Association of PLXND1 with a novel subtype of anomalous pulmonary venous return.

Anomalous pulmonary venous return (APVR) is a potentially lethal congenital heart disease. Elucidating the genetic etiology is crucial for understanding its pathogenesis and improving clinical practice, whereas its genetic basis remains largely unknown because of complex genetic etiology. We thus performed whole-exome sequencing for 144 APVR patients and 1636 healthy controls and report a comprehensive atlas of APVR-related rare genetic variants. Novel singleton, loss-of-function and deleterious missense variants (DVars) were enriched in patients, particularly for genes highly expressed in the developing human heart at the critical time point for pulmonary veins draining into the left atrium. Notably, PLXND1, encoding a receptor for semaphorins, represents a strong candidate gene of APVR (adjusted P = 1.1e-03, odds ratio: 10.9-69.3), accounting for 4.17% of APVR. We further validated this finding in an independent cohort consisting of 82 case-control pairs. In these two cohorts, eight DVars were identified in different patients, which convergently disrupt the GTPase-activating protein-related domain of PLXND1. All variant carriers displayed strikingly similar clinical features, in that all anomalous drainage of pulmonary vein(s) occurred on the right side and incorrectly connected to the right atrium, which may represent a novel subtype of APVR for molecular diagnosis. Studies in Plxnd1 knockout mice further revealed the effects of PLXND1 deficiency on severe heart and lung defects and cellular abnormalities related to APVR such as abnormal migration and vascular formation of vascular endothelial cells. These findings indicate the important role of PLXND1 in APVR pathogenesis, providing novel insights into the genetic etiology and molecular subtyping for APVR.

Protective efficacy of recombinant canine adenovirus type-2 expressing TgROP18 (CAV-2-ROP18) against acute and chronic Toxoplasma gondii infection in mice.

BACKGROUND: The use of recombinant viral vectors expressing T. gondii antigens is a safe and efficient approach to induce immune responses against the parasite, as well as a valuable tool for vaccine development. We have previously prolonged the survival time of mice challenged with the RH strain of T. gondii by immunizing the mice with a eukaryotic vector expressing the protein ROP18 of T. gondii. We are now looking for ways to improve this vaccination strategy and enhance protection. METHODS: In this study, we constructed and characterized a novel recombinant canine adenovirus type 2 expressing ROP18 (CAV-2-ROP18) of T. gondii by cytopathic effect (CPE) and indirect immunofluorescence assay (IFA) following transfection into MDCK cells. Intramuscular immunization of Kunming mice with CAV-2-ROP18 was carried out to evaluate humoral and cellular immune responses. RESULTS: The vaccination of experimental mice with CAV-2-ROP18 elicited antibody production against ROP18, including high levels of a mixed IgG1/IgG2a and significant production of IFN-γ or IL-2, and displayed a significant bias towards a helper T cell type 1 (Th1) profile. Furthermore, the presence of T. gondii-specific IFN-γ-production and TNF-α-production T cells was elicited in both CD4+ and CD8+ T cell compartments. Significantly higher survival rates (40%) occurred in the experimental group, and a reduction in brain cyst burden was detected in vaccinated mice. CONCLUSION: These results demonstrate the potential use of a CAV vector harboring the ROP18 gene in the development of a vaccine against acute and chronic toxoplasmosis.

Worldwide trends in mortality for hypertensive heart disease from 1990 to 2019 with projection to 2034: data from the Global Burden of Disease 2019 study.

