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Abstract and Introduction
Abstract
Recent reports from pulmonary arterial hypertension (PAH) registries suggest that the mean age at diagnosis is increasing, outlining a growing proportion of elderly male patients. As a consequence, the classical description of the disease is shifting and may no more be described as a rare disease typically affecting young women. Potential explanations of this changing picture may include an aging of populations in western countries, the increase in life expectancy and the growing awareness of PAH and emergence of potential efficient treatments. Diagnostic workup of severe pulmonary hypertension (PH) in the elderly should be performed in such a way as to discriminate between the expected consequences of aging, pulmonary vascular disease, and other frequent causes of secondary PH (left heart failure or lung disease). Careful exploration by right heart catheterization is mandatory, but special attention should be paid to several pitfalls specific to this procedure in this age group. This is a matter of concern as clinical trials that aim to study new specific drug therapy for PAH might be biased by the inclusion of misdiagnosed patients. The aim of this review is to highlight the main difficulties in diagnosing PAH in the elderly and to propose a practical approach to distinguish PAH from the other frequent causes of PH in this population.
Introduction
Pulmonary hypertension (PH) is a hemodynamic and pathophysiological condition defined by an increase in mean pulmonary artery pressure (mPAP) ≥ 25 mm Hg at rest as assessed by right heart catheterization (RHC). It is the consequence of an increasing pulmonary vascular resistance (PVR) that leads to right ventricular (RV) overload, hypertrophy, dilatation, reduction of cardiac output, and eventually to RV failure and death. PH is considered precapillary or postcapillary depending on the pulmonary artery wedge pressure (PAWP) measured lower or higher than 15 mm Hg, respectively. This can be associated with multiple clinical conditions forming different etiological groups with specific and diverse pathological and pathobiological characteristics. The first group (Group 1) of the current classification describes an insidiously progressive vasculopathy leading to a precapillary PH named pulmonary arterial hypertension (PAH). It is characterized by an intense and characteristic remodeling of small caliber pulmonary arteries, due to the proliferation of endothelial and smooth muscle cells and medial hypertrophy. PAH is a rare disease that is classically described as affecting young adults. However, recent data from several registries have shown that the mean age of patients with PAH is increasing in a growing proportion of elderly patients. This population has an increased likelihood of comorbidities or physiological age-related changes that may make PAH diagnosis challenging. These confounding factors must be carefully taken in account either before excluding or confirming PAH.
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