Abstract

Abstract Background despite therapeutic improvement, pulmonary arterial hypertension (PAH) is still a chronic, incurable, high mortality disease. Several clinical, laboratory, hemodynamic and instrumental prognostic factors have been already established in PAH patients. However, no electrocardiographic prognostic factors have been identified so far in a systematic multivariate analysis together with clinical, exercise and haemodynamic parameters. Methods patients with idiopathic/heritable/drug-induced PAH (I/H/D-PAH) referred to a single centre were included from 1993 to 2021. All patients underwent baseline clinical, 12-lead ECG, hemodynamic with vasoreactivity test, 6 minutes walking distance (6MWD), brain natriuretic peptide (BNP) or N-terminal pro-BNP (NT-proBNP), WHO functional class evaluation and risk stratification (according to a recently validated simplified version of 2015 ESC/ERS guidelines’ risk table). All cause mortality has been considered to identify the predictors of mortality using univariate and multivariate Cox regression analysis. Results 570 PAH patients were included: 448 idiopathic (79%), 105 heritable (18%) and 17 drug-induced (3.0%). 20 patients were on atrial flutter/fibrillation at baseline. Considering only electrocardiographic parameters the variables independently associated with prognosis are shown in the table below: Taking into account also the risk stratification the independent prognostic variables are: Considering also clinical, exercise and haemodynamic variables the independent prognostic variables are: Conclusions in patients with I/H/D-PAH heart rate is a prognostic factor independently from other electrocardiographic, clinical, exercise and haemodynamic parameters as reflects the attempt to preserve the cardiac output in patients with an increased right ventricular afterload causing a reduced stroke volume; both PR interval prolongation, marker of right atrial overload, and QTc prolongation, marker of right ventricle's electrical remodeling, are prognostic variables independently from baseline patient's risk level; right bundle branch block, expression of right ventricular electrical remodeling, has proved to be an independent predictor of mortality merely if we consider only the electrocardiographic parameters.

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