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

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.

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 Various types of conduits are available for right ventricular outflow tract reconstruction (RVOTR). The bovine jugular vein graft (BJVG) and expanded polytetrafluoroethylene graft (ePTFEG) have been descrived as an alternative to the homograft for RVOTR. Purpose- This study summarized the results to evaluate the single-center operation of RVOTR using BJVG and ePTFEG. Methods The valve functions of 27 patients under 20 years old who underwent primary RVOTR with BJVG and 26 patients with ePTFEG at our university hospital between 2013 and 2018 were retrospectively investigated. The valve conditions were assessed using echocardiography and cardiac catheterization. Results The median age at the time of operation was 1.8 years old (range, 6 days to 7.8 years old) with BJVG and 2.2 years old (range, 8 months to 9.1 years old) with ePTFEG. The median follow-up time was 3.4 years (range, 2 months to 5.2 years) with BJVG and 2.1 years (range, 1 month to 5.1 years) with ePTFEG. The peak RVOT gradient of BJVG was lower than ePTFEG (10.6±7.7 mmHg versus 18.1±16.2 mmHg, P=0.035). There were no differences in branch pulmonary stenosis defined as peak gradient up to 36mmHg (40.7% versus 50.0%, P=0.50) and pulmonary regurgitation graded worse than moderate (18.5% versus 11.5%, P=0.48) with BJVG and ePTFEG, respectively. Aneurysmal dilatation of the conduit was seen 22.2% with BJVG but none of patients with ePTFEG (P=0.01). All of patients with aneurysmal dilated BJVG had branch pulmonary stenosis. There were no differences in catheter intervention for branch pulmonary stenosis (22.2% versus 30.8%, P=0.48) and conduit replacement (11.1% versus 7.7%, log rank P=0.67) with BJVG and ePTFEG, respectively. There were no deaths during the fllow-up period in both groups. Conclusions The outcomes of RVOTR with BJVG and ePTFEG were clinically satisfactory. Aneurysmal dilatation was seen with BJVG and branch pulmonary stenosis was the risk factor for aneurysmal dilatation.

Background Donation after circulatory death (DCD) heart transplantation has been debated over the past decades because of the shortage of donor. The right ventricular dysfunction is one of the remaining problems for clinical implication of DCD heart transplantation. DCD hearts suffering from the volume overload have a potential to aggravate the right ventricular dysfunction after heart transplantation. The c-fos mRNA is one of the “immediate” response genes to mechanical stresses, such as myocardial cell stretch, without neural and humoral factors. In this study, we assessed myocardial stretch during asphyxiated cardiac arrest using c-fos mRNA expression. Purpose The purpose of this study is to reveal the impact of right ventricular volume overload during asphyxiated cardiac arrest. Methods Male Wistar rats (8 weeks of age, n=18) were anesthetized with paralyzed ventilation. The trachea was dissected and ligated to initiate asphyxiation. Hearts were harvested at 3 time points: 0, 15 and 30 minutes after termination of the ventilation. Free walls of right and left ventricle were sectioned and immersed in RNA stabilization solution as soon as possible. Total RNA was extracted from these tissues using a guanidine thiocyanate-phenol-chloroform method and cDNA was synthesized using a reverse transcriptase. Next, we measured the quantified expression level by using the droplet digital PCR method with a probe and primers for c-fos gene. Expression of c-fos level was divided by extracted TATA binding protein (TBP) level as a control marker, the ratio of c-fos and TBP was used in analysis. Results In the left ventricle, the expression of c-fos rapidly increased by 15 minutes (0.81±0.24 (c-fos/TBP), p Conclusion These results suggest that the volume overload to the right ventricle during asphyxiated cardiac arrest prolongs compared to that to the left ventricle, which may cause the right ventricular dysfunction after DCD heart transplantation.