AIMS: This study aims to analyse the worldwide trends in hypertensive heart disease (HHD) mortality and associations with age, period, and birth cohort and predict the future burden of HHD deaths. METHODS AND RESULTS: Mortality estimates were obtained from Global Burden of Disease 2019 study. We used age-period-cohort (APC) model to examine the age, period, and cohort effects on HHD mortality between 1990 and 2019. Bayesian APC model was utilized to predict HHD deaths to 2034. The global HHD deaths were 1.16 million in 2019 and were projected to increase to 1.57 million in 2034, with the largest increment in low- and middle-income countries (LMICs). Between 1990 and 2019, middle/high-middle socio-demographic index (SDI) countries had the largest mortality reductions (annual percentage change = -2.06%), whereas low SDI countries saw a lagging performance (annual percentage change = -1.09%). There was a prominent transition in the age distribution of deaths towards old-age population in middle/high-middle SDI countries, while the proportion of premature deaths (aged under 60 years) remained at 24% in low SDI countries in 2019. Amongst LMICs, Brazil, China, and Ethiopia showed typically improving trends both over time and in recent birth cohorts, whereas 63 countries including Indonesia, the Philippines, and Pakistan had unfavourable or worsening risks for recent periods and birth cohorts. CONCLUSION: The HHD death burden in 2019 is vast and is expected to increase rapidly in the next decade, particularly for LMICs. Limited progress in HHD management together with high premature mortality would exact huge human and medical costs in low SDI countries. The examples from Brazil, China, and Ethiopia suggest that efficient health systems with action on improving hypertension care can reduce HHD mortality effectively in LMICs.

This study provides the first comprehensive analysis of the age, period, and cohort trends in mortality for hypertensive heart disease (HHD) across 204 countries and territories from 1990 to 2019, with projection to 2034. The death burden of HHD is substantial and growing rapidly in most of the world, particularly in low- and middle-income countries (LMICs). Wide disparities exist within LMICs in HHD management, with most low socio-demographic index countries showing little progress in reducing HHD mortality. The examples from Brazil, China, and Ethiopia suggest that prevention policies for HHD can reduce risks for younger birth cohorts and shift the risks for all age groups over time.

Biventricular function after Ebstein anomaly repair from a single-center echocardiography study.

OBJECTIVE: We aimed to examine biventricular remodeling and function after Ebstein anomaly (EbA) surgical correction using echocardiographic techniques, particularly, the relations between the biventricular changes and the EbA types. METHODS: From April 2015 to August 2022, 110 patients with EbA were included in this retrospective study based on the Carpentier classification. Echocardiography assessments during the preoperative, early, and mid-term postoperative periods were performed. RESULTS: The 54 patients with types A and B EbA were included in group 1, whereas the 56 patients with types C and D were in group 2. Seventy-eight patients underwent surgical correction of EbA. The median age at operation was 8.8 years. During the mid-term follow-up, only 9.1% of the patients had moderate or severe tricuspid regurgitation. Right ventricular (RV) systolic function worsened in group 2 at discharge (fractional area change: 27.6 ± 11.2 vs. 35.4 ± 11.5 [baseline], P < 0.05; global longitudinal strain: -10.8 ± 4.4 vs. -17.9 ± 4.7 [baseline], P = 0.0001). RV function slowly recovered at a mean of 12 months of follow-up. Regarding left ventricular (LV) and RV systolic function, no statistical difference was found between before and after surgery in group 1. CONCLUSION: A high success rate of surgical correction of EbA, with an encouraging durability of the valve, was noted. Biventricular systolic function was maintained fairly in most patients with types A and B postoperatively. A late increase in RV systolic function after an initial reduction and unchanged LV systolic function were observed in the patients with types C and D postoperatively.

Effect of mitochondrial aldehyde dehydrogenase-2 genotype on cardioprotection in patients with congenital heart disease.

