SPC365

Chronic thromboembolic pulmonary disease from proximal to distal: what are the differences?

Session: Posters (Sessão 3 - Écran 6) - Hipertensão pulmonar
Speaker: Bárbara Marques Ferreira

Congress: CPC 2023
Topic: F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme: 21. Pulmonary Circulation, Pulmonary Embolism, Right Heart Failure
Subtheme: 21.7 Pulmonary Circulation, Pulmonary Embolism, Right Heart Failure - Other
Session Type: Pósters Electrónicos
FP Number: ---
Authors: Bárbara Marques Ferreira; Filipa Ferreira; Sofia Alegria; Rita Calé; Débora Repolho; Ana Francisco; Mário Ferraz; Alexandra Briosa; João Grade Santos; Mariana Martinho; Diogo Cunha; João Luz; Nazar Ilchysnyn; Oliveira Baltazar; Carla Saraiva; Hélder Pereira

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><strong>Background: </strong>Chronic thromboembolic pulmonary disease (CTEPD) is a rare, progressive pulmonary vascular disease. Most often results from obstruction of large and/or middle-sized pulmonary arteries by nonresolving thromboemboli. On the other hand, it is known that small-vessel abnormalities also have a substantial impact on the severity of CTEPD and postsurgical outcomes.</span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><strong>Aim: </strong>To analyze clinical, hemodynamical and prognostic differences between proximal and distal CTEPD.</span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><strong>Methods: </strong>We performed a longitudinal retrospective study of all patients (pts) with the diagnose of CTEPD being followed in a referral center for pulmonary hypertension (PH). Angio-Computed Tomography of pulmonary arteries have been analyzed and pts were divided into two different groups: Group 1 with predominance of proximal disease (main, lobar&nbsp;and proximal&nbsp;segmental&nbsp;pulmonary&nbsp;artery branches <span style="font-size:11.0pt">[</span>level I&ndash;III]); and Group 2 with predominance of distal disease (distal segmental or subsegmental disease). Clinical data including previous pulmonary embolism, risk factors for CTEPD, WHO functional class, plasma biomarkers, transthoracic echocardiogram, 6 minutes walking test (6MWT), right heart catheterization and death were collected.</span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><strong>Results: </strong>We included a total of 71 pts with CTEPD (<span style="color:black">66% female</span>, mean age <span style="color:black">57.56&plusmn;15.32 years), </span>pulmonary hypertension at rest was present in 90.5% of pts (CTEPH) while the remaining presented symptomatic exercise PH (CTED). <span style="color:black">78.8% presented proximal disease and 21.1% presented distal disease</span>. 39% of the pts had at least one risk factor for CTEPD with no differences between the groups. </span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif">Proximal disease presented more often with previous pulmonary embolism (PE) (78.5% vs 40.0%, p=0.007), and with less severe clinical parameters like greater distance in 6MWD (<span style="color:black">p=0.020), better right ventricular function (p=0.057) and lower mean pulmonary artery pressure in right heart catheterization (p=0.009</span>). Further variables were analyzed but no differences were found between the groups (Table 1).</span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif">Overall, 35 patients had pulmonary endarterectomy (49.3%). As expected, pts with proximal disease were submitted to pulmonary endarterectomy more often (61.0% vs 27.0%), but residual PH were similar after surgery in both groups (23.3% vs 25.0%, p=0.941).</span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif">With a median follow-up of 5.10 years (IQ25-75: 2.0-7.0), there was 12 deaths (survival 82.6%). Kapplan Meier analysis showed that survival was not different between the groups (Figure 1).</span></span></p>

<p style="text-align:justify"><strong><span style="font-size:12.0pt"><span style="font-family:&quot;Calibri&quot;,sans-serif">Conclusion: </span></span></strong><span style="font-size:12.0pt"><span style="font-family:&quot;Calibri&quot;,sans-serif">Pts with CTEPD that present proximal disease are more likely to have previous PE and present with less severe PH. Nonetheless, residual hypertension was similar between groups and prognostic was similar.</span></span></p>