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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.

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.

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.

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.

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.

Anomalous origin of the coronary artery from the aortic arch associated with hypoplastic left heart syndrome is an extremely rare anomaly. Coronary anomalies can significantly deteriorate the clinical outcomes of hypoplastic left heart syndrome. We describe the case of a newborn with concomitant hypoplastic left heart syndrome and abnormal origin of the left coronary artery arising from the distal aortic arch.

Purpose Giant mediastinal germ cell tumor (MGCT) requires a well-planned advanced surgical approach. We retrospectively reviewed our surgical strategy for giant MGCT. Methods Five children (median age, 5 years) with giant MGCT were treated in our institute from 2012 to 2016. Results The initial diagnosis was made by tumor markers and image inspection in all cases. Benign teratomas (2 girls) and malignancies (3 boys) were treated with upfront surgery and radical tumorectomy after neo-adjuvant chemotherapy, respectively. After detailed 3D-CT, radical tumor excision was performed supported by a skilled pediatric cardiovascular surgeon. The basic approach was as follows: under cardiopulmonary support (CPS) or with CPS on standby, via median sternotomy, the pericardium and phrenic nerve were resected en bloc with the tumor, followed by diaphragmatic plication. Open biopsy was performed via lateral thoracotomy in 1 patient who showed dense adhesion and fistula formation in the lung; lobectomy via hemi-clamshell incision was required. No deaths or severe sequelae occurred in this series. Conclusions Resectability is the most important predictor of outcomes for MGCTs. Preoperative 3D-CT and CPS can enable complete resection and ensure surgical safety. Well-functioned surgical team is critical success factor in such advanced surgery.

A male neonate presented with the aortic/mitral stenotic variant of hypoplastic left heart syndrome, wherein the suprasystemic left ventricular pressure and relatively large left ventricle had shifted the intraventricular septum. Despite bilateral pulmonary artery banding, the stroke volume was difficult to maintain owing to the compressed right ventricle, causing heart failure symptoms. Percutaneous balloon aortic valvuloplasty decreased the left ventricular pressure, restoring the right ventricular function. Norwood procedure with mitral valve closure after catheter intervention reduced the left ventricular size and improved the right ventricular function. This paper refers to the potential of mitral valve closure for hypoplastic left heart syndrome.

OBJECTIVES To clarify the risk factors for abnormal degeneration of autologous pericardium, the mechanical stress on the endothelial tissue caused by turbulent blood flow in the pulmonary artery (PA) reconstructed with autologous pericardium and major aortopulmonary collateral arteries was assumed in pulmonary atresia and ventricular septal defect. METHODS Patient-specific PA models were created for 6 patients based on their past computed tomography images taken after unifocalization of major aortopulmonary collateral arteries. Computational fluid dynamics models were created to simulate the physiological pulsatile flow including the peripheral reflection wave, characteristic impedance and autonomous regulation system. Flow streamline, wall shear stress (WSS) and the oscillatory shear index (OSI) were calculated from the simulated result. PA degeneration was evaluated with the computed tomography images before the intracardiac repair. RESULTS Regions with physiological high WSS had fewer abnormal changes. Excessively high WSS was often detected at the anastomosis site of the reconstructed PA, and intimal thickening was found in these regions during intracardiac repair. Regions with high OSI and low WSS had dilated change within several months. In 1 patient, in particular, detached vortex flow occurred at the rectangular angle anastomosis site of the right PA in flow streamline, resulting in high OSI and low WSS with abnormal enlargement in the pericardium. CONCLUSIONS Endothelial stress caused by blood flow would affect the degeneration of autologous pericardium and major aortopulmonary collateral arteries in the reconstructed PA. High OSI and low WSS might induce enlargement or dilatation, and excessively high WSS in the anastomosis site might induce intimal thickening.

The expanded polytetrafluoroethylene (ePTFE)-valved conduits as alternative material for right ventricular outflow reconstruction provides satisfactory long-term outcomes. The purpose of this study was to investigate degenerative changes in failed conduits through histopathologic analysis of the explanted specimens. All leaflets of explanted conduits were observed macroscopically, and their longitudinal sections were examined microscopically. Three typical findings, that is, calcification of the leaflet, neointimal proliferation, and proteinaceous infiltration into the leaflet, were evaluated quantitatively by measuring their degree and appearance probability. A total of 15 leaflets from 5 failed conduits (group F) and 12 leaflets from 5 nonfailed conduits (group non-F) were included. The median duration of implantation was 7.6 years (5.3-10.9 years) in group F and 1.3 years (0.7-3.9 years) in group non-F (P = 0.003). In group F, calcification tended to occur in the middle and upper third of the leaflet, causing stiffening, distortion, and exophytic concretion of the leaflet, and mean neointimal thickness on inflow and outflow surfaces were 0.33 ± 0.02 mm and 0.22 ± 0.01 mm, respectively. There was a moderately strong correlation between appearance probability of calcification in group F and that of proteinaceous infiltration in group non-F (correlation coefficient 0.67, P0.001). Proteinaceous infiltration into the leaflet was presumed be responsible for future calcification of the leaflet and subsequent stenotic conduit failure. Modification of the ePTFE material to prevent proteinaceous infiltration may contribute to improving the durability of ePTFE-valved conduit.