AIMS: About 40% of East Asians carry an aldehyde dehydrogenase-2*2 (ALDH2*2) allele, and the influence of the ALDH2*2 allele on human cardioprotection has not been studied. This study was designed to evaluate the effect of ALDH2*2 allele on cardioprotection of patients with congenital heart diseases after open-heart surgery. METHODS AND RESULTS: The right atrial appendage was harvested before performing cardiopulmonary bypass in cyanotic and acyanotic congenital heart disease groups (n = 20 per group). Tissues were assayed to determine the impact of cyanosis on metabolic remodelling. A prospective cohort of Tetralogy of Fallot (TOF) patients (n = 118) was recruited to investigate the influence of the ALDH2*2 allele on cardioprotection after surgical repair. Myocardium samples were dissected after cardioplegia. ALDH2 activity, oxidative stress and glutathione (GSH) levels, and activating transcription factor-4 (ATF4) were analysed. After genotyping and grouping, all of the experimental and clinical results were compared between ALDH2*2 carriers and non-carriers. Cyanosis inhibited ALDH2 activity and led to aldehyde accumulation in ALDH2*2 carriers. This accumulation in turn increased expression of ATF4 and resulted in larger myocardium GSH pools. The differences in ALDH2 activity and GSH level between carriers and non-carriers disappeared during cardioplegic arrest, and more aldehydes accumulated in the non-carriers. Consequently, ALDH2*2 carriers showed lower postoperative troponin I, inotrope score, and shorter postoperative length of ICU and hospital stay. CONCLUSIONS: ALDH2*2 carriers with cyanotic congenital heart disease were associated with an induced metabolic remodelling phenotype and a compensatory myocardium GSH pool. When ALDH2 activity was impaired during open-heart surgery, this larger GSH pool could lead to unexpectedly better cardioprotection. This may aid in the prediction of cardioprotection outcomes and identification of individualized cardioprotective strategies.

[Safety and efficacy of percutaneous transcatheter closure of atrial septal defect under transesophageal echocardiography guidance in children].

OBJECTIVE: To assess the safety and effectiveness of percutaneous transcatheter closure of atrial septal defect (ASD) under transesophageal echocardiography (TEE) guidance in children. METHODS: The study included 20 cases of patients with ASD. The patients were (4.2 ± 1.2) years old and the mean body weights were (18.2 ± 4.2) kg. The diameter of ASD before closure was (13.4 ± 3.3) mm . All procedures were guided under TEE. Procedure success was evaluated by TEE immediately after procedure. RESULTS: Closure devices were successfully implanted in all 20 patients under TEE guidance. The diameter of closure devices was 14-26 mm. There were no procedure related complications. The ventilation time was (2.9 ± 0.8)h and the hospitalization time was (3.2 ± 0.7) days. CONCLUSION: TEE guided percutaneous transcatheter closure is safe and effective for patients with ASD and avoids the radiation damages.

Mid-term results after correction of type I and type II persistent truncus arteriosus in older patients.

OBJECTIVE: This study aims to analyze long-term results after correction of type I and type II truncus arteriosus in older patients operated in one institution over five years. METHODS: Between 2006 and 2010, 12 patients, median age 4 years, underwent repair of truncus arteriosus. Repair with reconstruction of the right ventricular to pulmonary artery continuity was performed using a valved conduit in 12 patients. RESULTS: There was no early mortality. All patients are alive with their original conduit 0.6 to 5 years after correction. No patients required reoperations for conduit dysfunction. Recent clinical examination was undertaken in all patients and they are in good condition. CONCLUSIONS: Though mean age at operation was higher in this study than published results, the operation should be performed if the pulmonary vascular resistance is under 8 units.m(2) before operation.

Modified anastomosis for repair of supracardiac total anomalous pulmonary venous connection in infants.

Recurrent pulmonary venous obstruction after repair of total anomalous pulmonary venous connection (TAPVC) is usually restricted to the anastomosis between the pulmonary venous confluence and the left atrium. We describe a modified technique for repair of supracardiac TAPVC in infants. An L-shaped incision of left atrium is utilized and the right-sided anastomosis is enlarged by using autologous pericardium to create a large and tension-free anastomosis.

[Efficacy of hybrid balloon valvuloplasty via sternotomy for treating low-body weight infants with severe congenital valvular aortic stenosis].

OBJECTIVE: To observe the efficacy of hybrid balloon valvuloplasty for the treatment of low-body weight infants with severe congenital valvular aortic stenosis (AS). METHODS: Five infants with severe congenital valvular aortic stenosis underwent the hybrid balloon aortic valvuloplasty through median sternotomy in the hybrid operating room. The mean age was (40.2 ± 7.0) days, weight was (4.48 ± 0.75) kg. The patients were followed up by echocardiography for 9 - 13 months post procedure. RESULTS: Operation was successful in all 5 patients and they were discharged from hospital uneventfully. The gradient pressure decreased significantly from (98.8 ± 9.0) mm Hg (1 mm Hg = 0.133 kPa) to (13.8 ± 3.3) mm Hg (P < 0.05) post operation. There was no moderate or severe aortic insufficiency. All patients were alive, the gradient pressures was (18.8 ± 2.5) mm Hg and there was no moderate or severe aortic insufficiency during follow-up [9 - 13 (11.0 ± 1.4) months]. CONCLUSION: The hybrid balloon aortic valvuloplasty is an effective option for the low-body weight infants with severe congenital valvular aortic stenosis.

