freedom from more than moderate conduit regurgitation at 5 years was significantly better in group G (63.3% in group P vs 94.1% in group G, P = .04). Conclusions The conduit with ePTFE valves in the ePTFE tubes had better conduit function compared with the conduit with autologous pericardial or xenograft roll, especially in terms of conduit regurgitation.

Left ventricular aneurysms are rarely encountered in pediatric patients. A 4-year-old boy was diagnosed with severe mitral regurgitation and a posterior left ventricular aneurysm associated with a viral infection. The aneurysm was surgically resected and plicated longitudinally with a combination of an interrupted mattress and continuous over-and-over sutures with an outer felt reinforcement. The mitral regurgitation was reduced to a trivial degree postoperatively. The patient’s postoperative recovery was good. He was discharged 31 days after surgery without cardiac dysfunction or lethal arrhythmia. Considering the location and spread of the aneurysm, an appropriate surgical procedure should be adopted for pediatric patients.

OBJECTIVES Pulmonary artery (PA) compression by the neoaorta is a serious complication after the Norwood-type palliation (NP) for hypoplastic left heart syndrome. Either excess patch tailoring or limited use of autologous tissue may cause narrowing of the subaortic space. The chimney technique could theoretically provide a wide subaortic space. METHODS Twenty-nine patients with both pre- and post-NP computed tomography data available of the 37 consecutive patients who underwent NP in our institution were reviewed. Arch height, arch width, sinus of Valsalva diameter, area under the neoaortic arch and arch angle were measured. These patients were divided according to the neo-arch reconstruction technique, chimney reconstruction technique (CR) or conventional direct reconstruction technique (DR). RESULTS Median age and weight at NP were 2.1 months and 3.5 kg, respectively. Twenty-two patients underwent previous bilateral PA banding. During NP, 17 CR and 12 DR were performed. Four patients in the DR group developed PA compression. No neoaortic arch dilatation was found in either group. Post-NP arch width, area under the neo-arch and the arch angle were significantly larger in the CR group. Pre-NP arch height was significantly smaller in DR patients with PA compression than in those without. CONCLUSIONS The chimney technique decreased the risk of PA compression and provided a wider subaortic space and a less acute arch angle. This technique had no short-term effect on the neoaortic root. Small preoperative arch height is a potential risk factor for PA compression in DR, and the chimney technique could be an effective solution.

Cone reconstruction for Ebstein’s anomaly, although effective, is challenging in neonatal cases. Very few reports have discussed recurrent regurgitation. Herein, we report a successful redo tricuspid valve repair for recurrent regurgitation 5 years after cone reconstruction. A 5-year-old boy underwent cone reconstruction for Ebstein’s anomaly in the neonatal period. Although tricuspid regurgitation reduced initially, it subsequently worsened. The mechanisms of regurgitation are dilatation of the anteroseptal commissure, indentation in the septal leaflet, and foreshortening of the anterior leaflet. Augmentation of the anterior leaflet using an elliptic autologous pericardium and mending of the gaping commissure and indentation markedly reduced the regurgitation.

We evaluated the application of in vivo tissue-engineered vascular graft (in vivo TEVG) in pulmonary artery (PA) reconstruction as a substitute for autologous pericardium. From July 2017 to April 2020, 4 patients (male:female = 2:2) with major aortopulmonary collateral arteries underwent PA reconstruction with in vivo TEVGs. Graft moulds were embedded into the subcutaneous spaces in the first palliative surgery. In the second surgery used in vivo TEVGs were used as patch materials to treat PA stenosis. Preoperative and postoperative PA configurations were evaluated by computed tomography. Patients’ median age and body weight were 1.6 (1–4) years and 8.7 (7.3–15.4) kg, respectively. Two patients underwent PA reconstruction during staged repair and 2 underwent reconstruction during definitive repair. One patient had postoperative PA restenosis due to bronchial compression; re-PA reconstruction with in vivo TEVGs was performed. On histological examination, the in vivo TEVG wall mainly comprised collagen fibres and a small number of fibroblasts. The midterm results of this technique are satisfactory. in vivo TEVGs could be a promising alternative to autologous pericardium for paediatric cardiovascular surgeries requiring multi-stage operations. Clinical trial registration ERB-C-162

