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Connective Tissue Disease and Pulmonary Hypertension – an ominous association
Session:
Sessão de Posters 06 - Hipertensão Pulmonar
Speaker:
Inês Caldeira Araújo
Congress:
CPC 2024
Topic:
I. Hypertension
Theme:
21. Pulmonary Circulation, Pulmonary Embolism, Right Heart Failure
Subtheme:
21.2 Pulmonary Circulation, Pulmonary Embolism, Right Heart Failure – Epidemiology, Prognosis, Outcome
Session Type:
Cartazes
FP Number:
---
Authors:
Inês Caldeira Araújo; Catarina Simões de Oliveira; Ana Beatriz Garcia; Ana Margarida Martins; Pedro Alves da Silva; Miguel Raposo; Catarina Gregório; Tatiana Guimarães; Rui Plácido; Nuno Lousada; Susana Martins; Fausto J. Pinto
Abstract
<p><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong><span style="font-size:10.5pt"><span style="font-family:CalibriBold">Introduction</span></span></strong><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">: Pulmonary hypertension (PH) is a severe complica</span></span><span style="font-size:10.5pt">ti</span><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">on of connec</span></span><span style="font-size:10.5pt">ti</span><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">ve </span></span><span style="font-size:10.5pt">ti</span><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">ssue disease (CTD) and CTD associated pulmonary arterial hypertension (PAH) is the second most common cause of group 1 PH.</span></span></span></span></p> <p><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong><span style="font-size:10.5pt"><span style="font-family:CalibriBold">Purpose</span></span></strong><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">: To characterize patients with CTD associated PAH and to determine predictors of mortality.</span></span></span></span></p> <p><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong><span style="font-size:10.5pt"><span style="font-family:CalibriBold">Methods</span></span></strong><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">: Observa</span></span><span style="font-size:10.5pt">ti</span><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">onal single centre retrospec</span></span><span style="font-size:10.5pt">ti</span><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">ve study including pa</span></span><span style="font-size:10.5pt">ti</span><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">ents followed in a reference hospital for PH, who fulfilled basic requirements of RHC and echo at beginning and more than 3 appointments.</span></span></span></span></p> <p><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong><span style="font-size:10.5pt"><span style="font-family:CalibriBold">Results</span></span></strong><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">: We iden</span></span><span style="font-size:10.5pt">ti</span><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">fied 48 patients with CTD and associated PH followed during 9,1 ± 4,3 years. Mean age was 65,25 ± 14,4 years and female per male ra</span></span><span style="font-size:10.5pt">ti</span><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">o was 10:1; regarding CTD ae</span></span><span style="font-size:10.5pt">ti</span><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">ology, systemic sclerosis was the most prevalent (52,1%), followed by systemic lupus erythematous</span></span></span></span></p> <p><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">(22,6%) and limited systemic sclerosis (10,1%). Mean time between CTD diagnosis and beginning of PH follow-up was 5,25 years. </span></span></span></span></p> <p><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">Most pa</span></span><span style="font-size:10.5pt">ti</span><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">ents were classified as having pre-capillary hypertension (93,3%). As expected, most were under specific PH therapy, of which more than half had double (57,5%) and triple (17,5%) therapy; PDE5 inhibitors were prescribed in 35 pa</span></span><span style="font-size:10.5pt">??</span><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">ents (87,5%), endothelin receptor antagonists in 31 pa</span></span><span style="font-size:10.5pt">ti</span><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">ents (64,6%) and prostanoid analogs in 11 pa</span></span><span style="font-size:10.5pt">ti</span><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">ents (22%). Concomitant drug therapy for CTD was highly prevalent, as more than 80% were under corticotherapy and 57% with immunosuppressants. </span></span></span></span></p> <p><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">Almost all pa</span></span><span style="font-size:10.5pt">ti</span><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">ents were symptoma</span></span><span style="font-size:10.5pt">ti</span><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">c (95,6%) as dyspnoea (87,5%), fa</span></span><span style="font-size:10.5pt">ti</span><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">gue (75%) and chest pain (12%) were the most frequent complaints. Interstitial lung disease associated with CTD was present in 25% patients and 9% had serositis.</span></span></span></span></p> <p><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">Blood analysis, echo and right heart catheteriza</span></span><span style="font-size:10.5pt">ti</span><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">on parameters were performed at baseline and during follow-up. NTproBNP median was 204ng/mL and mean haemoglobin level was 12,63 ± 1,67g/dL. Mean sPAP by echocardiograpy was 58,73 ± 3,61mmHg and TAPSE was 19,72 ±</span></span></span></span></p> <p><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">0,73mm. Regarding invasive haemodynamic measurements, most pa</span></span><span style="font-size:10.5pt">ti</span><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">ents had severe PH with mean mPAP of 40,89 ± 2,32 mmHg and mean pulmonary vascular resistance of 9,84 ± 1,01mmHg.</span></span></span></span></p> <p><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">Risk stra</span></span><span style="font-size:10.5pt">ti</span><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">fication at the start of follow-up with COMPERA 4-strata analysis showed that most pa</span></span><span style="font-size:10.5pt">ti</span><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">ents were either low (39,6%) or intermediate low risk (35,4%).</span></span></span></span></p> <p><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong><span style="font-size:10.5pt"><span style="font-family:CalibriBold">Conclusion</span></span></strong><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">: CTD associated PH is an important cause of PH ranging a diverse popula</span></span><span style="font-size:10.5pt">ti</span><span style="font-size:10.5pt"><span style="font-family:CalibriRegular">on and encompassing several diseases. It is an ominous complication with an unfavourable prognosis, requiring specific therapy both for PH as for CTD as well as a thorough investigation.</span></span></span></span></p>
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