Objectives To achieve the growth of right-sided heart structures, our choice of the first palliation for patients with pulmonary atresia and intact ventricular septum (PA-IVS) includes a modified Blalock-Taussig shunt (BTS) with pulmonary valvotomy. We sought to analyse the impact of the first palliation on the growth of right-sided heart structures and factors associated with a choice of definitive surgical procedure. Methods Fifty patients with PA-IVS who underwent a staged surgical approach from 1991 to 2012 were retrospectively reviewed. Results Right ventricular (RV)-coronary artery fistulas were seen in 42% of patients at the time of birth. All 50 patients had a modified BTS with pulmonary valvotomy. Six patients died after the first palliation or inter-stage. Thirty patients achieved a biventricular repair (BVR group), 6 patients had a 1 + 1/2 ventricular repair (1 + 1/2V group) and 5 patients had a Fontan completion (Fontan group). After modified BTS with pulmonary valvotomy, tricuspid valve z-score did not increase in any of the group (BVR: pre -2.79 vs post -2.24, 1 + 1/2V: pre -5.25 vs post -6.69, Fontan: pre -6.82 vs post -7.94). Normalized RV end-diastolic volume increased only in BVR group after modified BTS with pulmonary valvotomy (BVR: pre 32% vs post 64%, 1 + 1/2V: pre 43% vs post 42%, Fontan: pre 29% vs post 32%). Major RV-coronary artery fistula was a strong factor with proceeding single-ventricle palliation [BVR: 4/30 (13%) patients, 1 + 1/2V: 1/6 (17%) and Fontan: 4/5 (80%)]. Conclusions Tricuspid valve growth was not obtained by modified BTS with pulmonary valvotomy; therefore, tricuspid valve size at birth appeared to be a predictor for achieving BVR. Proportionate RV growth was seen only in patients who achieved BVR. However, RV growth was not seen in patients having 1 + 1/2 ventricular repair. Major RV-coronary artery fistula was a strong predictor for proceeding single-ventricle palliation.

There is still no consensus on the optimal management to treat pediatric mediastinitis. We assessed the efficacy of continuous irrigation and vacuum-assisted closure (VAC) for mediastinitis in children. This study retrospectively reviewed 20 patients aged

Objective: A lateral tunnel-total cavopulmonary connection (LT-TCPC) using a right atrial (RA) free wall is the first choice of treatment for patients with a small body weight in this institute. Whether the growth of the LTis appropriate or not according to the growth of the body remains controversial. In addition, the optimal initial diameter of an LTis unknown. The purpose of this study was to verify the growth of the LT. Methods: Ninety-one patients of a total of 267 TCPC cases underwent an LT-TCPC at less than 5 years of age in this institute between March 1991 and June 2008. The data on 47 of the 91 patients, which were available to investigate the LT growth, were retrospectively reviewed. The mean age at LTTCPC was 37 11 months (16—57 months). The mean body weight at TCPC was 12.4 2.4 kg (7.6—20.0 kg). The initial LT diameter was determined with Hegar’s sizer of the estimated half-pulmonary arterial (PA) diameter, which is a diameter that results in half of the dimension of thenormalpulmonaryvalve.ThemeasuredmaximumLTdiameter(mm)dividedbytheestimatedhalf-PAdiameter(mm)wasconsideredastheLT index. The size of the LTwas evaluated using either echocardiography or angiography. Results: The mean follow-up period was 7.4 3.5 years (1.6—13.5years).TheLTindexwasinitially2.0 0.7(1.3—4.5),2.0 0.4(1.3—3.2)at1 yearafteroperation,2.1 0.5(1.5—3.2)at5 yearsafter operation, 1.9 0.4 (1.5—2.8) at 10 years after operation and 2.1 0.5 (1.6—2.5) at 13 years after operation, respectively. Conclusions: LT growth suitable for the body growth can be expected. Although there was some variation in the initial LT diameter, the LT index tended to converge at 2.0 with growth. # 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

The anatomy of a persistent left superior vena cava (SVC) to the left atrium (LA) without the innominate vein can make it challenging to complete intracardiac repair. We reviewed our five cases of the direct end-to-side anastomosis of SVCs to facilitate anatomical repair of SVC-right atrial connection for biventricular repair. Diagnoses were two partial atrioventricular septal defect with left isomerism, one complete atrioventricular septal defect (CAVSD) with left isomerism, one CAVSD without isomerism and one atrioventricular discordance and double outlet right ventricle with right isomerism. Mean age at the operation was 20+/-23 months (4-58 months) and body weight was 7.8+/-3.4 kg (4.8-12.7 kg). After completion of intracardiac repair, the SVC to LA was divided and end-to-side anastomosed to the SVC to the right atrium during cardiopulmonary bypass. No early or late death occurred during follow-up of 14.4+/-6.9 months (7-23 months). None of the patients developed an obstruction at the anastomosis site of the SVCs. The direct end-to-side anastomosis of SVCs achieved an excellent anatomical SVC-right atrium connection in complex congenital heart diseases.

Aortic root dilatation is rare in children, and is often secondary to Marfan syndrome (MFS). We experienced a case of a three-year-old boy (92 cm, 12 kg) with MFS presenting with progressive dilatation of aortic root. We electively performed a valve-sparing aortic root replacement using a 24-mm Gelweave Valsalva graft. Although the patient required a mitral valve repair due to infective endocarditis postoperatively, the recovery from the second surgery was uneventful. This case is one of the youngest children of valve-sparing aortic root replacement in the literature.