Clinical characteristics of 42 children with focal atrial tachycardia originated from the right atrial appendage / 中华儿科杂志

Objective: To investigate the feature and treatment of atrial tachycardia (AT) originated from right atrial appendage (RAA) in children. Methods: The data of 42 children with AT originated from RAA, who were admitted the First Hospital of Tsinghua University from January 2010 to September 2022 were analyzed retrospectively.The clinical characteristics, treatment and efficacy were analyzed. The children were divided into tachycardia cardiomyopathy group and normal cardiac function group. The differences in the ablation age and the heart rate during AT between two groups were compared by independent sample t-test. Results: Among 42 children, there were 20 males and 22 females. The age of onset was 2.7 (0.6, 5.1) years. Their age at radiofrequency ablation was (6.5±3.6) years, and the weight was (23.4±10.0) kg. Thirty-two children (76%) had sustained AT. The incidence of tachycardia cardiomyopathy was 43% (18/42). Compared to that of the normal cardiac function group, the ablation age and the heart rate at atrial tachycardia of the tachycardia cardiomyopathy group were higher ((8.1±3.8) vs. (5.3±3.1) years, t=-2.63, P=0.012; (173±41) vs. (150±30) beats per minute, t=-2.05, P=0.047. Thirty-eight children (90%) responded poorly to two or more antiarrhythmic drugs. The immediate success rate of radiofrequency ablation (RFCA) was 57% (24/42), and the AT recurrence rate was 17% (4/24). Twenty-two children underwent RAA resection, and their AT were all converted to sinus rhythm after the surgery. During the RAA resection, 10 cases of right atrial appendage aneurysm were found, 9/18 of which failed the RFCA. Conclusions: The AT originated from the RAA in children tend to present with sustained AT, respond poorly to antiarrhythmic drugs, and has a low success rate of RFCA as well as high recurrence rate. Resection of the RAA is a safe and effective complementary treatment.

Long-term outcome of 48 unoperated adult patients with Ebstein's anomaly / 中华心血管病杂志

Objective: To analyze the long-term outcome of unoperated Ebstein's anomaly (EA) patients aged over 18 years, and to evaluate the related factor of outcomes. Methods: The data of 48 unoperated EA patients from March 2004 to December 2008 in the First Hospital of Tsinghua University, were analyzed. The clinical data of the patients were collected, and patients received regular echocardiography, ECG and chest X-ray examinations. Septal leaflet attachment ratio (SLAr) was calculated based on transthoracic echocardiography imagines. The patients were divided into 3 groups according to SLAr: SLAr<0.45 (n=18), 0.45≤SLAr≤0.60 (n=21) and SLAr>0.60 (n=9). Chest X-ray was used for measurement of cardiothoracic ratio (CTR). Kaplan Meier survival curve was used to calculate the long-term survival rate. Cox proportional hazards regression model was used to analyze the influencing factors of death. Results: There were 19 males, and the mean age at diagnosis was (21.3±11.1) years. Forty-two patients (87.5%) were complicated with arrhythmia, including W-P-W syndrome (n=4), supraventricular tachycardia (n=16), right bundle branch block (n=37), and atrial fibrillation (n=2). The mean duration of follow-up was (148.8±16.8) months, the follow-up rate was 100% with no loss-to-follow up. Nine cases (18.8%) died during follow-up: 6 cases (12.5%) died of cardiac origin, including 3 cases of heart failure, 1 case of arrhythmia, and 2 cases of sudden death; 1 case died of accident; 2 cases died from unknown causes. During the follow-up period, the survival rates were 17/18, 19/21 (90.5%) and 3/9 in the SLAr<0.45, 0.45≤SLAr≤0.60 and SLAr>0.60 group, respectively. According to Kaplan-Meier survival curve, the 5-year survival rates among the three groups were 100%, 100% and 78%, respectively. The 10-year survival rates among the three groups were 94%, 95% and 44%, respectively. Decreased activity tolerance and heart failure were found in 7 patients (6 patients in SLAr>0.60 group and 1 patient in 0.45≤SLAr≤0.60 group). Two patients had cerebrovascular embolism. There were 3 cases with tachyarrhythmia lasting more than 24 hours. Cox regression analysis showed that the risk of death was higher in patients with SLAr>0.60 than in patients with SLAr<0.45 (HR=12.375, 95%CI 1.692-22.146, P=0.015); the risk of death in patients with CTR≥0.65 was 1.306 times higher than that in patients with CTR<0.65 (HR=1.306, 95%CI 0.417-12.754, P=0.038). Conclusions: EA patients often combines with arrhythmia. For unoperated EA patients, SLAr>0.60 and CTR≥0.65 are risk factors of death. EA patients with arrhythmia should be actively treated with drugs or radiofrequency ablation.