Clinical Characteristics of Congenital Atresia of the Left Main Coronary Artery in 12 Children.

Background: Left main coronary artery atresia (LMCAA) is an extremely rare abnormality and only <100 cases have been reported worldwide. We describe the clinical manifestations, imaging features, prognosis, and treatments of LMCAA who were admitted in our department, which aimed to improve the clinical diagnosis and treatments of LMCAA in children. Methods: A retrospective study identified 12 patients diagnosed with congenital left coronary artery atresia at Pediatric Heart Center of Beijing Anzhen Hospital from June 2010 to June 2019. The clinical characteristics, imaging data, and treatment follow-up were analyzed. Results: Among the 12 cases, 8 were boys and 4 were girls; the age of onset was 2 months to 2 years old (median age 7 months); the age of diagnosis was 7 months to 6 years old (median age 2 years and 11 months). At the initial diagnosis, there were 4 cases of respiratory tract infection with cardiac murmur, 3 cases of cardiac shadow enlargement, 1 case of recurrent syncope, 2 cases of feeding difficulty with cardiac enlargement, and 2 cases of simple cardiac murmur. In 12 cases of electrocardiogram examination, 7 cases showed pathological Q waves of lead I, AVL and v4-v6; in 12 cases of chest X-ray examination, 8 cases showed cardiac shadow enlargement; in 12 cases of our hospital's first cardiac ultrasound examination, 4 cases were definitely diagnosed, and 8 cases showed the possibility of left coronary artery abnormality; in 5 cases of cardiac coronary CT angiography examination, 2 cases were confirmed, 2 cases reported suspected left coronary artery abnormality, and 1 case did not report abnormality; All cases were definitely diagnosed in 8 cases of angiography. Follow-up was performed from 1 to 8 years; one case died suddenly, one case of syncope after activity was treated by oral medication, 3 cases received open coronary angioplasty and mitral valvuloplasty, recovered well after operation, the rest of the children were treated by oral medication, and the symptoms are stable at present. Conclusions: Left main coronary artery atresia is difficult to diagnose and can result in heart failure early in life. Timely diagnosis and reasonable treatment are the keys to improve the prognosis.

Pulmonary valve annular and right ventricular outflow tract size as predictions values for moderate to severe pulmonary regurgitation after repaired Tetralogy of Fallot.

OBJECTIVE: This study sought to investigate the variation of right heart structure pre- and post-operation as risk factors for moderate to severe pulmonary regurgitation (PR) after repaired Tetralogy of Fallot and the best "cutoff" values for the transannular patch (TAP). METHODS: We collected surgical, echocardiographic, and computed tomographic data of Teralogy of Fallot (TOF) patients over two years and calculated z-score values based on the echocardiographic data. Based on the PR level after follow-up, the patients were divided into two groups, trivial to mild PR and moderate to severe PR. A multivariate logistic regression analysis was performed, and the receiver operating characteristic curve analysis was used to find the best "cutoff" value for risk factors. RESULTS: A total of 104 TOF patients were included in our cohort study. From the multivariate analysis, correction strategy (P = .002), difference in zRVOT (OR 1.974, 95% CI 1.354 to 2.878, P < .0001), and zPVA (OR 3.605, 95% CI 1.980 to 6.562, P < .0001) were the significant risk factors for moderate to severe PR. The "cutoff" value for the difference in zPVA that could predict moderate to severe PR in the TAP group was 3, and the optimal "cutoff" value for TAP was -1.4. CONCLUSIONS: The TAP is a risk factor for significant PR after surgery. We recommend the optimal "cutoff" value for TAP is -1.4 calculated using Shan-Shan Wang's data set. During the procedure, to limit the RVOT resection and restrict the enlargement of pulmonary annulus within a variation of z-score as 3 would reduce significant PR.

[Survey on common pediatric drugs for renal diseases].

OBJECTIVE: Development and use of better medicine for children is a worldwide problem recently, especially in China. The current situation of drugs for children's renal diseases is far from well-understood now. This survey focused on drugs for pediatric renal diseases including immunosuppressants, corticosteroids, diuretics, anticoagulants, hypotensives and antilipemic agents.Information regarding the dosage, form, precaution, usage and administration in inserts was collected in this study. METHOD: Drugs for pediatric renal diseases were selected according to the guidelines established by the Chinese Society of Pediatric Nephrology. The detailed information about the dosage, form of drugs was searched on the website of China-State Food and Drug (SFDA). The information of the precaution, usage and administration was obtained from the China Pharmaceutical Reference, the first edition. RESULT: In this study, there were 5 categories of medicine including immunosuppressants, corticosteroids, diuretics, anticoagulants, hypotensives and antilipemic agents, and 89 kinds of medicine for renal diseases. Among these medicines, 65.2% were found not suitable for children in terms of drug dosage and form, 19.1% did not indicate the precaution, 51.7% did not indicate clearly the safety and effectiveness for children, and 56.2% lacked the detailed information about the usage and administration for children. There were only 4 kinds of these medicines which were studied via clinical trials in children population. CONCLUSION: There is a lack of drugs for children with renal diseases. Most of the time, the medicines used by doctors are not specially manufactured for children. The safety and efficacy of drugs that are currently used to treat pediatric renal diseases are not clear and definite.In addition, few clinical trials have been conducted for evaluation of drugs for pediatric renal diseases.In clinic, the situation of off-label drug treatment is very serious.