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.

Incremental value of myocardial global longitudinal strain in predicting major adverse cardiac events among patients with hypertrophic cardiomyopathy.

OBJECTIVES: Endocardial global longitudinal strain (endo-GLS) measured with echocardiography (echo) has been demonstrated to be associated with myocardial fibrosis (MF) and is a prognostic predictor in patients with hypertrophic cardiomyopathy (HCM). Late gadolinium enhancement cardiac magnetic resonance (LGE-CMR) imaging showed that MF is primarily located in the myocardial layer of the extremely hypertrophic septal or ventricular wall. We hypothesized that GLS of the myocardial layer (myo-GLS) is more strongly correlated with the extent of LGE (%LGE) and is a more powerful prognostic factor than endo-GLS. METHODS: A total of 177 inpatients (54.0 [IQR: 43.0, 64.0] years, female 37.3%) with HCM were retrospectively included from May 2019 to April 2021. Among them, 162 patients underwent echocardiographic examination and contrast-enhanced CMR within 7 days. Myo-GLS and %LGE were blindly assessed in a core laboratory. All the patients were followed after they were discharged. RESULTS: During a mean follow-up of 33.77 [IQR 30.05, 35.40] months, 14 participants (7.91%) experienced major adverse cardiac events (MACE). The MACE (+) group showed lower absolute endo-GLS and myo-GLS than the MACE (-) group. Myo-GLS was more associated with %LGE (r = -.68, P < .001) than endo-GLS (r = -.64, P < .001). Cox multivariable analysis indicated that absolute myo-GLS was independently associated with MACE (adjusted hazard ratio = .75, P < .05). Myo-GLS was better than endo-GLS at detecting MACE (+) patients (-8.64%, AUC .939 vs. - 16.375%, AUC .898, P < .05). CONCLUSIONS: Myo-GLS is a stronger predictor of MACE than endo-GLS in patients with HCM and is highly correlated with %LGE.

Exome-Based Case-Control Analysis Highlights the Pathogenic Role of Ciliary Genes in Transposition of the Great Arteries.

RATIONALE: Transposition of the great arteries (TGA) is one of the most severe types of congenital heart diseases. Understanding the clinical characteristics and pathogenesis of TGA is, therefore, urgently needed for patient management of this severe disease. However, the clinical characteristics and genetic cause underlying TGA remain largely unexplored. OBJECTIVE: We sought to systematically examine the clinical characteristics and genetic cause for isolated nonsyndromic TGA. METHODS AND RESULTS: We recruited 249 patients with TGA (66 family trios) and performed whole-exome sequencing. The incidence of patent ductus arteriosus in dextro-TGA (52.7%) and dextrocardia/mesocardia in congenitally corrected TGA (32.8%) were significantly higher than that in other subtypes. A high prevalence of bicuspid pulmonic valve (9.6%) was observed in patients with TGA. Similar results were observed in a replication group of TGA (n=132). Through a series of bioinformatics filtering steps, we obtained 82 candidate genes harboring potentially damaging de novo, loss of function, compound heterozygous, or X-linked recessive variants. Established congenital heart disease-causing genes, such as FOXH1, were found among the list of candidate genes. A total of 19 ciliary genes harboring rare potentially damaging variants were also found; for example, DYNC2LI1 with a de novo putatively damaging variant. The enrichment of ciliary genes supports the roles of cilia in the pathogenesis of TGA. In total, 33% of the TGA probands had >1 candidate gene hit by putatively deleterious variants, suggesting that a portion of the TGA cases were probably affected by oligogenic or polygenic inheritance. CONCLUSIONS: The findings of clinical characteristic analyses have important implications for TGA patient stratification. The results of genetic analyses highlight the pathogenic role of ciliary genes and a complex genetic architecture underlying TGA.

Doubly committed subarterial ventricular septal defect closure through tricuspid approach: a clinical analysis.

