Objective The influence of the coronary artery anatomy on the prognosis of patients receiving an arterial switch operation(ASO)is currently controversial,and the risk factors for this operation may change in more comp...Objective The influence of the coronary artery anatomy on the prognosis of patients receiving an arterial switch operation(ASO)is currently controversial,and the risk factors for this operation may change in more complicated patients.This study aimed to investigate the influence of coronary artery anomalies on the in-hospital and post-discharge outcomes of ASO in patients with transposition of the great arteries(TGA)and Taussig-Bing anomaly(TBA).Methods We retrospectively reviewed 206 patients who underwent ASO from January 2007 to December 2019.The median age at operation was 33[interquartile range(IQR):20–71]days.Median follow-up time was 7.2 years(IQR:4.0–10.3 years).Results Coronary anomalies were present in 86 patients(41.7%),with 9(4.4%)of them having a single coronary artery.Additional coronary features included intramural courses in 5(2.4%)patients,ostial stenosis in 1(0.5%)patient,and accessory coronary artery orifices in 5(2.4%)patients.There were 32(15.5%)in-hospital deaths and 8(4.6%)post-discharge deaths,yielding an overall survival of 81.3%,80.7%and 79.9%at 1,5 and 10 years,respectively.Mortality due to ASO has been drastically decreased since 2013.Patients with a single coronary artery had higher rate of in-hospital mortality,but this finding was not statistically significant.The earlier surgical era(OR:2.756)and a longer cardiopulmonary bypass time(OR:2.336)were significantly associated with in-hospital mortality,while coronary patterns were not.An intramural coronary artery(HR:10.034)and a patient age of older than 1 year at the time of ASO(HR:9.706)were independent predictors of post-discharge mortality.Conclusion ASO remains the procedure of choice for TGA with coronary anomalies with acceptable in-hospital and post-discharge outcomes in terms of overall survival and freedom of reoperation.However,intramural coronary artery is an independent risk factor for post-discharge mortality.Timely surgery within the 1st year of life helps improve overall midterm survival of ASO.展开更多
To the Editor: Periventricular nodular heterotopias (PNHs) represent a malformation of cortical development caused by an improper neuronal migration during forebrain formation, There is a wide spectrum of anatomica...To the Editor: Periventricular nodular heterotopias (PNHs) represent a malformation of cortical development caused by an improper neuronal migration during forebrain formation, There is a wide spectrum of anatomical and clinical presentations of PNH, ranging from asymptomatic small unilateral or bilateral nodules to extensive agglomerates of heterotopia lining the lateral ventricles with epilepsy and intellectual disabilities. The mutations in X-linked gene, filamin A (FLNA) gene, were identified in approximately 100% of familial PNHs and 25% of sporadic cases Here, we reported a novel frameshift mutation of FLNA gene in a Chinese family with PNH.展开更多
基金the National Natural Science Foundation of China(No.81727805 and No.82001854)the Shenzhen Science and Technology(No.SGDX20190917094601717 and No.JCYJ20210324141216040).
文摘Objective The influence of the coronary artery anatomy on the prognosis of patients receiving an arterial switch operation(ASO)is currently controversial,and the risk factors for this operation may change in more complicated patients.This study aimed to investigate the influence of coronary artery anomalies on the in-hospital and post-discharge outcomes of ASO in patients with transposition of the great arteries(TGA)and Taussig-Bing anomaly(TBA).Methods We retrospectively reviewed 206 patients who underwent ASO from January 2007 to December 2019.The median age at operation was 33[interquartile range(IQR):20–71]days.Median follow-up time was 7.2 years(IQR:4.0–10.3 years).Results Coronary anomalies were present in 86 patients(41.7%),with 9(4.4%)of them having a single coronary artery.Additional coronary features included intramural courses in 5(2.4%)patients,ostial stenosis in 1(0.5%)patient,and accessory coronary artery orifices in 5(2.4%)patients.There were 32(15.5%)in-hospital deaths and 8(4.6%)post-discharge deaths,yielding an overall survival of 81.3%,80.7%and 79.9%at 1,5 and 10 years,respectively.Mortality due to ASO has been drastically decreased since 2013.Patients with a single coronary artery had higher rate of in-hospital mortality,but this finding was not statistically significant.The earlier surgical era(OR:2.756)and a longer cardiopulmonary bypass time(OR:2.336)were significantly associated with in-hospital mortality,while coronary patterns were not.An intramural coronary artery(HR:10.034)and a patient age of older than 1 year at the time of ASO(HR:9.706)were independent predictors of post-discharge mortality.Conclusion ASO remains the procedure of choice for TGA with coronary anomalies with acceptable in-hospital and post-discharge outcomes in terms of overall survival and freedom of reoperation.However,intramural coronary artery is an independent risk factor for post-discharge mortality.Timely surgery within the 1st year of life helps improve overall midterm survival of ASO.
文摘To the Editor: Periventricular nodular heterotopias (PNHs) represent a malformation of cortical development caused by an improper neuronal migration during forebrain formation, There is a wide spectrum of anatomical and clinical presentations of PNH, ranging from asymptomatic small unilateral or bilateral nodules to extensive agglomerates of heterotopia lining the lateral ventricles with epilepsy and intellectual disabilities. The mutations in X-linked gene, filamin A (FLNA) gene, were identified in approximately 100% of familial PNHs and 25% of sporadic cases Here, we reported a novel frameshift mutation of FLNA gene in a Chinese family with PNH.