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Women With Pulmonary Arterial Hypertension Face Significant Disease BurdenACC 2025

A small, multi-country analysis of women of child-rearing age has found that these patients experience limits on activities of daily living, challenges with the healthcare system and a high overall financial burden.

Women with pulmonary arterial hypertension (PAH) experience a high burden of disease that impacts their lives regardless of where they reside, according to a small study presented as a poster at the American College of Cardiology Annual Scientific Session and Expo.

Pulmonary arterial hypertension is a rare, progressive, and life-threatening disease in which blood vessels in the lungs narrow, causing strain on the heart. About 40,000 people in the United States are living with pulmonary arterial hypertension. The five-year mortality rate is about 43%.

PAH is more common in women. Among all cases in 2021, 62% were among women, according to one study published last year. For women of childbearing and childrearing age, there is a significant risk, including maternal mortality as high as 56%.

Researchers — led by Ioana R. Preston, M.D., associate professor of medicine at Tufts University School of Medicine — wanted to understand the challenges that women with pulmonary arterial hypertension across different regions face. They recruited 26 women to participate in qualitative interviews: 12 in the United States, 4 in Germany, 4 in Italy, 3 in Canada and 3 in the United Kingdom. The participants answered open-ended questions on perceived burden and management of PAH, focusing on family planning, pregnancy, and child care.

The responses were assessed and stratified by region: the United States compared with Europe/Canada. The women in the study were between 21 and 50 years of age, with a mean age of 43 in the United States and 37 in Europe/Canada. In the United States, 41% of women were raising children. In Europe/Canada, 51% were raising children.

Patients reported that the burdens of their disease were high in both regions. Mental health issues, especially were high across both regions. Patients said they experienced depression, self-isolation, and complications with pregnancy.

Most patients reported limitations in activities of daily living. Additionally, patients across both regions reported challenges in interaction with the healthcare system and a high overall financial burden. Some patients reported a lack of empathy from treating physicians, a long wait time for appointments, and some were not satisfied with their specialist.

Researchers said one limitation of the study was its qualitative nature, which can lead to sampling bias and limits the generalizability of findings to broader populations. Additionally, participants may not accurately remember past events and experiences.

The study presented at ACC was sponsored by Merck, which markets Winrevair (sotatercept-csrk). Winrevair is currently approved in the United States and 40 countries to treat pulmonary arterial hypertension and to reduce the risk of clinical worsening events. The FDA approved Winrevair in March 2024.

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In PAH, Main Pulmonary Artery Diameter Predicts Adverse Outcomes

Main pulmonary artery (MPA) diameter is a prognostic indicator in patients with pulmonary arterial hypertension (PAH) without congenital heart disease, according to study findings published in Chest.

Investigators characterized the size and growth of the MPA in patients with PAH without congenital heart disease. The researchers also assessed the prognostic value of MPA relative to existing risk stratification scores. A composite of all-cause mortality, lung transplantation, and hospitalization for right heart failure was the primary outcome.

The study included patients from 2 pulmonary hypertension referral centers in Sydney, Australia, who had pulmonary artery imaging at least once between January 2010 and December 2021. Participants were identified through a retrospective review of electronic medical records of patients with PAH without congenital heart disease who also did not have concurrent chronic thromboembolic PH or vasculitis involving pulmonary circulation.

PAH risk scores based on the Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL) 2.0, REVEAL Lite 2, and the European Society of Cardiology/European Respiratory Society (ESC/ERS) risk assessment were evaluated using baseline variables. REVEAL 2.0 was used when there were at least 7 parameters available; REVEAL Lite 2 was used when there were at least 3 parameters available, with at least 2 being World Health Organization (WHO) functional class, 6-minute walk distance (6MWD), or N-terminal pro b-type natriuretic peptide (NT-proBNP); and ESC/ERS was used when there were at least 3 parameters available.

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MPA diameter is a significant independent predictor of adverse clinical events in PAH patients without congenital heart disease. It may potentially be a novel prognostic marker in addition to the existing risk scores.

Overall, 351 patients were included in analysis, 70% with contrast-enhanced CTs, 27% with non-contrast enhanced CTs, and 3% with MRIs. Connective tissue disease was the most common PAH subgroup (49%), followed by idiopathic PAH (30%), and portopulmonary hypertension (5%). Echocardiography showed that right ventricular function was normal in 55% of participants, mildly impaired in 17%, moderately impaired in 12%, and severely impaired in 15%. Pericardial effusion was noted in 18% of participants.

Mean pulmonary artery pressure (MPAP) was 43 mmHg at baseline. Mean PA diameter was 35.3 mm at baseline and grew by 0.4 mm per year. The investigators noted 190 primary events across mean 4.0 years follow-up, with MPA diameter as a predictor (hazard ratio [HR], 1.06; 95% CI, 1.04-1.07; P <.001).

After multivariable adjustments for the 3 risk scores and their individual components, MPA diameter remained an independent outcome predictor. Independent of baseline MPA diameter, MPA growth rate predicted the primary endpoint with every 1 mm per year presenting a 79% increased hazard (HR, 1.79; 95% CI, 1.52-2.11; P <.001).

The risk for the primary endpoint at 1 year was similar for MPA alone (area under the receiver-operator characteristic curve [AUC], 0.72) vs the 3 risk scores (AUCs, 0.72-0.75). Risk reclassification (primarily risk downgrading) in nearly a quarter of patients (23%) stemmed from MPA used in addition to REVEAL 2.0.

Study limitations include the retrospective design and the lack of data on causes of mortality.

"MPA diameter is a significant independent predictor of adverse clinical events in PAH patients without congenital heart disease," the investigators concluded. "It may potentially be a novel prognostic marker in addition to the existing risk scores."

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