CHOOSING WISELY Pulmonary Arterial Hypertension: redefining current screening, follow-up and prognostic procedures in patients with Pulmonary Arterial Hypertension (DSB.AD002.075)

Thematic area

Biomedical sciences

Project area

Structure responsible for the research project

Institute of clinical physiology (IFC)

Project manager

LUNA GARGANI
Phone number: 050 3153229
Email: luna.gargani@ifc.cnr.it

Abstract

Pulmonary arterial hypertension (PAH) is a clinical condition characterized by progressive vascular remodeling with
increased pulmonary vascular resistance and right ventricular overload that may ultimately lead to right heart failure and death (1).
Several clinical conditions are known to carry a high risk of developing PAH, such as connective tissue disease (CTD), HIV infection, Portal Hypertension and Congenital Heart Disease (2). Guidelines suggest to perform a yearly screening of patients carrying an intermediate/high risk to develop PAH (1). However, this recommendation is supported only by a level of evidence C (1). Moreover, the current suggested screening algorithm owes several limitations: 1) it is based on symptoms, sometimes very mild and non-specific (3) 2) echocardiography is performed only at rest, that may lead to false negative (4) 3) In patients with CTD, coexisting organ involvement such as interstitial lung disease makes the diagnosis of PAH even more challenging (3).
Furthermore, an early PAH diagnosis is of utmost importance considering that the earlier the treatment is implemented, the better is the prognosis (5,6).

Start date of activity

02/01/2019

Keywords

Pulmonary Arterial Hypertension

Last update: 26/08/2025