Search

Objectives We compared 2-patch repair (TP) with modified single-patch repair (MSP) for complete atrioventricular septal defects and evaluated their effect on the left atrioventricular valve (LAVV) competence. We also identified risk factors for unfavorable functional outcomes. Methods This retrospective study included 118 patients with complete atrioventricular septal defects who underwent intracardiac repair from 1998 to 2020 (MSP: 69; TP: 49). The median follow-up period was 10.4 years. The functional outcome of freedom from moderate or greater LAVV regurgitation (LAVVR) was estimated using the Kaplan–Meier method. Results The hospital mortality was 1.7% (2/118) and late mortality was 0.8% (1/118). Eight patients required LAVV-related reoperation (MSP: 4; TP: 4) and none required left ventricular outflow tract-related reoperation. In the MSP group without LAVV anomaly, the receiver operating characteristic curve analysis revealed that the ventricular septal defect (VSD) depth was strongly associated with moderate or greater postoperative LAVVR, with the best cutoff at 10.9 mm. When stratified according to the combination of intracardiac repair type and VSD depth, the MSP-deep VSD (VSD depth >11 mm) group showed the worst LAVV competence among the 4 groups (P = .002). According to multivariate analysis, weight Conclusions Postoperative LAVVR remains an obstacle to improved functional outcomes. MSP provides LAVV competence similar to TP unless deep VSD is present. The surgical approach should be selected on the basis of anatomical variations, specifically VSD depth.

The disadvantage of right ventricle-to-pulmonary artery (RV-PA) shunt is the need for more unplanned interventions to address stenosis in the shunt or branch pulmonary arteries, as compared to the modified Blalock-Taussig shunt group. Ring-enforced RV-PA PTFE conduit and dunk technique minimized these complications and right ventricle (RV) damage. Aortic arch obstruction increases afterload and leads to ventricular dysfunction and tricuspid regurgitation; therefore, most surgeons prefer to use homograft, autologous pericardium, or bovine pericardium to reconstruct the neoaorta. Artificial materials decrease the elastic properties, increase wall stiffness, and decrease the distensibility of the aorta; and as a result, RV function gradually deteriorates. This inelastic reconstructed aorta may be one of the reasons why long-term outcomes after the Fontan procedure are worse in hypoplastic left heart syndrome (HLHS) patients, in comparison to non-HLHS. Reconstruction of the neoaorta without any patch materials, or at least techniques that largely minimize the use of non-autologous materials, will offer a further refinement of our ability to optimize ventriculoarterial coupling and thereby long-term RV function.

Staphylococcus aureus is one of the main causative bacteria for polyurethane catheter and artificial graft infection. Recently, we developed a unique technique for coating diamond-like carbon (DLC) inside the luminal resin structure of polyurethane tubes. This study aimed to elucidate the infection-preventing effects of diamond-like carbon (DLC) coating on a polyurethane surface against S. aureus. We applied DLC to polyurethane tubes and rolled polyurethane sheets with our newly developed DLC coating technique for resin tubes. The DLC-coated and uncoated polyurethane surfaces were tested in smoothness, hydrophilicity, zeta-potential, and anti-bacterial properties against S. aureus (biofilm formation and bacterial attachment) by contact with bacterial fluids under static and flow conditions. The DLC-coated polyurethane surface was significantly smoother, more hydrophilic, and had a more negative zeta-potential than did the uncoated polyurethane surface. Upon exposure to bacterial fluid under both static and flow conditions, DLC-coated polyurethane exhibited significantly less biofilm formation than uncoated polyurethane, based on absorbance measurements. In addition, the adherence of S. aureus was significantly lower for DLC-coated polyurethane than for uncoated polyurethane under both conditions, based on scanning electron microscopy. These results show that applying DLC coating to the luminal resin of polyurethane tubes may impart antimicrobial effects against S. aureus to implantable medical polyurethane devices, such as vascular grafts and central venous catheters.

