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The Unusual Suspect – is Pulmonary Veno-Occlusive Disease underrecognized?
Session:
Sessão de Posters 06 - Hipertensão Pulmonar
Speaker:
Daniel Inácio Cazeiro
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:
Daniel Inácio Cazeiro; Miguel Azaredo Raposo; Catarina Simões de Oliveira; Ana Margarida Martins; Ana Beatriz Garcia; Ana Mineiro; Catarina Resende; Tatiana Guimarães; Susana Robalo Martins; Nuno Lousada; Fausto J. Pinto; Rui Plácido
Abstract
<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><strong>Introduction: </strong></span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000">Pulmonary hypertension (PH) with features of venous involvement (PVOD) is a rare, rapidly progressive type of PH, often underrecognized because of its nonspecific presentation. However, some clinical and diagnostic exam findings may allow a presumptive diagnosis (dx) of PVOD. Due to its lack of specific treatment, prognosis is generally poor.</span></span></span></p> <p> </p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><strong>Purpose</strong></span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000">: To characterize a population with probable/definitive dx of PVOD.</span></span></span></p> <p> </p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><strong>Methods</strong></span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000">: Retrospective single-center study which included patients (pts) from 2011 to 2023, with a dx of pre-capillary PH and findings suggestive of PVOD. Clinical and exam data were collected from pts’ records. A descriptive analysis was performed. Survival was analyzed using Kaplan Meier curves and log rank tests.</span></span></span></p> <p> </p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><strong>Results</strong></span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000">: A total of 17 pts were included (53% male), with mean age of 58.8</span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><span style="background-color:#ffffff">±</span></span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000">14.1 years (y). 65% of pts presented ≥1 risk factor for PVOD (associated mutation, systemic sclerosis or previous exposure to tobacco, chemotherapy, or organic solvents). Most were on functional class (FC) III. Median NTproBNP level was 1719 pg/mL and mean 6-minute walking test distance (6MWTD) was 259</span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><span style="background-color:#ffffff">±</span></span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000">157m, with mean O</span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><sub>2</sub></span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"> saturation nadir of 83</span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><span style="background-color:#ffffff">±5%</span></span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000">. </span></span></span></p> <p> </p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000">Mean echocardiographic values revealed right ventricular dilatation (basal diameter</span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><span style="background-color:#ffffff"> of </span></span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000">50</span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><span style="background-color:#ffffff">±6mm)</span></span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"> and dysfunction (</span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><span style="background-color:#ffffff">TAPSE of </span></span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000">15</span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><span style="background-color:#ffffff">±4mm and s’ of </span></span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000">9</span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><span style="background-color:#ffffff">±2 cm/s).</span></span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"> Median mean pulmonary artery pressure (PAP) was 43mmHg, with high pulmonary vascular resistance and low cardiac index (10</span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><span style="background-color:#ffffff">±7 WU and 2.0±0.5 L/min/m</span></span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><span style="background-color:#ffffff"><sup>2</sup></span></span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000">). </span></span></span></p> <p> </p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000">On chest imaging, 94%, 65% and 18% of pts presented 1, 2 or 3 signs of PVOD, respectively (mediastinal lymphadenopathy, septal lines, and ground glass opacities). Regarding pulmonary function tests, lung volumes were normal, and mean diffusion capacity for carbon monoxide was severely reduced (38</span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><span style="background-color:#ffffff">±19%). </span></span></span></span></p> <p> </p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><span style="background-color:#ffffff">Most pts were started on vasodilators. There were no significant clinical or hemodynamic improvements. Three pts experienced clinical worsening after drug initiation (1 developed acute pulmonary edema). From a total of 6 referrals, 2 pts (12%) were submitted to lung transplant. Median follow-up time was 13 months (mo). Nine pts were hospitalized, and 9 pts died (53%). Mean time to death was 21±17 mo. Pts with </span></span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000">≥</span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><span style="background-color:#ffffff">2 radiological signs, TAPSE<17mm, TAPSE/systolic PAP ratio <0.27 or hospitalizations had a higher death rate.</span></span></span></span></p> <p> </p> <p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><span style="background-color:#ffffff"><strong>Conclusion</strong></span></span></span></span><span style="font-size:12pt"><span style="font-family:Calibri,sans-serif"><span style="color:#000000"><span style="background-color:#ffffff">: PVOD is a rare form of PH, with a challenging dx and poor prognosis. Prompt clinical recognition and early referral for transplant are crucial for a successful outcome.</span></span></span></span></p>
Slides
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