BACKGROUND: The research was to introduce the experience of doubly committed subarterial ventricular septal defect (DCVSD) repaired through tricuspid approach. METHODS: From January, 2015 to September, 2019, 86 consecutive DCVSD paediatrics underwent repair via right subaxillary vertical incision (RAVI) through tricuspid approach. Perioperative and follow-up data were collected. RESULTS: The age and weight at operation were 28.1 ± 18.5 (range: 7-101) months and 12.2 ± 4.2 (6-26.5) kg. There were two patients combined with discrete subaortic membrane, two patients with patent ductus arteriosus, one patient with atrial septal defect, and two patients with abnormal muscle bundle in right ventricular outflow tract. The mean size of ventricular septal defect was 7.0 ± 2.4 (3-13) mm. The defect was repaired with a piece of Dacron patch in 68 patients or directly with 1-2 pledgetted polypropylene sutures in 18 patients. The cardiopulmonary bypass time and aortic cross-clamp time were 46.2 ± 13.3 (23-101) minutes and 29.2 ± 11.5 (12-84) minutes. After 3.1 ± 2.4 (0-14) hours' ventilator assist and 23.2 ± 32.1 (0-264) hours' ICU stay, all patients were discharged safely. At the latest follow-up (27.9 ± 14.6 months), echocardiography showed trivial residual shunt in two patients. There was no malignant arrhythmia occurred and there was no chest deformity or asymmetrical development of the breast was found. CONCLUSIONS: DCVSD repaired via right subaxillary vertical incision through tricuspid approach was safe and feasible, providing a feasible alternative to median sternotomy, and it can be performed with favourable cosmetic results.

Ultrasound-Guided Percutaneous Balloon Aortic Valvuloplasty for Aortic Stenosis.

Percutaneous balloon aortic valvuloplasty (PBAV), which is used to treat symptomatic aortic stenosis, requires ionizing radiation and contrast agent for imaging guidance. The aim of the study is to evaluate the feasibility and effectiveness of ultrasound-guided PBAV in patients with aortic stenosis. This case series included 30 patients (14 males; mean age, 61.5 ± 4.5 years) with moderate/severe aortic stenosis treated with ultrasound-guided PBAV at the Ultrasound Department, Fuwai Hospital, Beijing, China, between January 2016 and July 2019. Cardiac function (New York Heart Association grade) was assessed before PBAV and 1 month after the procedure. Aortic peak jet velocity, aortic valve orifice area (AVA), mean transvalvular pressure gradient (MTPG), left ventricular end-diastolic diameter (LVDD), left ventricular ejection fraction (LVEF), and left ventricular end-systolic diameter (LVESD) were determined before and immediately after PBAV using Doppler echocardiography. Preprocedural cardiac function was grade I in 3 cases, grade II in 9 cases, grade III in 10 cases, and grade IV in 8 cases. Postprocedural cardiac function was grade I in 22 cases, grade II in 4 cases, and grade III in 4 cases, suggesting that cardiac function was improved by PBAV. Ultrasound-guided PBAV resulted in significant improvements (P < 0.05) in aortic peak jet velocity (3.68 ± 0.811 m/s vs. 4.79 ± 0.63 m/s), MTPG (33.77 ± 13.85 mmHg vs. 54.54 ± 13.81 mmHg), AVA (1.96 ± 0.25 cm2 vs. 0.98 ± 0.12 cm2), LVEDD (51.90 ± 3.21 mm vs. 65.60 ± 6.81 mm), LVEF (63.46 ± 11.29% vs. 56.31 ± 11.04%), and LVESD (35.50 2.62 mm vs. 45.20 ± 2.42 mm). Ultrasound-guided PBAV is feasible and achieves good short-term effects in patients with aortic stenosis.

Left atrial reservoir strain combined with E/E' as a better single measure to predict elevated LV filling pressures in patients with coronary artery disease.