Pulmonary artery coarctation may pose a risk for pulmonary stenosis and subsequent failure to achieve definitive repair. We sought to assess the impact of pulmonary artery coarctation on pulmonary artery growth.A retrospective chart review was performed in 130 patients, including 37 single ventricles with a modified Blalock-Taussig shunt as first palliation. Pulmonary artery coarctation was defined as discrete stenosis of the pulmonary artery, with a diameter of less than 3 mm and with the ductus arteriosus connected. Preoperative echocardiography showed pulmonary artery coarctation in 29 patients (22%). Concomitant pulmonary artery plasty was performed in 14 patients with discrete stenosis having a diameter of less than 2 mm.Pre-modified Blalock-Taussig shunt left pulmonary artery z-scores were lower in patients with pulmonary artery coarctation than in those without (-4.0 [-5.8, -2.1] vs -1.7 [-2.6, -0.8], P .001), and this remained the same even after modified Blalock-Taussig shunt (-2.5 [-5.1, -0.5] vs -0.5 [-2.4, 0.8], P = .010). Concomitant pulmonary artery plasty did not result in catch-up growth of the left pulmonary artery (post-modified Blalock-Taussig shunt left pulmonary artery z-score in patients with pulmonary artery plasty: -3.0 (-6.5, -2.0) versus those without: -1.8 (-3.3, -0.3), P = .279). Definitive repair/Fontan completion was achieved in 111 patients (85%), and this was not affected by the presence of pulmonary artery coarctation.Pulmonary artery coarctation affected disproportionate pulmonary artery growth throughout the staged repair, but did not result in failure of definitive repair/Fontan completion. Pulmonary artery plasty during the neonatal period did not contribute to catch-up growth of the left pulmonary artery; therefore, surgical indications and timing should be carefully considered.

OBJECTIVES The goal of this study was to investigate the long-term outcome of aortic valve replacement (AVR) for severe aortic insufficiency with a focus on pre- and postoperative left ventricular (LV) function to explore predictive factors that influence the recovery of LV function and clinical outcome. METHODS A total of 478 patients who underwent AVR for pure severe aortic insufficiency were grouped according to the preoperative echocardiographical LV ejection fraction (EF): low (LO) EF 50% (n = 285). RESULTS Actuarial survival at 10 years post-AVR was 64% with a LO EF, 92% with a moderate EF and 93% with a normal EF (P = 0.016), whereas 10-year rates of freedom from major adverse cerebral and cardiovascular events were 47%, 79% and 84%, respectively (P 365 pg/mL (P = 0.0022) and echocardiographic LV mass index) >193 g/m2 (P = 0.0018). CONCLUSIONS Long-term outcome post-AVR for severe aortic insufficiency was largely influenced by preoperative LV function. Predictive factors of failure to recover ventricular function post-AVR included EF 365 pg/mL or LV mass index >193 g/m2.

Even today, when the surgical outcome of congenital heart disease in the neonatal period has improved, the prognosis for heterotaxy syndrome and functional single ventricle complicated with total anomalous pulmonary venous connection (TAPVC), especially the infra-cardiac type, is catastrophic. We describe a strategy that combines percutaneous ductus venosus (DV) stent placement and occlusion after TAPVC repair to ensure survival from initial surgery to bidirectional cavopulmonary shunt (BCPS) procedure and facilitate subsequent treatment. Three consecutive patients with heterotaxy syndrome and functional single ventricle complicated by infra-cardiac TAPVC treated with our own strategy were retrospectively studied. In two infants, DV stent placement was performed on the day of birth. In one case at 11 days of age. The risk of pulmonary vein obstruction was reduced, and on-pump surgery, including TAPVC repair, was performed on a standby basis. Since the rapid increase in hepatic enzymes occurred on postoperative day 0 to 1 in all cases, percutaneous stent occlusion was performed until postoperative day 3. The procedure improved liver function. One patient died due to severe atrioventricular valve regurgitation, one case underwent BCPS, and one patient was waiting to undergo. DV stent placement can avoid TAPVC repair in the early neonatal period. After TAPVC repair, the portosystemic shunt remained, resulting in hepatic dysfunction, but this could be improved by stent and vertical vein occlusion. A series of stepwise treatments can be useful to help such critically ill infants survive the high-risk neonatal period and achieve good BCPS circulation.

