A recent study published in BMC Cardiovascular Disorders has unveiled alarming findings regarding patients suffering from connective tissue disease–associated pulmonary arterial hypertension (CTD-PAH). The research indicates that patients who score 5 or higher on the Heart Failure Association–preserved ejection fraction (HFA-PEFF) algorithm are at an increased risk of all-cause mortality and clinical worsening events.

The HFA-PEFF algorithm, a tool that helps diagnose heart failure with preserved ejection fraction (HFpEF), has shown to be particularly beneficial for assessing patient prognosis in this vulnerable demographic. Researchers emphasized that a higher HFA-PEFF score correlates with a significantly poorer overall prognosis, primarily due to the exacerbated left heart dysfunction present in these patients. The algorithm involves four critical steps: a pretest assessment, calculation of the HFA-PEFF score, functional testing for intermediate scores, and investigation of specific etiologies.

In this single-center retrospective study, the participants were divided into two groups based on their HFA-PEFF scores: one group scored below 5, constituting a low to intermediate probability for HFpEF, while the other group scored 5 or higher, indicating a high probability. The study focused on 147 adult patients diagnosed through right heart catheterization between June 2016 and January 2024, all of whom met the diagnostic criteria for PAH.

Key findings highlighted that the patients with a high HFA-PEFF score experienced significantly worse clinical outcomes. These individuals exhibited a substantially shorter time from CTD diagnosis (0.90 vs 31.50 months), reduced 6-minute walking distances (386 meters vs 465 meters), and elevated serum NT-proBNP levels (2094 pg/mL vs 202 pg/mL). Moreover, a staggering 68.1% of this group were classified as World Health Organization functional class III-IV, a stark contrast to just 28.4% in the lower scoring group.

Further analysis revealed that patients with an HFA-PEFF score of 5 or higher had more severe measurements across several critical indicators, including higher baseline mean pulmonary arterial pressure (mPAP), pulmonary vascular resistance (PVR), and reduced cardiac index. Metrics of right and left heart function were also significantly poorer in this high-risk group, underscoring the multifaceted challenges faced by those with CTD-PAH.

The researchers utilized univariable and multivariable Cox regression analyses, which confirmed that an HFA-PEFF score of 5 or higher is strongly linked to increased all-cause mortality and clinical worsening events. Follow-up data indicated a trend of worsening serum NT-proBNP levels and functional impairment in this cohort.

In conclusion, the study affirms the acute dangers facing patients with CTD-PAH who score high on the HFA-PEFF scale. The researchers emphasize the urgent need for effective risk stratification and targeted treatment strategies in this population, suggesting that the high incidence of right ventricular enlargement and myocardial changes play critical roles in the trajectory of disease progression.

This groundbreaking research serves as a reminder of the complex relationship between pulmonary arterial hypertension and heart function, prompting a call to action for enhanced clinical management and potential therapeutic interventions. Stay informed as we follow further developments in this pivotal area of cardiovascular health!