BACKGROUND: The 2016 guidelines for left ventricular diastolic dysfunction diagnosis has been simplified from previous versions; however, multiparametric diagnosis approach still exists indeterminate left ventricular diastolic dysfunction category. Left atrial (LA) strain was recently found useful to predict elevated left ventricular (LV) filling pressures noninvasively. This study aimed to (1) analyze the diagnostic value of LA strain for noninvasive assessment of LV filling pressures in patients with stable coronary artery disease (CAD) with preserved LV ejection fraction (LVEF), using invasive hemodynamic assessment as the gold standard, and (2) explore whether LA strain combined with conventional diastolic parameters could detect elevated LV filling pressures alone. METHODS: Sixty-four patients with stable CAD having LVEF > 50% and 30 healthy controls were enrolled. Two-dimensional speckle-tracking echocardiography was used to measure LA strain during the reservoir (LASr), conduit, and contraction phases. LV end-diastolic pressure (LVEDP), as a surrogate for LV filling pressures, was invasively obtained by left heart catheterization. Logistic regression was used to calculate the odds ratio to predict LV filling pressures. Pearson's correlation was used to analyze associations between echocardiographic parameters and LVEDP. The area under the receiver-operating characteristic curve was calculated to determine the capability of the echocardiographic parameters to detect elevated LVEDP. Inter-technique agreement was analyzed by contingency tables and tested by kappa statistics. RESULTS: LASr and the ratio of early-diastolic transmitral flow velocity (E) to tissue Doppler early-diastolic septal mitral annular velocity (E/E'septal) significantly predicted elevated LV filling pressures. LASr was combined with E/E'septal to generate a novel parameter (LASr/E/E'septal). LASr/E/E'septal had the best predictive ability of elevated LV filling pressures. LVEDP was negatively correlated with LASr and LASr/E/E'septal but positively correlated with E/E'septal. The area under the receiver-operating characteristic curve of LASr/E/E'septal was higher than that of LASr alone (0.83 vs. 0.75), better than all conventional LV diastolic parameters. Inter-technique agreement analysis showed that LASr/E/E'septal had good agreement with the invasive LVEDP measurement, better than the 2016 guideline (kappa = 0.63 vs. 0.25). CONCLUSIONS: LASr provided additive diagnostic value for the noninvasive assessment of LV filling pressures. LASr/E/E'septal had the potential to be a better single noninvasive index to predict elevated LV filling pressures in patients with stable CAD and preserved LVEF.

Evaluation of the Left Ventricular Hemodynamic Status of Double-Root Translocation Patients Through Vector Flow Mapping.

The double-root translocation (DRT) procedure is considered as a new choice for surgical management of complex congenital heart disease. Our study aims to investigate the left ventricular hemodynamic status after DRT by energy loss (EL), involving 42 patients who underwent DRT as well as 42 healthy volunteers as controls. The EL of left ventricle (LV) during different periods (rapid filling-RF, atrial contraction-AC, isovolumic contraction-IVC, rapid ejection-RE) of the same cardiac cycle were obtained and analyzed. Compared to the controls, global LV and apical three chamber-EL at AC and IVC periods in DRT group were higher (p < 0.05), and EL at RE period of DRT group was moderately lower. In the correlation analysis, the global EL at the RF was correlated with E wave (r = 0.47, p = 0.03), E/e' (r = 0.50, p = 0.02), BSA (r = - 0.54, p = 0.01), and age (r = - 0.67, p < 0.001). AC and RE- global EL were significantly correlated with E/e' (r = 0.49, r = 0.59, p < 0.05). There was a strong positive correlation between E/e' and global EL at the IVC (r = 0.62, p = 0.003) and a moderate negative correlation with age (r = - 0.44, p = 0.04). The present study confirms that EL is a feasible and reproducible indicator for quantitatively evaluating LV hemodynamic status in patients who underwent DRT and reveals that DRT can lead to approximatively normal long-term hemodynamic performance of LV.

Modified Single Repair Technique for Complete Atrioventricular Septal Defect: A Propensity Score Matching Analysis.