Background: Accurate coronary translocation is very important for a successful arterial switch operation (ASO) for transposition of the great arteries (TGA) and to provide good long-term outcomes. We have previously reported the “bay window” technique as a useful option for coronary translocation with excellent midterm results. However, the long-term results of this technique and the morphological changes in the coronary channel have not yet been reported. Patients and Methods: Between September 2001 and December 2012, 33 patients with TGA underwent coronary translocation using the bay window technique concomitantly with ASO. The diagnoses were TGA with intact ventricular septum in 21, TGA with ventricular septal defect in 7, and Taussig-Bing anomaly in 5. The median age of the patients at operation was 12 days, and their median body weight was 2.93 kg. Coronary artery patterns were as follows: Shaher and Puddu’s type 1 in 23, single coronary artery in 4, intramural type in 1, and others in 5. Results: The median follow-up period was 4.5 years. There were no operative deaths, but there was one late death because of sepsis 13 months after ASO. Coronary artery evaluation with angiography, computed tomography, or myocardial scintigraphy was performed in 22 patients. There was no abnormal coronary morphology or perfusion. Coronary channel dilatation was not observed. Three patients underwent reoperation for pulmonary stenosis. Conclusions: The bay window technique provides excellent long-term results in ASO for TGA. Three-dimensional computed tomography revealed no aneurysmal changes in the bay window channel at nine-year follow-up examination.

Background Pulmonary regurgitation is a common complication after tetralogy of Fallot repair, resulting in right ventricular dysfunction, arrhythmia, and sudden death. However, the indications and optimal timing for pulmonary valve replacement are not fully known. We describe a case in which a four-dimensional imaging tool was useful in the decision to re-operate, thus resulting in decreased energy loss and improved right ventricular function after the re-operation for tetralogy of Fallot. Case presentation A 54-year-old Japanese woman visited our hospital due to palpitations and wide QRS tachycardia with persistent tiredness for several months. She underwent repair of tetralogy of Fallot when she was 2-years old. An electrocardiogram showed prolonged QRS duration (199 msec) with a complete right bundle branch block and an echocardiograph demonstrated that her right ventricle was highly enlarged and had poor contraction, and severe pulmonary valve regurgitation with one leaflet flail. Four-dimensional flow magnetic resonance imaging demonstrated that regurgitant volumes and regurgitant fractions of pulmonary regurgitation were calculated as 63.12 ml and 54.0%, respectively. Right ventricular end-diastolic/end-systolic volume index was 169.54/99.76 mL/m2, and the cardiac index was 1.78 L/minute per m2. Flow energy loss was 2.93 mW, which is estimated to be three times higher than normal controls. An electrophysiological study showed an intact anterior internodal pathway and a slow pathway just through the outside of the right atriotomy line scar, which is supposed to cause a re-entry circuit. We decided to perform a pulmonary valve replacement and a right maze procedure. A 27 mm bioprosthetic valve was implanted in the native pulmonary annulus with a supra-annular position. Concomitantly, the right maze procedure was performed. A four-dimensional flow magnetic resonance imaging done 3 months later showed that right ventricular end-diastolic/end-systolic volume index had significantly reduced to 85.24/55.41 mL/m2 and the cardiac index had increased from 1.78 to 2.58 L/minute per m2. Energy loss had greatly improved from 2.93 to 1.48 mW. Conclusions A four-dimensional imaging tool was useful in the decision to re-operate, thus resulting in decreased energy loss and improved right ventricular function after the re-operation for tetralogy of Fallot. Electronic supplementary material The online version of this article (10.1186/s13256-018-1964-9) contains supplementary material, which is available to authorized users.

