CONVENTIONAL ECHOCARDIOGRAPHIC FINDINGS IN SYSTEMIC SCLEROSIS-RELATED PULMONARY HYPERTENSION

SESSION TITLE: Pulmonary Vascular Disease Posters SESSION TYPE: Original Investigation Posters PRESENTED ON: October 18-21, 2020 PURPOSE: Systemic sclerosis (SSc) is a rare systemic disease in which pulmonary complications constitute the single most common cause of mortality. Pulmonary hypertension (PH) in SSc is phenotypically diverse and can result from Group 1 pulmonary arterial hypertension, Group 2 PH due to left-sided heart disease, Group 3 PH due to interstitial lung disease and/or chronic hypoxia, and possibly, Group 4 PH from chronic thromboembolic disease. The goal of this study was to investigate common findings on conventional echocardiography in systemic sclerosis patients with and without PH. METHODS: We conducted a retrospective study using a clinical registry of SSc patients from 2002 to 2017 and identified 183 SSc patients who have undergone at least one right heart catheterization procedure in which PH was defined by a mean pulmonary arterial pressure of greater than or equal to 25 mmHg. For these SSc patients, we collected the following conventional echocardiographic findings: 1) Left ventricular ejection fraction (LVEF); 2) Left ventricular diastolic dysfunction; 3) Left atrial enlargement; 4) Right atrial enlargement; 5) Right ventricular dysfunction; and, 6) Pericardial effusion. RESULTS: There were a total of 135 SSc patients who met criteria for PH and 48 SSc patients who did not. The mean LVEF was 60.6 ± 7.0% in SSc patients with PH and 60.9 ± 11.2% (P = 0.89) in SSc patients without PH. There were no statistically significant differences in the presence of left-sided cardiac abnormalities (i.e., the presence of left ventricular diastolic dysfunction or left atrial enlargement) between SSc patients with and without PH. However, right atrial enlargement (37.0% versus 1.1%; P = 0.0002) and right ventricular dysfunction (21.1% versus 1.1%; P = 0.03) were significantly more common among SSc patients with PH versus those without PH. CONCLUSIONS: As would be expected, right-sided cardiac abnormalities are more common than left-sided cardiac abnormalities on conventional echocardiography in SSc patients with PH compared to those without PH. CLINICAL IMPLICATIONS: These data support the need for novel imaging techniques to discern more subtle evidence of left ventricular dysfunction as conventional methods may be inadequate. DISCLOSURES: No relevant relationships by Andreea Bujor, source=Web Response No relevant relationships by Deepa Gopal, source=Web Response Advisory Committee Member relationship with Pfizer Please note: $20001 - $100000 Added 02/14/2020 by Elizabeth Klings, source=Web Response, value=Grant/Research Support Advisory Committee Member relationship with Micelle Please note: $1001 - $5000 Added 02/14/2020 by Elizabeth Klings, source=Web Response, value=Consulting fee Site PI for clinical trial relationship with Bayer Please note: $5001 - $20000 Added 02/14/2020 by Elizabeth Klings, source=Web Response, value=Grant/Research Support Site PI for clinical trial relationship with Actelion Please note: $5001 - $20000 Added 02/14/2020 by Elizabeth Klings, source=Web Response, value=Grant/Research Support Site PI for clinical trial relationship with Reata Please note: $5001 - $20000 Added 02/14/2020 by Elizabeth Klings, source=Web Response, value=Grant/Research Support site PI for clinical trial relationship with Arena/United Therapeutics Please note: $5001 - $20000 Added 02/14/2020 by Elizabeth Klings, source=Web Response, value=Grant/Research Support Research support relationship with Novartis Please note: $5001 - $20000 Added 07/10/2020 by Elizabeth Klings, source=Web Response, value=Consulting fee No relevant relationships by Justin Lui, source=Web Response no disclosure on file for Brett Martin; No relevant relationships by Ruchika Sangani, source=Web Response no disclosure on file for marcin Trojanowski; No relevant relationships by Renda Wiener, source=Web Response