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.

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.

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.

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.

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.

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.

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.

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.

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 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.

Bacillus anthracis edema but not lethal toxin challenge in rats is associated with depressed myocardial function in hearts isolated and tested in a Langendorff system.

Although direct myocardial depression has been implicated in the lethal effects of Bacillus anthracis lethal toxin (LT), in hearts isolated from healthy rats and perfused under constant pressure, neither LT or edema toxin (ET) in typically lethal concentrations depressed myocardial function. In the present study, we challenged rats with LT and ET and performed in vivo and ex vivo heart measures. Sprague-Dawley rats infused over 24 h with LT (n = 94), ET (n = 99), or diluent (controls; n = 50) were studied at 8, 24, or 48 h. Compared with control rats (all survived), survival rates with LT (56.1%) and ET (37.3%) were reduced (P < 0.0001) similarly (P = 0.66 for LT vs. ET). LT decreased mean arterial blood pressure from 12 to 20 h (P ≤ 0.05), whereas ET decreased it progressively throughout (P < 0.05). On echocardiography, LT decreased left ventricular (LV) ejection fraction at 8 and 48 h but increased it at 24 h and decreased cardiac output (P ≤ 0.05 for the time interaction or averaged over time). ET decreased systolic and diastolic volumes and increased LV ejection fraction at 24 h (P ≤ 0.05). In isolated hearts perfused for 120 min under constant pressure, LT did not significantly alter LV systolic or developed pressures at any time point, whereas ET decreased both of these at 24 h (P < 0.0001 initially). ET but not LT progressively increased plasma creatine phosphokinase and cardiac troponin levels (P < 0.05). In conclusion, despite echocardiographic changes, in vivo lethal LT challenge did not produce evidence of myocardial depression in isolated rat hearts. While lethal ET challenge did depress isolated heart function, this may have resulted from prior hypotension and ischemia.

Serum UCH-L1 as a Novel Biomarker to Predict Neuronal Apoptosis Following Deep Hypothermic Circulatory Arrest.

BACKGROUND: Deep hypothermic circulatory arrest (DHCA) has been used in cardiac surgery involving infant complex congenital heart disease and aortic dissection. DHCA carries a risk of neuronal apoptotic death in brain. Serum ubiquitin C-terminal hydrolase L1 (UCH-L1) level is elevated in a number of neurological diseases involving neuron injury and death. We studied the hypothesis that UCH-L1 may be a potential biomarker for DHCA-induced ischemic neuronal apoptosis. METHODS: Anesthetized piglets were used to perform cardiopulmonary bypass (CPB). DHCA was induced for 1 hour followed by CPB rewarming. Blood samples were collected and serum UCH-L1 levels were measured. Neuron apoptosis and Bax and Bcl-2 proteins in hippocampus were examined. The relationship between neuron apoptosis and UCH-L1 level was determined by receiver operating characteristics (ROC) curves and correlation analysis. RESULTS: DHCA resulted in marked neuronal apoptosis, significant increase in Bax:Bcl-2 ratio in hippocampus and UCH-L1 level elevations in serum (all P<0.05). Positive correlation was obtained between serum UCH-L1 level and the severity of neuron apoptosis (r= 0.78, P<0.01). By ROC, the area under the curve were 0.88 (95% CI: 0.74-0.99; P<0.01), 0.81 (95% CI: 0.81-0.96; P<0.05), 0.71 (95% CI: 0.47-0.92; P=0.11) for UCH-L1, Bax/Bcl-2 ratio and Bax, respectively. Using a cut-off point of 0.25, the UCH-L1 predicted neuronal apoptosis with a sensitivity of 85% and specificity of 57%. CONCLUSION: Serum UCH-L1, as an easy and quick measurable biomarker, can predict neural apoptosis induced by DHCA. The elevation in UCH-L1 concentration is consistent with the severity of neural apoptosis following DHCA.

Safety and Efficacy of Ventricular Septal Defect Repair Using a Cosmetic Shorter Right Lateral Thoracotomy on Infants Weighing Less than 5 kg.

OBJECTIVES: This study evaluated the safety and efficacy of a cosmetic right lateral thoracotomy for ventricular septal defect (VSD) repair in infants weighing less than 5 kg. METHODS: Eighty-nine infants who underwent a right lateral thoracotomy (right group) and 116 infants who underwent a median sternotomy (median group) were included. Patient characteristics, and perioperative and follow-up data were retrospectively analysed. RESULTS: Patient characteristics were comparable between the two groups as were cardiopulmonary bypass and aortic cross-clamping time, length of intensive care unit stay, ventilation duration and application of inotropic drugs. Compared to the median group, there was shorter procedure time (p < 0.05), shorter length of incision (p<0.05), and less drainage and transfusion in the right group (p<0.05). The peak airway pressure, partial oxygen pressure, partial carbon dioxide pressure, oxygenation index and alveolar-arterial oxygen gradients were similar for these two groups at the four time points analysed. No deaths occurred in either group and differences in morbidity were insignificant. No cardiac or thoracotomy-related complications occurred within the 18.4 ± 9.9 month follow-up. CONCLUSIONS: A right lateral thoracotomy reduces operative traumas and is as safe and effective as a median sternotomy at VSD repair in infants under 5 kg in weight.