Echocardiography in the diagnosis of Shone's complex and analysis of the causes for missed diagnosis and misdiagnosis.

BACKGROUND: Shone's complex is a rare syndrome characterized by congenital left heart defects that can differ among the patients. AIM: To use echocardiography in the diagnosis of Shone's complex and analyze the causes of missed diagnosis and misdiagnosis. METHODS: This was a retrospective study of patients who underwent echocardiography and repair surgery from February 14, 2008, to November 22, 2019. The patients were followed once a year at the outpatient clinic after surgery. RESULTS: Sixty-six patients were included. The patients were 2.7 (0.8-5.6) years of age, and 54.5% were male. Ten (15.2%) had a history of heart surgery. The most common heart defect was the Annulo-Leaflet mitral ring (ALMR) (50/66, 75.8%), followed by coarctation of the aorta (CoA) (43/66, 65.2%). The patients had a variety of combinations of defects. Only two (3.0%) patients had all four defects. None of the patients had a family history of congenital heart disease. The preoperative echocardiographic findings were examined against the intraoperative findings. Echocardiography missed an ALMR in 31 patients (47.0%), a parachute mitral valve (PMV) in one patient (1.5%), subaortic stenosis in one patient (1.5%), and CoA in two patients (3.0%). CONCLUSION: Echocardiography is an effective method to diagnose the Shone's complex. Due to this disease's complexity and interindividual variability, Improving the understanding of the disease can reduce misdiagnosis and missed diagnosis.

Handmade tri-leaflet ePTFE conduits versus homografts for right ventricular outflow tract reconstruction.

BACKGROUND: This study aimed to investigate the performance of handmade tri-leaflet expanded polytetrafluoroethylene (ePTFE) conduits in the absence of a suitable homograft. METHODS: Patients who underwent right ventricular outflow tract reconstruction with tri-leaflet ePTFE conduits or homografts between December 2016 and August 2020 were included. The primary endpoint was the incidence of moderate or severe conduit stenosis (≥ 36 mmHg) and/or moderate or severe insufficiency. The secondary endpoint was the incidence of severe conduit stenosis (≥ 64 mmHg) and/or severe insufficiency. RESULTS: There were 102 patients in the ePTFE group and 52 patients in the homograft group. The median age was younger [34.5 (interquartile range: 20.8-62.8) vs. 60.0 (interquartile range: 39.3-81.0) months, P = 0.001] and the median weight was lower [13.5 (10.0-19.0) vs. 17.8 (13.6-25.8) kg, P = 0.003] in the ePTFE group. The conduit size was smaller (17.9 ± 2.2 vs. 20.5 ± 3.0 mm, P < 0.001) and the conduit Z score was lower (1.48 ± 1.04 vs. 1.83 ± 1.05, P = 0.048) in the ePTFE group. There was no significant difference in the primary endpoints (log rank, P = 0.33) and secondary endpoints (log rank, P = 0.35). Multivariate analysis identified lower weight at surgery [P = 0.01; hazard ratio: 0.75; 95% confidence interval (CI) 0.59-0.94] and homograft conduit use (P = 0.04; hazard ratio: 8.43; 95% CI 1.14-62.29) to be risk factors for moderate or severe conduit insufficiency. No risk factors were found for moderate or severe conduit stenosis or conduit dysfunction on multivariate analysis. CONCLUSION: Handmade tri-leaflet ePTFE conduits showed acceptable early and midterm outcomes in the absence of a suitable homograft, but a longer follow-up is needed.

Two approaches for newborns with critical congenital heart disease: a comparative study.