Purpose The ideal material for pediatric pulmonary artery (PA) augmentation is autologous pericardium. However, its utility for multistaged operations is limited. In this study, we applied an in vivo tissue-engineered autologous Biotube graft to a patient with congenital heart disease for the first time. Description For molds of the Biotubes, two silicone 19F drain tubes were embedded in the subcutaneous spaces of a 2-year-old girl with a diagnosis of pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries during palliative surgical procedures. When definitive repair was performed after 8 months, the implants were removed to prepare Biotubes, one of which was cut open and autologously implanted into the PA for patch augmentation. Evaluation Seven months after implantation, the Biotube patch-augmented PA tolerated balloon angioplasty (BAP) for residual stenosis of the peripheral PA. Computed tomography images after BAP showed the well-preserved shape of the Biotube patch-augmented PA. Neither aneurysm formation nor stenosis was observed. Conclusions The safety and feasibility of Biotubes for pediatric PA patch augmentation are described. Because Biotubes are completely autologous, they may be ideal material for pediatric PA augmentation.

Background One of the critical factors limiting conduit longevity in right ventricular outflow tract (RVOT) reconstruction with homografts and xenografts is the small size of the conduit. The aim of our study was to assess the outcome of using smaller-sized expanded polytetrafluoroethylene (ePTFE) conduits with a fan-shaped valve and bulging sinuses for RVOT reconstruction. Methods This retrospective review examined 303 patients who underwent RVOT reconstruction with ePTFE conduits at 63 Japanese hospitals between 2003 and 2014. Inclusion criteria were a conduit size less than or equal to 16 mm and the use of operative treatment as the primary correction for underlying heart disease. Patients undergoing palliative procedures were excluded. Results Median follow-up was 1.7 years. Freedom from conduit replacement and freedom from conduit reintervention were 90.1% ± 4.8% and 77.2% ± 5.6%, respectively. The most common indication for conduit reintervention was pulmonary artery (PA) bifurcation stenosis (82%). Modeling z -scores as a dichotomous variable revealed that freedom from conduit reintervention for PA bifurcation stenosis was significantly decreased for conduits with a z -score greater than or equal to 1.4 compared with z -scores less than 1.4 ( p = 0.036). There were 30 patients (9.9%) who experienced at least moderate conduit stenosis and 21 patients (6.9%) with at least moderate conduit insufficiency. Univariate Cox regression analysis showed that conduit size was a significant factor for conduit stenosis ( p = 0.006). Conclusions Excellent midterm outcomes were achieved with ePTFE valved conduits, even when using smaller sizes. Conduit z -scores around 1.4 were optimal for RVOT reconstruction in younger patients.

We developed an expanded polytetrafluoroethylene (ePTFE) valved conduit for use in right ventricular outflow tract reconstruction, a procedure used in various congenital heart diseases with decreased pulmonary blood flow, particularly Tetralogy of Fallot. The valve was designed with a fan-shaped configuration and consisted of a 0.1-mm-thick ePTFE membrane. To improve valve mobility, bulging sinuses were constructed in the conduit. The follow-up duration was 4.9 ± 3.4 years (maximum, 14.2 years), and the reoperation-free rates were 98.8% at 5 years and 93.8% at 10 years with large-caliber conduits (16-24 mm) and 89.9% at 5 years and 68.6% at 10 years with small-caliber conduits (6-16 mm), results superior to those seen with previous commercially available conduits and homograft. The incidence of infection caused by the ePTFE conduit was very low, at 0.3%. An ePTFE conduit with bulging sinuses and a fan-shaped valve made of an ePTFE membrane is useful for right ventricular outflow tract reconstruction.

The double-switch operation for congenitally corrected transposition of the great arteries, in which the morphologic left ventricle is restored to the systemic circulation, is an effective surgical option. This case report describes an atrial switch technique without using supplemental material during the double-switch operation for congenitally corrected transposition of the great arteries associated with persistent left superior vena cava, with the systemic venous blood flow rerouted through the enlarged coronary sinus.

Objective Conventional procedures for partial anomalous pulmonary venous return (PAPVR) to the superior vena cava (SVC) still have serious complications, such as late SVC and/or pulmonary venous obstruction and supraventricular arrhythmia. We aimed to introduce our newly developed surgical technique with minimum right atriotomy and double-barreled arrangement of systemic and pulmonary venous channels (double-decker technique). Methods From 1998 to 2018, 21 consecutive patients with PAPVR to the SVC underwent this new procedure. The patients' median age and body weight were 4.4 years and 16.5 kg, respectively. Two female patients underwent lateral thoracotomy. Postoperative hemodynamics of both venous channels were assessed using time-resolved 3-dimensional magnetic resonance phase contrast imaging in 6 patients. Results The median follow-up period was 11.0 years. There was no early mortality and late death. No patient required reoperation and/or intervention. All patients maintained normal sinus rhythm, and supraventricular arrhythmia did not occur. The median blood flow velocity of the neo-SVC and neopulmonary venous channel was 0.40 and 0.30 m/s, respectively. Using time-resolved 3-dimensional magnetic resonance phase contrast imaging, the straight and nonrestrictive flow and low wall shear stress were visualized in both venous channels. Conclusions Our newly developed double-decker technique is a useful alternative surgical procedure for PAPVR to the SVC. Late complications can be completely avoided using this method. Growth potential of both channels is also maintained.

