Mid-Term Results and Risk Factors For 10 Years of Functional Single Ventricle Associated With Total Anomalous Pulmonary Venous Connection.

BACKGROUND: There are few surgical treatment results in elderly patients with functional single ventricle (FSV) and total anomalous pulmonary venous connection (TAPVC). We retrospectively analyzed 10 years of mid-term surgical treatment results and risk factors of these age-specific people. METHODS: Between March 2008 and December 2018, 43 consecutive patients with FSV and TAPVC received initial surgical palliation in our center. There were 20 cases of supracardiac TAPVC, 21 of cardiac type, and two cases of mixed type. Initial surgical palliation procedures involved pulmonary artery banding (PAB) for patients, modified Blalock-Taussing shunt (mBTs) for five patients, and bidirectional Glenn (BDG) for 34 patients. TAPVC repair was performed in 12 patients during BDG. RESULTS: The 1-year and 5-year overall survival rates were 69.7% and 62.8%, respectively. In TAPVC repair group and non-TAPVC repair group, the 1-year overall survival rates after initial surgical palliation were 41.7 and 80.5%, respectively, and the 3-year ones were 25% and 77%, respectively. There were significant differences in the type of TAPVC (P < 0.001), preoperative pulmonary venous obstruction (P = 0.001), and overall mortality (P = 0.001) between these two groups. Cox univariate and multivariable analysis indicated concomitant TAPVC repair was the only risk factor for mortality. CONCLUSIONS: The mid-term results of surgical treatment of FSV and TAPVC, especially for patients who underwent concomitant TAPVC repair, remain poor. TAPVC repair may be a priority over single-ventricular palliative surgery for patients with FSV and TAPVC.

Experience of repair ventricular septal defect with left superior vena cava through right axillary thoracotomy.

OBJECTIVE: To summarize the experience in the treatment of repair ventricular septal defect with left superior vena cava (LSVC) through right axillary thoracotomy. To explore the surgical strategy of treating VSD with LSVC through right axillary thoracotomy. METHODS: right axillary thoracotomy and median sternotomy were performed in 73 cases of ventricular septal defect with LSVC in our center from 2018 to 2019. Perioperative data and surgical information were analyzed retrospectively. RESULTS: There were 54 cases of R-group and 19 cases of S-group with median age of 0.8 years (0.5-1.6years). In the 73 patients, 21(38.9%) were men and 52 (61.1%) women. The operation time of R-group was shorter than S-group, p<0.05. The postoperative drainage in R-group was less than S-group, p<0.05. The mechanical ventilation time was longer in the S-group than in the R-group, p<0.05. There were no deaths, serious complications and readmission in the follow-up 6 months(3-10months). CONCLUSION: Right axillary thoracotomy is a safe procedure with excellent cosmetic and clinical results for ventricular septal defect with left superior vena cava. It has the advantages of short operation time, less bleeding and short postoperative time.

Targeted treprostinil delivery inhibits pulmonary arterial remodeling.

INTRODUCTION: Pulmonary arterial hypertension (PAH) is a fatal disease caused by the progressive remodeling of pulmonary arteries (PAs). Treprostinil (TPS) is a tricyclic benzidine prostacyclin clinically used for PAH treatment. However, due to low bioavailability, short half-times, and severe systemic side effects, TPS efficacy remains limited. METHODS: In this study, glucuronic acid (GlcA)-modified liposomes were developed to improve the site-specific delivery of TPS to pulmonary arterial smooth muscle cells (PASMCs) by targeting the glucose transporter-1 (GLUT-1) in vitro and in vivo. RESULTS: Non-GlcA-modified and GlcA-modified liposomes encapsulating TPS were 106 ± 1.12 nm in diameter. The drug encapsulation efficiency (EE) was 92%. Data from rat PASMCs showed that GlcA-liposomes enhanced the inhibitory effects of TPS on PASMC proliferation and migration by suppressing growth factor expression, including transforming growth factor-ß1 (TGF-ß1), connective tissue growth factor (CTGF), and cAMP, which was possibly mediated by the cAMP-C/EBP-α p42-p21 signaling pathway. In PAH model rats, GlcA-modified liposomes significantly improved TPS bioavailability and sustained its release over time. Most importantly, the selective inhibition of pulmonary arterial pressure, rather than systemic arterial pressure, indicated the increased pulmonary-specific accumulation of TPS. Of the three TPS formulations, TPS-loaded GlcA-modified liposomes exhibited the most potent activity by inhibiting PA remodeling and muscularization, decreasing PA medial thickening, suppressing collagen deposition in PAs, and attenuating right ventricle hypertrophy (RVH) in sugen-5416-induced PAH rats. CONCLUSIONS: The GLUT-1-targeted delivery of TPS increased pulmonary specificity and enhanced TPS anti-PAH activities in vivo and in vitro.

