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Case Report: Rethinking pulmonary arterial hypertension: immune and metabolic adaptations in a 34-year case of insidious progression

Erickson-Keith B

Frontiers in cardiovascular medicine, 2026

1 citation

Abstract

Background: Pulmonary arterial hypertension (PAH) is traditionally characterized as a rapidly progressive, fatal disease. Yet increasing evidence suggests that immune dysregulation, endothelial injury, and metabolic adaptation can sustain subclinical disease for decades before recognition.

Case presentation: We report a 73-year-old U.S. Army veteran with terminal PAH whose symptoms began in 1972-nearly fifty years before diagnosis. Despite a childhood splenectomy and extensive exposure to pulmonary toxins during a 21-year career in military aviation (JP-4/JP-8 fuels, asbestos, burn pits, and talc pleurodesis), no follow-up imaging was performed after retirement in 1992. After a 24-year gap, a 2016 chest x-ray revealed pulmonary artery enlargement consistent with pulmonary hypertension, but the finding was not pursued until 2021, when right-heart catheterization confirmed severe PAH with pulmonary artery pressures of 82/29 mm Hg (mean ≈47 mm Hg). A transthoracic echocardiogram in October 2025 demonstrated an estimated right-ventricular systolic pressure (RVSP) of 66 mm Hg with right-atrial pressure 8 mm Hg, preserved biventricular function, normal ejection fraction (LVEF 68%), mild concentric left-ventricular hypertrophy, and only trace tricuspid and mitral regurgitation. Brain natriuretic peptide (BNP) levels remained normal, suggesting right-ventricular metabolic adaptation rather than decompensation.

Discussion: This case supports a model of slowly progressive PAH driven…

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