To address various problems with conventional surgical procedures for transposition of the great arteries with left ventricular outflow obstruction, the aortic translocation procedure has been reconsidered as an alternative surgical procedure. We developed another innovative translocation technique, the half-turned truncal switch operation, to make use of various anatomical characteristics. The truncal block involving both semilunar valves is harvested en bloc and anastomosed to the opposite ventricular outflow tract after a half-turn. Temporarily detached coronary arteries are re-anastomosed to the reversed aortic wall defects. The key advantage of this half-turned truncal switch operation is the creation of hemodynamically faultless right and left ventricular outflow tracts including competent valve functions and a coronary circulation ensured by the posteriorly translocated aortic valve, the maximally used autologous pulmonary valve, and anastomosis of the coronary arteries to the confronting aortic wall defects.

This report describes the case of a 3-year-old boy with supravalvular aortic stenosis after an arterial switch operation in whom the stenosis was successfully repaired using an ascending sliding arch aortoplasty without using a patch. Because patches were avoided, growth of the surgical site is expected. Ascending sliding arch aortoplasty and longitudinal expansion of the pulmonary bifurcation are useful for relieving stenosis and preventing supravalvular aortic stenosis recurrence after an arterial switch operation.

Purpose This study sought to evaluate the histologic and mechanical properties of autologous in vivo tissue-engineered vascular grafts (in vivo TEVGs) used for pediatric heart surgery. Description Molds of in vivo TEVGs made of silicone drain tubes were embedded into subcutaneous spaces in 2 boys during their first operation and were used as patch materials to treat pulmonary artery stenosis during the second operation. The remaining pieces of the patches were evaluated histologically and mechanically. Evaluation In vivo TEVGs had very smooth luminal surfaces, and their walls mainly comprised collagen fibers and small numbers of fibroblasts. Mean wall thickness was 200 μm, mean suture retention strength was 2.26 N, and burst pressure was 3057 mm Hg. Conclusions Human in vivo TEVGs mainly comprise collagen fibers, and their mechanical properties prove them safe for pulmonary arterioplasty. Therefore, human in vivo TEVGs may be promising alternatives to autologous pericardium for pediatric cardiovascular surgical procedures that often require multistage operations.

OBJECTIVES The aim of this study was to clarify the impact of valved systemic ventricle–pulmonary artery (SV–PA) shunt on outcomes after stage-1 Norwood-type palliation (NP) compared with the modified Blalock–Taussig shunt. METHODS Consecutive patients who underwent NP between 2003 and 2019 were enrolled. SV–PA shunts using the expanded polytetrafluoroethylene valved conduit were implanted in 18 patients (valved SV–PA group), and another 18 patients underwent modified Blalock–Taussig shunt during NP (modified Blalock–Taussig shunt group). All valved conduits were made in our institution in advance. RESULTS No differences in baseline characteristics were found between the groups, except for shunt size. During a median 2.9 (interquartile range 0.4–6.4, maximum 14.2) years of follow-up, 8 (22.2%) patients died across both groups. There were no statistically significant differences in early mortality (5.5% vs 11.1%, P = 0.55) and overall survival rates at 5 years (80.8% vs 71.4%, P = 0.48) in the valved SV–PA and modified Blalock–Taussig shunt groups. No statistically significant difference was observed in the frequency of interventions between the groups (31% vs 33%, P = 1.0). At the time of the bidirectional Glenn procedure, the systemic ventricular end-diastolic volume index was significantly lower (84 ± 24 vs 106 ± 31 ml/m2, P = 0.05) and the ejection fraction was significantly greater (62 ± 8% vs 55 ± 9%, P = 0.03) in the valved SV–PA group. There was no statistically significant difference in the pulmonary artery index (228 ± 85 vs 226 ± 60 mm2/m2, P = 0.92). CONCLUSIONS A valved SV–PA shunt using an expanded polytetrafluoroethylene valved conduit was associated with preserved ventricular function after NP and did not impair pulmonary artery growth by controlling pulmonary regurgitation.

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.