Emergency surgical treatment of total anomalous pulmonary venous connection.

BACKGROUND: This study explored the strategy and effect of emergency surgical treatment for total anomalous pulmonary venous connection (TAPVC). METHODS: From March 2009 to February 2020, 78 patients with TAPVC underwent emergency surgical correction. There were 51 males and 27 females. The median age was 39.5 days, and the median weight was 4.0 kg. Preoperative percutaneous oxygen saturation was 80.8% ± 4.5%. RESULTS: Of the cases investigated, seven died during the perioperative period; 16 had a delayed chest closure; 19 had early pulmonary vein obstruction; two had secondary tracheal intubation; one had a brain complication; and one had a third-degree atrioventricular block. Low weight, younger age, cardiopulmonary bypass time, and aortic cross-clamp time were identified as risk factors for early mortality. During the follow-up from 4 to 137 months, 12 cases did not respond to follow-up requests. Ten patients died within 1-6 months after discharge. One patient underwent reoperation due to pulmonary vein obstruction. The longer hospital stay after the operation and intensive care unit time were identified as risk factors for late mortality. CONCLUSIONS: Emergency surgery for severe TAPVC patients after admission achieved good short-term results. Prenatal diagnosis should be strengthened to save more patients. The higher late mortality rate of such patients indicates that post-discharge management should be strengthened to reduce the occurrence of post-discharge deaths.

Higher Serum Lysophosphatidic Acids Predict Left Ventricular Reverse Remodeling in Pediatric Dilated Cardiomyopathy.

Background: The prognosis of pediatric dilated cardiomyopathy (PDCM) is highly variable, ranging from death to cardiac function recovery. Left ventricular reverse remodeling (LVRR) represents a favorable prognosis in PDCM. Disturbance of lipid metabolism is associated with the change of cardiac function, but no studies have examined lipidomics data and LVRR. Methods: Discovery analyses were based on 540 targeted lipids in an observational, prospective China-AOCC (An Integrative-Omics Study of Cardiomyopathy Patients for Diagnosis and Prognosis in China) study. The OPLS-DA and random forest (RF) analysis were used to screen the candidate lipids. Associations of the candidate lipids were examined in Cox proportional hazards regression models. Furthermore, we developed a risk score comprising the significant lipids, with each attributed a score of 1 when the concentration was above the median. All significant findings were replicated in a validation set of the China-AOCC study. Results: There were 59 patients in the discovery set and 24 patients in the validation set. LVRR was observed in 27 patients (32.5%). After adjusting for age, left ventricular ejection fraction (LVEF), and left ventricular end-diastolic dimension (LVEDD) z-score, lysophosphatidic acids (LysoPA) 16:0, LysoPA 18:2, LysoPA 18:1, and LysoPA 18:0 were significantly associated with LVRR in the discovery set, and hazard ratios (HRs) were 2.793 (95% CI, 1.545-5.048), 2.812 (95% CI, 1.542-5.128), 2.831 (95% CI, 1.555-5.154), and 2.782 (95% CI, 1.548-5.002), respectively. We developed a LysoPA score comprising the four LysoPA. When the LysoPA score reached 4, LVRR was more likely to be observed in both sets. The AUC increased with the addition of the LysoPA score to the LVEDD z-score (from 0.693 to 0.875 in the discovery set, from 0.708 to 0.854 in the validation set) for prediction of LVRR. Conclusions: Serum LysoPA can predict LVRR in PDCM patients. When the LysoPA score was combined with the LVEDD z-score, it may help in ascertaining the prognosis and monitoring effects of anti-heart failure pharmacotherapy.

