Comparison between the modified Blalock-Taussig shunt and right ventricular outflow tract stent in the palliative treatment for tetralogy of Fallot. / 改良Blalock-Taussigåˆ†æµæœ¯ä¸Žå³å¿ƒå®¤æµå‡ºé“æ”¯æž¶æ¤å ¥æœ¯å§‘æ¯æ€§æ²»ç–—æ³•æ´›å››è”ç—‡çš„æ¯”è¾ƒ.

OBJECTIVES: For patients with tetralogy of Fallot (TOF) who are not suitable candidates for primary corrective surgery or have a high surgical risk, transcatheter right ventricular outflow tract (RVOT) stent implantation is considered a safe and effective palliative intervention. This study aims to investigate the therapeutic outcomes of RVOT stent implantation in neonates and infants with TOF in comparison with the modified Blalock-Taussig shunt (mBTS) and to compare the impact of the 2 palliative interventions on arterial oxygen saturation and pulmonary artery development in pediatric patients. METHODS: Clinical data of 32 patients with TOF admitted to the Second Xiangya Hospital of Central South University from March 2011 to March 2021 were retrospectively collected. The patients were divided into an mBTS group (undergoing mBTS, n=15) and a stent implantation group (undergoing RVOT stenting, n=17) according to the surgical procedures. The 2 groups were assessed and compared in the surgical-related arterial oxygen saturation, postoperative complication rate, mortality rate, and re-intervention rate. The development of the patients' main pulmonary artery, right pulmonary artery, and left pulmonary artery was assessed by z-scores according to echocardiographic results. RESULTS: The children in the stent implantation group were younger and less weighed compared with the mBTS group (both P<0.05). Compared with the preoperative period, children in the stent implantation group had significantly higher arterial oxygen saturation [(75±17)% vs (96±3)%, P=0.026]; z-scores of pulmonary trunk [(-2.82±1.27) points vs (0.86±0.77) points, P=0.014], right pulmonary artery [(-1.88±0.59) points vs (-0.28±0.71) points, P=0.011], and left pulmonary artery [(-2.34±0.36) points vs (-1.67±0.36) points, P=0.036] were significantly increased. However, there were no significant differences in arterial oxygen saturation and pulmonary artery z-scores between pre- and post-mBTS procedures (all P>0.05). CONCLUSIONS: RVOT stent would have good surgical outcomes used in TOF patients with low weight and severe comorbidities. It also leads to an higher postoperative oxygen saturation and better promotion of pulmonary artery growth with RVOT stent compared to mBTS.

Atrial septal defect with a rare occupying lesion in heart.

BACKGROUND: Cardiac epicardium hemangiomas are exceedingly rare; however, they can cause significant hemodynamic impairment and large pericardial effusions. On rare occasion, cardiac tumors coexist with malformations of the heart. CASE PRESENTATION: We present the case of a 10-month-old female infant with a rare cardiac surface hemangioma coexisting with malformations of the heart. It revealed an atrial septal defect (ASD) coexisting with an abnormal occupying lesion with high echogenicity. It was 35*12*9 mm in size and was found in the anterior atrioventricular junction to the posterior atrioventricular junction at the bottom of the ventricular septum by transthoracic echocardiography. We performed surgical treatment of the atrial septal defect and performed biopsy with the occupying lesion. The histopathological examination reported a benign tumor as hemangioma. As far as we know, this is the first case in which cardiac surface hemangioma was found to coexist with an atrial septal defect. CONCLUSIONS: Cardiac epicardium hemangiomas is a rare solid tumor of the heart. If the mass is impossible to resect and does not cause hemodynamic impairment, only mass biopsy is possible.

The M310T mutation in the GATA4 gene is a novel pathogenic target of the familial atrial septal defect.