Purified cell wall from the probiotic bacterium Lactobacillus gasseri activates systemic inflammation and, at higher doses, produces lethality in a rat model.

INTRODUCTION: One proposed benefit of probiotic therapy is that probiotic bacterial cell-wall binding to intestinal cell pathogen-recognition receptors activates protective innate immunity. However, in critically ill patients, intestinal epithelium disruption by shock or other insults may compromise this compartmentalized response and cause systemic bacteria and cell-wall translocation. The effects of intravascular introduction of probiotic bacterial cell wall are unclear. METHODS: We investigated 24-hour infusions of purified cell wall from Lactobacillus gasseri ATC33323 (L. gasseri), a probiotic bacterium, in Sprague-Dawley rats (n = 49). RESULTS: Increasing cell-wall doses (0 (control), 10, 20, 40, 80, or 160 mg/kg over 24 hours) produced dose-ordered decreases in survival measured after 168 hours (11 survivors/11 total (100%), seven of seven (100%), seven of seven (100%), six of eight (75%), five of eight (63%), and one of nine (11%), respectively, P < 0.0001). The L. gasseri cell wall was equally or more lethal than Staphylococcus aureus cell wall, which was previously studied (100% to 88% survival with the same increasing doses). During challenge, compared with controls, L. gasseri cell wall produced increases in blood IL-1ß, IL-10, tumor necrosis factor-α, migratory inhibitory protein-1α, monocyte chemotactic protein-1, and nitric oxide, and decreases in neutrophils, lymphocytes, and platelets that were greater with higher versus lower doses (P ≤ 0.05). Medium-dose cell wall (40 and 80 mg/kg combined) progressively decreased blood pressure and increased heart rate, and all doses increased lactate, hepatic transaminases, and creatinine phosphokinase (P ≤ 0.05). CONCLUSION: Although L. gasseri, like other probiotic bacteria, is considered safe, its cell wall can stimulate the maladaptive inflammatory response associated with pathogenic bacteria. Such effects deserve study, especially regarding critically ill patients.

The follow-up surgical results of coarctation of the aorta procedures in a cohort of Chinese children from a single institution.

OBJECTIVES: The aim of this study was to evaluate the results following surgeries for the treatment of coarctation of the aorta in Chinese paediatric patients and to compare the surgery outcomes between simple and complex coarctation procedures. METHODS: Between January 2006 and December 2011, 107 consecutive paediatric patients with coarctation of the aorta underwent surgery. Forty-four patients (41.12%) were classified as having simple coarctations (group A), and 54 patients (50.47%) were classified as having complex coarctations (group B). Echocardiography and the resting systolic blood pressure were evaluated prior to the operation, at one month following the operation, and then once annually. RESULTS: Follow-up was 93.5% complete (100 patients), without significant differences between the two groups. Arch hypoplasias and bicuspid aortic valves were initially present in 10 (9.35%) and 11 (10.28%) of 107 patients, respectively. There were no deaths among the group A patients and three (5.56%) early deaths among the group B patients. There was a significant difference in the restenosis incidence rate between the two groups during the most recent follow-up consultations (p<0.05). Additionally, only 10 of 43 group A and 10 of 51 group B patients had persistently abnormal blood pressures during the annual follow-up consultations. CONCLUSIONS: The postoperative restenosis ratio was increased in the complex coarctation group compared with the simple coarctation group. Additionally, the complex coarctation patients who did not have restenosis at follow-up had a lower proportion of hypertension.

ACE2 activation confers endothelial protection and attenuates neointimal lesions in prevention of severe pulmonary arterial hypertension in rats.

