The impact of cardiovascular comorbidities associated with risk for left heart disease on idiopathic pulmonary arterial hypertension: Data from the Hellenic Pulmonary Hypertension Registry (HOPE)

Abstract

Whereas younger female patients were diagnosed with idiopathic pulmonary arterial hypertension (IPAH) in 1980s, it is now frequently encountered in elderly patients with cardiovascular comorbidities (CVCs) associated with increased risk for left heart disease. We present data until November 2019 regarding specific features and clinical outcomes of IPAH population from the Hellenic Pulmonary Hypertension Registry (HOPE). Patients were divided into two groups based on the presence of ≥ or <3 CVCs, arterial hypertension, diabetes mellitus, obesity, presence of coronary artery disease, or atrial fibrillation. Overall, 77 patients with IPAH (55.1 [interquartile range, IQR: 24.1] years, 62.8% women) have been recorded. Fifteen patients (19.2%) had ≥3 CVCs, while 25 (32%) were over 65 years old. Patients with ≥3 CVCs were older, presented an almost equal female to male ratio, walked less in 6‐min walk test, and had lower mean arterial pulmonary pressure and pulmonary vascular resistance at baseline than patients with less CVCs. Fewer patients with ≥3 CVCs received PAH‐specific treatment compared to patients with less comorbidities ( n = 11 [73.3%] versus n = 58 [95.5%], p = 0.02). During a median follow‐up period of 3.8 (IQR: 2.7) years, 18 patients died (all‐cause mortality 24.3%). Male sex and older age were independent predictors of mortality and/or lung transplantation, while CVCs did not have a significant impact on clinical outcomes. In this nationwide, register‐based study, the epidemiology of IPAH involves older patients with CVCs, who seem to have less hemodynamic compromise, but worse functional impairment and are treated less aggressively with PAH pharmacotherapy.

Real‐world clinical practice of pulmonary arterial hypertension in Japan: Insights from a large administrative database

“…Although there is no clear epidemiological information on the prevalence of comorbidities in PAH patients in Japan, a national research study on PAH indicates that patients aged 65 and above accounted for almost half of the newly diagnosed cases in 2013 11 . Several registries have also reported common comorbidities, like systemic arterial hypertension, in PAH patients, particularly among the elderly 16,17,23 . These findings are consistent with our results, suggesting that Japanese PAH patients may have multiple comorbidities.…”

“…11 Several registries have also reported common comorbidities, like systemic arterial hypertension, in PAH patients, particularly among the elderly. 16,17,23 These findings are consistent with our results, suggesting that Japanese PAH patients may have multiple comorbidities. Nevertheless, further research is required to better understand this association.…”

Elderly Patients with Idiopathic Pulmonary Hypertension: Clinical Characteristics, Survival, and Risk Stratification in a Single-Center Prospective Registry

“…Patients over 60 years represented 25.2% of the entire population compared to 63% of patients over 65 years in the COMPERA registry [9]. The provocative tests with fluid loading or exercise challenge during the RHC were not part of routine clinical practice at the time of working on the majority of the PAH registries [6,9,17,18,45] and conducting our study. At present, methodological approaches that could improve the reliability of hemodynamic data during RHC are well defined in elderly patients with a presence of LHD [46].…”

Pulmonary Arterial Hypertension in the Elderly: Peculiar Features and Challenges for a Proper Phenotyping Approach

“…Compared to the younger patients without cardiovascular comorbidities, elderly subjects with PAH and risk predictors for LHD have shown to have a higher mortality risk and a worse prognostic outcome, as well as poorer exercise capacity and functional class, a weaker response to PAH-targeted treatment, and a higher rate of PAH drug discontinuation [ 1 , 13 , 14 , 66 ] ( Figure 2 ). The first plausible explanation seems to be attributable to the persistent delay in the diagnosis of PAH over time in this patient population, usually due to the longer duration of symptoms, which are commonly attributed to the older age and other more prevalent comorbidities, than suspecting a timely diagnosis of PAH [ 15 , 16 , 37 ].…”