OBJECTIVES The ideal configuration of a reconstructed aortic arch in the Norwood procedure for hypoplastic left heart syndrome is still a matter of debate. Chimney reconstruction was developed to avoid postoperative complications and turbulent flow in the aortic arch. This study sought to clarify early outcomes of the procedure and verify its haemodynamic advantages using computational fluid dynamics (CFD). METHODS Fourteen consecutive patients with hypoplastic left heart syndrome or a variant who underwent chimney reconstruction in the Norwood procedure between January 2013 and March 2018 were enrolled. Median age and body weight at the time of operation were 2.5 months and 4.1 kg, respectively. Thirteen patients (93.9%) had been palliated with previous bilateral pulmonary artery (PA) banding. In addition, patient-specific CFD models of neoarches based on postoperative computed tomograms from 6 patients were created and the flow profiles analysed. RESULTS Survival rates at 1, 3 and 5 years were 76.6%, 67.3% and 67.3%, respectively. No patient developed left PA compression by neoaorta, neoaortic dilation or neoaortic insufficiency. Only 2 patients (14.3%) required surgical intervention for recoarctation. Fontan completion was performed on 5 patients. On CFD analysis, all reconstructed aortic arches showed low energy loss (9.16–14.4 mW/m2) and low wall shear stresses. CONCLUSIONS Chimney reconstruction was a feasible technique when homografts were not readily available. CFD analyses underscored the fact that this technique produced excellent flow profiles. Larger studies should be conducted to clarify long-term outcomes.

OBJECTIVES: The objective of this study was to evaluate our late outcomes using expanded polytetrafluoroethylene (ePTFE) valved patches with bulging sinuses and a fan-shaped valve for right ventricular outflow tract (RVOT) reconstruction. METHODS: Six hundred and ninety patients underwent RVOT reconstruction using fan-shaped ePTFE valves and ePTFE valved patches with a bulging sinus. The patients’ median age and weight were 1.3 years [range 4 days–64.2 years, interquartile range (IQR) 0.9–2.3 years] and 8.7 (range 2.8–83.1, IQR 7.4–10.5) kg, respectively. The patches were monocuspid in 634 patients, bicuspid in 49 patients and tricuspid in 7 patients. Preoperative and postoperative data were collected retrospectively from the patients’ medical records. The longest follow-up period was 17.5 (7.6 ± 3.9) years. RESULTS: There were no deaths related to the ePTFE patch. Pulmonary insufficiency was less than mild in 77.3%, and the peak RVOT gradient was CONCLUSIONS: Satisfactory long-term outcomes were achieved with ePTFE patches with a bulging sinus and a fan-shaped valve. This ePTFE valved patch could be the optimal choice for RVOT reconstruction.

Background Conventional Rastelli, Lecompte, and Nikaidoh operations are accepted as standard techniques for complete transposition of the great arteries (TGA) with left ventricular outflow tract (LVOT) obstruction. These operations show serious drawbacks, however, including postoperative obstruction of both ventricular outflow tracts. We developed the half-turned truncal switch operation (HTTSO) to address these problems. Methods Between 2002 and 2017, 14 patients underwent HTTSO. Median age was 1.2 years and median body weight was 8.3 kg. Diagnosis was TGA with pulmonary stenosis in 9 cases, TGA-type double-outlet right ventricle in 4, and TGA with degenerative pulmonary valve after pulmonary arterial banding in 1. The coronary artery was Yacoub type A in 13 and type D in 1. Four patients had a small right ventricle. Pulmonary-aortic annular diameter ratio ranged from 0.43 to 1.00. The right ventricular outflow tract was augmented using a monocuspid polytetrafluoroethylene valved patch in 8 cases. Autologous pulmonary annulus was preserved in 6 cases. Results Median follow-up was 5.2 years. No early mortality was encountered. Only 1 patient was lost due to arrhythmia, 11 months after HTTSO. No patients showed coronary insufficiency and no outflow tract obstruction was identified. Aortic regurgitation was within mild degree in 12 cases. Additional mitral valvular annuloplasty was required in 3 cases late after HTTSO for moderate-to-severe mitral regurgitation. Risk factors for late death and reoperation were low age and body weight at HTTSO. Conclusions HTTSO is useful for TGA with LVOT obstruction, ensuring wide, straight ventricular outflow tracts and growth potential.

