SPC365

Pulmonary hypertension in connective tissue diseases with overt of features of venous/capillaries involvement: clinical implications and prognostic impact of this phenotype.

Session: Comunicações Orais (Sessão 4) - Cardiopatias Congénitas, Doença Vascular Pulmonar e Embolia Pulmonar 1 - Foco no TEP e Hipertensão Pulmonar
Speaker: Bárbara Marques Ferreira

Congress: CPC 2022
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: Comunicações Orais
FP Number: ---
Authors: Bárbara Marques Ferreira; Filipa Ferreira; Sofia Alegria; Alexandra Briosa; Ana Rita Pereira; Débora Repolho; João Grade Santos; Mariana Martinho; Diogo Cunha; Maria José Loureiro; Hélder Pereira

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><strong><span style="background-color:white"><span style="font-family:&quot;Segoe UI&quot;,sans-serif"><span style="color:#212121">Background:</span></span></span></strong><span style="background-color:white"><span style="font-family:&quot;Segoe UI&quot;,sans-serif"><span style="color:#212121"> Pulmonary hypertension (PH) is a serious complication of connective tissue disease (CTD) and remains one of the leading causes of mortality in this subset of patients (pts). Pulmonary veno-occlusive disease (PVOD), recently reclassified as pulmonary arterial hypertension (PAH) with overt features of venous/capillaries involvement, is a subgroup of group 1 PH, which has been rarely reported in CTD pts. Due to clinical similarities between CTD-related PAH and PVOD, some pts are misdiagnosed and this could explain, in part, the worse prognosis associated with this clinical condition.</span></span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><strong><span style="background-color:white"><span style="font-family:&quot;Segoe UI&quot;,sans-serif"><span style="color:#212121">Purpose:</span></span></span></strong><span style="background-color:white"><span style="font-family:&quot;Segoe UI&quot;,sans-serif"><span style="color:#212121"> To analyze signs evocative of venous and capillary (pulmonary veno-occlusive disease/pulmonary capillary haemangiomatosis) involvement in pts with PAH associated with CTD (CTD-PAH).</span></span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><strong><span style="background-color:white"><span style="font-family:&quot;Segoe UI&quot;,sans-serif"><span style="color:#212121">Methods:</span></span></span></strong><span style="background-color:white"><span style="font-family:&quot;Segoe UI&quot;,sans-serif"><span style="color:#212121">&nbsp; We retrospectively included all pts with diagnosis of CTD-PAH&nbsp; (group 1.4.1) followed in a pulmonary hypertension referral center. Clinical parameters, pulmonary function tests and high-resolution computed tomography were reviewed looking for signs evocative of PVOD involvement: decreased diffusing capacity of the lung for carbon monoxide (DLCO) (&lt;50%), severe hypoxemia (PaO2&lt;60 mmHg without supplementary oxygen), presence of septal lines, centrilobular ground-glass opacities/nodules (CLGGO) and mediastinal lymph node enlargement (MLNE). Follow-up period was up to 10 years. Cox&#39;s proportional hazards analyses was used to determine predictors of event-free survival defined as initiation of parenteric prostanoid therapy, referral for pulmonary transplant or death.</span></span></span></span></span></p>

<p style="text-align:justify">&nbsp;</p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><strong><span style="background-color:white"><span style="font-family:&quot;Segoe UI&quot;,sans-serif"><span style="color:#212121">Results:</span></span></span></strong><span style="background-color:white"><span style="font-family:&quot;Segoe UI&quot;,sans-serif"><span style="color:#212121"> In a universe of 91 pts with PAH, we included 17 pts with the diagnosis of PAH associated with CTD. All were females, mean age 60.5&plusmn;14.8. 82.4% were in WHO functional class (FC)&ge;III. Compared to all other PAH groups, CTD-PAH were older, presented higher NT proBNP, lower mean pulmonary artery pressure and lower DLCO (Table 1). DLCO was &lt;50% in 71.4%, severe hypoxemia was present in 43.8%, MLNE in 38.5%, CLGGO in 35.7% and septal lines in 28.6% of the pts. Only 4 pts did not present any finding suggestive of PVOD.</span></span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="background-color:white"><span style="font-family:&quot;Segoe UI&quot;,sans-serif"><span style="color:#212121">With a mean follow-up of 4,5 years, 8 pts had no adverse event and 3 died. WHO FC, DLCO and cardiac index were predictors of adverse events in this population. Kaplan-Meier survival analyses showed that the median survival time without events was 11 months and was significantly worst in pts with at least 1 sign evocative of PVOD (log Rank p=0,028; Figure 1).</span></span></span></span></span></p>

<p style="text-align:justify"><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><strong><span style="background-color:white"><span style="font-family:&quot;Segoe UI&quot;,sans-serif"><span style="color:#212121">Conclusion: </span></span></span></strong><span style="background-color:white"><span style="font-family:&quot;Segoe UI&quot;,sans-serif"><span style="color:#212121">Signs evocative of PVOD involvement are frequent in PAH associated with CTD and their presence determine worse event-free survival.</span></span></span></span></span></p>