BACKGROUND: Prenatal diagnosis and planned peripartum care is an unexplored concept in China. This study aimed to evaluate the effects of the "prenatal diagnosis and postnatal treatment integrated model" for newborns with critical congenital heart disease. METHODS: The medical records of neonates (≤ 28 days) admitted to Fuwai Hospital were reviewed retrospectively from January 2019 to December 2020. The patients were divided into "prenatal diagnosis and postnatal treatment integrated group" (n = 47) and "non-integrated group" (n = 69). RESULTS: The age of admission to the hospital and the age at surgery were earlier in the integrated group than in the non-integrated group (5.2 ± 7.2 days vs. 11.8 ± 8.0 days, P < 0.001; 11.9 ± 7.0 days vs. 16.5 ± 7.7 days, P = 0.001, respectively). The weight at surgery also was lower in the integrated group than in the non-integrated group (3.3 ± 0.4 kg vs. 3.6 ± 0.6 kg, P = 0.010). Longer postoperative recovery time was needed in the integrated group, with a median mechanical ventilation time of 97 h (interquartile range 51-259 h) vs. 69 h (29-168 h) (P = 0.030) and with intensive care unit time of 13.0 days (8.0-21.0 days) vs. 9.0 days (4.5-16.0 days) (P = 0.048). No significant difference was observed in the all-cause mortality (2.1 vs. 8.7%, P = 0.238), but it was significantly lower in the integrated group for transposition of the great arteries (0 vs. 18.8%, log rank P = 0.032). CONCLUSIONS: The prenatal diagnosis and postnatal treatment integrated model could significantly shorten the diagnosis and hospitalization interval of newborns, and surgical intervention could be performed with a lower risk of death, especially for transposition of the great arteries.

Platelet Aggregation Before Aspirin Initiation in Pediatric Patients With Congenital Heart Disease at High Risk of Thrombosis.

Background: Aspirin following unfractionated heparin is the most common anticoagulation strategy for pediatric patients who experienced cardiac surgery at high risk of thrombosis. The platelet aggregation test is the golden method to evaluate the aspirin effect on platelet function. However, the platelet aggregation basal status before postoperative aspirin initiation and the related clinical influencing factors hasn't been investigated systemically in this population. Methods: In a prospective cohort of 247 children, arachidonic acid-induced platelet aggregation (PAG-AA) was measured by means of light transmission aggregometry (LTA) before the first dose of aspirin after cardiac surgical procedure and the perioperative variables were also collected. Distribution of this population's PAG-AA basal status was described. Univariate and multivariate logistic regression analysis were performed to identify the main influencing factors of PAG-AA. Results: The median time of aspirin administration was 2 (1-27) days after surgery and the corresponding median value of basal PAG-AA was 20.70% (1.28-86.49%), with 67.6% population under 55% and 47.8% population under 20%. Patients undergoing cardiopulmonary bypass (CPB) had a significantly lower basal PAG-AA than those without (30.63 ± 27.35 vs. 57.91 ± 27.58, p = 0.013). While patients whose test done within 3 days after CPB had a significantly lower PAG-AA than those out of 3 days (25.61 ± 25.59 vs. 48.59 ± 26.45, p = 0.001). Univariate analysis implied that the influencing factors of the basal PAG-AA including CPB use, test time point, cyanosis, and platelet count. Multivariate regression analysis indicated that only CPB use, test time point, and platelet count were the main independent influencing factors for the basal PAG-AA. Conclusion: The majority of children have impaired basal platelet aggregometry responses before postoperative aspirin initiation. The main influencing factors are CPB use, test time point, and platelet count. To establish the platelet aggregometry baseline prior to commencement of aspirin therapy, testing should be performed 3 days later following the procedure when effect of CPB is basically over.

Design, synthesis, and insecticidal and fungicidal activities of quaternary ammonium salt derivatives of a triazolyphenyl isoxazoline insecticide.