Background The purposes of this study were to review our early outcomes using valved expanded polytetrafluoroethylene (ePTFE) conduits, with or without bulging sinus structure, for right ventricular outflow tract reconstruction and to examine the mechanical properties of the ePTFE material after bulging sinuses were created. Methods A retrospective review was performed of all patients who received the valved ePTFE conduit between 2008 and 2014 at a single institution. The surface morphologies and the mechanical strengths of the ePTFE conduit with bulging sinuses examined by scanning electron microscopy and unidirectional pull test were compared with those of the original ePTFE material. Results There were 120 operations with the valved ePTFE conduit (60 with bulging sinuses). The patients median age and weight were 6.9 years and 23.7 kg. The conduits were a median size of 22 mm. At 5 years, freedom from conduit reoperation was 92.7% (95% confidence interval, 82.7% to 97.0%), and freedom from severe conduit insufficiency or more than a 50 mm Hg gradient was 74.8% (95% confidence interval, 60.8% to 84.4%). No significant differences in the surface morphologies were observed by the scanning electron microscopy or in the maximum tolerated loads obtained by the pull test between the original ePTFE material and the ePTFE with bulging sinuses (121 and 122 N in longitudinal direction and 115 and 121 N in circumferential direction; p = 0.88 and p = 0.68). Conclusions The valved ePTFE conduits demonstrated excellent early clinical outcomes. The mechanical property examinations showed no obvious difference after bulging sinuses were created on the ePTFE material.

Neoaortic root dilatation is a significant and inevitable late complication in patients after the Norwood operation. In this report, we describe a patient who had left pulmonary artery compression and severe neoaortic regurgitation from a severely dilated neoaortic root 11 years after the Norwood operation and subsequent Fontan procedure. We performed successful valve-sparing neoaortic root replacement in this patient.

This report describes an infantile case of Loeys-Dietz syndrome (LDS) with spontaneous mitral leaflet rupture. The patient was diagnosed with a type B interruption of the aortic arch. Bilateral pulmonary artery banding was performed 5 days after birth. On the 53rd day, intra-cardiac repair was performed without valvuloplasty. Although the operation was successful, mitral regurgitation deteriorated at 4 weeks after operation. On the 88th day, a mitral valvuloplasty was performed and a severely ruptured anterior leaflet was observed. Seven days after valvuloplasty, the mitral valve insufficiency again worsened and a fourth operation was performed. Two tears were observed in the anterior and posterior mitral valve leaflets, and a mitral valve replacement was required. Subsequently, the patient was diagnosed with LDS according to gene mutational status. LDS is known to have a poor prognosis with cardiovascular complications, but valve rupture has not been previously reported in other cases.

Background Staged palliative surgery markedly shifts the balance of volume load on a single ventricle and pulmonary vascular bed. Blalock-Taussig shunt necessitates a single ventricle eject blood to both the systemic and pulmonary circulation. On the contrary, bidirectional cavopulmonary shunt release the single ventricle from pulmonary circulation. Case presentation We report a case of tricuspid atresia patient who underwent first palliative surgery and second palliative surgery. Volume loading condition was assessed by energetic parameters (energy loss, kinetic energy) intraoperatively using vector flow mapping. These energetic parameters can simply indicate the volume loading condition. Conclusion Vector flow mapping was useful tool for monitoring volume loading condition in congenital heart disease surgery. Electronic supplementary material The online version of this article (10.1186/s12947-017-0118-3) contains supplementary material, which is available to authorized users.

The authors examined the hemodynamic characteristics of expanded polytetrafluoroethylene (ePTFE) pulmonary valved conduits quantitatively by our originally developed pediatric pulmonary mechanical circulatory system, in order to suggest the optimal shape design. The system consisted of pneumatically driven right atrium and ventricle model, a pulmonary valve chamber, and elastic pulmonary compliance model with peripheral vascular resistance units, a venous reservoir. We employed two different types of ePTFE valve and evaluated the relationship between the leaflets motion and hemodynamic characteristics by using a high-speed video camera. As a result, we successfully reproduced hemodynamic simulations in our pediatric pulmonary mock system. We confirmed that the presence of bulging sinuses in the pulmonary valved conduit reduced the transvalvular energy loss and increased the valve opening area during systolic period. Our engineering-based in vitro analysis could be useful for proposing a shape design optimization of sophisticated pediatric ePTFE pulmonary valve.