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.

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.

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.
Adolescent ; Child ; Child, Preschool ; Echocardiography, Transesophageal ; methods ; Female ; Heart Septal Defects, Atrial ; surgery ; Humans ; Infant ; Male ; Minimally Invasive Surgical Procedures ; methods ; Septal Occluder Device ; Ultrasonography, Interventional

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.
Adolescent ; Child ; Child, Preschool ; Echocardiography, Doppler, Color ; Female ; Heart Septal Defects, Ventricular ; diagnostic imaging ; surgery ; Humans ; Infant ; Male ; Minimally Invasive Surgical Procedures ; instrumentation ; methods ; Retrospective Studies ; Septal Occluder Device

OBJECTIVE: To retrospectively analyze sex chromosomal abnormalities and clinical manifestations of children with disorders of sex development (DSD). METHODS: A total of 14 857 children with clinical features of DSD including short stature, cryptorchidism, hypospadia, buried penis and developmental delay were recruited from Zhengzhou Children's Hospital from January 2013 to March 2022. Fluorescence in situ hybridization (FISH) and chromosomal karyotyping were carried out for such children. RESULTS: In total 423 children were found to harbor sex chromosome abnormalities, which has yielded a detection rate of 2.85%. There were 327 cases (77.30%) with Turner syndrome and a 45,X karyotype or its mosaicism. Among these, 325 were females with short stature as the main clinical manifestation, 2 were males with short stature, cryptorchidism and hypospadia as the main manifestations. Sixty-two children (14.66%) had a 47,XXY karyotype or its mosaicism, and showed characteristics of Klinefelter syndrome (KS) including cryptorchidism, buried penis and hypospadia. Nineteen cases (4.49%) had sex chromosome mosaicisms (XO/XY), which included 11 females with short stature, 8 males with hypospadia, and 6 cases with cryptorchidism, buried penis, testicular torsion and hypospadia. The remainder 15 cases (3.55%) included 9 children with a XYY karyotype or mosaicisms, with main clinical manifestations including cryptorchidisms and hypospadia, 4 children with a 47,XXX karyotype and clinical manifestations including short stature and labial adhesion, 1 child with a 46,XX/46,XY karyotype and clinical manifestations including micropenis, hypospadia, syndactyly and polydactyly, and 1 case with XXXX syndrome and clinical manifestations including growth retardation. CONCLUSION Among children with DSD due to sex chromosomal abnormalities, sex chromosome characteristics consistent with Turner syndrome was most common, among which mosaicism (XO/XX) was the commonest. In terms of clinical manifestations, the females mainly featured short stature, while males mainly featured external genital abnormalities. Early diagnosis and treatment are particularly important for improving the quality of life in such children.
Humans ; Male ; Female ; Turner Syndrome/genetics* ; In Situ Hybridization, Fluorescence ; Cryptorchidism ; Hypospadias ; Retrospective Studies ; Quality of Life ; Sex Chromosome Aberrations ; Karyotyping ; Mosaicism ; Disorders of Sex Development/genetics*

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.
Child ; Echocardiography, Transesophageal ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Humans ; Infant ; Pulmonary Valve Stenosis ; Treatment Outcome