Anomalous origin of the left coronary artery from the opposite sinus of Valsalva with an intramural aortic course (L-ACAOS-IM) can cause syncope, sometimes as a prodrome of lethal events, including sudden cardiac death, in young athletes. The detailed mechanism of syncope in patients with L-ACAOS-IM is still unclear. This case report describes a 17-year-old boy who presented to the hospital because of syncope following chest pain with increasing frequency during exercise, such as playing soccer and running. In a treadmill exercise test, a decrease in blood pressure was seen (from 99/56 mm Hg to 68/38 mm Hg); chest pain and faintness accompanied by ST-segment elevation in lead aVR and ST-segment depression at other leads on electrocardiography were noted. These findings and symptoms disappeared spontaneously within a few minutes while clinicians prepared for emergency medications. Coronary computed tomography angiography (CCTA) showed that the origin of the left coronary artery (LCA) was the opposite sinus of Valsalva, and the course of the LCA was through the aortic wall toward the left coronary sinus. He was diagnosed with L-ACAOS-IM. After surgical treatment by unroofing the intramural part of the LCA and reconstructing a neo-ostium, he no longer experienced syncope during exercise. This case suggests that low cardiac output caused by myocardial ischemia, not life-threatening arrythmia, is a main mechanism of syncope in patients with L-ACAOS-IM. Consideration should be given to performing CCTA before an exercise stress test for young patients with syncope and chest pain to avoid the risk of severe myocardial ischemia.

We report a case of ventricular septal defect (VSD) in which we attempted to treat pulmonary arterial hypertension (PAH) with the goal of VSD closure in an adult with suspected Eisenmenger syndrome in childhood. Four years previously (age 41 years), she was referred to our department due to repeated hemoptysis requiring further treatment of PAH. We started combination therapy with several pulmonary vasodilators. Two years later, her pulmonary vascular resistance (PVR) was improved but still not at the level where VSD closure was possible. To control the increased PA flow resulting from intensive PAH treatment and to reduce the risk of hemoptysis, we performed pulmonary artery banding (PAB). As the risk of hemoptysis decreased, a prostacyclin analog was introduced, and the dose was increased. More than 1 year after PAB, active vasoactivity testing became positive, suggesting that the pulmonary vascular lesion was now “reversible”. We performed VSD closure and atrial septal defect creation even though her PVR was still high. After the operation, her exercise capacity was remarkably improved. We suggest that stepwise surgical repair with pulmonary vasodilators is an important treatment option for select patients with VSD with severe PAH.

Aortic root abscess is one of the most catastrophic complications of aortic valve endocarditis. Complete debridement is recommended regardless of the kind of infected lesion. A 37-year-old man with extensive aortic root abscess due to prosthetic aortic valve endocarditis was surgically treated. The main lesion was the aortomitral continuity extending to the commissure between the left and right coronary cusps. After debridement, the aortic annulus underneath the left coronary artery was reconstructed using a handmade aortomitral monobloc valve without aortic annuloplasty. This valve was required for the extensive root abscess of the left and noncoronary sinus to achieve complete debridement.