BACKGROUND: Insect pests seriously decrease the yield and quality of agricultural crops. Resistance to commonly used insecticides is increasingly undermining their effectiveness, and therefore the development of agents with novel modes of action is desirable. Isoxazolines are a new class of insecticides that act on γ-aminobutyric acid (GABA) gated chloride channels. In this work, we used the highly active 4-triazolyphenyl isoxazoline DP-9 as a parent structure to design and synthesize a series of quaternary ammonium salt (QAS) derivatives, and we systematically evaluated their insecticidal and antifungal activities. RESULTS: Many of the synthesized QASs exhibit insecticidal activities equivalent to or higher than that of DP-9. In particular, compounds I-31 (93%, 0.00005 mg/L) and I-34 (80%, 0.00001 mg/L) showed insecticidal activities against diamondback moth larvae that were 2-10 times higher than those of fluralaner (70%, 0.0001 mg/L) and DP-9 (80%, 0.0001 mg/L), in addition to showing excellent activities against oriental armyworm, fall armyworm, cotton bollworm, corn borer, and mosquito larvae. Furthermore, all of the synthesized compounds also showed broad-spectrum fungicidal activities. CONCLUSION: The insecticidal activities of QAS derivatives of DP-9 were the same as or better than the activity of DP-9. Compounds I-31 and I-34 showed better insecticidal activities against diamondback moth larvae than fluralaner and DP-9, and thus are promising new candidates for insecticide research.

Safety and protective effects of an avirulent Salmonella Gallinarum isolate as a vaccine candidate against Salmonella Gallinarum infections in young chickens.

Fowl typhoid is an important disease of chickens and turkeys, which is caused by Salmonella Gallinarum (S. Gallinarum). Vaccines with high levels of protective effects against fowl typhoid need to be developed for the poultry industry. In this study, a S. Gallinarum strain, named SG01, was isolated from a poultry farm in Mashan region of Wuxi City, China, and identified through biochemical tests and specific PCR amplifications. Then, safety evaluations of the SG01 strain were performed in young chickens. No clinical symptom including depression and diarrhea and gross lesion involved in the cardiac nodule, hepatic necrotic lesion and splenic necrotic lesion, was determined on fifteen-day-old chickens after immunization with 1 × 1010 CFU of the SG01 strain through the oral route. However, diarrhea symptoms and hepatic lesions were identified from chickens immunized with the commercial vaccine strain SG9R by the same dose and route. At 14 days post inoculation, SG01 strain was eliminated in the liver and spleen from SG01-immunized chickens, while the SG9R strain still could be identified from SG9R-immunized chickens. After challenge with the virulent S. Gallinarum strain, significant reduction of the morbidity rate was found in the SG01 immunized group (20 %) compared to the challenge group (100 %) according to signs scoring systems for clinical symptoms and gross lesions. Additionally, immunization with the SG01 strain could provide more than 8 weeks of protection periods against fowl typhoid. These results demonstrate the SG01 strain is avirulent to young chickens and might be safer compared to the SG9R strain. In addition, SG01 strain is a potential vaccine candidate against fowl typhoid in young chickens.

[Risk factors affecting in-hospital mortality of the arterial switch operation for transposition of the great arteries].

OBJECTIVE: To analyze the in-hospital mortality and factors affecting in-hospital mortality for patients with transposition of the great arteries (TGA) undergoing arterial switch operation (ASO). METHODS: Between January 2004 and December 2007, ASO was performed in 169 patients [129 male, 40 female; mean age (11.71 ± 26.3) months] with TGA. The patients were divided in intact ventricular septum group (n = 56): TGA with intact ventricular septum and ventricular septal defect group (n = 113): TGA with ventricular septal defect. Multiple logistic regression analysis was performed to identify the risk factors of in-hospital mortality. RESULTS: The overall in-hospital mortality was 11.24% (19/169). The yearly in-hospital mortality was similar between intact ventricular septum group and ventricular septal defect group. With the improvement of perioperative treatment, the in-hospital mortality decreased from 16.67% in 2004 to 3.92% in 2007. The multivariate analysis revealed that body weight ≤ 3 kg (OR: 4.571, P = 0.0409), complicating ventricular septal defect (OR: 4.444, P = 0.0406), complex TGA (OR: 4.321, P = 0.0140), coronary anomalies (OR: 4.867, P = 0.0104) and non-type A coronary arteries (OR: 3.045, P = 0.0243) were independent predictors for poor early postoperative survival. CONCLUSION: Body weight ≤ 3 kg, complicating ventricular septal defect, complex TGA, coronary anomalies are independent predictors for increased in-hospital mortality in patients with transposition of TGA and undergoing arterial switch operation.