BACKGROUND: Although most cases of atrial septal defect (ASD) are sporadic, familial cases have been reported, which may be caused by mutation of transcription factor GATA binding protein 4 (GATA4). Herein we combined whole-exome sequencing and bioinformatics strategies to identify a novel mutation in GATA4 accounting for the etiology in a Chinese family with ASD. METHODS: We identified kindred spanning 3 generations in which 3 of 12 (25.0%) individuals had ASD. Punctilious records for the subjects included complete physical examination, transthoracic echocardiography, electrocardiograph and surgical confirming. Whole-exome capture and high-throughput sequencing were performed on the proband III.1. Sanger sequencing was used to validate the candidate variants, and segregation analyses were performed in the family members. RESULTS: Direct sequencing of GATA4 from the genomic DNA of family members identified a T-to-C transition at nucleotide 929 in exon 5 that predicted a methionine to threonine substitution at codon 310 (M310T) in the nuclear localization signal (NLS) region. Two affected members (II.2 and III.3) and the proband (III.1) who was recognized as a carrier exhibited this mutation, whereas the other unaffected family members or control individuals did not. More importantly, the mutation GATA4 (c.T929C: p.M310T) has not been reported previously in either familial or sporadic cases of congenital heart defects (CHD). CONCLUSIONS: We identified for the first time a novel M310T mutation in the GATA4 gene that is located in the NLS region and leads to family ASD with arrhythmias. However, the mechanism by which this pathogenic mutation contributes to the development of heart defect and tachyarrhythmias remains to be ascertained.

Application value of echocardiography in transthoracic punctural closure of postoperative residual ventricular septal defect of congenital heart disease. / è¶ å£°å¿ƒåŠ¨å›¾åœ¨ç»èƒ¸å£ç©¿åˆºæ²»ç–—å ˆå¤©æ€§å¿ƒè„ç— æœ¯åŽæ®‹ä½™å®¤é—´éš”ç¼ºæŸä¸­çš„åº”ç”¨ä»·å€¼.

OBJECTIVES: Residual ventricular septal defect (VSD) after congenital heart disease (CHD) is one of the major postoperative complications in cardiac surgery. At present, the commonly used clinical treatment methods for this complication are reoperation to redo surgical repair with cardiopulmonary bypass (CPB) and percutaneous transcatheter device closure, but these 2 methods have their own advantages and disadvantages. Transthoracic punctural closure of residual VSD is a feasible, safe, and novel technique for patients with residual VSD, which avoids not only the risk of difficulties in reoperation under another CPB due to thoracic adhesion, but also the risk of radiation exposure. Moreover, the operation is easier to handle due to short and direct operation path. This study aims to explore the role and value of echocardiography in transthoracic punctural closure of postoperative residual VSD of CHD. METHODS: A total of 25 patients, who were admitted in the Department of Cardiovascular Surgery, Second Xiangya Hospital, Central South University and accepted transthoracic punctural closure of postoperative residual VSD, were collected. The morphology of the residual VSD and the distance from tricuspid valve and aortic valve were assessed by trans-esophageal echocardiography (TEE) preoperatively, and the location of the punctural point and the direction of puncture were determined. The establishment of delivery track and releasing of occluder device were accurately guided by TEE intraoperatively. The position and morphology of the occluder device, residual shunt, aortic regurgitaion, and outflow obstruction were required close attention in immediately postoperative evaluation. If any dislocation or residual shunt was found, adjustments were needed immediately. Follow-ups were performed at 3-5 days, 1 month, 3 months, 6 months, and 1 year after operation. Occluder location, residual shunt, valvular function, and other complications were observed by transthoracic echocardiography (TTE) to assess the effect of the closure by occluder. Ventricular size and cardiac function were determined to evaluate the state of ventricular remodeling. In addition, cardiac rhythm was monitored by ECG periodically. RESULTS: Of the 25 patients underwent transthoracic punctural closure of postoperative residual VSD, except 1 double outlet right ventricle (DORV) and 1 tetralogy of fallot (TOF) postoperative patients failured and immediately received a thoracotomy surgery with CPB due to excessive size of residual defect and the irregular morphology, the rest 23 patients were successfully closed by the occluders (92.0%). Among the 23 occluders (diameters range from 5 mm to 10 mm), membrane symmetrical VSD occluders were applied to 17 cases, small-waist-large-edge VSD occluder was applied to 1 case, and eccentric VSD occluders were applied to 5 cases. TEE, applied immediately after occlusion, showed the satisfactory position and the shaping of the occluders. There were no residual shunts, no cardiac tamponade, no thrombosis and outflow obstruction. Two patients had small amounts of pericardial effusion. No newly emerging valve reflux was observed. After 3-48 months of observation, there was no device displacement, newly emerging valve reflux, and residual shunt. One case had incomplete right bundle branch block. CONCLUSIONS: Guided by TEE, transthoracic punctural closure of postoperative residual ventricular septal defect of CHD is safe and effective. This procedure has broadened the indications for the minimally invasive treatment of CHD and improved the technical system of the minimally invasive treatment of CHD. TEE which can provide accurate diagnosis and guide the whole process plays a decisive role in this operation technique.