OBJECTIVES Coronary artery bypass grafting (CABG) has been reported for coronary artery diseases in patients with Kawasaki disease and coronary artery complications after arterial switch operations for transposition of the great arteries. However, only a few studies have explored this modality for congenital coronary artery anomalies. As congenital coronary artery anomalies, particularly left coronary artery atresia and stenosis, are one of the reasons for sudden death, coronary revascularization is often required in infants and young children. Therefore, we aimed to investigate the outcome of CABG for such anomalies in infants and young children. METHODS From 2014 to 2018, 3 infants and 2 children (median age: 10 months; range: 6–40 months) with coronary artery anomalies underwent CABG at our hospital. The indications for the procedure included left main coronary artery atresia and stenosis in 2 and 3 patients, respectively. Graft patency was evaluated postoperatively by contrast-enhanced computed tomography or coronary angiography, and postoperative outcomes (including death and cardiac events) were assessed during the follow-up period. RESULTS No 30-day or in-hospital mortalities were noted. Postoperative examinations revealed patent grafts in all patients. They were discharged without any cardiac complications. Regarding the outcomes at the follow-up period, the graft patency rate was 80.0% (4/5 grafts), with no deaths or cardiac events. CONCLUSIONS CABG is a useful strategy for coronary revascularization in infants and young children with coronary artery anomalies. Although the mid-term outcomes and patency are satisfactory, careful follow-up is necessary because the long-term outcomes remain unknown.

Background: The purpose of this study was to assess autograft function after the Ross procedure and to review surgical outcomes associated with autograft reoperations. Methods: This is a retrospective study of patients undergoing the Ross procedure since 1993. Autograft function and autograft reoperation were studied. Autograft failure was defined as more than moderate autograft regurgitation or autograft dilatation to more than 50 mm diameter or z-score of more than +4 in children. One hospital death was excluded from analysis as were patients with unknown late autograft status. Results: Among 75 patients analyzed, preoperative diagnosis before the Ross procedure included aortic regurgitation in 26, aortic stenosis in 19, combined lesions in 28, and 2 mechanical valve malfunctions. Median age at the Ross procedure was 12.1 (0.4-43.6) years with 44 children less than 15 years old. Six patients had greater than mild autograft regurgitation at post-Ross hospital discharge. During median follow-up of 14.9 years, there were 23 autograft failures. Eighteen autograft reoperations were performed on 17 patients (13 children), including 12 aortic valve replacements, 5 aortic root replacements (including 1 valve-sparing root replacement), and 1 Konno procedure. Freedom from autograft failure and autograft reoperation at 20 years after the Ross procedure was 52.0% and 66.3%, respectively. Multivariate analysis identified greater than mild autograft regurgitation at hospital discharge from Ross procedure as a risk factor for autograft failure ( P < .01). All patients who underwent autograft reoperation survived and had good health status at a median of 6.9 years after the reoperation. Conclusions: The Ross procedure is effective in delaying prosthetic aortic valve replacement, although the time-related risk of autograft failure is a real consideration.

We report two cases of symptomatic cardiac ischemia caused by left coronary artery compression. One was a 16-year-old boy with history of the Ross procedure, aortic root aneurysm, and right ventricle-to-pulmonary artery conduit stenosis. The other was a 32-year-old woman with history of pulmonary atresia with ventricular septal defect repair and a giant right ventricle-to-pulmonary artery conduit aneurysm. In both cases, the left coronary artery was compressed by the previously placed valved autologous pericardial roll right ventricle-to-pulmonary artery conduit. Conduit replacement without direct coronary intervention relieved the coronary ischemia. Early diagnosis and relief of external compression avoided potentially fatal outcomes.

Aortic arch repair is an important technical element in the Norwood operation for hypoplastic left heart syndrome that could adversely affect long-term results. Therefore, there are many controversies surrounding this procedure. We developed an alternative chimney technique for aortic arch repair without any supplemental materials. Both pulmonary branch orifices were resected en bloc from the posterior wall of the pulmonary trunk. The pulmonary defect was closed by longitudinal direct anastomosis. Consequently, the neo-aortic trunk was completed as a gradually converged, chimney-like shape. The effective length of the neo-aortic trunk elongated longitudinally, and the caliber of the trunk was reduced transversely. The elongated neo-aortic trunk could be anastomosed directly to the aortic arch without excessive tension. The neo-aortic trunk was joined to the aortic arch with a gentle shape. A hydrodynamically advantageous aortic configuration was completed, and a large subaortic arch space was provided. Chimney technique is an innovative procedure in the Norwood operation that requires no supplemental material.