Objectives The aim of this study was to investigate whether milrinone infusion improved one-year survival in patients who underwent the Norwood-Sano procedure. Design A retrospective observational study. Setting A single-institution university hospital. Participants Children who underwent the Norwood-Sano procedure from January 2008 to December 2014. Interventions Patients were categorized into two groups: group E+D, who received routine epinephrine and dopamine infusion, and group M, who received routine milrinone infusion for intra- and postoperative inotropic support. Measurements and Main Results The primary outcome of this study was one-year survival after the Norwood-Sano procedure. A total of 45 patients were included (group E+D, 22; group M, 23). One-year survival in group M was significantly higher than that in group E+D (95.7% [22/23] v 72.7% [16/22], p = 0.03). A Kaplan-Meier curve also showed that one-year survival in group M was significantly higher than that in group E+D (p = 0.04), from the result of the log-rank test. The number of patients who had any arrhythmias in the intensive care unit (ICU) was significantly lower in group M than in group E+D (21.7% [5/23] v 50% [11/22], p = 0.03). The duration of ICU stay did not have statistical difference between groups (group M 19; interquartile range [IQR], 15-28) v group E+D 19.5 (IQR, 16.3-35.5) days, p = 0.57). Conclusions Perioperative milrinone infusion improved the mortality after the Norwood-Sano procedure. Potential advantages of milrinone compared with epinephrine are fewer arrhythmias and better systemic perfusion, which could decrease lethal cardiac events in the ICU.

We report 3 cases in sinus plication technique utilized to repair aortic or neoaortic root dilation and valve regurgitation after congenital heart disease operation.

Surgical restoration of subvalvular geometry is very important to prevent recurrence of ischemic/functional mitral regurgitation. We evaluated an approach to the radical repair of left ventricular (LV) remodeling for three different cases with responsible coronary lesions. Leaflet tethering was corrected by tugging of the papillary-ventricular complex, which consists of the base of papillary muscles and posterior LV wall. The main lesion of the postinfarction scar was concomitantly excluded. Restoration of LV remodeling diminished mitral regurgitation with minimal leaflet tethering and improved systolic LV function. This technique may be an aggressive and encouraged approach for patients with ischemic/functional mitral regurgitation.

Transvenous lead extraction (TLE) is necessary because of system infection, lead malfunction, or system upgrade. Patients with severe left ventricular dysfunction (SLVD) undergoing TLE may be at a higher risk because hemodynamic parameters may change unfavorably during or after TLE; however, this has not yet been clarified.The purpose of this study was to examine whether patients with SLVD undergoing TLE have higher mortality.All patients who underwent TLE were stratified as follows: patients with ejection fraction ≤ 35% (SLVD group) and those with ejection fraction35% (non-SLVD group).We assessed the data of 200 patients [SLVD group, 36 (18%); non-SLVD group, 164 (82%)]). Brain natriuretic peptide level and cardiac resynchronization therapy rate were higher in the SLVD group than in the non-SLVD group. There were no significant between-group differences in major complications and clinical success rates. Patients with SLVD were more likely to require additional hemodynamic support, such as catecholamine infusion, temporary atrium-ventricle sequential pacing, and temporary cardiac resynchronization therapy pacing (27.8% vs 1.2%; P.001). The survival rate was not significantly different between the groups at 30 days and 1 year after TLE (SLVD vs non-SLVD: 30 days: 97.2% vs 99.4%; P = .215; 1 year: 80.6% vs 91.5%; P = .053). Multivariate Cox regression analysis revealed log brain natriuretic peptide and serum hemoglobin levels as predictors for 1-year mortality.The prognosis after TLE was comparable between patients with and without SLVD. However, additional hemodynamic support was often necessary for patients with SLVD.

The number of patients with adult congenital heart disease (ACHD) has been dramatically increasing and adults with congenital heart disease now outnumber children with congenital heart disease. However, patients with ACHD are still at increased risk of morbidity and mortality due to residua and sequelae. Although echocardiography is an indispensable imaging modality in the comprehensive assessment of ACHD, accurate echocardiographic assessment of ACHD is challenging especially for physicians or sonographers who are not familiar with ACHD because of its complex morphology, physiology, and hemodynamics. A recently developed fusion imaging technology can provide synchronized display of real-time echocardiographic images and multiplanar reconstruction images of computed tomography or magnetic resonance imaging corresponding to the image plane of real-time echocardiography. We have reported the clinical utility of this fusion imaging technology for the precise evaluation of complex ACHD. On the other hand, conventional echocardiographic technology also plays an important role in assessing unique ACHD pathophysiology. For example, restrictive right ventricular physiology is a common finding after tetralogy of Fallot or pulmonary stenosis repair and can be evaluated by conventional pulsed-wave Doppler. In this review, we discuss the clinical usefulness of modern and conventional echocardiographic technologies for the evaluation of ACHD by presenting a case series.

