(371) - Initial Results of the Prospective Registry of Sarcoidosis Associated Pulmonary Hypertension (ReSAPH)

Abstract

s S143 artery pressure (mPAP) ≥ 25 mmHg, pulmonary artery wedge pressure (PAWP) 3 Wood Units. Data reported as mean ± standard deviation. Results: Of 5420 patients with Sjogren’s Syndrome evaluated between 1995 and 2014, 100 had evidence of right ventricular systolic pressure (RVSP) > 40 mmHg on transthoracic echocardiography. Of those 100 patients, 40 underwent right heart catheterization (RHC); and 36 (90%) had mPAP ≥ 25 mmHg. Of those 36 all-female patients, the average age was 65 ± 9 years. 20 (56%) had primary SS, and 16 (44%) had secondary SS. An underlying lung disorder was present in 13 (36%), with five carrying a diagnosis of interstitial lung disease. A left ventricular ejection fraction (EF) < 45% was present in 14 (39%). Transthoracic echocardiography for the 36 patients revealed a right atrial pressure (RAP) of 13 ± 4 mmHg and a mean RVSP of 72 ± 21 mmHg. Moderate-severe right atrial enlargement was present in 21 (58%) with moderate-severe right ventricular enlargement present in 21 (58%) also. 19 (53%) had ≤ mild right ventricular (RV) dysfunction and 17 (47%) had moderate-severe RV dysfunction. RHC in the 36 patients demonstrated a mPAP of 45 ± 10 mmHg, RAP of 11 ± 7 mmHg, PAWP of 14 ± 8 mmHg, and PVR of 7.3 ± 4.7 Wood Units. 16 (44%) had PAH and 15 (42%) were treated with PAH-specific medications. Median survival of patients with PAH was 39 months. Adverse prognostic indicators for those 36 patients with coexisting SS and PH included moderate-severe right atrial enlargement (p= 0.03), moderate-severe RV dysfunction (p= 0.035), and pre-capillary pulmonary arterial hypertension (p= 0.05). Conclusion: In patients with primary or secondary Sjogren’s Syndrome, a minority develop pulmonary hypertension. There is no significant difference in outcome between primary or secondary SS in the setting of concomitant PH. Right atrial enlargement, RV dysfunction, and pre-capillary pulmonary arterial hypertension are associated with worse survival.