Objective The objective of this study was to evaluate the long-term surgical results of transposition of the great arteries with left ventricular outflow tract obstruction. Methods We conducted a retrospective study of patients with transposition of the great arteries or double outlet right ventricle with left ventricular outflow tract obstruction undergoing biventricular repair between 1980 and 2017. Results One hundred and eleven patients were enrolled and classified into five groups: atrial switch (n = 20), arterial switch (n = 12), Nikaidoh (n = 7), Rastelli (n = 48), and REV operation groups (n = 24). Early mortality was highest in Nikaidoh group (29%). Median follow-up was 18.2 years. Long-term survival was by far lowest in Nikaidoh group and comparable among the other 4 groups. Freedom from reoperation at 20 years was lowest in Rastelli group (32.1%) due to right ventricular outflow tract-related reoperations. While having no recurrence of left ventricular outflow tract obstruction, the arterial switch operation group had a high proportion of substantial neo-aortic regurgitation (29%). Conclusions The long-term survival was satisfactory regardless of the surgical technique except Nikaidoh group. The surgical option for transposition of the great arteries with left ventricular outflow tract obstruction should be selected based on the features of the respective procedures.

OBJECTIVES To compare conventional procedures with the half-turned truncal switch operation (HTTSO) for the management of complete transposition of the great arteries with left ventricular outflow tract (LVOT) obstruction using time-resolved 3-dimensional magnetic resonance phase-contrast imaging. METHODS We identified 2 cases that underwent the Rastelli procedure and one case that underwent the Réparation a l'étage ventriculaire before 2002 [conventional procedures group (group C)], and 16 cases of HTTSO that were performed between 2002 and 2020 [HTTSO group (group H)]. Postoperative haemodynamics were assessed using time-resolved 3-dimensional magnetic resonance phase-contrast imaging in cases in both groups. RESULTS The median follow-up period was 20.4 years in group C, and 6.1 years in group H. In group C, all 3 patients underwent reoperation because of postoperative right ventricular outflow tract obstruction and/or insufficiency. In addition, permanent pacemaker implantation was needed in 1 patient because of complete atrioventricular block complicated by ventricular septal defect enlargement. In group H, reoperation for LVOT/right ventricular outflow tract obstruction was not needed. A time-resolved 3-dimensional magnetic resonance phase-contrast imaging examination revealed high energy loss and wall shear stress in the winding LVOT in the group C. In contrast, low energy loss and wall shear stress, with straight and smooth LVOT, were identified in group H. CONCLUSIONS HTTSO was shown to be superior to conventional procedures because a straight and wide LVOT could be obtained. Therefore, HTTSO should be the first choice for complete transposition of the great arteries with LVOT obstruction.

OBJECTIVES Late complications of arterial switch operations (ASO) for transposition of the great arteries, such as neo-pulmonary artery (PA) stenosis and/or neoaortic regurgitation, have been reported. We developed an alternative reconstruction method called the longitudinal extension (LE) method to prevent PA bifurcation stenosis (PABS). METHODS We identified 48 patients diagnosed with transposition of the great arteries and performed ASO using the Lecompte manoeuvre for neo-PA reconstruction. In 9 consecutive patients (from 2014), the LE method was performed (LE). Before 2014, conventional techniques were performed in 39 patients (C). The median body weight and age in the LE and C groups were 3.0 and 3.1 kg and 12 and 26 days, respectively. In the LE group, 1 patient underwent bilateral PA banding before ASO. In C, PA banding and arch repair were performed in 1 patient each. Patients who received concomitant procedures were included. RESULTS The median follow-up in LE and C groups was 1.9 and 10.1 years, respectively. Early mortality/late death was not found in group LE and in 1 patient in group C. Only 1 case required ascending aorta sliding plasty in LE, and 8 patients needed PA augmentation for PABS in C. The median velocity of right/left PA was measured as 1.6/1.9 m/s in LE and 2.1/2.3 m/s in C, so it showed a lower value in LE. CONCLUSIONS Excellent mid-term results were obtained with the LE method. It was considered a useful procedure in preventing PABS, which is a primary late complication of ASO. Further follow-up and investigations are needed.

