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Lung involvement in Pulmonary Hypertension associated with Systemic Sclerosis
Session:
Posters - F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Speaker:
Rita Soares Rosa
Congress:
CPC 2021
Topic:
F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme:
21. Pulmonary Circulation, Pulmonary Embolism, Right Heart Failure
Subtheme:
21.2 Pulmonary Circulation, Pulmonary Embolism, Right Heart Failure – Epidemiology, Prognosis, Outcome
Session Type:
Posters
FP Number:
---
Authors:
Rita Soares Rosa; Filipa Ferreira; Sofia Alegria; Paula Fazendas; Ana Cordeiro; Alexandra Briosa; Débora Repolho; Ângela Manuel; Maria José Loureiro; Hélder Pereira
Abstract
<p style="text-align:justify"><span style="font-size:medium"><span style="font-family:"Times New Roman",serif"><span style="color:#000000"><strong><em><span style="font-family:Calibri,sans-serif"><span style="color:black"><span style="background-color:white">Introduction: </span></span></span></em></strong><span style="font-family:Calibri,sans-serif">Systemic sclerosis (SS) is an auto-immune disease characterized multiple organ involvement, including the lungs. Pulmonary hypertension (PH) is a common finding that can significantly impact prognosis. To this day, it is still a matter of debate the classification of PH in SS patients, specifically the impact of lung involvement. </span></span></span></span></p> <p style="text-align:justify"><span style="font-size:medium"><span style="font-family:"Times New Roman",serif"><span style="color:#000000"><strong><em><span style="font-family:Calibri,sans-serif"><span style="color:black"><span style="background-color:white">Aim: </span></span></span></em></strong><span style="font-family:Calibri,sans-serif"><span style="color:black"><span style="background-color:white">To analyse the population of patients followed in a referral centre for PH, from 2012 to 2020, with a diagnosis of SS and PH, according to the presence of interstitial lung disease (ILD).</span></span></span></span></span></span></p> <p style="text-align:justify"><span style="font-size:medium"><span style="font-family:"Times New Roman",serif"><span style="color:#000000"><strong><em><span style="font-family:Calibri,sans-serif"><span style="color:black"><span style="background-color:white">Methods: </span></span></span></em></strong><span style="font-family:Calibri,sans-serif"><span style="color:black"><span style="background-color:white">Observational study including patients with the diagnosis of SS and PH</span></span></span><span style="font-family:Calibri,sans-serif"> followed between January 2012 and December 2020. </span><span style="font-family:Calibri,sans-serif"><span style="color:black"><span style="background-color:white">Patients with a </span></span></span><span style="font-family:Calibri,sans-serif">chest CT scan showing more than 20% total lung involvement due to ILD or with FVC inferior to 70% </span><span style="font-family:Calibri,sans-serif"><span style="color:black"><span style="background-color:white">were classified as patients with lung involvement (LI), and the remaining as patients with no lung involvement (NLI).</span></span></span> <span style="font-family:Calibri,sans-serif">Demographic, clinical, analytical, echocardiographic (</span><span style="font-family:Calibri,sans-serif"><span style="color:black"><span style="background-color:white">pulmonary artery systolic pressure </span></span></span><span style="font-family:Symbol"><span style="color:black"><span style="background-color:white">[</span></span></span><span style="font-family:Calibri,sans-serif"><span style="color:black"><span style="background-color:white">PASP</span></span></span><span style="font-family:Symbol"><span style="color:black"><span style="background-color:white">]</span></span></span><span style="font-family:Calibri,sans-serif">, tricuspid annular plane systolic excursion </span><span style="font-family:Symbol"><span style="color:black"><span style="background-color:white">[</span></span></span><span style="font-family:Calibri,sans-serif">TAPSE</span><span style="font-family:Symbol"><span style="color:black"><span style="background-color:white">]</span></span></span><span style="font-family:Calibri,sans-serif">, s’ wave, Fractional area change </span><span