Predictors of operability in children with severe pulmonary hypertension associated with congenital heart disease / 中华医学杂志(英文版)
Chinese Medical Journal
;
(24): 811-818, 2019.
Article
in English
| WPRIM
| ID: wpr-774805
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Pathology
/
Prognosis
/
General Surgery
/
Echocardiography
/
Retrospective Studies
/
Risk Factors
/
ROC Curve
/
Kaplan-Meier Estimate
/
Heart Defects, Congenital
/
Hypertension, Pulmonary
Type of study:
Etiology study
/
Observational study
/
Prognostic study
/
Risk factors
Limits:
Adolescent
/
Child
/
Child, preschool
/
Female
/
Humans
/
Male
Language:
English
Journal:
Chinese Medical Journal
Year:
2019
Type:
Article
Similar
MEDLINE
...
LILACS
LIS