There were controversies about the priority of modified single patch (MSP) technique compared with two-patch (TP) technique for patients with complete atrioventricular septal defect (CAVSD). From 2002 to 2013, patients who diagnosed as balanced CAVSD in our institution were retrospectively included. Patients with unbalanced ventricles or associated anomalies were excluded. The primary endpoint was all-cause mortality and the secondary endpoint consist of left atrioventricular dysfunction or left atrioventricular outflow tract obstruction (LVOTO). A total of 74 patients underwent MSP repair and 102 patients underwent TP repair. After 1:1 propensity matching, there were 46 Rastelli type A CAVSD patients in each group. Patients in MSP group had shorter cardiopulmonary bypass time [median (interquartile range) 73.5 (65.5-95.0) versus 105.0 (88.8-130.0) min, P < 0.001] and aortic cross-clamp time [105.5 (90.0-128.0) versus 143.0 (122.0-184.0) min, P < 0.001]. In total, the primary endpoint occurred in 12 patients, including 2/46 (4.3%) in MSP group and 10/46 (21.7%) in TP group. That 1-year, 3-year, 5-year survival rate can be calculated as 95.6%, 95.6%, 95.6% in MSP group and 92.6%, 80.4%, 78.2% in TP group, P = 0.015. No significant statistical difference found for the secondary endpoints that 7/46 (15.2%) patients occurred moderate or severe LAVVR in MSP group versus 11/46 (23.9%) patients in TP group, P = 0.293. No LVOTO occurred in both groups. Besides the simplicity of technique, MSP technique may be safer.

Tricuspid valvuloplasty for isolated tricuspid regurgitation in children.

BACKGROUND: Isolated congenital tricuspid regurgitation other than Ebstein's anomaly was rare especially for children. The objective of this study was to investigate the clinical characteristics and to assess the results of tricuspid valvuloplasty for children with isolated tricuspid regurgitation. METHODS: From January 2010 to June 2019, 10 consecutive patients with isolated tricuspid regurgitation who were unresponsive to drug therapy underwent tricuspid valvuloplasty in our hospital. Patients' clinical data were analysed retrospectively. RESULTS: Mean age at operation was 48.5 ± 31.0 (range: 9-106) months and mean weight at operation was 16.1 ± 6.9 (range: 8.6-33.0) kg. All patients presented severe isolated tricuspid regurgitation. According to pathological lesions, the main causes accounted for chordae tendinea rupture (3/10), leaflet cleft (2/10), mal-connected chordal tendinea to leaflets (2/10), elongated chordae (1/10) and chordae absent (1/10), and severe anterior leaflet dysplasia (1/10). Individualised tricuspid valvuloplasty was adapted to all of them successfully. Post-operative echocardiography showed no tricuspid regurgitation in two patients and mild regurgitation in eight patients. The cardiothoracic ratios on their chest roentgenograms decreased from 0.59 ± 0.05 to 0.54 ± 0.05. At the latest follow-up (50.4 ± 47.2 months), echocardiography showed that mild to moderate tricuspid regurgitation in seven patients, moderate tricuspid regurgitation in three patients, and no patient with severe tricuspid regurgitation. All patients were in NYHA functional class I. CONCLUSIONS: For patients with isolated tricuspid regurgitation who were not well responsive to drug therapy, individualised tricuspid valve repair can achieve an excellent result.

Outcomes of Common Atrioventricular Valve Repair in Patients With Single-Ventricle Physiology　- Indication, Timing and Repair Techniques.

BACKGROUND: Common atrioventricular valve (CAVV) repair in patients with a single ventricle remains a great challenge and a refractory issue for pediatric cardiac surgeons. Methods and Results: From January 2007 to April 2018, 37 consecutive patients with a single ventricle who underwent CAVV repair were included in the study group. Patients were divided into 2 groups based on the repair technique: patients in Group A were treated using the bivalvation technique, and patients in Group B underwent conventional repair techniques; baseline data were similar between groups. The inhospital and follow-up mortality were 5.4% (2/37) and 11.4% (4/35), respectively. After a follow-up of 65.5±29.3 months, the estimated 1-, 5-, and 10-year overall survival rates were 94.6%, 83.4%, and 77.0%, respectively. The rates of freedom from CAVV failure were 94.3%, 72.7%, and 62.9% after 1, 5, and 10 years, respectively. In the multivariate analysis, the independent factors for CAVV repair failure were repair technique (P=0.004) and heterotaxy syndrome (P=0.003). A total of 30 patients (81.1%) completed total cavopulmonary connection (TCPC); 3 patients required re-intervention; 24 of 31 patients (77.4%) were in New York Heart Association classes II and I at the latest follow-up. CONCLUSIONS: Outcomes of CAVV repair in patients palliated by single-ventricular surgery are acceptable. The bivalvation technique is a simple and effective technique.