Image diagnosis: Eisenmenger's syndrome in patients with simple congenital heart disease.

BACKGROUND: Early identification of congenital heart disease (CHD) allows detection of the pulmonary arteriopathy in an early stage, and timely shunt closure can permanently reverse pulmonary arterial hypertension (PAH). However, surgical correction is not recommended in patients with irreversible PAH. Herein we report our experience about Eisenmenger's syndrome in simple CHD. CASE PRESENTATION: From January 2017 to November 2018, a total of 8 CHD patients (3 ventricular septal defects (VSD), 2 atrial septal defects (ASD), and 3 patent ductus arteriosus (PDA), median age, 15.5 years [range, 3-18 years]) with PAH were detected by chest X-ray, electrocardiogram, transthoracic echocardiography (TTE), computed tomographic angiography (CTA) and cardiac catheterization. The median defect diameter, pulmonary artery pressure (PAP), pulmonary vascular resistance (PVR) were 16.5 mm (range, 3-30 mm), 75 mmHg (range, 60-86 mmHg), and 16 Woods units (range, 12-19 Woods units), respectively. Here, we report the representative cases of three types of simple CHD with irreversible PAH. The surgical correction was not performed in all patients who had fixed PAH and were referred to medical treatment. CONCLUSIONS: PAH in CHD can be reversed by early shunt closure, but this potential is lost beyond a certain point of no return. This article highlights the essence of enhancing the level of healthcare and services in Chinese rural areas. Failure to accurately and timely assess PAH will delay effective treatment past optimal treatment time, and even lead to death.

Echocardiography-guided percutaneous closure of perimembranous ventricular septal defects without arterial access and fluoroscopy.

BACKGROUND: Traditional percutaneous device closure of perimembranous ventricular septal defects (PmVSDs) is a minimally invasive technique, but can result in high radiation exposure and can result in potential arterial complications. Here, we aimed to assess the safety and feasibility of device closure of PmVSDs via the femoral vein approach under transesophageal echocardiography (TEE) guidance in children. METHODS: From January 2014 to December 2017, a total of 46 PmVSD patients (mean age, 6.5 ± 2.3 years [range, 4.2-12.0 years]; mean body weight 22.1 ± 6.6 kg [range, 16.0-38.5 kg]; VSD diameter, 4.1 ± 0.6 mm [range, 3.2-5.0 mm]) underwent attempted transcatheter closure via the femoral vein approach under the guidance of TEE without fluoroscopy. RESULTS: The transcatheter occlusion procedure under TEE guidance was successful in 44 (95.7%) patients. Surgery was necessary in 2 (4.3%) patients. The procedure duration was 28.2 ± 8.7 min (range, 12.0-42.0 min). One patient had immediate post-operative trivial residual shunt and three patients had immediate incomplete right bundle branch block (IRBBB) after operation; the new IRBBB in 1 case was noted in the first postoperative month. No residual shunt was noted at 3 months after the procedure, and no intervention related complications were detected at 1-24 months follow-up. CONCLUSIONS: Percutaneous device closure of PmVSDs under TEE guidance solely by femoral vein approach is effective and safe, avoids radiation exposure, potential arterial complications and a surgical incision.

[Establishment and preliminary mechanism study of the zebrafish strain of KIAA0196: A candidate pathogenic gene for heart development].

OBJECTIVE: To explore the effects of KIAA0196 gene on cardiac development and the establishment of zebrafish strain.
 Methods: Peripheral blood and gDNA from patients were extracted. Copy number variation analysis and target sequencing were conducted to screen candidate genes. The KIAA0196 knockout zebrafish was generated by CRISPR/Cas9 to detect whether KIAA0196 deficiency could affect cardiac development. Finally, the wild-type and mutant zebrafish were anatomized and histologically stained to observe the phenotype of heart defects.
 Results: The KIAA0196 knockout zebrafish strain was successfully constructed using CRISPR/Cas9 technology. After 60 hours fertilization, microscopic examination of KIAA0196 knockout zebrafish (heterozygote + homozygote) showed pericardial effusion, cardiac compression and severely curly tail. Compared with wild-type zebrafish, the hearts of mutant KIAA0196 zebrafish had cardiac defects including smaller atrium and larger ventricle, and the myocardial cells were looser.
 Conclusion: KIAA0196 gene plays an important regulatory role in the development of heart. It might be a candidate gene for congenital heart disease.