Surgical repair of subaortic stenosis resection: 10 years of single-center experience in 65 patients.

BACKGROUND: Subaortic stenosis (SAS) was a rare congenital heart disease of left ventricular outflow tract (LVOT), ranging from "isolated" lesions to "tunnel" or "diffuse" lesions. We conducted a retrospective study to describe the characteristics of patients with different lesions and analyze the risk factors for reoperation. METHODS: In this study, we examined a single-center retrospective cohort of SAS patients undergoing resection from 2010 to 2019. Patients were classified as simple lesion group (n = 37) or complex lesion group (n = 28). Demographics, perioperative findings, and clinical data were analyzed. RESULTS: The surgical effect of the two groups was significantly lower than that before the operation (p < .05). The median age at operation was 6 (3-11.8) years. There was no operative mortality. In complex lesion group, cardiopulmonary bypass time (CPB time), aortic cross-clamping time (ACC time), mechanical ventilation time, and intensive care unit (ICU) stay time were longer. The median follow-up period was 2.8 years (range: 1-3.8), with two late death. Six patients (9.2%) required reoperation due to restenosis or severe aortic insufficiency. The freedom from reoperation rates at 5 years was 66.7% for simple lesion but only 52.3% for complex lesion (p = .036). CONCLUSIONS: Although the lesions include many forms, SAS resection was still satisfactory. However, the reoperation after initial surgical treatment was not infrequent, especially in patients with complex lesion.

RNA sequencing analyses in infants patients with coarctation of the aorta.

BACKGROUND: Coarctation of the aorta (CoA) is a serious innate heart disease. Although surgery results are generally good, some complications such as recoarctation and aortic aneurysm or persistent hypertension were serious threats to patient's health. To better understand the pathology of CoA and its underlying molecular mechanism is particularly important for early diagnosis and preventing the occurrence of its complications. However, the mechanisms of CoA remain unclear, especially for infants. METHODS: RNA sequencing (RNA-seq) was used to identify the differentially expressed genes (DEGs) in vascular tissues of 12 patients with CoA and 10 normal participants form 3- to 34-month-old infants. The characteristic of DEGs were validated by quantitative reverse transcription-polymerase chain reaction (qRT-PCR) and immunochemical staining (IHC) in vessels of patients with CoA and normal infants. RESULTS: A total of 2491 DEGs with the false discovery rate less than 0.05(> 1.5-fold, P < 0.05 change) were identified, including 443 upregulated genes and 2048 downregulated genes. The Gene Ontology enrichment analysis showed that 26 out of the 2491 DEGs identified were associated with cardiovascular diseases. These 26 genes were mainly associated with extracellular matrix (ECM) and smooth muscle cells (SMCs) differentiation. Three DEGs, that is, CNN1 (calponin), α-actinin1 and myosin heavy chain 11 MYH11, were validated using qRT-PCR and Western blot analysis. In addition, immunochemical staining showed that calponin and MYH11 were highly expressed on the surface and in the deep layers of the thickened intima respectively. CONCLUSION: This study comprehensively characterized the CoA transcriptome. Migration of extracellular matrix (ECM) and smooth muscle cells (SMCs) to the subendothelial space may be the major characteristic of CoA in infants.

Prognostic Model to Predict Postoperative Adverse Events in Pediatric Patients With Aortic Coarctation.