style="font-family:Symbol"><span style="color:black"><span style="background-color:white">[</span></span></span><span style="font-family:Calibri,sans-serif">FAC</span><span style="font-family:Symbol"><span style="color:black"><span style="background-color:white">]</span></span></span><span style="font-family:Calibri,sans-serif">, presence of pericardial effusion </span><span style="font-family:Symbol"><span style="color:black"><span style="background-color:white">[</span></span></span><span style="font-family:Calibri,sans-serif">PE</span><span style="font-family:Symbol"><span style="color:black"><span style="background-color:white">]</span></span></span><span style="font-family:Calibri,sans-serif">) and hemodynamic parameters (</span><span style="font-family:Calibri,sans-serif"><span style="color:black"><span style="background-color:white">mean pulmonary arterial pressure </span></span></span><span style="font-family:Symbol"><span style="color:black"><span style="background-color:white">[</span></span></span><span style="font-family:Calibri,sans-serif"><span style="color:black"><span style="background-color:white">m</span></span></span><span style="font-family:Calibri,sans-serif">PAP</span><span style="font-family:Symbol"><span style="color:black"><span style="background-color:white">]</span></span></span><span style="font-family:Calibri,sans-serif">, mean right atrial pressure </span><span style="font-family:Symbol"><span style="color:black"><span style="background-color:white">[</span></span></span><span style="font-family:Calibri,sans-serif">mRAP</span><span style="font-family:Symbol"><span style="color:black"><span style="background-color:white">]</span></span></span><span style="font-family:Calibri,sans-serif">, pulmonary vascular resistance </span><span style="font-family:Symbol"><span style="color:black"><span style="background-color:white">[</span></span></span><span style="font-family:Calibri,sans-serif">PVR</span><span style="font-family:Symbol"><span style="color:black"><span style="background-color:white">]</span></span></span><span style="font-family:Calibri,sans-serif">, mixed venous oxygen saturation </span><span style="font-family:Symbol"><span style="color:black"><span style="background-color:white">[</span></span></span><span style="font-family:Calibri,sans-serif">SvO2</span><span style="font-family:Symbol"><span style="color:black"><span style="background-color:white">]</span></span></span><span style="font-family:Calibri,sans-serif">, cardiac index </span><span style="font-family:Symbol"><span style="color:black"><span style="background-color:white">[</span></span></span><span style="font-family:Calibri,sans-serif">CI</span><span style="font-family:Symbol"><span style="color:black"><span style="background-color:white">]</span></span></span><span style="font-family:Calibri,sans-serif">) were evaluated. Distance recorded in the 6-minute walk test (6MWT) was also assessed. Given the small sample size relevant results are represented by the effect size (Cohen’s d considered relevant >0,7).</span></span></span></span></p> <p style="text-align:justify"><span style="font-size:medium"><span style="font-family:"Times New Roman",serif"><span style="color:#000000"><strong><span style="font-family:Calibri,sans-serif"><span style="color:black">Results:</span></span></strong><span style="font-family:Calibri,sans-serif"><span style="color:black"> 15 patients, all female, with PH due to SS were included, 7 in the group LI and the remaining 8 in the group NLI. Patients with LI had lower DLCO (34 vs 53%, p=0,027), were younger (58,6</span></span><span style="font-family:Symbol"><span style="color:black">±</span></span><span style="font-family:Calibri,sans-serif"><span style="color:black">15,8 vs 66,8</span></span><span style="font-family:Symbol"><span style="color:black">±</span></span><span style="font-family:Calibri,sans-serif"><span style="color:black">13,7, p=0,296) and had lower pO2 (64,4 vs 74,1 mmHg, p=0,36). Regarding the hemodynamic parameters: CI (1,9</span></span><span style="font-family:Symbol"><span style="color:black">±</span></span><span style="font-family:Calibri,sans-serif"><span style="color:black">0,62 vs 2,5</span></span><span style="font-family:Symbol"><span style="color:black">±</span></span><span style="font-family:Calibri,sans-serif"><span