Application of descending aortic translocation in tracheal or bronchial stenosis due to aortic deformity / 中华胸心血管外科杂志

Objective:To investigate the efficacy of descending aortic translocation in relieving the compression of trachea or bronchus caused by aortic deformity.Methods:From January 2017 to July 2019, a total of 11 patients with distal trachea or proximal bronchial stenosis caused by aortic deformity were treated with descending aortic translocation. Cardiac CT and fiberoptic bronchoscopy were performed before surgery. The median age was 55(23-540) days, and the body weight was 4(2.1-9.0)kg. Five patients had a special type of vascular ring(left aortic arch with right descending aorta, small aortic window with funnel chest; left aortic arch with right descending aorta and right artery ligament, vagus right subclavian artery, combined with trachea, carina and left and right bronchial stenosis in 1 case; Right aortic arch with left descending aorta, combined with tracheomalacia stenosis in 1 case; Right aortic arch with left descending aorta combined with broad tracheal stenosis and left pulmonary dysplasia in 1 case) compressed tracheal or bronchial tube in 5 cases. Three patients with left main bronchus constriction after traditional arch disconnection surgery. 3 patients with left main bronchus stenosis before coarctation or interrupted aortic arch.Surgical methods: Descending aortic translocation was performed through a midline sternotomy with cardiopulmonary bypass and deep hypothermia.The proximal descending aorta was transected distal to the left subclavian artery, proximal sutures were performed, and the distal brought up though the transverse sinus caudad to the right pulmonary artery and tracheal carina, and anastomosed in end-to-end fashion to the ascending aorta, and simultaneous correction was performed with intracardiac malformations, such as airway plasty was performed at the same time without improvement after compression of tracheal stenosis.Results:There was no death in the whole group. Median cardiopulmonary bypass was 180(136-337)min with an median aortic cross-clamp time of 51(30-84)min; Median absence of perfusion to the descending thoracic aorta 34(21-50)min .Tracheal compression was effectively relieved in 5 patients, and airway plasty was performed simultaneously in 6 patients due to persistent tracheal softening and stenosis. All patients had resolution of symptoms.There was only one case anastomotic stenosis after descending aortic translocation.The mean follow-up was(18.0±9.4)months.Conclusion:Descending aortic translocation can effectively relieve this kind of pressure due to aortic deformity.

Single-stage repair of interrupted aortic arches and associated cardiac anomalies in neonatal: the outcomes and follow-up / 中华胸心血管外科杂志

Objective:This study defined mid-term results of a policy of single-stage repair of interrupted aortic arch associated cardiac anomalies.Methods:Between October 2012 and June 2019, 38 patients with interrupted aortic arch were evaluated for short- and mid-term results after surgical treatment, the average age of patients was(12±7) days and the mean body weight was(3.3±0.5)kg.29 patients belonged to IAA type A, and 7 patients to type B, and 2 patients belonged to IAA type C, 30 patients were complicated with ventricular septal defect, atrial septal defect, and patent ductus arteriosus, two complicated with bicuspid aortic valve, four complicated with Taussig-Bing malformation and two with double outlet right ventricle, one with truncus arteriosus(A4), one with aortopulmonary window, one with left ventricle outflow tract obstruction. Single-stage repair was performed in all patients with cardiac anomalies.Results:Early mortality was 5.2%(2 cases), one was die for low cardiac output syndrome, the other die of septic shock. Median CPB time was 138 min(90-305 min), and median aortic cross clamping time was 68 min(47-163 min). Hospital day was 25d( 13-52 days).32 patients were followed-up, median time 34 months(9-85months). 2 patients(6.0%) were mild pulmonary stenosis pressure(20-25 mmHg), 3 patients(9.3%) suffer aortic anastomosis mild stnosis(21-44)mmHg.1 patient(3.1%) LVOTO was repaired ten months after the repair of interrupted aortic arch. The left broncus of of one patient was mild stenosis but without dyspnea.Conclusion:Single-stage end -to-side anastomosis repair of interrupted aortic arches in neonatal and repair of associated cardiac anomalies is safe and have low effective with low motality.

Clinical characteristics and treatment of congenital vascular rings in 54 infants / 中华胸心血管外科杂志