Echocardiography-guided percutaneous closure of patent ductus arteriosus without arterial access: Feasibility and safety for a new strategy.

OBJECTIVE: To evaluate the feasibility and safety of device closure of patent ductus arteriosus (PDA) using only venous access under echocardiography guidance alone.
 Methods: A total of 102 consecutive pediatric patients underwent transcatheter PDA closure without arterial access, under the guidance of only echocardiography. The patients were followed up by clinical examination, electrocardiogram, and echocardiogram at 1, 3, 6 12, and 24 months.
 Results: Transvenous PDA closure under echocardiographic guidance was successful in 99 (97.1%) patients. There were no acute procedural complications or severe adverse events. The duration ranged from 10 to 65 minutes (median, 21 minutes). Immediate complete closure of PDA was achieved in 87 patients (87.9%), and 100% of the patients were completely closed after 24 h. There were no severe adverse events in the period of 1-24 months (median, 12 months) follow up.
 Conclusion: Transvenous PDA closure without fluoroscopy avoids radiation exposure, contrast agent usage and potential arterial complications. It can be used as an alternative procedure, especially for children.

A Targeted, Next-Generation Genetic Sequencing Study on Tetralogy of Fallot, Combined With Cleft Lip and Palate.

BACKGROUND: Congenital heart disease (CHD), plus cleft lip and palate (CLP) are currently the most common types of structural malformation in infants. Many genes have been investigated for their involvement in CHD with CLP. Targeted next-generation sequencing can analyze large amounts of genetic information rapidly, and thus address this question. METHODS: The authors designed a targeted, next-generation sequencing gene panel for 455 genes previously implicated in CHD or CLP. A single-subject patient served as a genetic source. Variants that affect protein-coding regions were classified into silico and filtered through databases, such as the Single-Nucleotide Polymorphism Database, Yan Huang, the Exome Sequencing Project, and the 1000 Genomes Project. The authors then predicted the function of gene mutations by PolyPhen-2, SIFT, and Mutation Taster. To confirm the related disease genes, the authors surveyed relevant literature on PubMed. Finally, the variant was verified by Sanger sequencing. RESULTS: A total of 1520 mutations were successfully found in a patient using combined tetralogy of Fallot and CLP by the targeted next-generation sequencing. However, there were 6 gene mutations (ZNF528, PVRL2, methylenetetrahydrofolate reductase [MTHFR], EVC2, DAND5, CCDC39) that were not found on Single-Nucleotide Polymorphism Database, Yan Huang, Exome Sequencing Project, and 1000 Genomes Project. Four genes (ZNF528, PVRL2, EVC2, CCDC39) were all predicted to be "tolerated," "benign," or "polymorphic" by SIFT, PolyPhen-2, and Mutation Taster. The DAND5 gene was predicted to be "possibly damaging" and "disease causing" respectively by PolyPhen-2 and Mutation Taster, but the SIFT program predicted this mutation to be "tolerated." Likewise, the MTHFR gene mutation was predicted to be "damaging," "possibly damaging," and "disease causing" respectively by SIFT, PolyPhen-2, and Mutation Taster. There is no relevant report about MTHFR gene mutation (c.G586A, p.G196S) on PubMed. CONCLUSION: Using targeted, next-generation sequencing technology, the authors identified for the first time a mutation (c.G586A, p.G196S) in the MTHFR gene as a possible cause of TOF and CLP in a patient.

[Application of perimembranous ventricular septal defects closure solely by femoral vein approach under transesophageal echocardiography guidance].

