Descending aorta translocation for bronchial stenosis caused by shorter interaortic distance.

BACKGROUND AND AIM OF THE STUDY: Numerous anatomic relationships of arteries could cause extrinsic compression of the trachea or bronchus. We report a rare left bronchial stenosis just caused by shorter inter-aortic distance. METHODS: One patient wih recurrent coughing and wheezing was diagnosed as left emphysema.Cardiac computed tomography (CTA) shows a shorter distance between ascending aorta (AAo) and descending aorta (DAo) caused left bronchial stenosis with extrinsic compression of right pulmonary artery. RESULTS: A translocation of the descending aorta was performed in this patient, and postoperative CTA showed that the DAo was translocated to the AAo and the left main bronchial stenosis was relieved. CONCLUSIONS: Translocation of the DAo was necessary for the rare left bronchial stenosis caused by shorter inter-aortic distance and could bring a good outcome.

Cardiac-specific methylation patterns of circulating DNA for identification of cardiomyocyte death.

BACKGROUND: Correct detection of human cardiomyocyte death is essential for definitive diagnosis and appropriate management of cardiovascular diseases. Although current strategies have proven utility in clinical cardiology, they have some limitations. Our aim was to develop a new approach to monitor myocardial death using methylation patterns of circulating cell-free DNA (cf-DNA). METHODS: We first examined the methylation status of FAM101A in heart tissue and blood of individual donors using quantitative methylation-sensitive PCR (qMS-PCR). The concentrations and kinetics of cardiac cf-DNA in plasma from five congenital heart disease (CHD) children before and after they underwent cardiac surgery at serial time points were then investigated. RESULTS: We identified demethylated FAM101A specifically present in heart tissue. Importantly, our time course experiments demonstrated that the plasma cardiac cf-DNA level increased quickly during the early post-cardiac surgery phase, peaking at 4-6 h, decreased progressively (24 h) and returned to baseline (72 h). Moreover, cardiac cf-DNA concentrations pre- and post-operation were closely correlated with plasma troponin levels. CONCLUSIONS: We proposed a novel strategy for the correct detection of cardiomyocyte death, based on analysis of plasma cf-DNA carrying the cardiac-specific methylation signature. Our pilot study may lead to new tests for human cardiac pathologies.

The V-shaped double-layer patch technique for complete atrioventricular septal defect: A novel surgical technique.

OBJECTIVE: Several surgical techniques for repair of a complete atrioventricular septal defect have been developed. However, the postoperative complications with these methods may lead to reoperation during follow-up. The aim of this report is to share our experience with a modified surgical technique for complete atrioventricular septal defect that has anatomic advantages postoperatively and could reduce the reoperation rate. METHODS: Twenty-nine patients who underwent repair of complete atrioventricular septal defect using a V-shaped double-layer patch between April 2011 and September 2019 were retrospectively investigated. RESULTS: There were no deaths (0%) and only 1 reoperation (3.4%) in the series. The aortic crossclamp and cardiopulmonary bypass times were 62.7 ± 16.0 minutes and 113.9 ± 25.9 minutes, respectively. The median follow-up duration was 5.1 years. To date, no significant residual ventricular septal defects have been detected and no left ventricular outflow tract obstruction has been seen on echocardiography in any patient. During follow-up, the left atrioventricular valve status was assessed as no incompetence in 9 patients (31.0%), trivial in 18 patients (62.1%), and mild in 2 patients (6.9%). CONCLUSIONS: The V-shaped double-layer patch technique is a valuable surgical option for patients with complete atrioventricular septal defects. The midterm results in our series document excellent performance of this technique, which augments the area of the anterior valve of the left atrioventricular valve to make it closer to a normal mitral valve and may also reduce the need for reoperation.

A mid- and long-team follow-up study of the application of single-valved bovine pericardium patches in right ventricular outflow tract reconstruction.

Background: Pulmonary regurgitation following right ventricular outflow tract (RVOT) reconstruction may cause right heart dysfunction and even right heart failure. Installation of a single valve at this time point can effectively reduce pulmonary regurgitation, thereby protecting right heart function. Here, we analyzed the outcomes and mid- and long-term follow-up data of patients undergoing single-valved bovine pericardium patch (svBPP) placement for reconstruction and explored the effectiveness and gaps of svBPP in preventing right heart failure. Methods: A retrospective analysis was performed on patients undergoing RVOT reconstruction using BalMonocTM svBPP from October 2010 to August 2020. The follow up procedures included outpatient visits and collection of outcomes. The cardiac ultrasound-related indicators during the follow-up visits included ejection fraction (EF), right ventricular end-diastolic diameter (EDD), pulmonary regurgitation, and pulmonary artery stenosis. The survival rates and reoperation-free rate were analyzed by Kaplan-Meier method. Results: Patients includes tetralogy of Fallot, pulmonary atresia and other complex congenital heart disease. A total of 5 patients (5.7%) died during the perioperative period. Early complications included pleural effusion, cardiac insufficiency, respiratory insufficiency, chylothorax, and atelectasis, all of which were cured. After discharge, 83 patients (94.3%) were effectively followed up. During follow-up, 1 patient died and 1 patient underwent reoperation. The 1-, 5-, and 10-year survival rates were 98.8%, 98.8%, and 98.8%, respectively, and the reintervention-free rates for the same intervals were 98.8%, 98.8%, and 98.8%, respectively. The last follow-up ultrasound revealed severe pulmonary stenosis in 0 cases, moderate stenosis in 2 cases, mild stenosis in 7 cases, and no stenosis in 73 cases. Pulmonary regurgitation was not found in 12 patients; however, there were 2 cases of severe pulmonary regurgitation, 20 cases of moderate pulmonary regurgitation, and 48 cases of mild pulmonary regurgitation. Conclusions: As shown in the mid- and long-term follow-up studies, BalMonocTM svBPP has good performance in RVOT reconstruction. It can effectively eliminate or reduce pulmonary valve regurgitation and protect right heart function. Both réparation à l'Etage ventriculaire (REV) and the modified Barbero-Marcial procedure can bring growth potential and reduce reoperation rate.

[Genetics and pedigree analysis of primary carnitine deficiency cardiomyopathy in 6 cases].

OBJECTIVE: To investigate the mutation and background of SLC22A5 in 6 patients with primary carnitine deficiency (PCD) who only presented as cardiomyopathy. METHOD: 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. RESULT: 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. CONCLUSION: R254X, R289X and C113Y might be associated with primary carnitine deficiency.