Transthoracic Echocardiography-Guided Percutaneous Patent Ductus Arteriosus Occlusion: A New Strategy for Interventional Treatment.

INTRODUCTION: Percutaneous patent ductus arteriosus (PDA) occlusion has become the preferred therapeutic option, which uses fluoroscopy as the guidance. To reduce the x-ray exposure, PDA occlusion using the Amplatzer Duct Occluder II (ADO II) under guidance of transthoracic echocardiography only was conducted. This single center study aims to access the safety and efficiency of this new strategy. METHODS AND RESULTS: From June 2013 to May 2015, 63 consecutive PDA patients underwent transthoracic echocardiography-guided PDA occlusion through the femoral artery. Outpatient follow-up was conducted at 1, 3, and 6 months, and yearly. Sixty-two patients successfully underwent echocardiography-guided percutaneous PDA occlusion. One patient was converted to minimally invasive transthoracic occlusion due to failure of delivery sheath passage through tortuous PDA. Mean procedure duration was 24.3 ± 7.0 minutes; ADO II diameter averaged 4.6 ± 0.9 mm; 8 cases showed traces of residual shunt immediately after operation which resolved after 24 hours; and mean hospital stay was 3.4 ± 0.5 days. There was no occluder migration, hemolysis, pericardial effusion, pulmonary branch or aortic stenosis at mean 13.5 ± 4.8 months follow-up. CONCLUSIONS: This study demonstrated that percutaneous PDA occlusion can be successfully performed under guidance of transthoracic echocardiography only and appears safe and effective while avoiding radiation and contrast agent use.

Percutaneous Closure of Atrial Septal Defects Under Transthoracic Echocardiography Guidance Without Fluoroscopy or Intubation in Children.

OBJECTIVE: Demonstrate the benefits of percutaneous atrial septal defect (ASD) closure under guidance of transthoracic echocardiography (TTE) without fluoroscopy. METHODS: From February 2013 to April 2014, 127 consecutive patients with an isolated type II ASD were recruited to undergo percutaneous closure under either TTE (n = 60, TTE group) or TEE (n = 67, TEE group) guidance. The TTE group received local anesthesia or sedation with propofol, and the TEE group received general anesthesia with endotracheal intubation. Follow-up examinations were performed for both groups at 1 month, 3 months, 6 months, and 1 year after discharge and annually thereafter. RESULTS: The TTE group had a significantly shorter procedure time and respirator ventilation duration than the TEE group. The dose of propofol required, the cost, and the pharyngeal complication rate were significantly lower in the TTE group than in the TEE group. The median follow-up of 11.6 months was uneventful in all patients. CONCLUSIONS: Percutaneous ASD closure with TTE guidance as the only imaging tool avoids fluoroscopy, endotracheal intubation, and probe insertion and is associated with a satisfactory procedural success rate and lower costs. This procedure is a safe and reliable treatment for ASD.

[Application research of percutaneous patent ductus arteriosus closure by femoral vein approach under echocardiography guidance].