Background Re-intervention after Ross procedure into the right ventricular outflow tract might be needed in patients in the long term. However, right ventricular outflow tract re-intervention indications are still unclear. Comprehensive assessment of total hemodynamics is needed. Case summary A 42-year-old Japanese woman was referred to our hospital for moderately severe pulmonary regurgitation and severe tricuspid regurgitation after a Ross–Konno procedure. Thirteen years after surgery, she developed atrial fibrillation and atrial flutter and complained of dyspnea. Electrophysiological studies showed re-entry circuit around the low voltage area of the lateral wall on the right atrium. Four-dimensional flow magnetic resonance imaging revealed moderate pulmonary regurgitation, severe tricuspid regurgitation, and a dilated right ventricle. Flow energy loss in right ventricle calculated from four-dimensional flow magnetic resonance imaging was five times higher than in normal controls, suggesting an overload of the right-sided heart system. Her left ventricular ejection fraction was almost preserved. Moreover, the total left interventricular pressure difference, which shows diastolic function, revealed that her sucking force in left ventricle was preserved. After the comprehensive assessments, we performed right ventricular outflow tract reconstruction, tricuspid valve annuloplasty, and right-side Maze procedure. A permanent pacemaker with a single atrial lead was implanted 14 days postoperatively. She was discharged 27 days postoperatively. Echocardiography performed 3 months later showed that the size of the dilated right ventricle had significantly reduced. Discussion A four-dimensional imaging tool can be useful in the decision of re-operation in patients with complex adult congenital heart disease. The optimal timing of surgery should be considered comprehensively.

Background Biventricular physiological interaction remains a challenging problem in cardiology. We developed a four-dimensional (4D) flow magnetic resonance imaging (MRI) scan and clinically available analysis protocol based on beat tracking of the cardiovascular lumen without contrast medium, which enabled measurement of the biventricular hemodynamics and energetic performance by calculating flow energy loss (EL) and kinetic energy (KE). The aim of this study was to observe the flow patterns and energy dynamics to reveal the physiology of the right and left ventricular systems. Methods 4D flow MRI studies were performed in 19 healthy volunteers including 11 male and 8 female. The right and left ventricular systems were segmented to visualize the flow patterns and to quantify the hemodynamics and energy dynamics. Results A large vortex was observed in the left ventricle (LV), along the longitudinal axis, during end diastole and early systole. At early systole, the vortex appeared to facilitate smooth ejection with little EL. In contrast, in the right ventricle (RV), there were vortices near the free wall in both the short and long axes during the diastolic filling phase. Mean EL index during a single cardiac cycle in the right and left heart systems was 0.63 ± 0.16 (0.42–0.99) mW/m2, and 1.02 ± 0.26 (0.58–1.58) mW/m2, respectively. EL is inevitable loss caused by the vortex flow to facilitate smooth right and left ventricular function and left-sided EL tended to correlate positively with heart rate and right ventricular stroke volume. Kinetic energy at the aortic valve was influenced by LV end-diastolic volume/stroke volume. No gender difference was observed. Conclusions The RV appears to function as a regulator of the energy dynamics of the LV system.

Background The Harmonization By Doing (HBD) program was established in 2003 as a partnership among stakeholders of academia, industry and regulatory agencies in Japan and the United States, with a primary focus on streamlining processes of global medical device development for cardiovascular medical devices. While HBD has traditionally focused on development of devices intended to treat conditions prevalent in adults, in 2016, HBD established the "HBD-for-Children" program, which focuses on the development of pediatric devices as the development of medical devices for pediatric use lags behind that of medical devices for adults in both countries.Methods and Results:Activities of the program have included: (1) conducting a survey with industry to better understand the challenges that constrain the development of pediatric medical devices; (2) categorizing pediatric medical devices into five categories based on global availability and exploring concrete solutions for the early application and regulatory approval in both geographies; and (3) facilitating global clinical trials of pediatric medical devices in both countries. Conclusions The establishment of the HBD-for-Children program is significant because it represents a global initiative for the introduction of pediatric medical devices for patients in a timely manner. Through the program, academia, industry and regulatory agencies can work together to facilitate innovative pediatric device development from a multi-stakeholder perspective. This activity could also encourage industry partners to pursue the development of pediatric medical devices.

