Background/Purpose: Prevalence of pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) is 8-12%, and 3-year survival rate is 47-56%. Rest echocardiogram is used to screen for this pathology; recent studies suggest that systolic pulmonary artery pressure (sPAP) increase >18 mmHg with exercise may be a marker for early PAH diagnosis in SSc patients. However, left diastolic dysfunction, comorbidities, and sarcopenia may impair exercise echocardiogram interpretation. We explored its use as a screening tool for early PAH detection in SSc patients.

Methods: Cross-sectional, observational study that included SSc patients (ACR/EULAR 2013 criteria) NYHA classes I-II, and peak tricuspid regurgitant jet velocity at rest echocardiogram <3m/s. We excluded patients with established PAH, interstitial lung disease (ILD) >20% of extent, cardiac disease, renal failure, diabetes mellitus, lipid disorders, systemic arterial hypertension, untreated thyroid disease, pulmonary embolism, tobacco use (>2 pkg/year), use of vasodilators, pregnancy or contraindication to perform the tests (echocardiogram with cycloergometer, starting at 10W and increasing 5W every min, maximum 60W; cardiopulmonary exercise test (CPET) with modified Bruce protocol on a computer-controlled cycle ergometer, and body mass composition). All the patients read and signed an informed consent form. The study was approved by the local IRB and was performed according to the Declaration of Helsinki contents.

Results: We included 19 patients. Fourteen (73.7%) with limited cutaneous (lc) SSc and 5 (26.3%) with diffuse cutaneous (dc) SSc; 17 were female. Mean age was 50.8 (± 8.6 SD) years and mean duration of the disease was 78.2 (± 53.7 SD) months. Mean modified Rodnan Skin Score (mRSS) was 3.6 (± 3.1 SD) in lcSSc and 13.4 (± 6.1 SD) in dcSSc patients. Twelve patients had NYHA I (63.2%). Mean BNP was 47.8 (± 48.1 SD). Mean forced vital capacity (FVC) was 87.4%. Cardiovascular evaluation: Mean rest sPAP was 28 mmHg (± 6.4 SD), sPAP at maximum effort was 52 mmHg (± 8.4 SD), with mean delta sPAP of 24 mmHg (± 6.4 SD). A sPAP increase of >18mmHg was observed in 15 (78.9%) of our patients. Pulmonary Vascular Reserve (PVR) was elevated (mean 3.4 ± 0.8 mmHg/ml/m2 SD), suggesting pulmonary vasculopathy, while E/E’ ratio was normal (8.3 ± 1.9 SD), suggesting normal left diastolic function. At CPET, mean peak oxygen uptake was normal (80.6%), suggesting early, non-severe, pulmonary vasculopathy. The normal relative skeletal muscle mass index (RSMI) (6.5 ± 0.95 kg/m² SD) ruled out sarcopenia as a cause of low exercise performance.

Conclusion: Seventy nine percent of SSc patients without cardiovascular risk factors and with normal mPAP at rest echocardiogram, showed changes suggestive of pulmonary vasculopathy in exercise echocardiogram. This finding was not related to diastolic dysfunction or muscular impairment, and may reflect vascular changes that may lead to overt PAH. Standardized exercise echocardiogram may be useful to screen SSc patients for PAH.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/should-we-perform-exercise-echocardiogram-as-a-screening-test-for-pulmonary-arterial-hypertension-pah-for-all-systemic-sclerosis-ssc-patients/