OBJECTIVE: To avoid the injuries of radiation and contrast agent, we assess the efficacy and safety of percutaneous patent ductus arteriosus (PDA) closure by femoral vein approach solely under echocardiography guidance. METHODS: From January 2014 to December 2014, 25 patients in Fuwai hospital with PDA were selected, with mean age (4.5 ± 2.1) years and mean body weight (19 ± 7) kg. The mean diameter of PDA was (5.9 ± 1.2) mm. Patients were all treated by percutaneous PDA closure solely by echocardiography guidance in femoral vein. The effect of the procedure was evaluated by echocardiography. Follow-up was given at one month after procedure. RESULTS: Twenty-three cases were successfully treated with percutaneous PDA closure by femoral vein approach solely under echocardiography guidance, while two patients was closed by femoral artery approach because guide wires could not pass through PDA. The procedural time was (33 ± 5) min. The mean diameter of PDA occluder was (11.4 ± 1.5) mm. Postoperative early trivial residual shunt occurred in three patients. All patients survived with no peripheral vascular injury or complications such as cardiac perforation. Hospitalization time was (3.6 ± 0.8) days. At one month follow-up, no complications such as residual shunt or pericardial effusion were occurred. CONCLUSION: Echocardiography guided percutaneous PDA closure by femoral vein approach is safe and effective, and avoids the use of radiation and contrast agents.

[Value of a novel categorization of congenital double-outlet right ventricle on guiding the choice of surgical approaches].

OBJECTIVE: To explore the feasibility and value of a new categorization of double outlet right ventricular (DORV) on guiding the optimal choices of surgical approaches. METHODS: Five hundred and twenty one DORV patients diagnosed by echocardiography, angiocardiography and CT at Fuwai Hospital from May 2003 to September 2014 were enrolled in this retrospective study. Congenital DORV was categorized according to three basic factors as follows: the positional relationships of great arteries (normal relation or abnormal relation), the relationships of the ventricular septal defect (VSD) to the great arteries (committed VSD or remote VSD), the presence or absence of pulmonary outflow tract obstruction (POTO). Eight types of DORV were established: type I (normal relation, committed VSD, without POTO), type II (normal relation, committed VSD, POTO), type III (normal relation, remote VSD, without POTO), type IV (normal relation, remote VSD, POTO), type V (abnormal relation, committed VSD, without POTO), type VI (abnormal relation, committed VSD, POTO), type VII (abnormal relation, remote VSD, without POTO), type VIII (abnormal relation, remote VSD, POTO). Feasibility of this classification and the value of this classification on guiding the choice of surgical approaches were analyzed. RESULTS: Among the five hundred and twenty one patients, there were 90 patients (17.3%) with type I DORV, 94 patients (18.0%) with type II, 33 patients (6.3%) with type III, 34 patients (6.5%) with type IV, 64 patients (12.3%) with type V, 61 patients (11.7%) with type VI, 33 patients (6.3%) with type VII, 112 patients (21.5%) with type VIII. Thus, all patients could be typed by this classification method. The echocardiography diagnosis was consistent with the intra-operative and or cardiac catheterization/CT findings. Excluding the contraindications of bi-ventricular repair, different surgical approaches were performed in every subtype of DORV according the classification, which indicated that this novel categorization could accurately guide the clinic managements. CONCLUSION: This novel DORV categorization can accurately diagnose DORV lesions, and guide the clinic therapy choice.

[Safety and efficacy of percutaneous patent ductus arteriosus closure solely under thoracic echocardiography guidance].

OBJECTIVE: To avoid the radiation injuries and use of contrast agent, we assessed the safety and efficacy of percutaneous patent ductus arteriosus closure solely under thoracic echocardiography guidance. METHODS: From June 2013 to June 2014, thirty patients (mean age: (6.3 ± 2.5) years, mean body weight:(22.5 ± 7.3) kg) with pure patent ductus arteriosus were continuously included in this study. The mean diameter of patent ductus arteriosus was (3.8 ± 0.9) mm. Patients were all treated by percutaneous patent ductus arteriosus closure via right femoral artery solely under thoracic echocardiography guidance. The efficacy of the procedure was evaluated by thoracic echocardiography. Follow-up was performed at one month after procedure. RESULTS: All 30 cases were successfully treated with percutaneous patent ductus arteriosus closure solely under thracic echocardiography guidance. The procedural time was (32.8 ± 5.7) minutes. The mean diameter of Amplatzer ADO II was (4.9 ± 1.0) mm. Postoperative trivial residual shunt occurred in six patients immediately after the procedure. All patients survived without peripheral vascular injury or complications such as cardiac perforation. Hospitalization time was (3.4 ± 0.7) days. At one-month follow-up, no complications such as residual shunt or pericardial effusion were observed. CONCLUSION: Echocardiography guided percutaneous patent ductus arteriosus closure by femoral artery approach is safe and effective, and can avoid X-ray and the use of contrast agents.