Takeuchi repair is a unique surgical approach in anomalous left coronary artery from the pulmonary artery. We present an adult patient with anomalous left coronary artery from the pulmonary artery with multiple late structural complications after Takeuchi repair who was evaluated using multimodality imaging, including newly developed cardiac fusion imaging with computed tomography and echocardiography. (Level of Difficulty: Advanced.), Central Illustration

Background Congenital heart disease (CHD) is the most common neonatal and pediatric cardiac indication for extracorporeal membrane oxygenation (ECMO). Risk factors of survival and neurologic complication were different in many centers. We sought to evaluate survival and neurological outcome after ECMO in patients with CHD. Methods We retrospectively reviewed the medical records of 37 patients (Results The median ECMO duration was 160 (91–286) h. Twenty-nine patients (78%) were successfully weaned off ECMO. Overall survival to hospital discharge was 59%. Risk factors of mortality were as follows: ECMO duration >1 week and urine output Conclusions Longer ECMO duration and lower urine output were associated with increased mortality. Neurologic outcomes were not satisfactory when CPR was required for a longer period before ECMO establishment.

Pathological mineralization, such as that in atherosclerosis or following transplantation of biomaterials, is associated with millions of deaths worldwide. A deeper understanding of the mechanisms underlying mineral nucleation in pathological mineralization will facilitate the development of novel bioinspired materials/techniques as therapeutics for either disease prevention or bone tissue reconstruction. The objective of this study was to perform a systematic and ultrastructural analysis of the nucleation sites of atherosclerotic mineralization from the viewpoint of material science. Histological staining, ultrastructural analysis, and selected area elemental mapping of human atherosclerotic tissues revealed two different nucleation sites in a collagenous and a lipidic area, respectively, comprising phospholipidic large vesicles (average 827 ± 470 nm) and non-vesicular phospholipids. In both sites, the initial minerals were identified to be amorphous calcium phosphate (ACP), which then transformed into hydroxyapatite (HAp). Mineralization assays in vitro was performed by incubating non-vesicular low- (LDL) and high- (HDL) density lipoprotein and cholesterol in 2 mM CaCl2 for 2 days, without additional phosphate. Calcium ions in the formed minerals, measured using atomic absorption (AA) spectroscopy, showed that only LDL was mineralized. We then evaluated the mineralization of the major phospholipids, phosphatidylserine (PS) and phosphatidylcholine (PC) under the same conditions. The results of AA and mineral dry weight showed that PS was more abundantly mineralized than PC, most likely due to its higher hydrolysis rate. Similar to the in vivo findings, PS mineralization initially formed ACP, which then crystallized into HAp. Taken together, these results indicated that atherosclerosis involves two distinct nucleation sites and that non-vesicular phospholipids were one of the major nucleation sites for atherosclerotic mineral formation.

In donation after circulatory death heart transplantation, the donor heart is exposed to circulatory load. The right ventricle, due to its structure, has high compliance for volume load but is particularly vulnerable to increased pressure load. This study used a porcine model to conduct a functional assessment of the hemodynamics of the heart, with a focus on the right ventricle. Six pigs weighing 24.6 ± 1.4 kg were used. Circulatory death was induced by asphyxiation after median sternotomy. After 30 minutes in the state of global warm ischemia, the ascending aorta was clamped, followed by a 20-minute reperfusion of the heart with a 20°C blood cardioplegia solution. Systemic circulation was established by cardiopulmonary bypass after aortic cross-clamping. After initial reperfusion, the blood cardioplegia solution was replaced with blood. The blood was then rewarmed while the heart was still in a non-working state. Cardiac function was assessed twice in situ, first by the thermodilution method, and then, by the pressure-volume measurement both at preischemia and at three hours after initiation of reperfusion. The recovery rate of cardiac output was 75%. End-systolic elastance (P = .02) and pulmonary arterial elastance significantly increased (P = .03), but the ratio of arterial elastance to end-systolic elastance was preserved (P = .91) in the right ventricle. Despite a decrease in cardiac output after reperfusion from warm ischemia, the right ventricle had a potential to respond the elevated afterload. It is important that donations after circulatory death heart transplantation should be performed with attention to avoiding right ventricular distension.