Background: Postoperative adverse events remain excessively high in surgical patients with coarctation of aorta (CoA). Currently, there is no generally accepted strategy to predict these patients' individual outcomes. Objective: This study aimed to develop a risk model for the prediction of postoperative risk in pediatric patients with CoA. Methods: In total, 514 patients with CoA at two centers were enrolled. Using daily clinical practice data, we developed a model to predict 30-day or in-hospital adverse events after the operation. The least absolute shrinkage and selection operator approach was applied to select predictor variables and logistic regression was used to develop the model. Model performance was estimated using the receiver-operating characteristic curve, the Hosmer-Lemeshow test and the calibration plot. Net reclassification improvement (NRI) and integrated discrimination improvement (IDI) compared with existing risk strategies were assessed. Results: Postoperative adverse events occurred in 195 (37.9%) patients in the overall population. Nine predictive variables were identified, including incision of left thoracotomy, preoperative ventilation, concomitant ventricular septal defect, preoperative cardiac dysfunction, severe pulmonary hypertension, height, weight-for-age z-score, left ventricular ejection fraction and left ventricular posterior wall thickness. A multivariable logistic model [area under the curve = 0.8195 (95% CI: 0.7514-0.8876)] with adequate calibration was developed. Model performance was significantly improved compared with the existing Aristotle Basic Complexity (ABC) score (NRI = 47.3%, IDI = 11.5%) and the Risk Adjustment for Congenital Heart Surgery (RACHS-1) (NRI = 75.0%, IDI = 14.9%) in the validation set. Conclusion: Using daily clinical variables, we generated and validated an easy-to-apply postoperative risk model for patients with CoA. This model exhibited a remarkable improvement over the ABC score and the RACHS-1 method.

Pulmonary artery banding in patients with functional single ventricle associated with pulmonary hypertension.

Background: To assess the impact of our surgical strategy for the treatment of patients with functional single ventricle and pulmonary hypertension, especially in patients>24 months old.Methods We retrospectively analyzed the clinical data of 97 patients with functional single ventricle and pulmonary hypertension undergoing pulmonary artery banding (PAB) in our hospital between April 2010 and December 2018. The surgical results, transition to Glenn operation and subsequent transition to Fontan operation were analyzed.Results: The 97 patients underwent PAB included 34 patients>24 months old, hospital mortality was 2.1% (2/97). 62 patients underwent the second-stage Glenn operation, and 21 patients underwent third-stage Fontan operation. On competing risk analysis, at 80 months after PAB, 81% had undergone the Glenn operation, and 13% were awaiting the Glenn operation. At 35 months after the Glenn operation, 2% of patients had died, 63% had undergone the Fontan operation, and 36% were awaiting the Fontan operation.Conclusion: PAB is an acceptable strategy for patients with functional single ventricle associated with pulmonary hypertension. Outcomes and results of subsequent Glenn and Fontan procedures are generally good included patients>24 months. Accompanied with unbalanced atrioventricular septal defect for lower ratio of transition to Glenn and Fontan operation.

Application of Left Axillary Incision in Patent Ductus Arteriosus Ligation.

BACKGROUND: Left posterolateral incision has been a conventional incision for patent ductus arteriosus ligation. This study aimed to evaluate the efficacy and safety of left axillary thoracotomy for patent ductus arteriosus ligation. METHODS: Between January 2013 and December 2019, the clinical data of 76 patients who underwent left axillary thoracotomy for patent ductus arteriosus ligation were compared with the data of a paired group of 101 patients who underwent left posterolateral thoracotomy. RESULTS: Compared with the left posterolateral group, the left axillary group showed less drainage (P < 0.05). Operation time, postoperative mechanical ventilation time, and postoperative hospitalization duration were similar between the groups. Complications were rare in both groups with no mortality during follow up. In total, 72 patient families (95%) in the left axillary group and 81 patient families (80%) in the left posterolateral group were satisfied with their cosmetic results (P < 0.01). CONCLUSIONS: A left axillary thoracotomy is as safe and effective as a left posterolateral thoracotomy for patent ductus arteriosus ligation. With lower trauma and better cosmetic results, this procedure provides a good alternative to the standard left posterolateral thoracotomy.