style="color:black">0,51, Cohen’s d=1,1, p=0,057), TAPSE (15,5</span></span><span style="font-family:Symbol"><span style="color:black">±</span></span><span style="font-family:Calibri,sans-serif"><span style="color:black">2,9 vs 18,1</span></span><span style="font-family:Symbol"><span style="color:black">±</span></span><span style="font-family:Calibri,sans-serif"><span style="color:black">3,7, Cohen’s d=0,79) and s’ wave (9,4</span></span><span style="font-family:Symbol"><span style="color:black">±</span></span><span style="font-family:Calibri,sans-serif"><span style="color:black">2 vs 10,9</span></span><span style="font-family:Symbol"><span style="color:black">±</span></span><span style="font-family:Calibri,sans-serif"><span style="color:black">2,1, Cohen’s d=0,73) were lower in patients with LI. No significant differences were noticed in the remaining parameters: PASP (71,3</span></span><span style="font-family:Symbol"><span style="color:black">±</span></span><span style="font-family:Calibri,sans-serif"><span style="color:black">22,8 vs 78,5</span></span><span style="font-family:Symbol"><span style="color:black">±</span></span><span style="font-family:Calibri,sans-serif"><span style="color:black"> 17,5mmHg, p=0,5), FAC (31,2</span></span><span style="font-family:Symbol"><span style="color:black">±</span></span><span style="font-family:Calibri,sans-serif"><span style="color:black">14,4 vs 35,1</span></span><span style="font-family:Symbol"><span style="color:black">±</span></span><span style="font-family:Calibri,sans-serif"><span style="color:black">8,3%, p=0,63), mPAP (40,1</span></span><span style="font-family:Symbol"><span style="color:black">±</span></span><span style="font-family:Calibri,sans-serif"><span style="color:black">9,9 vs 40,4</span></span><span style="font-family:Symbol"><span style="color:black">±</span></span><span style="font-family:Calibri,sans-serif"><span style="color:black">14,2mmHg, p=0,96), PVR (9,9</span></span><span style="font-family:Symbol"><span style="color:black">±</span></span><span style="font-family:Calibri,sans-serif"><span style="color:black">6,9 vs 11,5</span></span><span style="font-family:Symbol"><span style="color:black">±</span></span><span style="font-family:Calibri,sans-serif"><span style="color:black">5,8 UW, p=0,65). Other markers of poor prognosis such as distance in 6MWT (322,9</span></span><span style="font-family:Symbol"><span style="color:black">±</span></span><span style="font-family:Calibri,sans-serif"><span style="color:black">145,1 vs 333,3</span></span><span style="font-family:Symbol"><span style="color:black">±</span></span><span style="font-family:Calibri,sans-serif"><span style="color:black">50,5m, p=0,16), </span>SvO2<span style="color:black"> (63,7</span></span><span style="font-family:Symbol"><span style="color:black">±</span></span><span style="font-family:Calibri,sans-serif"><span style="color:black">8,9 vs 67,49</span></span><span style="font-family:Symbol"><span style="color:black">±</span></span><span style="font-family:Calibri,sans-serif"><span style="color:black">11,44, p=0,49) and presence of PE (62,5 vs 57,1%) were similar in both groups. There were also no significant differences regarding PWP (p=0,61), PADm (p=1), or NT-proBNP (p=0,28). Mortality rates were of 28,5% and 37,5% (p=0,71) in the LI and NLI group, respectively.</span></span></span></span></span></p> <p style="text-align:justify"><span style="font-size:medium"><span style="font-family:"Times New Roman",serif"><span style="color:#000000"><strong><span style="font-family:Calibri,sans-serif"><span style="color:black"><span style="background-color:white">Conclusion</span></span></span></strong><strong><span style="color:black"><span style="background-color:white">:<em> </em></span></span></strong><span style="font-family:Calibri,sans-serif"><span style="color:black">In SS, patients with LI had worse CI and right ventricular longitudinal function when compared to patients with NLI. This difference was particularly marked for the CI. </span></span><span style="font-family:Calibri,sans-serif"><span style="color:black">The remaining clinical and hemodynamic parameters were similar irrespective of lung involvement by SS. </span></span></span></span></span></p>
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