Abstract 17922: Scleroderma Associated Pulmonary Arterial Hypertension is Associated With a High Prevalence of Aortic Stenosis

Introduction: Traditional risk factors for aortic stenosis (AS) include age, male sex, hypertension, smoking, and dyslipidemia, while inflammation and increased bone morphogenetic protein (BMP) signaling have been implicated in disease progression. BMP signaling is suppressed in pulmonary arterial hypertension (PAH), and these patients are usually younger at the time of diagnosis, mostly female, and typically do not have traditional AS risk factors. On the other hand, PAH, and particularly scleroderma associated PAH (Scl-PAH) patients, have a high burden of systemic inflammation. Hypothesis: Following serial clinical observations, we hypothesized that the prevalence of AS would be higher in patients with Scl-PAH or idiopathic PAH (iPAH) compared to the normal population, where it is observed in 1% of women and 1.5% of men 65-75yo and in even less (0.6%) of patients 55-65yo. Methods: We reviewed ECHO and clinical data from a prospective database in our pulmonary hypertension clinic since 2002. This included Scl-PAH (n=116), iPAH (n=99), and chronic thromboembolic pulmonary hypertension (CTEPH, as a control group, n=98). The presence of AS, grade of disease, aortic valve area, peak aortic jet velocity and maximum aortic pressure gradient were analyzed. Results: In Scl-PAH vs iPAH vs CTEPH groups, the mean age was 55.8±1.3 vs 54.8±1.7 vs 59.9±1.7 years; the %females was, 87.1% vs 68.7% vs 61%; the mean PA pressure by catheterization was 34.8±2.4 vs 49.3±1.6 vs 43.1±1.2 mmHg, respectively. AS was present in 13.8% of Scl-PAH vs 6.1% of iPAH and 4.1% of CTEPH patients. The rate of progression of AS in Scl-PAH was 0.42m/s/year, a rate that in the general population is associated with worse outcomes. Conclusions: Patients with Scl-PAH may be a previously unidentified high-risk group for AS development, with high prevalence and fast progression rates. The AS risk is particularly high in these younger and mostly female patients considering the typical absence of known AS risk factors in Scl-PAH. If our data are confirmed in larger and multicenter cohorts, they would suggest a need for increased awareness for AS in Scl-PAH patients, whose serious co-morbidities and PAH-therapies (vasodilators, transplantation) can significantly complicate the management of AS.

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