Preoperative percutaneous oxygen saturation is a predictor of postoperative adverse events after Ebstein's anomaly reconstruction.

BACKGROUND: Ebstein's anomaly (EA) is a kind of congenital heart disease, which is currently widely treated by cone reconstruction. However, the prediction of postoperative recovery is still challenging. METHODS: A retrospective analysis was performed on EA cases undergoing cone reconstruction from January 2010 to January 2016. Univariate and multivariate logistic regression analyses were performed, with postoperative adverse events defined as dependent variable and pre- and intraoperative parameters defined as independent variables. The predictive capacity of preoperative percutaneous oxygen saturation (SPO2 ) and Great Ormond Street (GOS) score was evaluated using areas under the curve of the receiver operating characteristic (ROC). RESULTS: Preoperative SPO2 was 95.7 ± 5.20%. Cardiopulmonary bypass, aortic cross-clamp, postoperative mechanical ventilation, and hospitalization time were 101.7 ± 28.26 min, 60.9 ± 18.04 min, 16 h (8, 22), and 8 days (7, 11), respectively. The incidence of total postoperative adverse events, including low cardiac output syndrome, mechanical ventilation more than 3 days, postoperative hospitalization more than 2 weeks, postoperative reintubation, extracorporeal membrane oxygenation assistance, and death, was 13.1% (n = 13). Low preoperative SPO2 (p = .001, odds ratio [OR] = 0.834), GOS score (p = .021, OR = 0.368), and cardiopulmonary bypass time (p = .034, OR = 1.021) were risk factors for adverse events. Multivariate logistic regression analysis showed that low preoperative SPO2 (p = .002, OR = 0.846) and GOS score (p = .043, OR = 0.577) were independent risk factors for adverse events. The areas of SPO2 and GOS score under the ROC curve were 0.764 and 0.740, respectively. CONCLUSIONS: Low preoperative SPO2 and GOS score were predictors of adverse events after cone reconstruction, and SPO2 was more convenient and objective than the GOS score.

A summary of second systemic pulmonary shunt for congenital heart disease with pulmonary hypoxemia.

BACKGROUND: There has been an increasing number of children with congenital heart disease that undergo primary or second systemic-pulmonary shunt, while there are few reports on the second systemic-pulmonary shunt. Therefore, this study summarizes the experience of second systemic-pulmonary shunt for congenital heart disease in our hospital. METHODS AND RESULTS: Sixty-five children with congenital heart disease who underwent systemic-pulmonary shunt for the second time in our hospital were analyzed. At the early stage after the operation, cyanosis improved and SpO2 significantly increased. One patient died in hospital (1.54%) and the causes of death were aggravated atrioventricular regurgitation, low cardiac output syndrome, and liver failure. Early complications occurred in 18 patients (27.7%). All the children were rechecked in our hospital every 3-6 months and the McGoon index significantly increased. CONCLUSION: Systemic-pulmonary artery shunt can promote pulmonary vascular development, improve cyanosis symptoms, and increase the chance of radical treatment in children with pulmonary vascular dysplasia.

The complete chloroplast genome sequence of Elaeocarpus braceanus: the first Celastraceae plastome.

The first complete chloroplast genome (cpDNA) sequence of Elaeocarpus braceanus was determined from Illumina HiSeq pair-end sequencing data in this study. The cpDNA is 158,225 bp in length, contains a large single-copy region (LSC) of 85,731 bp and a small single-copy region (SSC) of 17,654 bp, which were separated by a pair of inverted repeats (IR) regions of 27,420 bp. The genome contains 133 genes, including 88 protein-coding genes, 8 ribosomal RNA genes, and 37 transfer RNA genes. Further phylogenomic analysis showed that E. braceanus clustered in a clade in Celastrales order.