[A cross-sectional study of pulmonary arterial hypertension in congenital heart disease by right heart catheterization].

OBJECTIVE: To explore the prevalence of pulmonary arterial hypertension (PAH) in congenital heart disease (CHD) and analyze its independent risk factors. METHODS: During May 2007 to December 2008, a total of 704 consecutive CHD patients initially admitted into Fuwai Hospital were recruited. Their baseline data were collected and right heart catheterization (RHC) was performed. Those with a mean pulmonary arterial pressure (mPAP) ≥ 25 mmHg (1 mmHg = 0.133 kPa) during RHC were diagnosed as PAH. Multiple Logistic regression was performed to analyze the independent risk factors of PAH. RESULTS: There were 319 males and 385 females with a median age of 5 years. The causes included atrial septal defect (n = 185), ventricular septal defect (VSD, n = 452), patent ductus arteriosus (PDA, n = 48) and a combinations of the above lesions (n = 19). And 280 (39.8%) CHD patients had PAH. Logistic regression analysis revealed that age, systemic artery systolic blood pressure, VSD, PDA and combined lesions were independent risk factors of PAH in CHD (age: OR = 1.017, 95%CI: 1.001-1.033, P = 0.040; systemic artery systolic blood pressure: OR = 0.959, 95%CI: 0.944-0.975, P < 0.001; VSD: OR = 4.402, 95%CI: 2.615-7.411, P < 0.001; PDA: OR = 6.417, 95%CI: 3.079-13.373, P < 0.001; combined lesions: OR = 10.513, 95%CI: 3.641-30.355, P < 0.001). CONCLUSIONS: PAH is a common complication of CHD. Age, peripheral artery systolic blood pressure and type of lesion are independent risk factors of PAH in CHD.

[Feasibility of transcatheter closure of atrial septal defect under the guidance of transthoracic echocardiography].

OBJECTIVE: To explore the feasibility of transcatheter closure of atrial septal defect (ASD) under transthoracic echocardiography (TTE) guidance. METHODS: Retrospective analysis was performed in 65 patients with simple ASD who underwent transcatheter closure under echocardiography guidance in Fuwai hospital from February to August 2013. They were divided into TTE group (n = 30) and transesophageal echocardiography (TEE) group (n = 35). The TTE group patients who underwent localized anesthesia or basal anesthesia received transcatheter closure of ASD under the guidance of TTE. The TEE group patients who underwent tracheal intubation and general anesthesia received transcatheter closure of ASD under the guidance of TEE. The patients were followed up with TTE and electrocardiogram at one month after procedure at outpatient department. RESULTS: In the TTE group, 28 occluders were implanted successfully and 2 patients were subsequently switched to TEE guidance because of unclear TTE images, and the occluder implantation in these 2 patients was successful. There were no obvious differences in age, sex, body weight, ASD size, and time of hospital stay between the two groups (all P > 0.05) . Compared with TEE group, the TTE group had a significantly shorter operation time ((52.77 ± 9.00 ) min vs. (60.11 ± 9.15) min, P < 0.05), respirator ventilation duration ((0.25 ± 0.95) h vs. (3.17 ± 0.69) h, P < 0.05), and stay time in ICU ((1.50 ± 1.96) h vs. (16.43 ± 6.99) h, P < 0.05). The dose of propofol required was significantly lower in the TTE group compared to TEE group ((2.41 ± 2.97) mg/kg vs. (9.43 ± 3.70) mg/kg, P < 0.05). The patients in both groups had no complications such as residual shunt, peripheral vascular injury or cardiac perforation at the time of hospitalization.No complications, such as occluder dislocation, residual shunt, or pericardial effusion were seen during follow-up at one month post procedure in both groups. CONCLUSION: Transcatheter closure of ASD under TTE guidance is feasible and has a broad application prospects.