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 The aim of this study was to evaluate the long-term outcomes of the Norwood procedure with right ventricle–pulmonary artery (RV–PA) conduit for hypoplastic left heart complex. METHODS A retrospective observational study was performed in 136 patients with hypoplastic left heart complex who underwent a Norwood procedure with RV–PA conduit between 1998 and 2017. The probabilities of survival, reintervention and Fontan completion were analysed. RESULTS Stage 1 survival was 91.9% (125/136). Reintervention for PA stenosis was needed for 22% and 30% at stages 2 and 3, respectively, while 15% underwent reintervention for aortic arch recoarctation. Among 106 bidirectional Glenn survivors, 93 (68% of the total number of patients) had a Fontan completion, while 4 were not considered to be Fontan candidates. Risk factors for overall mortality included weighing CONCLUSIONS Probabilities of survival and Fontan completion were acceptable under the current surgical strategy incorporating RV–PA Norwood procedure as the first palliation. Incorporating a strategy to maintain PA growth and ventricular function through the staged repair is of prime importance. Further studies are necessary to observe changes in atrioventricular regurgitation as well as in right ventricular function, in patients who require atrioventricular valve interventions during the staged Fontan completion.

Previous studies demonstrated that the incidence of atrial fibrillation (AF) was significantly increased when patients aged ≥40 years had surgical atrial septal defect (ASD) closure (sASD). However, limited information is available on such findings in transcatheter ASD closure (tASD). The purpose of this study was to investigate the incidence of newly developed AF after tASD in patients aged ≥40 years in whom preoperative AF or atrial flutter (AFL) had not been detected and to compare with the incidence after sASD.The medical records of patients aged ≥40 years without a history of AF or AFL who underwent tASD (n = 281) or sASD (n = 24) were reviewed. Patients who had catheter ablation before the ASD closure were excluded. Patients with a patent foramen ovale were also excluded. The incidence of newly developed AF after ASD closure and the risk factors for that were evaluated statistically.Eleven patients had newly developed AF (5 in tASD and 6 in sASD) postoperatively including 6 persistent AF (3 each after tASD and sASD). The cumulative incidence of newly developed AF was 0.7% in tASD and 16.7% in sASD, and 2.7% and 20.8% at 1 and 5 years, respectively (p 0.001). ASD diameter ≥30 mm and sASD were potential risk factors for newly developed AF after ASD closure and postoperative persistent AF.In patients aged ≥40 years without a history of AF or AFL, the incidence of newly developed AF after tASD closure was lower than that after sASD. A large ASD more than 30 mm diameter was a potential risk factor for development of AF even if it is closed by transcatheter procedure. Further long-term evaluation after tASD is required to clarify preventive benefit for new onset AF in adult ASD population.