Palliative Operations for Cyanotic Congenital Heart Disease with Severely Asymmetrical Pulmonary Arteries.

BACKGROUND: Surgically managing patients with complex congenital heart disease and severely asymmetrical pulmonary arteries is challenging. Here, we report our experience using combined palliative procedures. METHOD: The medical records of 28 patients with complex congenital heart disease and severely asymmetrical pulmonary arteries who underwent combined palliative procedures between January 2004 and April 2013 were retrospectively reviewed until January 2018. The patients were divided into three groups according to shunt procedure timing: in group A (n = 15), cavopulmonary and systemic-pulmonary shunting were performed simultaneously; in group B (n = 11), systemic-pulmonary shunting was performed first; and in group C (n = 2), cavopulmonary shunt was performed first. Patients were followed for a mean ± standard deviation of 4.18 ± 2.22 years. RESULTS: No operative deaths occurred. There were no postoperative complications in groups B or C, but there was one case of pulmonary effusion and one of chylothorax in group A. The superior vena cava pressures were higher in patients in groups A and B than in those in group C. The ventilatory support duration and intensive care unit stays were longer in group A than in groups B and C (p < 0.01). Hypoplastic pulmonary artery development significantly improved after the use of three systemic-pulmonary shunts (p < 0.05), while the peripheral oxygen saturation increased from 67%±17% preoperatively to 85%±8% postoperatively (p < 0.001). Haemoglobin concentration decreased from 190 ± 34 g/L preoperatively to 136 ± 26 g/L postoperatively (p < 0.001). Two patients underwent double ventricle correction. Two patients underwent Fontan procedure. One patient underwent one and a half ventricle correction. One patient underwent collateral occlusion. CONCLUSIONS: Combined palliative procedures can achieve acceptable arterial oxygen saturation without extra volume loading and rescue the hypoplastic pulmonary artery.

Correction of simple congenital heart defects by right axillary thoracotomy in adults.

BACKGROUND: Right axillary thoracotomy has been performed for open-heart procedures as a more aesthetic alternative to standard median sternotomy. This study aimed to evaluate the efficacy and safety of right axillary thoracotomy for the correction of simple congenital heart defects in adults. METHODS: Between January 2014 and December 2017, the clinical data of 180 adults who underwent right axillary thoracotomy for simple congenital heart defects were compared with the data of a paired group of 192 adults who underwent median sternotomy. RESULTS: Compared with the median sternotomy group, the right thoracotomy group showed shorter operative time, postoperative mechanical ventilation time, and postoperative hospitalization duration, as well as less drainage and transfusion volumes (P < .05). Aortic cross-clamping time, cardiopulmonary bypass time, and length of intensive care unit stay were similar between groups. No mortality or significant residual defects were reported in either group during follow-up. In total, 172 patients (95.6%) in the right thoracotomy group and 134 patients (69.8%) in the median sternotomy group were satisfied with their cosmetic results (P < .01). CONCLUSIONS: A right axillary thoracotomy is as safe and effective as a median sternotomy for the correction of simple congenital heart defects in adults. With lower trauma and better cosmetic results, this procedure provides a good alternative to the standard median sternotomy.

The expression of survivin in irreversible pulmonary arterial hypertension rats and its value in evaluating the reversibility of pulmonary arterial hypertension secondary to congenital heart disease.

