SPC365

Risk factors for chronic thromboembolic pulmonary hypertension – a real life experience from a Portuguese tertiary center

Session: Posters - F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Speaker: Alexandra Briosa

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: Alexandra Briosa; Filipa Ferreira; Sofia Alegria; Débora Repolho; Mario Ferraz; Tiago Judas; Rita Calé; Maria José Loureiro; Helder Pereira

<p style="text-align:start"><span style="font-size:medium"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><strong><span style="font-size:10pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">Introduction: </span></span></strong><span style="font-size:10pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">Chronic thromboembolic pulmonary hypertension (CTEPH) is an increasingly diagnosed type of pulmonary hypertension, that is characterized by a chronic thrombotic obstruction of the pulmonary vessels. Although its pathogenesis remains unclear, there are multiple risk factors (RF) identified as being associated with this pathology. </span></span></span></span></span></p>

<p style="text-align:start"><span style="font-size:medium"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><strong><span style="font-size:10pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">Aim: </span></span></strong><span style="font-size:10pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">To evaluate the risk profile of the patients (pts) with CTEPH treated in our center, as well as to determine the representativeness of the established RF and to evaluate the impact on clinical outcomes.&nbsp; </span></span></span></span></span></p>

<p style="text-align:start"><span style="font-size:medium"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><strong><span style="font-size:10pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">Methods: &nbsp;</span></span></strong><span style="font-size:10pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">Single center retrospective study analyzing<strong> </strong>pts with established CTEPH in what concerns presence of well-known RF, clinical and imaging characteristics, type of treatment chosen as well as long term outcomes. </span></span></span></span></span></p>

<p style="text-align:start"><span style="font-size:medium"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><strong><span style="font-size:10pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">Results: </span></span></strong><span style="font-size:10pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">82 pts, 72% from&nbsp;female sex, with a mean age of 62&plusmn;14 years old.&nbsp;70%&nbsp;presented in NYHA class 3 or higher, the mean and median NT-proBNP levels were 2242&plusmn;5108 pg/mL and 599 pg/mL respectively, and the median PSAP value on echocardiogram was 86 mmHg. The first right-cath evaluation showed a median PAP value of 44 mmHg, median cardiac index of 2,24 L/min/m<sup>2</sup> and median pulmonary vascular resistance (PVR)&nbsp;of 9.33 wood U. 36 pts were submitted to pulmonary endarterectomy (PEA) (3 of them with subsequent need of balloon pulmonary angioplasty-BPA), 13 pts integrated the program of BPA in the first place and the rest stayed on vasodilator therapy. By the time of the analysis, 5 pts were still waiting to integrate BPA program and 2 pts were waiting for PEA confirmation. </span></span></span></span></span></p>

<p style="text-align:start"><span style="font-size:medium"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><span style="font-size:10pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">At the 6 month-follow-up, there was a significant reduction in NTproBNP levels (422 vs 1606 pg/mL, p&lt; 0.001), PSAP levels (37 vs 82 mmHg, p&lt;0.001), PAP levels (25 vs 43 mmHg, p&lt;0.001) and on PVR (4 vs 10 WU, p&lt;0.001). 23% of pts died. </span></span></span></span></span></p>

<p style="text-align:start"><span style="font-size:medium"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><span style="font-size:10pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">In what concerns established RF: 69,5% (n=57) had at least one episode of clinical pulmonary embolism (PE), 12 of them submitted to fibrinolysis therapy during hospitalization. Previous deep venous thrombosis (DVT) was observed in 17 pts. Additionally, 18 pts were obese, 13 had history of neoplasm</span></span></span></span></span><span style="font-size:medium"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><span style="font-size:10pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">, 10 had hypothyroidism under replacement therapy, 4 had hematological disease such as antiphospholipid antibody syndrome. Pacemaker leads and splenectomy were present in 2 pts each.Regarding genetic mutations, 3 had the presence of antiphospholipid antibodies, 2 had mutation on beta 2 glycoprotein antibody and 1 had a mutation on the prothrombin gene. The majority of pts had a total of 1 or 2 RF (44% and 29% respectively). Between pts with 2RF, the most common combination was PE and DVT (29%), followed by PE and neoplasm (17%)</span></span></span></span></span></p>

<p style="text-align:start"><span style="font-size:medium"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><strong><span style="font-size:10pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">Conclusions: </span></span></strong><span style="font-size:10pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">In our study, we confirmed the presence of those well-known RF for CTEPH, showing that our population has the same risk profile as those presented in other studies. The early identification of these common RF&nbsp;can help to identify at-risk pts, shifting the paradigm of treatment into a more preventive one and improving prognosis.</span></span></span></span></span></p>