OBJECTIVES In Japan, homograft and bovine jugular vein are available in very limited institutions for the reconstruction of the right ventricular outflow tract, and handmade expanded polytetrafluoroethylene (ePTFE)-valved conduits have been widely used instead. This study aimed to clarify the long-term outcomes and the durability of the ePTFE-valved conduits purely by narrowing down to those with large sizes to eliminate the influence of the body growth. METHODS Between January 2002 and December 2015, patients who underwent right ventricular outflow tract reconstruction in 34 Japanese institutions using ePTFE-valved conduits with a diameter of ≥18 mm were included. All the valved conduits were made in the authors’ institution and delivered to each participating institution. RESULTS Overall, 502 patients were included. Early mortality was 1.4% and not related to conduit failure. The overall survival rate was 98.2% at 5 years and 96.6% at 10 years. Freedom from conduit explantation was 99.5% at 5 years and 89.0% at 10 years. Three patients (0.13 per 100 patient-years) developed infective endocarditis of the conduit, and only 1 patient required conduit removal. Pulmonary insufficiency was mild or less in 480 (96%) patients, and conduit stenosis was mild or less in 436 (88%) patients at the latest follow-up. CONCLUSIONS By narrowing the analyses down to only ePTFE conduits with a large size, satisfactory long-term outcomes of these conduits with a fan-shaped valve and bulging sinuses were shown. These conduits would be among the optimal choices for right ventricular outflow tract reconstruction.

To investigate mid- to long-term morbidity and mortality after the repair of partial anomalous pulmonary venous connections (PAPVCs). We retrospectively analyzed the cases of 29 consecutive patients who underwent surgery in 1991–2010 at Tokyo Women's Medical University Hospital. Median follow-up period: 9.9 years. Median age at operation: 7.4 years. Median body weight: 22.4 kg. Right PAPVC (n = 23), left-PAPVC (n = 5), and mixed PAPVC (n = 1) were identified. In the 14 patients with a right upper pulmonary vein (PV) connected to the superior vena cava (SVC) and/or cavo-atrial junction, the following were performed: single-patch diversion (n = 6), double-patch diversion (n = 4), Warden procedure (n = 1), and atrial septation with the atrial wall (n = 3). In the nine patients with a right anomalous PV connected to the right atrium (RA) and/or coronary sinus, intra-atrial rerouting was performed (n = 7) and translocation of the atrial septum (n = 2). Intra-atrial rerouting of the left upper and lower PVs to the coronary sinus (n = 1) and direct anastomosis of the left upper PV to the left atrial appendage (n = 4) were performed. Double-patch diversion and direct anastomosis of the left upper PV to the left atrial appendage was performed in a patient with mixed PAPVC. There were no post-operative deaths or reoperations, although transient sinus node dysfunction occurred (n = 2 with atrial septation and intra-atrial rerouting), while mild stenosis of the SVC occurred (n = 1 with double-patch diversion). The various methods which we used for PAPVC repair all appear to have reasonable outcomes, although rhythm disturbance and stenotic complications were confirmed in a few patients.

Background Echocardiography vector flow mapping can assess dynamic flow to treat congenital heart diseases. We evaluated intracardiac flow, energy loss, left ventricular output kinetic energy, and energetic performance index using vector flow mapping during Glenn and Damus-Kaye-Stansel procedures in order to assess the efficacy of the surgery. Case presentation A 9-month-old boy underwent Glenn and Damus-Kaye-Stansel procedures. The energy loss depends on the left ventricular preload; therefore, energy loss decreased after the Glenn procedure. After the Damus-Kaye-Stansel procedure, the kinetic energy would increase owing to the integrated systemic outflow; however, in our case, kinetic energy decreased, which was potentially explained by the fact that kinetic energy also depends on the left ventricular preload. After the Glenn and Damus-Kaye-Stansel procedures, we detected an improvement in energetic performance index, indicating that the cardiac workload improved as well. Conclusion We revealed the efficiency of the Glenn and Damus-Kaye-Stansel procedures using vector flow mapping.