The reversibility of pulmonary arterial hypertension (PAH) determines the operability of congenital heart disease (CHD) complicating with PAH, but it lacks a method for evaluating the reversibility. The current study aims to investigate the serum survivin level in irreversible PAH rats and to explore its potential as a biomarker for evaluating the reversibility of PAH in CHD patients. Irreversible PAH rats were characterized by prominent obstructive lesions resulting from the intimal formation, which was associated with decreased apoptosis and increased survivin expression, while reversible PAH rats were featured by medial hypertrophy resulting in mild occlusion, with increased apoptosis and unchanged survivin expression. In addition, the serum survivin was significantly increased in irreversible PAH rats when compared to both reversible PAH and control rats, and a positive correlation of serum survivin with survivin expression in the lung was confirmed. Third, the preoperative serum survivin was significantly higher in patients with irreversible CHD-PAH than in these with reversible CHD-PAH, and significant correlations between the serum survivin and BNP, preoperative pulmonary vascular resistance index, and postoperative mean pulmonary arterial pressure were also identified. In conclusion, the increased survivin level is a feature of irreversible PAH and the serum survivin represents a candidate biomarker reflecting the operability of CHD-PAH patients.

The efficiency of systemic-to-pulmonary shunts in older children with hypoplastic pulmonary arteries.

BACKGROUND: To investigate the effects of systemic-to-pulmonary shunts (SPSs) in older children with hypoplastic pulmonary arteries and the factors affecting the development of the pulmonary arteries. METHODS: Eighty-six children (older than 3 years) who received SPSs were retrospectively analyzed. The perioperative parameters, the postoperative diameter of the pulmonary artery were collected, and the factors influencing the growth of the pulmonary arteries after an initial palliative shunt operation were analyzed. RESULTS: Two patients died postoperatively (2.33%), and the pulse oxygen saturation (SpO2 ) increased from 71.70 ± 6.75% preoperatively to 85.20 ± 11.07% at discharge. During the follow-up period of 56 (10-99) months, 37 patients (43.02%) underwent subsequent procedures, and in the remaining patients, the McGoon ratio was increased from 0.96 ± 0.48 at the surgery to 1.30 ± 0.31 at the final assessment (P < 0.05). Univariate analysis indicated that age younger than 5 years old (P < 0.05), pulmonary artery forward flow (P < 0.05) and a diagnosis of tetralogy of Fallot (P < 0.05) played positive roles in the growth of the pulmonary artery after surgery, while children with a McGoon ratio less than 0.6 showed poor development of the pulmonary arteries (P < 0.05). Multivariate analysis showed that age younger than 5 years old (P < 0.05) and pulmonary artery forward flow (P < 0.05) were positive effectors on the growth of the pulmonary artery. CONCLUSIONS: Older children with cyanotic congenital heart disease benefited from a systemic-pulmonary shunt and showed increased postoperative oxygen saturation and development of the pulmonary arteries. Age younger than 5 years and pulmonary artery antegrade flow were the positive factors influencing the growth of the pulmonary arteries postoperatively.

Reverse Remodeling of Pulmonary Arterioles After Pulmonary Artery Banding in Patients ≥ 2 Years Old with Severe Pulmonary Arterial Hypertension and Congenital Heart Disease.

The purpose of this study was to evaluate the pathological changes of the pulmonary arterioles in patients ≥ 2 years of age who first underwent a pulmonary artery banding (PAB) procedure, followed by bidirectional Glenn or Fontan according to their specific conditions. This was a prospective study of 15 children diagnosed and treated with PAB at the Department of Cardiothoracic Surgery of Anzhen Hospital between January 2009 and December 2012. The percentage of media area (%MS) of pulmonary arteriole, the percentage of media thickness (%MT), and pulmonary arterial density (APSC) were calculated from lung tissue sections. Pulmonary artery pressure decreased significantly after PAB surgery (P < 0.01). Postoperative mean pulmonary artery pressure declined significantly (P < 0.01), the cardiothoracic ratio was reduced (P < 0.05), and percutaneous oxygen saturation (inhaled air) decreased to 80-85% (P < 0.01). %MT (from 35.1 ± 5.6% to 26.9 ± 4.3%, P < 0.01), %MS (from 51.4 ± 6.7% to 32.2 ± 7.4%, P < 0.01), and APSC (from 108.3 ± 38.5 to 83.6 ± 19.6, P < 0.01) were decreased significantly after PAB. Five patients underwent the bidirectional Glenn procedure and four underwent Fontan. In conclusion, the results suggest that PAB can reduce pulmonary artery pressure and that pulmonary arterial lesions can be reversed after PAB.