Objective To summarize the clinical characteristics and therapeutic effect of congenital vascular rings in in-fants. Methods The clinical data of 54 children with vascular rings were retrospectively analyzed from August 2011 to June 2017 at our center for surgical treatment. Age 2 days to 11 months,the median age was 1 months;weight 1. 4 - 9. 4 kg,the average weight of(4. 52 ± 1. 89)kg. The type of vascular ring includes 21 cases of double aortic arch,right aortic arch with left aortic duct / ligament with or without vagal left subclavian artery in 14 cases,9 cases of pulmonary artery suture,left aortic arch with right aortic duct / ligament with or without 5 cases of oblique right subclavian artery,3 cases of nameless arterial com-pression syndrome,2 cases of other vascular rings. All cases underwent echocardiography,chest radiography,and CT scan of the heart,and 42 cases were examined before operation or intraoperative bronchoscopy. 39 cases(72％)had tracheal stenosis. Results Twenty-six patients underwent extracorporeal circulation underwent vascular anteroposterior surgery with an average extracorporeal circulation time of( 160 ± 61)min and 28 patients underwent extracorporeal circulation. Postoperative mechani-cal ventilation 1 to 22 days,the median number of days 2 days;hospitalization time 7 to 62 days,the median of 19 days,hos-pital death in 2 cases(3. 7％). Discharge patients were followed up for 2 to 74 months,respiratory symptoms to varying degrees to ease or disappear,discharged in 3 cases. Conclusion Airway stenosis is a serious complication of the vascular ring. CT is the best way to determine the vascular ring. To avoid serious airway complications,the vascular ring should be operated as soon as possible,and the airway stenosis and intracardiac malformation Children suggested concurrent treatment,this can improve the survival rate of surgery and improve the prognosis of children.

Echocardiography in evaluation of pulmonary venous lesion in total anomalous pulmonary venous connection / 中国医学影像技术

Objective To explore the value of echocardiography in evaluation of pulmonary venous in total anomalous pulmonary venous connection (TAPVC).Methods Fifty-five children with TAPVC were enrolled in the study.The data of echocardiography and CT angiography were retrospectively analyzed and compared with intraoperative findings.Results Totally 55 patients with TAPVC were classified into supra-cardiac type (n=24),cardiac type (n=20),infra-cardiac type (n=7) and mixed type (n=4) according to the sites of drainage of pulmonary venous in echocardiography.In 15 patients with obstruction of pulmonary vertical vein,the sites of obstruction in the supra-cardiac type mostly presented between vertical vein and superior vena cava or innominate vein,and the sites of obstruction in the infra-cardiac presented all between vertical vein and hepatic or portal vein.In 4 patients with pulmonary vein stenosis,3 cases with local pulmonary vein stenosis were all cardiac type,which presented between individual pulmonary vein and common confluence or right atrium;1 patient with diffuse pulmonary vein stenosis was infra-cardiac type.In 9 patients of abnormal individual pulmonary vein,8 cases were not detected by echocardiography,but all were detected by CT angiography.Conclusion Echocardiography is able to make more comprehensive evaluation for the pulmonary venous drainage,obstruction,and proximal stenosis of individual pulmonary vein in TAPVC.CT angiography is superior in evaluation of abnormalities of connection and amount of individual pulmonary vein,and imaging of distal pulmonary vein.

Genetics and pedigree analysis of primary carnitine deficiency cardiomyopathy in 6 cases / 中华儿科杂志

<p><b>OBJECTIVE</b>To investigate the mutation and background of SLC22A5 in 6 patients with primary carnitine deficiency (PCD) who only presented as cardiomyopathy.</p><p><b>METHOD</b>Genomic DNA were abstracted from the blood of the patients and their parents. Using high-throughput sequencing to determine the mutation site.Using Sanger method to confirm the mutated alleles in PCD patients and detect the corresponding sequences in their patients. Using SIFT and PolyPhen to predict the function of protein for detected missense mutations.</p><p><b>RESULT</b>Three different mutations were identified, including 2 nonsense mutations (R254X and R289X), 1 missense mutation (C113Y), R254X was the most frequently seen mutation. Four patients had compound heterozygous mutations and 2 patients had homozygous mutations. Their parents were found to have heterozygous mutations in corresponding alleles.</p><p><b>CONCLUSION</b>R254X, R289X and C113Y might be associated with primary carnitine deficiency.</p>

The clinical experience of childhood cardiomyopathy caused by inborn errors of metabolism in 11 cases / 临床儿科杂志

Objectives To summarize the diagnosis and treatment of cardiomyopathy caused by inborn errors of metabo-lism (IEM). Methods The retrospective study included 11 cases diagnosed as metabolic cardiomyopathy through tandem mass spectrometry, activity of serum enzyme, detection of urine mucopolysaccharide and gene analysis from 2012 to 2013. Six cases were diagnosed as primary carnitine deficiency (PCD). Four cases were diagnosed as glycogen storage disease (GSD) and only 1 case was diagnosed as mucopolysaccharidosis. Six PCD cases received carnitine supplementation and anti-heart failure thera-py and received follow-up for 2-10 months. Other 5 cases received supportive treatment and follow-up. Results Patients with PCD recovered soon after treatment but other 5 cases have died within 5 months. Conclusion IEM is an important cause of chil-dren cardiomyopathy which varied in clinical manifestation, diagnosis, treatment and prognosis of different kinds of metabolic cardiomyopathy. Early diagnosis and treatment could be lifesaving for cardiomyopathy caused by IEM.