Aims Angiotensin II (AngII) is a potential contributor to the development of abdominal aortic aneurysm (AAA). In aortic vascular smooth muscle cells (VSMCs), exposure to AngII induces mitochondrial fission via dynamin-related protein 1 (Drp1). However, pathophysiological relevance of mitochondrial morphology in AngII-associated AAA remains unexplored. Here, we tested the hypothesis that mitochondrial fission is involved in the development of AAA. Methods and results Immunohistochemistry was performed on human AAA samples and revealed enhanced expression of Drp1. In C57BL6 mice treated with AngII plus β-aminopropionitrile, AAA tissue also showed an increase in Drp1 expression. A mitochondrial fission inhibitor, mdivi1, attenuated AAA size, associated aortic pathology, Drp1 protein induction, and mitochondrial fission but not hypertension in these mice. Moreover, western-blot analysis showed that induction of matrix metalloproteinase-2, which precedes the development of AAA, was blocked by mdivi1. Mdivi1 also reduced the development of AAA in apolipoprotein E-deficient mice infused with AngII. As with mdivi1, Drp1+/− mice treated with AngII plus β-aminopropionitrile showed a decrease in AAA compared to control Drp1+/+ mice. In abdominal aortic VSMCs, AngII induced phosphorylation of Drp1 and mitochondrial fission, the latter of which was attenuated with Drp1 silencing as well as mdivi1. AngII also induced vascular cell adhesion molecule-1 expression and enhanced leucocyte adhesion and mitochondrial oxygen consumption in smooth muscle cells, which were attenuated with mdivi1. Conclusion These data indicate that Drp1 and mitochondrial fission play salient roles in AAA development, which likely involves mitochondrial dysfunction and inflammatory activation of VSMCs.

Background: There is limited information on outcomes of adult patients with pulmonary atresia with intact ventricular septum (PA-IVS) due to the low incidence of disease and the large variation of surgical histories. Methods: Among 58 patients with repaired PA-IVS, a total of 32 patients aged ≥16 years and who were followed at our institution between January 2003 and December 2018 were reviewed. Surgical history, clinical outcomes, and laboratory, echocardiographic and electrocardiographic data were obtained by chart review. Results: Follow-up was from the age of 16 years and the median age at the latest follow-up was 23.7 years. Twenty-four patients had undergone biventricular repair (BVR), 3 had undergone one-and-a half ventricular repair (1.5VR), and 5 had undergone univentricular repair. Over a median follow-up period of 7.7 years (interquartile range: 4.1–11.0 years), 1 BVR patient died suddenly and 7 patients had heart failure. Arrhythmias were present in 5 patients. Ten patients underwent surgical re-interventions, including 4 BVR take-downs with conversion to 1.5VR and 3 Fontan conversions. Overall survival, heart failure-free, arrhythmia-free, and surgical re-intervention-free rates at 5 years and 10 years from the age of 16 years were 96.2% (95% confidence interval [CI], 77.2–99.4) and 96.2% (95% CI, 77.2– 99.4), 81.4% (95% CI, 62.1–92.1) and 74.6% (95%CI, 52.3–88.7), 88.7% (95% CI, 70.1–96.3) and 75.9% (95% CI, 51.7–90.2), and 80.7% (95% CI, 60.8–91.8) and 70.8% (95% CI, 49.7–85.7), respectively. Conclusion: Adults with PA-IVS have preserved long-term survival regardless of the early operative strategy, while they are at risk for heart failure, arrhythmia, and surgical re-intervention. Thus, detailed and continued follow-up is mandatory for all PA-IVS patients from childhood to adulthood.

An increasing number of patients will require pulmonary valve replacement following repair of tetralogy of Fallot. Most of these patients have a history of surgical repair with a transannular patch (ie, pulmonary valve not preserved). Ten to twenty years after the initial repair, severe pulmonary regurgitation results in right ventricular dilation and dysfunction. There are multiple prosthetic valve options and techniques to use when performing pulmonary valve replacements. Herein, we describe our technique of bioprosthetic pulmonary valve replacement.