Role of miR-223-3p in pulmonary arterial hypertension via targeting ITGB3 in the ECM pathway.

OBJECTIVES: To investigate the functions of miR-223-3p and ITGB3 in pulmonary arterial hypertension (PAH). MATERIALS AND METHODS: Microarray analysis was used to detect differentially expressed genes and microRNAs. In in vitro models, the expressions of miR-223-3p and ITGB3 were detected by qRT-PCR and Western blot. α-SMA expression and cell proliferation were analysed by immunofluorescence and MTT assay, respectively. In in vivo models, PAH progressions were determined by measuring the levels of mPAP and RVSP. Lung and myocardial tissues were subjected to HE staining and Masson and Sirius red-saturated carbazotic acid staining to investigate the pathological features. RESULTS: The microarray analysis revealed that ITGB3 was upregulated, while hsa-miR-223-3p was downregulated in PAH. After the induction of hypoxia, miR-223-3p was downregulated and ITGB3 was upregulated in PASMCs. Hypoxia induction promoted cell proliferation and inhibited α-SMA expression in PASMCs. Both the upregulation of miR-223-3p and the downregulation of ITGB3 attenuated the aberrant proliferation induced by hypoxia conditions. After approximately 4 weeks, the mPAP and RVSP levels of rats injected with MCT were decreased by the overexpression of miR-223-3p or the silencing of ITGB3. The staining results revealed that both miR-223-3p overexpression and ITGB3 knockdown alleviated the pulmonary vascular remodelling and improved the PAH pathological features of rats. CONCLUSIONS: MiR-223-3p alleviated the progression of PAH by suppressing the expression of ITGB3, a finding which provides novel targets for clinical treatment.

Sirtuin 1 Mediates Hydrogen Sulfide-induced Cytoprotection Effects in Neonatal Mouse Cardiomyocytes.

BACKGROUND: Current knowledge indicates that oxidative damage and the following inflammation is pivotal pathway for myocardial cell death. In recent decades, hydrogen sulfide (H2S) has been identified as a novel endogenous vasodilator and neuromodulator due to its antioxidation capacity. However, whether H2S pretreatment in neonatal mouse cardiomyocytes is a protection effect against oxidative stress remains elusive. METHODS: Primary neonatal mouse cardiomyocytes were isolated and cultured, subsequently, pretreated with the H2S donor, sodium hydrosulfide (NaHS). Cell viability, lactate dehydrogenase (LDH) release, and reactive oxygen species (ROS) production are evaluated. The levels of superoxide dismutase (Sod2) and Sirtuin 1 (Sirt1), a deacetylation enzyme, were detected by Western blotting. The statistics was performed using independent-sample t-test. RESULTS: NaHS (100 µmol/L) had no toxicity to primary neonatal mouse cardiomyocytes. Furthermore, NaHS pretreatment significantly improved neonatal mouse cardiomyocytes survival after H2O2-induced cell death, indicated by the decrease in LDH release (40.00 ± 2.65% vs. 65.33 ± 4.33%, P < 0.01) and ROS production (1.90 ± 0.33 vs. 4.56 ± 0.56, P < 0.05), and that the salubrious effect was accompanied by the upregulation of Sod2 expression. In addition, the study showed that NaHS pretreatment improved mitochondrial DNA number in neonatal mouse cardiomyocyte. Furthermore, the result demonstrated NaHS increased the expression of Sirt1 in neonatal mouse cardiomyocyte. Ex 527, an inhibitor of Sirt1, could attenuate these effects of NaHS-induced Sod2 expression and mtDNA number increase, furthermore, abrogate the cytoprotective effects of NaHS for neonatal mouse cardiomyocytes. CONCLUSION: Sirt1 mediated H2S-induced cytoprotection effects in neonatal mouse cardiomyocytes.