BACKGROUND: Angiotensin-converting enzyme 2 (ACE2), an ACE homolog, hydrolyzes angiotensin II and opposes its actions, and plays a protective role in the pathogenesis of pulmonary arterial hypertension (PAH). However, the underlying mechanisms involved in the effect of ACE2 on PAH are still uncertain. In this study, we observed the effects of ACE2 activation on endothelial dysfunction and vascular remodeling in the development of severe PAH in rats. METHODS: Severe PAH was induced by monocrotaline injection 1 week following left pneumonectomy, and ACE2 was activated by continuous injection of resorcinolnaphthalein. The PAH-related hemodynamics, pathological changes, and endothelium-dependent vasorelaxation were examined to assess the effects of ACE2 activation. In addition, the changes of the main components of the renin-angiotensin system were identified by ELISA or Western blotting. RESULTS: Severe PAH was established at 3 weeks and was characterized by high pulmonary arterial pressure (45 mmHg), significant right ventricular hypertrophy, neointimal occlusive lesions, and impaired endothelium-dependent relaxation in pulmonary arteries. Coadministration of resorcinolnaphthalein reduced pulmonary arterial pressure, right ventricular hypertrophy, and neointimal formation and shifted the endothelial-dependent responses toward values measured in normal rats. Theses changes were associated with an increase in ACE2 and angiotensin-(1-7) levels and a decrease in ACE and angiotensin II levels, in addition to a decrease in the ACE/ACE2 ratio and the angiotensin II/angiotensin-(1-7) ratio. The beneficial effects of resorcinolnaphthalein were abolished by A-779. CONCLUSIONS: These findings suggested that ACE2 activation by resorcinolnaphthalein improved endothelial function and suppressed neointimal formation in the prevention of severe PAH by the mechanism of mediating the levels of the components of the renin-angiotensin system.

Lung remodeling in a porcine model of cyanotic congenital heart defect with decreased pulmonary blood flow.

BACKGROUND: Hypoperfusion of the pulmonary vascular bed under the condition of congenital cardiac malformations may lead to progressive pulmonary vascular disease. To improve the mechanistic understanding of this disease, we examined the biochemical and morphological changes of the lung in a relevant animal model and provided valuable insights into the underlying mechanisms of the pathogenesis of pulmonary hypotension. MATERIALS AND METHODS: A model of congenital heart defect with decreased pulmonary blood flow was implemented into 8 piglets (the cyanosis group). Another 8 piglets underwent a sham operation (the control group). Two months postoperatively, lung biopsy specimens were harvested for the measurement of the expression levels of MMP-2, MMP-9, TIMP-1, VEGF, and type I and type III collagens. Moreover, the light-microscopic morphology, morphometry, and ultrastructure of lobes were examined. RESULTS: Compared to the controls, the histopathological changes of the pulmonary vasculature in the cyanosis group showed evident hypoplasia and degeneration. The expression levels of MMP-2, MMP-9, TIMP-1, VEGF, and type I collagen, as well as the microvessel density, in the cyanosis group were significantly lower than those in the control group, whereas the level of type III collagen in the cyanosis group was significantly higher than that in the control group. CONCLUSIONS: The observed morphological changes may represent an adaptive reaction to the prolonged decrease of pulmonary blood flow. The underlying mechanism of lung remodeling may be attributed to the changes in the expression of structural proteins and cytokines in the pulmonary extracellular matrix induced by modulating factors.

[Diagnosis and surgery of anomalous origin of the left coronary artery from the pulmonary artery in children].

OBJECTIVE: To explore the clinical features, diagnosis and management of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in children. METHODS: The clinical manifestations, examinations, diagnostic modalities and treatments of children identified as ALCAPA at Beijing Anzhen Hospital from September 1993 to September 2011 were retrospectively reviewed. RESULTS: A total of 27 children were recruited. There were 16 males and 11 females with an age range of 1 month to 13 years. Among them, 19 patients had an onset age of ≤ 1 years. The initial symptoms were acute heart failure (n = 15), pneumonia (n = 7) and cardiac dilation with murmur (n = 5). The pre-admission misdiagnoses included endocardial fibroelastosis (EFE) (n = 13), dilated cardiomyopathy (DCM) (n = 4) and mitral severe regurgitation (n = 2). The definite diagnosis was established on the basis of electrocardiography (ECG), echocardiography and 64-slice computed tomography or angiography. Twenty-six cases underwent immediate surgery after an accurate diagnosis, including left coronary artery (LCA) ligation (n = 1), LCA ligation plus coronary artery bypass grafting (n = 1), Takeuchi operation (n = 7) and LCA reimplantation (n = 17). Six died postoperatively. And 20 cases were routinely followed up with symptoms, signs, ECG and echocardiography for 3-192 months. The outpatient visits were at Months 3, 6 and 12 post-operation and then annual check-ups by returning to hospital, telephone, letters and other forms. The cohort had no overt symptoms and left ventricular ejection fraction returned to normal range. No complications occurred after LCA reimplantation. CONCLUSIONS: As a rare congenital heart anomaly, ALCAPA may be definitely diagnosed with clinical features and serial diagnostic methods. It can be treated with several types of cardiac surgery. Coronary reimplantation is the technique of choice for surgical correction of ALCAPA due to its excellent prognosis.