Predictors of operability in children with severe pulmonary hypertension associated with congenital heart disease.
10.1097/CM9.0000000000000145
- Author:
Shi-Bing XI
1
;
Shu-Shui WANG
;
Ming-Yang QIAN
;
Yu-Mei XIE
;
Jun-Jie LI
;
Zhi-Wei ZHANG
Author Information
1. Department of Pediatric Cardiology, Guangdong Cardiovascular Institute, Guangdong Academy of Medical Science/Guangdong Provincial People's Hospital, Guangzhou, Guangdong 510080, China.
- Publication Type:Journal Article
- MeSH:
Adolescent;
Child;
Child, Preschool;
Echocardiography;
Female;
Heart Defects, Congenital;
pathology;
surgery;
Humans;
Hypertension, Pulmonary;
pathology;
surgery;
Kaplan-Meier Estimate;
Male;
Prognosis;
ROC Curve;
Retrospective Studies;
Risk Factors
- From:
Chinese Medical Journal
2019;132(7):811-818
- CountryChina
- Language:English
-
Abstract:
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.