Safety and efficacy of arterial switch operation in previously inoperable patients.

BACKGROUND: Patients older than six months with complete transposition of the great arteries and ventricular septal defect or Taussig-Bing anomaly and severe pulmonary arterial hypertension (PAH), previously thought to be inoperable, usually are not indicated for arterial switch operation (ASO) or even repair. This study aimed to evaluate the safety and efficacy of ASO in these selected subset patients. METHODS: The records of 86 patients older than six months with complete transposition of the great arteries and ventricular septal defect or Taussig-Bing anomaly and severe PAH who underwent ASO at our institution from May 2000 to October 2008 were reviewed retrospectively. Eighty survivors were followed-up. RESULTS: There were six hospital deaths (7.0%, 95% confidence limit 1.6 to 12.4%). From January 2006 to October 2008, 46 consecutive ASOs were performed with no death. Operative mortality and mobility decreased significantly (p = 0.008 and p = 0.046, respectively). The median duration of follow-up was 42.1 +/- 28.8 months (range, 2.0 to 99.5). Two late deaths occurred. Latest follow-up data showed that 2.8% of survivors were in New York Heart Association (NYHA) class II and 97.2% were in NYHA class I. CONCLUSIONS: Excellent early and mid-term results of ASO are obtained from patients older than six months with complete transposition of the great arteries and ventricular septal defect or Taussig-Bing anomaly and severe PAH in current era, and ASO is safe and effective in these selected subset patients.

[Influence of coronary artery variation on the outcome of arterial switch operation].

OBJECTIVE: To investigate the influence of coronary artery variation on the outcome of arterial switch operation for transposition of great arteries. METHODS: Among 280 patients undergoing arterial switch operations at our hospital from 2001 to 2008, 73 (26.1%) had concurrent coronary arteries variation (54 males and 19 females; median age: 0.6 ± 1.1 years old; mean body weight: 5.8 ± 2.6 kg). Of these 73 patients (variant group), 21 cases had transposition of great arteries with a ventricular septal defect and 30 cases with an intact ventricular septum. The other 22 cases were of Taussig-Bing anomalies. Another 207 cases had usual coronary arteries (usual group). Coronary artery transfer was achieved by implantation of buttons to the previously anastomosed neo-aorta. RESULTS: There were 29 early death (10.4%) including 12 cases (16.4%) in variant group and 17 cases (8.21%) in usual group (P < 0.05). Mean cardiopulmonary bypass and cross-clamp durations were 229 ± 84 and 146 ± 48 min in variant group while 206 ± 59 and 137 ± 40 min in usual group (P < 0.05). Six cases were confirmed intra-operatively as coronary compression or obstruction. Complications included infection (n = 11), low output syndrome (n = 7), diaphragm paralysis (n = 3), pericardial effusion (n = 2) and atrioventricular block (n = 2). CONCLUSION: Coronary artery variation increases the operative difficulty and influences the outcome. The operative proficiency may decrease the mortality.

[One-stage repair of the interrupted aortic arch associated with cardiac anomaly].