OBJECTIVE: To investigate the feasibility and safety of perimembranous ventricular septal defects (PmVSD) closure solely by femoral vein approach under transesophageal echocardiography (TEE) guidance.
 Methods: From January 1, 2014 to May 31, 2016, 26 patients with PmVSD in Second Xiangya Hospital were selected, with age at 3.2-6.0 (4.3±0.7) years old and body weight at 15.0-19.5 (16.7±1.4) kg. The diameter of VSD was 3.5-4.8 (4.1±0.3) mm. All patients were treated by percutaneous PmVSD closure solely by femoral vein approach under TEE guidance. The effect of the procedure was evaluated by TEE and transthoracic echocardiography (TTE). The clinical follow-up study was conducted by TTE at 1, 3, 6 and 12 month (s) after the procedure.
 Results: Twenty cases were successfully treated with percutaneous PmVSD closure solely by femoral vein approach under TEE guidance, and the success rate was 76.9%. Six patients were converted to perventricular closure under TEE guidance because the guide wire in two cases or catheter in other cases could not pass through PmVSD. The diameter of symmetrical VSD occluder was 6.0-7.0 (6.2±0.4) mm. The procedural time was 12.0-64.0 (26.8±6.3) min. The residence time at ICU was 1.8-2.4 (26.8±6.3) h. The in-hospital time was 4.0-5.0 (4.4±0.5) d. There were 3 patients with immediate post-operative trivial residual shunt and incomplete right bundle branch block (IRBBB). All patients survived with no peripheral vascular injury or complications such as tricuspid regurgitation, pericardial tamponade and pulmonary infection. The residual shunt disappeared in 3 patients and IRBBB became normal rhythm in 3 patients at 1 month follow-up time point. No patients suffered from occluder malposition, residual shunt, pericardial effusion, arrhythmia (atrio-ventricular block), aortic valve regurgitation and tricuspid regurgitation.
 Conclusion: TEE-guided percutaneous PmVSD closureby femoral vein approach is safe and effective.

[Hybrid procedure for infants/children treatment with pulmonary stenosis under transesophageal echocardiographic guidance].

OBJECTIVE: To investigate the outcomes of hybrid procedure in treating 10 infants/children with pulmonary stenosis under transesophageal echocardiographic guidance.
 METHODS: Between September, 2009 and December, 2015, 10 infants/children underwent hybrid procedure of transthoracic balloon pulmonary valvuloplasty for pulmonary stenosis in the Second Xiangya Hospital, Central South University. The age, height and weight at the time of admission were 0.7-42 (14.8±15.8) months, 53-97 (74.8±16.3) cm, and 4-15.5 (9.3±4.1) kg, respectively. Atrial septal defect, patent foramen ovale, patent ductus arteriosus, muscular ventricular septal defect, persistent left superior vena cava and tricuspid regurgitation were found in 2, 6, 1, 2, 1 and 5 cases, respectively.
 RESULTS: After the operation, all patients were sent into ICU. The mean duration mechanical ventilation, ICU stay and hospitalization were 0.5-41(6.8±12.3) h, 2-85 (31.1±22.8) h, and 6-20 (11.4±5.1) d, respectively. Postoperative transvalvular pressure gradient reduced to 16-45 (31.1±9.8) mmHg, which was decreased significantly compared with that in preoperative (P<0.001). There was no death during hospitalization and follow-up.
 CONCLUSION: Hybrid procedure of transthoracic balloon pulmonary valvuloplasty for pulmonary stenosis under transesophageal echocardiographic guidance is a safe and effective treatment.

Duplication of 10q22.3-q23.3 encompassing BMPR1A and NGR3 associated with congenital heart disease, microcephaly, and mild intellectual disability.

Chromosome region 10q22.3-q23.3 contains several low copy repeats (LCRs) and is prone to recombination. Deletions with breakpoints within LCR3 and LCR4 have been described to be associated with intellectual disability and dysmorphic features, while the reciprocal duplications are rarely reported. We present an additional case with multiple congenital anomalies that include microcephaly, cardiac defect, and mild intellectual disability, in which a de novo interstitial 8.2-Mb duplication of 10q22.3-q23.3, including BMPR1A and NGR3, was identified by Illumina SNP array platform. Our study is consistent with the hypothesis that the BMPR1A is a plausible candidate gene for congenital heart disease (CHD) and should contribute to the diagnosis and treatment of these genomic diseases.

A minimally invasive technique for occluding large muscular ventricular septal defects in infants.

