SPC365

Reducing the risk in patients with pulmonary arterial hypertension – from guidelines to practice

Session: Posters (Sessão 2 - Écran 5) - Doença Arterial Pulmonar e Tumores Cardíacos
Speaker: Alexandra Briosa

Congress: CPC 2022
Topic: F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme: 21. Pulmonary Circulation, Pulmonary Embolism, Right Heart Failure
Subtheme: 21.4 Pulmonary Circulation, Pulmonary Embolism, Right Heart Failure - Treatment
Session Type: Pósters Electrónicos
FP Number: ---
Authors: Alexandra Briosa; Barbara Ferreira; Mariana Martinho; Joao Santos; Filipa Ferreira; Sofia Alegria; Debora Repolho; Sofia Alves; Maria Jose Loureiro; Helder Pereira

<p style="text-align:start"><span style="font-size:medium"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="color:#000000"><span style="font-size:9pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">Pulmonary arterial hypertension (PAH) is a progressive and fatal disease. Current guidelines recommend the use of risk stratification model encompassing a range of parameters, allowing patients (pts) to be categorized as low risk (LR), intermediate risk&nbsp;(IR) and high-risk (HR)&nbsp;score. Treatment strategy with pulmonary vasodilator therapy (PVT) in based is risk stratification in order to achieve or maintain LR&nbsp;status </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:9pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">Aim:</span></span></strong><span style="font-size:9pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">&nbsp;Determine if pts achieve LR status after PVT initiation based in the actual guidelines and to define its long-term impact.</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:9pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">Methods</span></span></strong><span style="font-size:9pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">: We&nbsp;analyzed&nbsp;all pts with PAH submitted to PVT in our center. <span style="color:#211e1e">Risk was assessed at baseline and at FUP&nbsp;(6-12 months after PVT) using COMPERA registry parameters. Survival and clinical worsening-free survival were assessed in each risk group.</span></span></span></span></span></span></p>

<p style="text-align:start"><span style="font-size:medium"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="color:#000000"><strong><span style="font-size:9pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">Results: </span></span></strong><span style="font-size:9pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">84 pts with PAH, mostly females&nbsp;(71.4%), with a mean age of 47&plusmn;18y. The majority had congenital heart disease (n=27), 16 pts&nbsp;idiopathic PAH (iPAH), 14&nbsp;PAH associated with connective tissue disease (CTD), 11&nbsp;associated with HIV, 6&nbsp;portal hypertension, 5&nbsp;heritable PAH, 2 PVOD, 2 mixed disease and 1&nbsp;drug-associated. At admission, most pts were in WHO class &ge; 3, had a&nbsp;mean 6-minute walking test (6MWT) of 389&plusmn;120m,&nbsp;median NT-proBNP value of 904 pg/mL, mean cardiac index (CI) of 2,3&plusmn;0,6 ml/m<sup>2</sup> and&nbsp;mean PAPm of 52&plusmn;14 mmHg. </span></span></span></span></span></p>

<p style="text-align:start"><span style="font-size:medium"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="color:#000000"><span style="font-size:9pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">Most&nbsp;pts were in the IR and HR&nbsp;group (51,3% and&nbsp;43%). 75%&nbsp;started combination therapy: double therapy in 43% (mainly with sildenafil and bosentan) and triple therapy in 32%. Parenteral prostanoids (PP) were initiated upfront in 26 pts (18 in triple upfront combination therapy).</span></span></span></span></span></p>

<p style="text-align:start">After 6-12 months,&nbsp;<span style="color:#000000; font-family:&quot;Calibri Light&quot;,sans-serif; font-size:9pt">there was a significant improvement in 6MWT (387 vs 422m, p= 0.008), SVO2 (65 vs 70%, p=0.002) and CI (2,15 vs 2,66 l/min/m<sup>2</sup> p&lt; 0.001), as well as a significant reduction in functional class (p&lt; 0.001), PVR&nbsp;(12 vs 8WU, p&lt;0.001), PAPm (50 vs 44 mmHg, p=0.018) and RAP (8 vs 7 mmHg, p=0.036).Most</span><span style="font-size:medium"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="color:#000000"><span style="font-size:9pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">&nbsp;pts were in the LR group (n=30,&nbsp;48%), but there was 25 pts (40%)&nbsp;in IR and 7 (8,3%) in HR group. 76,1% lowered at least 1 risk group, 28,8% maintained same risk and 5,1% increased risk group. Pts who improved risk were younger (44 vs 53, p=0,04), male sex (83,3 vs 58.1%, p=0,05), and were more frequent in iPAH vs CTD-PAH (82 vs 58,3%). No difference found in the number of vasodilators used (2,11 vs 1,97). All pts that reduced from HR to LR&nbsp;group (20,3%) were on combination PVT including PP.</span></span></span></span></span></p>

<p style="text-align:start"><span style="font-size:medium"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="color:#000000"><span style="font-size:9pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">In a median FUP time of 4 years, 35 pts had &ge;1 hospitalization, 29 died and 3 had transplantation. As expected, pts with LR on admission, had more event-free time (Log rank: 10,3, p=0.006). The presence of &ge;1&nbsp;LR criteria after 6-months&nbsp;was also related with increase survival (Log rank: 6,5, p=0.011).</span></span></span></span></span></p>

<p style="text-align:start"><span style="font-size:medium"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="color:#000000"><strong><span style="font-size:9pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">Conclusions: </span></span></strong><span style="font-size:9pt"><span style="font-family:&quot;Calibri Light&quot;,sans-serif">Our study confirms and supports the importance of risk stratification, showing a clear benefit of maintaining pts in the LR&nbsp;possible. Importantly, after initiation PVT&nbsp;as recommended by the guidelines, there is still a high percentage of pts that remain in IR category.</span></span></span></span></span></p>