SPC365

A tale of a deadly duo – Estimating prognosis in CTD associated PH

Session: Comunicações Orais - Sessão 10 - Hipertensão Pulmonar Tromboembólica Crónica
Speaker: Pedro Alves Da Silva

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: Comunicações Orais
FP Number: ---
Authors: Pedro Alves Da Silva; Joana Brito; Beatriz Silva; Ana Margarida Martins; Catarina Simões Oliveira; Ana Beatriz Garcia; Ana Abrantes; Miguel Raposo; Catarina Gregório; João Fonseca; Tatiana Guimarães; Nuno Lousada; Rui Plácido; Fausto J. Pinto

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>Introduction</strong>: Pulmonary arterial hypertension (PAH) is a severe complication of connective tissue disease (CTD), conveying a poor prognosis in this population. PAH specific therapies improved the outcome for PAH patients in the modern treatment era, but few data are available regarding risk stratification and prognosis in this specific pts. Pulmonary vascular compliance is diminished due to decreased proximal pulmonary arterial elasticity and increased distal pulmonary arterial vasculopathy<span style="background-color:white"><span style="font-family:&quot;Helvetica&quot;,sans-serif"><span style="color:black">. </span></span></span>A recent paper proposed that pulmonary artery compliance (PAC) could be a marker of prognosis in Chinese patients with LES and PH, but its application in other CTD is yet to be confirmed. </span></span></p>

<p><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>Purpose</strong>: We aimed to identify&nbsp;factors that could influence mortality in this subset of pts and to determine if PAC could act as a valid predictor in pts with CTD other than LES.</span></span></p>

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>Methods</strong>: Observational single centre retrospective study including pts followed in a reference hospital for PH related to CTD. Clinical, lab, echo and&nbsp; RHC&nbsp;data were collected at beginning and during FUP. Uni and multivariate analysis were performed with Cox regression and survival analysis was done using Kaplan Meyer curves. </span></span></p>

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>Results</strong>: 48 patients with CTD and associated PH were gathered, mean age was 65,25 &plusmn; 14,4 years and female per male ratio 10:1 (9% male). Systemic sclerosis was the most prevalent CTD (52,1%), followed by LES and Sj&ouml;gren sydrome. During a mean FUP&nbsp;of 5 years, 39,6% pts died (n=39) and 43,8% were hospitalized (n=21) due to CV cause. There were no statistical differences between different CTD aetiologies in respect to mortality and admissions. </span></span></p>

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">On univariate Cox analysis, NTproBNP (p&lt;0,001), alkaline phosphatase (p=0,001); uric acid (p=0,014), TAPSE/sPAP ratio (p=0,015) and COMPERA at beginning FUP (p&lt;0,001) correlated with CV events (composite endpoint of admissions and mortality). TAPSE/sPAP ratio&lt;0,55 has been described as a non-invasive marker of severity in PAH. After ROC curve analysis, TAPSE/sPAP ratio&lt;0,379 had the best specificity and sensitivity in our population.</span></span></p>

<p><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Pulmonary vasculature is a highly compliant system but pts with PAH have a lower PAC. PAC can be estimated by a simplified calculus, dividing stroke volume per pulse pressure. A cut-off of lower than 1,39mL/mmHg had been proposed as conveying worse prognosis and we thus divided our population using this value. In our cohort 40 pts had all the parameters at RHC that enabled calculation of PAC: 20 above and 20 below 1,39mLmmHg . Kaplan-Meyer analysis showed a significant difference between two groups (p=0,001, 95% CI 6,82-11,1).</span></span></p>

<p><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>Conclusion</strong>: In this specific population NTproBNP, alkaline phosphatase, uric acid and COMPERA at beggining&nbsp;were predictors of CV events.&nbsp;In line with proposed pathophysiology, PAC showed to be a marker of severity and a previously proposed cut-off of 1,39mL/mmHg revealed a positive association with prognosis not only in LES but in other CTD.</span></span></p>