OBJECTIVE: This study investigated the feasibility, timing and indications for closure of large muscular ventricular septal defects (VSDs) in infants without use of a cardiopulmonary bypass. METHODS: A total of 9 infants with large muscular VSDs ranging in size from 7 to 15 mm were treated by minimally invasive closure without cardiopulmonary bypass between April 2010 and February 2013. RESULTS: All surgeries were successful without resulting in uncontrolled systemic infection, a pulmonary hypertensive crisis or a severely low cardiac output, and with an apparently shorter operation time and postoperative tracheal cannula. CONCLUSIONS: VSD closure without cardiopulmonary bypass can improve the efficacy and prognosis of infants with large muscular VSDs and concurrent pulmonary infections or respiratory failure.

Transesophageal echocardiography-guided minimally invasive surgical device closure of an unusually shaped residual ventricular septal defect in a child.

Closure of residual ventricular septal defect with an occluder is traditionally performed by a percutaneous transcatheter approach under radiographic guidance. However, this procedure may be of limited use in cases with unusually shaped defects and in patients with low body weight. Here, we report minimally invasive surgical device closure of a 6 mm residual ventricular septal defect under transesophageal echocardiographic guidance, in a patient weighing 10 kg that had previously undergone surgical correction of a double outlet right ventricle. The defect was positioned in the suture line between the Gore-Tex vascular graft and the remnant ventricular septum, and was unusual in that it formed a 135° angle with the Gore-Tex graft. The defect was closed successfully with a 10 mm asymmetric occluder. To the best of our knowledge, this is the first report of transesophageal echocardiography-guided minimally invasive surgical device closure of an unusually shaped residual ventricular septal defect after surgical correction of a double-outlet right ventricle.

A novel approach: trans-ascending aorta balloon aortic valvuloplasty via sternotomy for treating severe valvular aortic stenosis in a low-weight infant.

BACKGROUND: Severe congenital aortic stenosis in infants is a life-threatening congenital heart anomaly that is typically treated using percutaneous balloon aortic valvuloplasty. METHODS: The usual route is the femoral artery under radiographic guidance. However, this procedure may be limited by the small size of the femoral artery in low-weight infants. An infant weighing only 7 kg with severe aortic stenosis (peak gradient was 103 mmHg) was successfully treated with a novel approach, that is trans-ascending aorta balloon aortic valvuloplasty guided by transesophageal echocardiography. RESULTS: The patient tolerated the procedure well, and no major complications developed. After the intervention, transesophageal echocardiography indicated a significant reduction of the aortic valvular peak gradient from 103 mmHg to 22 mmHg, no aortic regurgitation was found. Eighteen months after the intervention, echocardiography revealed that the aortic valvular peak gradient had increased to 38 mmHg and that still no aortic regurgitation had occurred. CONCLUSIONS: In our limited experience, trans-ascending aorta balloon aortic valvuloplasty for severe aortic stenosis under transesophageal echocardiography guidance effectively reduces the aortic peak gradient. As this is a new procedure, long-term follow up and management will need to be established. It may be an alternative technique to treat congenital aortic stenosis in low-weight patients.

Echocardiographic assessment and guidance in minimally invasive surgical device closure of perimembranous ventricular septal defects.

BACKGROUND: The primary aim of this study was to explore the safety and feasibility of minimally invasive surgical device closure of perimembranous ventricular septal defects (PMVSDs) in children using echocardiography for preoperative assessment and intraoperative guidance. METHODS: We enrolled 942 children diagnosed with PMVSDs from April 2010 to October 2013. All children underwent full evaluation by transthoracic echocardiography (TTE) and multiplane transesophageal echocardiography (MTEE) to determine the sizes, types and spatial positions of defects and their proximity to the adjacent tissues. The PMVSDs were surgically occluded using MTEE for guidance. RESULTS: Eight hundred eighty-nine (94.37%) of 942 children underwent successful closure of PMVSDs. Symmetric devices were used in 741 children (including 38 A4B2 occluders) and asymmetric devices were used in the other 148. All patients received follow-ups at regular intervals after successful occlusion. The occluders remained firmly in place. No noticeable residual shunt or valvular regurgitation was discovered, with the exception of one child whose original mild aortic regurgitation progressed to moderate by the 18 month follow-up. Overall there were no significant arrhythmias with the exception of 3 children, all of whom experienced postsurgical acute attacks of Adams-Stokes syndrome. CONCLUSIONS: Minimally invasive surgical device closure of PMVSDs is safe and feasible. TTE and MTEE play vital roles in all stages of treatment of PMVSDs.

