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Background: Data regarding the prognosis of Eisenmenger syndrome (ES) and effect of targeted drugs are limited. This study aimed to analyze the prognosis and impact of targeted drug therapy on the survival rate of patients with ES in the Chinese population., Methods: The data of patients with ES referred to our hospital between January 2010 and December 2020 were retrospectively analyzed. Data included baseline demographics, echocardiographic parameters, and clinical diagnoses. All patients were followed up via telephone interviews in February 2022. The primary endpoint was mortality., Results: Overall, 1,021 patients with ES were included. The 1-, 3-, 5-, 7-, 10-, and 12-year survival rates were 91.6%, 84.2%, 80.7%, 73.8%, 71.4%, and 69.9%, respectively. Patients with atrial septal defects had the best prognosis than those with ventricular septal defects, patent ductus arteriosus, and complex congenital heart disease (CHD) (P < 0.0001). Patients who visited between 2016 and 2020 received increased targeted drug therapy and had a better prognosis than those who visited between 2010 and 2015 (all P < 0.05). Cox regression analysis revealed age, pulmonary arterial systolic pressure, post-tricuspid shunt CHD, targeted drugs, and year of the first hospital visit to be predictors of death (P < 0.05)., Conclusions: Survival rates associated with an increased use of combined targeted drugs significantly improved in patients with ES. However, numerous factors that predict increased mortality remain to be elucidated., (Copyright © 2023 Hellenic Society of Cardiology. Published by Elsevier B.V. All rights reserved.)

Aims: This study aimed to provide a simple index for predicting the definitive indication for transcatheter closure of atrial septal defect (ASD) with pulmonary arterial hypertension (PAH)., Methods and Results: A positive response after attempted occlusion was defined as mean pulmonary artery pressure (MPAP) ≤30 mmHg or the decrement percentage of it ≥20% compared with baseline. If a positive response was achieved, the occluder would be released, and the procedure was defined as successful. In 209 patients who underwent a successful procedure without PAH-specific medicine, there was a dramatic decrease in the percentage of patients with pulmonary arterial systolic pressure (PASP) ≥50 mmHg from baseline to the one-year follow-up (79.4% to 14.0%, p<0.001). The optimal cut-off value of MPAP to predict a positive response without PAH-specific medicine was 35.0 mmHg, with an area under the curve (AUC) of 0.919 (p<0.001). Administration of inhaled iloprost extended the cut-off point to 50.0 mmHg to reach a positive response, with an AUC of 0.774 (p=0.003)., Conclusions: This large-scale study indicated that MPAP could be a simple but powerful index to predict benefit from closure in adult ASD patients with PAH.

Background: Recently, assessment of left ventricular (LV) torsional deformation has become an important approach for quantifying LV function. This study sought to evaluate LV torsion using speckle tracking imaging in patients with atrial septal defect (ASD)., Methods and Results: Basal and apical LV short-axis images were analyzed in 45 asymptomatic adults with isolated secundum ASD and 45 matched normal subjects. The apical rotation parameters were similar between 2 groups; however, the peak basal clockwise rotation was significantly depressed (-5.4 +/-2.8 degrees vs -6.9 +/-2.6 degrees , P<0.001) and time to the peak was significantly delayed (118.3 +/-18.8% vs 96.1 +/-12.6% of systolic period, P<0.001) in patients with ASD. The peak basal initial counterclockwise rotation in the ASD group was significantly higher (5.1 +/-3.3 degrees vs 1.8 +/-1.4 degrees , P<0.001) and longer (75.4 +/-26.7% vs 42.5 +/-24.4% of systolic period, P<0.001) than that in the control group. LV peak twist was also reduced significantly in patients with ASD (11.9 +/-5.9 degrees vs 14.6 +/-3.5 degrees , P<0.05) in comparison with the controls., Conclusions: LV systolic twist was significantly reduced in patients with ASD mainly because of the heterogeneous basal rotation.

Left ventricular (LV) torsional deformation plays an important role with respect to LV ejection and filling. However, no data are available on the impact of overload relief on LV torsional deformation after transcatheter ASD closure. This study sought to evaluate LV twist and untwisting before and early after device closure of ASD using the speckle tracking imaging (STI). We acquired basal and apical LV short-axis ultrasound images in 30 asymptomatic patients (29 +/- 9 years, 9 males) scheduled for percutaneous closure of an ASD before and 1-day after transcatheter ASD closure. All data were offline analyzed with Echopac 7.0 software. After transcatheter ASD closure, there was no significant difference in peak apical rotation and time to the peak (P > 0.05 for both). However, a significantly improved basal rotation was recorded, including significantly increased peak clockwise rotation (-7.1 +/- 3.2 degrees vs. -5.4 +/- 2.9 degrees , P = 0.014), decreased initial counterclockwise rotation (2.0 +/- 1.8 degrees vs. 5.1 +/- 3.2 degrees , P < 0.001) and shortened time to peak clockwise rotation (105.5 +/- 16.5% vs. 118.0 +/- 18.5% of systolic period, P = 0.001). LV twist was significantly improved in patients with ASD after the device closure (16.1 +/- 6.7 degrees vs. 12.2 +/- 6.3 degrees , P = 0.001), whereas there was no significant difference in peak untwisting rate, time to the peak and untwisting during IVRT (P > 0.05 for all). In conclusion, LV systolic twist could be significantly improved but diastolic untwisting remained unchanged after transcatheter ASD closure. This improvement was mainly attributed to the improved LV basal rotation rather than the unchanged apical rotation.