Objective:To evaluate the electrophysiological characteristics and radiofrequency catheter ablation of atrial tachycardia (AT) in patients with repaired atrial septal defects(ASD). Methods: In 76 consecutive patients ...Objective:To evaluate the electrophysiological characteristics and radiofrequency catheter ablation of atrial tachycardia (AT) in patients with repaired atrial septal defects(ASD). Methods: In 76 consecutive patients with AT who underwent the electrophysiological study and radiofrequency catheter ablation (RFCA). 4 patients (one male and three female aged 35. 5±11. 5 years) had AT-related myocardial scar or incision. Earliest activation combined with entrainment mapping was adopted to determine a critical isthmus. Results: Re-entry related to the lateral atriotomy scar was inducible in 3 of 4 patients. With en-trainment mapping, the PPI-TCL difference was <30 ms when pacing at the inferior margins of the right lateral atriotomy scar. Among them, 2 patients had successful linear ablation between scar area to inferior vena cava, and 1 patient between scar areas to tricuspid annulus. Re-entry involving an ASD patch was demonstrated in 1 of 4 patients. PPI-TCL differences <30 ms were found when entraining tachycardia at sites near the septal patch. But linear ablation failed in terminating AT. There was no complication during procedure. No recurrence of incision-related AT was found during follow-up except for the failed patient. Conclusion: Under conventional electrophysiological mapping, adopting linear ablation from scar area to anatomic barrier, successful ablation also can be obtained in patients with IRAT related to myocardial scar or incision.展开更多
Congenital heart disease （CHD） is the leading cause of birth defects, and its etiology is not completely understood. Atrial septal defect （ASD） is one of the most common defects of CHD. Previous studies have demon...Congenital heart disease （CHD） is the leading cause of birth defects, and its etiology is not completely understood. Atrial septal defect （ASD） is one of the most common defects of CHD. Previous studies have demonstrated that mutations in the transcription factor T-box 20 （TBX20） contribute to congenital ASD. Whole-exome sequencing in combination with a CHD-related gene filter was used to detect a family of three generations with ASD. A novel TBX20 mutation, c.526G〉A （p.D176N）, was identified and co-segregated in all affected members in this family. This mutation was predicted to be deleterious by bioinformatics programs （SIFT, Polyphen2, and MutationTaster）. This mutation was also not presented in the current Single Nucleotide Polymorphism Database （dbSNP） or National Heart, Lung, and Blood Institute （NHLBI） Exome Sequencing Project （ESP）. In conclusion, our finding expands the spectrum of TBX20 mutations and provides additional support that TBX20 plays important roles in cardiac development. Our study also provided a new and cost-effective analysis strategy for the genetic study in small CHD pedigree.展开更多
文摘Objective:To evaluate the electrophysiological characteristics and radiofrequency catheter ablation of atrial tachycardia (AT) in patients with repaired atrial septal defects(ASD). Methods: In 76 consecutive patients with AT who underwent the electrophysiological study and radiofrequency catheter ablation (RFCA). 4 patients (one male and three female aged 35. 5±11. 5 years) had AT-related myocardial scar or incision. Earliest activation combined with entrainment mapping was adopted to determine a critical isthmus. Results: Re-entry related to the lateral atriotomy scar was inducible in 3 of 4 patients. With en-trainment mapping, the PPI-TCL difference was <30 ms when pacing at the inferior margins of the right lateral atriotomy scar. Among them, 2 patients had successful linear ablation between scar area to inferior vena cava, and 1 patient between scar areas to tricuspid annulus. Re-entry involving an ASD patch was demonstrated in 1 of 4 patients. PPI-TCL differences <30 ms were found when entraining tachycardia at sites near the septal patch. But linear ablation failed in terminating AT. There was no complication during procedure. No recurrence of incision-related AT was found during follow-up except for the failed patient. Conclusion: Under conventional electrophysiological mapping, adopting linear ablation from scar area to anatomic barrier, successful ablation also can be obtained in patients with IRAT related to myocardial scar or incision.
基金Project supported by the National Natural Science Foundation of China （Nos. 81370204, 81300072, and 81101475） Electronic supplementary materials： The online version of this article （htlp：//dx.doi.org/10.1631/jzus.B1400062） contains supplementary materials, which are available to authorized users
文摘Congenital heart disease （CHD） is the leading cause of birth defects, and its etiology is not completely understood. Atrial septal defect （ASD） is one of the most common defects of CHD. Previous studies have demonstrated that mutations in the transcription factor T-box 20 （TBX20） contribute to congenital ASD. Whole-exome sequencing in combination with a CHD-related gene filter was used to detect a family of three generations with ASD. A novel TBX20 mutation, c.526G〉A （p.D176N）, was identified and co-segregated in all affected members in this family. This mutation was predicted to be deleterious by bioinformatics programs （SIFT, Polyphen2, and MutationTaster）. This mutation was also not presented in the current Single Nucleotide Polymorphism Database （dbSNP） or National Heart, Lung, and Blood Institute （NHLBI） Exome Sequencing Project （ESP）. In conclusion, our finding expands the spectrum of TBX20 mutations and provides additional support that TBX20 plays important roles in cardiac development. Our study also provided a new and cost-effective analysis strategy for the genetic study in small CHD pedigree.