The Norwood procedure consists of a complex reconstruction resulting in: (1) unobstructed systemic outflow from the right ventricle to neo-aorta, (2) creation of large atrial septal defect, and (3) a controlled source of pulmonary blood flow (PBF) from the systemic circulation. The Sano modification provides the source of PBF from the right ventricle. Both procedures have advantages and disadvantages, therefore, there have been many debates regarding which procedure is better as a first stage palliation. Another ongoing debate of first stage palliation is the method of reconstruction of the neo-aorta. Aortic arch obstruction increases afterload and leads to ventricular dysfunction and tricuspid regurgitation. Recent SVR Trial identified recurrent coarctation requiring intervention in 18% of patients. We believe that another important point regarding reconstruction of the neo-aorta is the use of only native tissue and minimizing the use of inelastic patch material. Because elastic properties of the arterial tree are known to have a profound influence on ventricular function, arterial elasticity may therefore be of particular importance to those patients with hypoplastic left heart syndrome. During the past 20 years, we have modified our original technique of aortic arch reconstruction. Proximal arch plication and creation of elongated flaps of the main pulmonary artery are 2 of our recent modifications which facilitate neo-aortic reconstruction with native tissue. We describe a technique where direct anastomosis of neo-aorta is achieved with avoidance of foreign material in a majority of our patients.

Sudden cardiac events in young athletes are a major concern in the field of sports cardiology. Although coronary artery anomalies remain a major cause of cardiac events in young athletes, only a few cases have been diagnosed prior to critical events. Here, we present the case of a previously asymptomatic young male runner who experienced sudden cardiac arrest at the end of a marathon. The patient immediately received cardiopulmonary resuscitation from a bystander and was transported to an emergency hospital. As his electrocardiogram showed ventricular fibrillation, he was treated with electric shock, and his rhythm was successfully converted to a normal sinus rhythm. Following successful resuscitation, the patient was diagnosed with an anomalous origin of the right coronary artery from the left sinus of Valsalva with an intramural course. The patient underwent coronary artery bypass using the right internal thoracic artery. Fifteen years later, the coronary bypass was found to be blocked, but the patient was asymptomatic. However, an exercise electrocardiogram revealed ST-segment elevation in the inferior leads. The patient then underwent an unroofing procedure. He has remained asymptomatic without complications for two years after the second surgery.

We present our experience with transcatheter vascular occlusion using 0.035-inch hydrogel expandable coils, which has been reported only in a few cases in the pediatric cardiology fields.This study is a retrospective analysis of all patients who underwent transcatheter embolization with 0.035-inch hydrogel coils at the Department of Pediatrics, Okayama University Hospital, between October 2018 and September 2020.Twenty patients with a median age of 5.1 years (0.05-26.0 years) and a median weight of 13.8 kg (3.0-56.8 kg) were included. A total of fifty-four 0.035-inch hydrogel coils, including 35 Azur 35 and nineteen Azur CX 35 coils (Terumo, Tokyo, Japan), were successfully deployed in 22 target vessels. The target vessels consisted of 10 aortopulmonary collaterals, 8 veno-venous collaterals, and 4 pulmonary arteriovenous malformations. We achieved technical success in all the target vessels. In total, the mean target vessel diameter was 4.4 mm, the mean number of 0.035-inch hydrogel coils was 2.5 per vessel. The mean device to vessel ratio was 1.6 for the anchor coil and 1.2 for the additional coil. Post-implantation angiograms revealed that the primary occlusion rate was 18/22 (82%). There were no periprocedural complications.The 0.035-inch hydrogel expandable coils are effective and safe in patients with congenital heart disease and vascular anomalies. These occlusion devices could be valuable options for interventional pediatric cardiologists.

In patients with midventricular obstructive hypertrophic cardiomyopathy (HCM), a transapical approach allows excellent exposure for midventricular myectomy. Although the ventriculotomy is required on the antero-lateral left ventricular wall, the minimal ventriculotomy would be ideal, especially for cases with concomitant apical aneurysm. We report a case of midventricular obstructive HCM with concomitant apical aneurysm. Through the minimal ventriculotomy within the aneurysm, the hypertrophic septum was successfully hollowed out under repeated ultrasound guidance. Intraoperative ultrasound guidance is useful for transapical septal myectomy in midventricular obstructive HCM.

Graphical abstract, Highlights • BVR can be used in patients with PA-IVS to avert abnormal hemodynamics. • Restrictive RV physiology is a unique Doppler echocardiographic feature in patients undergoing BVR. • Although 1.5VR conversion from BVR relieves congestive symptoms, restrictive RV physiology is not diminished.