Ozaki technique versus ross operation for complex aortic valve diseases in children: A retrospective cohort study.

BACKGROUND: The Ozaki technique demonstrated promising results in adults, but few studies reported on pediatric patients with limited follow-up time. This study aimed to evaluate the mid-term results of Ozaki technique compared with Ross operation for complex aortic valve (AV) diseases in children. MATERIALS AND METHODS: One hundred and seventeen children underwent either Ozaki (n = 64) or Ross (n = 53) operation from January 2017 to December 2023. The primary endpoint was incidence of moderate or severe regurgitation/stenosis (AR/AS) post procedure. RESULTS: No significant difference was observed in age (6.5±3.4 vs. 7.9±4.3 years) and weight (25.9±15.5 vs. 31.0±25.9 kgs) at surgery. The Ozaki group had significantly more patients in heart failure (20.3% vs. 1.9%, P = 0.003) before surgery and more patients needed ECMO installation (6.3% vs. 0, P = 0.125) after surgery. The Ozaki group were in worse status with more patients occurred heart failure (20.3% vs. 1.9%, P = 0.003) before surgery and needed ECMO installation (6.3% vs. 0, P = 0.125) after surgery. During follow up (20.4±17.3 vs. 22.7±22.8 months, P = 0.526), five patients (7.8%) in Ozaki group but no patients in Ross group required reoperations. The incidence of moderate or severe AR (28.1% vs. 3.1%) and AS (31.3% vs. 5.7%) were significantly higher than Ross group. Multivariate analysis identified lower age [HR:1.282 (95%CI:1.075-1.529), P = 0.006] and ECMO installation [HR:0.126 (0.018-0.887), P = 0.037] to be risk factors for moderate or severe AR, and higher aortic transvalvular gradient before discharge was confirmed as the only risk factor for moderate or severe AS (≥36 mmHg) at follow up in Ozaki group. CONCLUSION: Ozaki technique may be used as a palliative procedure for complex AV diseases in children, but its' mid-term results were not durable as Ross surgery, especially younger patients.

[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.

Fate of transposition of the great arteries with pulmonary stenosis after double-root translocation, Rastelli, and Réparation à l'Etage Ventriculaire.

OBJECTIVES: For transposition of the great arteries with unrestricted ventricular septal defect and pulmonary stenosis, double-root translocation is reported to reconstruct ideal double artery roots with growth potential. However, prospective long-term studies describing the long-term outcomes are still scarce. Therefore, the aim was to assess development of double artery roots, hemodynamics, and freedom from death and heart failure 17 years after double-root translocation, Rastelli, and Réparation à l'Etage Ventriculaire procedures. METHODS: In this prospective population-based study, 266 patients with transposition of the great arteries/ventricular septal defect/pulmonary stenosis (from July 2004 to August 2021) were consecutively included before surgery. All patients were divided into 3 groups based on the type of operation: double-root translocation (174), Rastelli (68), and Réparation à l'Etage Ventriculaire (24), who accepted postoperative evaluations annually. Generalized linear mixed model analysis was performed to determine growth potential of artery roots. RESULTS: Longitudinal repeated computed tomography measurements show the pulmonary root has significantly increased diameter (0.62 [0.03] mm/y, P < .001) over time and an adequate Z-score (-0.18) at the last follow-up only in the double-root translocation group. The pressure gradients of double outflow tracts in the double-root translocation group were the least among 3 groups. The probabilities of freedom from death/heart failure at the 15th year were 73.1%, 59.3%, and 60.9% in the double-root translocation, Rastelli, and Réparation à l'Etage Ventriculaire groups, respectively (double-root translocation vs Rastelli, P = .026; double-root translocation vs Réparation à l'Etage Ventriculaire, P = .009; Rastelli vs Réparation à l'Etage Ventriculaire, P = .449). CONCLUSIONS: By reconstructing ideal double artery roots, double-root translocation can provide postoperative long-term excellent hemodynamics and minimal death and heart failure for patients with transposition of the great arteries/ventricular septal defect/pulmonary stenosis.