A Correlative Study of Spirometric Parameters and Markers of Right Ventricular Dysfunction in Pediatric Patients with Pulmonary Arterial Hypertension

Abstract

Background: Alteration in lung function is present in children with pulmonary arterial hypertension (PAH). We aimed to study the pulmonary function indices obtained by spirometry in pediatric patients with PAH, and to correlate them with B-type natriuretic peptide (BNP) and echocardiographic parameters. Methods: Thirty-one children with PAH were enrolled in this study, of those 26 patients (83.87%) associated shunt defect and 5 patients (16.12%) were diagnosed with idiopathic PAH. Forced vital capacity (FVC), forced expiratory volume in one second (FEV1), FEV1/FVC, peak expiratory flow rate (PEFR), forced expiratory flow at 25–75% of the pulmonary volume (FEF 25–75) were evaluated by spirometry and correlated with BNP and echocardiographic parameters that reflect right ventricle function. Results: Restrictive pattern of pulmonary function was present in 51.6% (n = 16) of all PAH children. There were significant correlations between BNP with FVC (p = 0.001), FEV1 (p = 0.001), and FEV1/FVC (p = 0.001). Serum BNP level was significantly increased in the group of patients without shunt. Of those echocardiographic parameters that reflect right ventricle function, we found that TAPSE significantly correlated with PEFR (r = −0.47, p <0.01), and with FEF 25–75 (r = −0.39, p <0.01). Conclusions: Deterioration of the pulmonary function indices are correlated with BNP and echocardiographic parameters, markers of RV dysfunction. Being easy and reliable tests, pulmonary functions can be introduced among the follow-up tools in children with PAH.

Biomarkers and Risk Assessment — an Interdisciplinary Approach

“…This study used BNP as a measure of ventricular dysfunction, and proved that spyrometry-derived parameters such as forced vital capacity, forced expiratory volume in one second, peak expiratory flow rate and forced expiratory flow at 25-75% of the pulmonary volume are good indicators of the severity of right ventricular failure, correlating well with validated serum or imaging biomarkers such as BNP (p <0.0001) or echocardiographic parameters reflecting right ventricular function (p = 0.01). 7 At the same time, in another study carried out by Muntean et al, published in the current issue of this journal, echocardiography-derived parameters characterizing left ventricular function, such as longitudinal systolic strain and synchronicity index were positively correlated with the severity of pulmonary arterial hypertension in children (p <0.0001), proving the role of imaging-derived biomarkers for assessing ventricular function. 8 These two studies published in this issue by Șuteu et al and Muntean et al succeed to prove that beyond the traditional serum biomarkers, there is a significant role for more complex biomarkers, such as those provided by imaging techniques, for assessing the evolution of patients with left or right heart failure of different etiologies.…”