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Background: Pulmonary artery hypertension associated with congenital heart disease (PAH-CHD) occurs predominantly among patients with uncorrected CHD. Treatment of severe pediatric PAH-CHD remains a major intractability. This study evaluated the predictors and prognoses of children with PAH-CHD who underwent surgical correction., Methods: The data for 59 children with severe PAH-CHD who underwent surgical correction, with or without postoperative medication, between May 2011 and June 2015 at the Guangdong Provincial People's Hospital were analyzed retrospectively. A regression analysis, receiver-operating characteristic (ROC) curves, and Kaplan-Meier curves were used for survival analysis., Results: Fifty-nine children with severe PAH-CHD underwent heart catheterization and correction, with or without specific anti-PAH drugs postoperatively, were included in this study. The pulmonary pressure, heart function, and ending events were observed and median observation period was 49 ± 20 months. Twenty-eight patients (50%) received at least one additional anti-PAH drug after correction. The survival rate after 2 years was 91.5% (54/59); two patients were in a critical condition, and three were lost to follow-up. Twelve patients (29%) still received over one additional PAH-specific therapy at follow-up, whereas 42 (75%) had successfully stopped drug treatment. Two patients (3.5%) died and one underwent a second thoracotomy to remove the ventricular septal defect patch. Acute vasoreactivity test (AVT) criteria had limited efficacy in predicting pediatric PAH-CHD, whereas pulmonary vascular resistance (PVR) ≤ 6.65 Wood units (WU)/m or PVR/systemic vascular resistance (SVR) ≤ 0.39 during AVT indicated a good prognosis after surgical correction with an AUC of 98.3% (95% confidence interval [CI]: 96.0-100%), 98.4% (95% CI: 96.0-100%) sensitivity of 100%, 100% and specificity of 82.1%, 92.9%, respectively., Conclusions: Although the criteria for positive AVT currently used are unsuitable for pediatric patients with PAH-CHD, PVR and PVR/SVR during AVT are excellent predictors of outcome in pediatric PAH-CHD. Surgery aided by anti-PAH drugs is an effective strategy and should be recommended for severe pediatric PAH-CHD with PVR ≤ 6.65 WU/m and PVR/SVR ≤ 0.39 after iloprost aerosol inhalation.

Objective: To report the results of transcatheter interventional therapy (TIT) of congenital heart disease (CHD) register from 23 medical centers in China., Method: In this retrospective multicenter registry study, clinical data from 5808 patients who underwent TIT between January 2008 to December 2010 in 23 Chinese medical centers in 14 cities were analyzed., Results: Procedure was successful in 5720 cases (98.5%), success rate was 99.5% for PDA, 98.8% for ASD, 97.4% for VSD and 98.5% for pulmonary stenosis (PS). Multivariate regression analysis showed that PDA size and procedure time, age and procedure time, distance from VSD to AV were significantly associated with the procedure success rate of PDA, ASD and VSD closure, respectively. Early complications occurred in 306 cases (5.3%), 36 cases (0.6%) experienced major complications including device embolization in 7 cases, serious aorta regurgitation in 5 cases, serious tricuspid regurgitation in 4 cases, tricuspid stenosis in 2 cases, heart block (HB) in 13 cases (2 in ASD and 11 in VSD), cardiac tamponade in 2 cases (1 ASD and 1 PS) and hemolysis in 3 cases. Procedure time and PDA size, ASD size, device size, age and PS degree were risk factors related to the occurrence of the early complications for PDA, ASD and VSD closure and PBPV respectively. The median follow-up time was 15 months (range 1-36 months). The complete closure rate during follow up was 100% for ASD, PDA and VSD and the pressure gradient in PS decreased to normal range in all PS patients. Late complications occurred in 15 cases (0.2%), of which 3 cases needed surgery intervention and permanent pacemaker was implanted in 1 patient. There was no death during procedure and at follow-up period., Conclusions: TIT of CHD offers encouraging results in China. Follow up is warranted to monitor the occurrence of serious complications, especially late complications.

Objective: To evaluate the efficacy of interventional therapy for complex congenital heart defects patients with un-repaired or postoperative residual lesions., Methods: Between March 1998 and April 2009, 42 patients (12 females), mean age 6 years (5 months to 30 years) received interventional therapy, 17 cases underwent occlusion of major aorto-pulmonary collateral arteries (MAPCAs), 15 underwent device closure of residual ventricular or atrial septal shunting, 12 underwent balloon angioplasty (n = 10) and stenting (n = 2) for stenosis of the anastomosis of vessels or branched pulmonary arteries., Results: Twenty-three MAPCAs were performed in 17 patients without residual shunting. One patient died of multiple organ failure after intervention therapy and the remaining patients discharged without complication, successful device closure was performed in 15 patients and there was minimal residual shunting in 1 patient. There were no severe arrhythmias such as complete atrio-ventricular block during and post procedure. Exercise capacities were significantly improved in 12 patients underwent balloon angioplasty or stenting. Pressure gradients were significantly decreased and there was no aneurysmal or thromboembolic formation post procedure., Conclusions: Interventional therapy is a safe and effective therapy option for treating complex congenital heart defects patients with un-repaired or postoperative residual lesions.