Abstract
Background: Reversibility of pulmonary hypertension (PH) is closely related to the treatment options for and prognosis of children with congenital heart disease. Objective: We combined patient-specific clinical features including diagnosis, age and echocardiographic results, and biomarkers of pulmonary vascular dysfunction to explore the noninvasive methods that can be used to accurately evaluate the reversibility of pulmonary hypertension in congenital heart disease (PH-CHD). Methods: Based on the preoperative systolic pulmonary arterial pressure (sPAP), 70 CHD patients were divided into normal, PH-CHD suspected, and confirmed groups. Additionally, biomarkers of circulating endothelial cells (CECs), endothelin-1 (ET-1), and endothelial nitric oxide synthase (eNOS) were detected. Patients were categorized into reversible (RPH) and irreversible (IRPH) groups according to the sPAP 6 months after surgery. Risk stratification was performed according to the clinical features and biomarkers. Results: CECs and ET-1 levels in the confirmed group were significantly higher. eNOS was higher in the confirmed and suspected groups than that in the normal group. CECs in the IRPH group were significantly higher compared to the RPH group. No such intergroup differences were observed with respect to ET-1 and eNOS levels. The ROC curve showed that the risk stratification was of high diagnostic value to evaluate reversibility. Conclusion: The CECs, eNOS, and ET-1 were closely related with PH-CHD. CECs and risk stratification have high practical value in assessing the reversibility of PH-CHD.
Highlights
Pulmonary hypertension in congenital heart disease (PH-CHD) is a severe condition threatening children’s physical and mental health
In the irreversible pulmonary hypertension (IRPH) group, four patients were diagnosed with functional single ventricle (FSV), three with ventricular septal defect (VSD), two with double outlet right ventricle (DORV), two with truncus arteriosus, two with atrioventricular septal defect (CAVSD), and one with complete anomalous pulmonary vein connection (TAPVC)
The results indicated that the combination of clinical features and circulating endothelial cells (CECs) for risk stratification may be a potentially valuable tool to noninvasively evaluate the reversibility of pulmonary hypertension (PH)-CHD
Summary
Pulmonary hypertension in congenital heart disease (PH-CHD) is a severe condition threatening children’s physical and mental health. Irreversible pulmonary vascular remodeling occurs, with significantly increased PAP and PVR and decreased pulmonary blood flow, referred to as Eisenmenger syndrome. Structural and functional assessments of the pulmonary vascular endothelium have become important noninvasive methods of evaluating the reversibility of PH-CHD. Reversibility of pulmonary hypertension (PH) is closely related to the treatment options for and prognosis of children with congenital heart disease. Objective: We combined patient-specific clinical features including diagnosis, age and echocardiographic results, and biomarkers of pulmonary vascular dysfunction to explore the noninvasive methods that can be used to accurately evaluate the reversibility of pulmonary hypertension in congenital heart disease (PH-CHD). CECs in the IRPH group were significantly higher compared to the RPH group No such intergroup differences were observed with respect to ET-1 and eNOS levels. CECs and risk stratification have high practical value in assessing the reversibility of PH-CHD
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