OBJECTIVE: To evaluate the clinical experiences of one-stage repair of the interrupted aortic arch (IAA) associated with cardiac anomaly in neonates and infants. METHODS: From July 1996 to October 2008, 48 neonates or infants with IAA associated with cardiac anomaly underwent one-stage repair. There are 30 males and 18 females ranging from 0.08 to 7.00 years old (1.97 +/- 2.05) with body weight 3 - 20 (9 +/- 5) kg. 42 patients had the middle-severe pulmonary hypertension (PH), the pulmonary pressure was 61 - 106 (82 +/- 14) mm Hg and the pulmonary arterial resistance was 66 - 762 (315 +/- 259) dynxsxcm(-5). There are 40 patients with type A IAA and 8 patients with type B IAA, all patients had an associated patent ductus arteriosus, other combined malformations included ventricular septal defect (VSD) in 33 cases, atrial septal defect (ASD) in 8 cases, mitral valve insufficiency in 6 cases, tricuspid valve insufficiency in 5 cases, aortopulmonary window in 4 cases, double outlet of the right ventricule in 3 cases, mitral valve stenosis in 2 cases, and et al. the operation was performed through median sternotomy under the general anesthesia and cardiopulmonary bypass (CPB, the deep hypothermia with circulatory arrest, the deep hypothermia with low flow rate, or the deep hypothermia with low flow rate and circulatory arrest), the interrupted aortic arch repair was achieved with the arch anastomosis in 30 patients and with an interposition graft in 18 patients, the concomitant operations included VSD repair in 33 cases, ASD repair in 8 cases, mitral valve plasty in 7 cases, tricuspid valve plasty in 4c ases, aortopulmonary window repair in 4 cases, intraventricular baffle tube repair in 2 cases, arterial switch operation in 1 case, and et al. RESULTS: The time of the CPB and the aortic occlusion were 112 to 375 min (182 +/- 52) and 24 to 287 min (99 +/- 45) respectively. The endotracheal intubation time were 8 to 936 hours (179 +/- 133) and the ICU stay were 1 - 57 days (14 +/- 14). By echocardiograph examination after operation, the pressure gradient across the arch was obvious relieved and the combined cardiac malformations was satisfied corrected. 3 patients (3/48, 6.25%) died during the perioperative period. The cause of death included severe pulmonary hypertension crisis, dysfunction of the pulmonary or the serious low cardiac output syndrome. the main complications included reopen for bleeding in 2 cases, tracheostomy in 2 cases, the right diaphragma paralysis in 1 case, delayed sternal closure in 1 case, hydrothorax in 1 case, the severe pulmonary infection in 1 case. Physical activities of the survivals increased obviously and discharged from hospital uneventfully, all surviving patients had no late complications and death during the follow-up 3 months to 12 years. CONCLUSION: One-staged primary repair of IAA with other intracardiac anomalies can be the preferred surgical approach and safely applied with good results as to lead a better life.

[The analysis of the recent effectiveness of the total anomalous pulmonary venous connection repairs on infants].

OBJECTIVE: To review the efficacy of total anomalous pulmonary venous connection (TAPVC) repair and to conclude the factors impacting the peri-operative death rate. METHODS: The clinical data of 145 infants under 1 year old who underwent the TAPVC repair from January 2001 to July 2008 was analyzed. There were 94 male and 51 female patients. The mean age when the repair was performed was (7 ± 3) months, and the average weight was (6.3 ± 1.6) kg. As to the pulmonary connection type, 77 patients were supracardiac (53.1%), 47 patients were cardiac (32.4%), 9 patients were intracardiac (6.2%), and the remaining 12 patients were mixed (8.3%). Pre-surgery echocardiography showed that 21 patients had pulmonary venous obstruction (12 patients were supracardiac type, 3 patients were cardiac type, 3 patients were intracardiac type, and 3 patients were mixed type). RESULTS: All patients underwent two-ventricle anatomy correction (the cases of complex malformations had been excluded). Peri-operative mortality was 11.7% (17/145). Because of the significant improvement in the surgical techniques, anesthesiology, cardiopulmonary bypass and the management of ICU in January 2006, the population was divided into two groups: A (before January 2006) and B (after January 2006). Peri-operative mortality decreased from 19.0% in group A to 6.2% in group B(P = 0.020). After analysis, it was determined that the factors impacting mortality were which group the patient belongs to, whether he/she had preoperative pulmonary vein obstruction and how big the atril septel connection was. The operative technique to keep the anastomotic aperture adequate and prophylaxis pulmonary hypertensive episodes contributed to the improvement on the mortality rate. There had been no case of repeating the surgery because of pulmonary venous obstruction during peri-operative care period. CONCLUSIONS: Improvements of the surgical technique as well as the treatment in preoperative and postoperative have led to the reduction of the mortality. Preoperative pulmonary vein obstruction is still an important factor that contributes to early mortality.