Pulmonary arterial hypertension: guidelines and unmet clinical needs

Abstract

The term pulmonary arterial hypertension (PAH) identifies a heterogeneous group of diseases characterized by a progressive increase in pulmonary arterial resistance (PVR), which causes a significant burden in terms of quality of life, right heart failure and premature death. The pathogenesis of PAH is not completely clear: the remodeling of the small pulmonary vessels is crucial, causing an increase in the resistance of the pulmonary circle. Its diagnosis is based on cardiac catheterization of the right heart. According to the present hemodynamic definition of pulmonary hypertension (PH) proposed by the Guidelines of the European Society of Cardiology/European Respiratory Society (ESC-ERS), the mean pulmonary arterial pressure (mPAP) values are ≥25 mmHg. In case of PAH, apart from an mPAP value ≥25 mmHg, patients must have a >3 Wood units increase in PVR and normal pressure values of the left heart. PH is a pathophysiological condition observed in more than 40 different diseases, while PAH is a primary disease of the pulmonary bloodstream potentially treatable with specific drugs. PAH is a severe complication of systemic sclerosis (SSc) affecting about 10% of the patients. Due to the devastating nature of SSc-PAH, there is a clear need to systematically adopt appropriate screening programs. In fact, despite awareness of the negative impact of SSc-PAH on quality of life and survival, as well as on the severity of lung function, at the moment standardized and shared guidelines and/or screening programs for the diagnosis and the subsequent early treatment of PAH in SSc are not available. The aim of the present paper is to highlight the lights and shadows of SSc-PAH, unraveling the unmet clinical needs on this topic with a proposal of clinical-diagnostic and therapeutic guidelines.

Primary systemic sclerosis heart involvement: A systematic literature review and preliminary data-driven, consensus-based WSF/HFA definition

“…6 The prevalence of clinical SSc primary heart involvement (SSc-pHI) is still unclear, as most studies have not sought to distinguish primary from secondary heart involvement, due to other SSc-related (i.e. renal, pulmonary vascular or pulmonary parenchymal disease) 7–11 or non-SSc related condition (i.e. ischemic heart disease).…”

Novel Prognostic Predictor for Primary Pulmonary Hypertension: Focus on Blood Urea Nitrogen

“…PAH is a life-threatening disease associated with increased mortality regardless of the classification and underlying etiology ( 13 , 14 ). Moreover, PAH is a progressive pulmonary circulatory disease characterized by vascular remodeling followed by increased pulmonary vascular resistance (PVR) and pulmonary artery pressure (PAP) that can lead to right ventricular (RV) overload and failure, ultimately resulting in premature death ( 15 ). The survival rate of PAH is between 68 and 93% at 1 year and 39 and 77% at 3 years ( 13 , 16 ).…”

An Overview of Different Techniques for Improving the Treatment of Pulmonary Hypertension Secondary in Systemic Sclerosis Patients

“…The use of algorithms within SSc-PAH screening is associated with a better outcome. Here, we describe three (Table 2) of the most widely used algorithms for PAH screening in SSc [37,[53][54][55][56][57][58][59][60].…”

“…If either or both parameters (step A) are present patients must then undergo echocardiography associated with other confirmatory tests like HRTC and 6 MWT (step B). If there is no parenchyma lung involvement, a RHC should be performed on the patient [19,37,[53][54][55][56][57][58][59].…”