[Asymmetric occluder in minimal-invasive surgical device closure of ventricular septal defects].

OBJECTIVE: To discuss the suitable types of ventricular septal defects for asymmetric occluders, and elucidate the critical role of echocardiography in choosing occluders, guiding successful occlusion and avoiding injury during operation. METHODS: We retrospectively studied 179 patients with ventricular septal defects who received minimal-invasive surgical device closure with asymmetric occluder. We analyzed the types, size and morphology of ventricular septal defects suitable for asymmetric occluders. The therapeutic efficiency was evaluated by follow-ups. RESULTS: Out of the 179 successful cases treated with asymmetric occluders, 86.59% had perimembranous ventricular septal defects, and double-committed sub-arterial ventricular septal defects accounted for 13.41%. In general, the size of occluders to be selected was the maximum diameter of the defects plus 2-3 mm. Follow-ups showed that occluders were placed and fixed properly. No severe residual shunt, valve regurgitation or heart block were discovered. CONCLUSION: Application of asymmetric occluders expands the range of indications for occlusion via small chest incision. Accurate echocardiography helps to improve the safety and successful rate of the surgery.

[Echocardiography in mini-invasive surgical device closure of secundum atrial septal defects].

OBJECTIVE: To discuss the preoperative, intraoperative, and postoperative application of echocardiography in mini-invasive surgical device closure of secundum atrial septal defects, including those special and difficulty-occluded defects. METHODS: We performed mini-invasive surgical device closure of secundum atrial septal defects on 287 patients. Before the surgery, transthoracic echocardiography was applied for screening; during the surgery we reassessed the sizes of the defects and their remaining margins, designated the suitable occluders, and guided the placement of the occluders by multiplane transesophageal echocardiography. The patients were postoperatively followed up at regular intervals by multiplane transesophageal echocardiography (MTEE) which was employed to assess the therapeutic efficacy. RESULTS: Out of the 287 atrial septal defects, 276 (96.17%) were successfully closed. There were 37 porous defects and 23 cases with short posterior-inferior margin of defects. Follow-ups at intervals showed the occluders stayed firmly and echoed clearly. No notable residual shunt or valve regurgitation was observed. CONCLUSION: Echocardiography plays a vital and reliable role in mini-invasive surgical device closure of secundum atrial septal defects, especially those special and difficulty-occluded defects.

Diagnosis of covert coarctation of the aorta in adolescents.

Objectives: By reviewing the diagnostic process for adolescents with coarctation of the aorta (CoA) in our institution, we analyzed the reasons for delayed diagnosis of CoA. We also proposed a diagnostic protocol to improve the detection rate of CoA. Methods: In this retrospective study, we included 48 patients aged 12-18 years who were diagnosed with CoA in our hospital from January 2000 to November 2022. Clinical data from involved cases in local hospitals and our institution were collected. Results: All patients had blood pressure (BP) measurements in upper and lower extremities in our institution. They all had hypertension, 29 (60.4%) of whom had known histories of the same. BP in the upper limbs of 47 (97.9%) patients was ≥20 mmHg higher than that in the lower limbs, and BP in the upper limb of 1 (2.1%) patient was greater than 0 and less than 20 mmHg than that in the lower limb. Echocardiography (ECHO) was performed in all patients, computed tomography (CT) or magnetic resonance imaging (MRI) was performed in 44 patients (91.7%). There were 38 (79.2%) patients who visited local hospitals. Among them, a total of 20 (52.6%) patients had their right upper extremity BP measured, 18 (47.4%) only had their left upper extremity BP measured, and 16 (42.1%) had their lower extremity BP measured. ECHO was performed in 27 (56.2%) patients and CT/MRI was performed in 18 (37.5%) patients. The detection rate for CT/MRI was 100%, and those of ECHO were 72.9% and 18.5% at our institution and a local hospital, respectively. Forty-eight (100%) and 23 (60.5%) patients were detected in our institution and local hospitals (P < 0.0001). Conclusion: We recommend measuring BP in the bilateral upper extremities. Measurement of BP in the lower extremities is recommended if hypertension is diagnosed. MRI/CT is recommended when BP in the upper extremity is greater than that in the lower extremity.