SPC365

The Dual Challenge: Understanding and Managing Pulmonary Hypertension in Congenital Heart Defects

Session: Sessão de Posters 49 - Cardiopatias Congénitas
Speaker: Ana Margarida Martins

Congress: CPC 2024
Topic: F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Theme: 20. Congenital Heart Disease and Pediatric Cardiology
Subtheme: 20.6 Congenital Heart Disease – Clinical
Session Type: Cartazes
FP Number: ---
Authors: Ana Margarida Martins; Ana Beatriz Garcia; Catarina Oliveira; Daniel Cazeiro; Pedro Alves da Silva; Tatiana Guimarães; Ana Rita Francisco; Ana Mineiro; Susana Martins; Nuno Lousada; Fausto J Pinto; Rui Plácido

<p><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>Introduction</strong>:</span></span></p>

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Pulmonary arterial hypertension (PAH) is a well-known consequence of congenital heart disease (affecting 5-10% of this pts) and conveying a worse prognosis. This subgroup comprises an increasing proportion of&nbsp;PAH pts, adding complexity due to underlying anatomical and hemodynamic abnormalities. Additionally, most PAH clinical trials focus on pts with idiopathic or CTD-associated PH, with little evidence regarding CHD.</span></span></p>

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Our aim was to characterize a population with CHD and PH followed in a tertiary centre from 2012-2022&nbsp;and assess clinical, laboratory and echocardiographic parameters that could influence prognosis.</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 associated with CHD. Clinical, laboratorial, echocardiographic and invasive hemodynamic data were collected at beginning and during follow-up. Uni- and multivariate analyses were performed with Cox regression&nbsp;and Kaplan-Meier curves were used for survival analysis.</span></span></p>

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

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>Results</strong>: We analysed 30 pts with CHD-associated PH. Mean age was 53,8 &plusmn; 17 years, with female predominantly represented (63,3%). Atrial septal defect (ASD) was the most prevalent CHD (66,6% - 80% simple ASD, 15% with anomalous venous return, and 5% with patent ductus arteriosus), followed by ventricular septal defect (16,6%) and patent ductus arteriosus (10%). During a mean follow-up of 4,7 &plusmn; 3 years, 26.7 % pts died (n=8), and 46,7% were hospitalized (n=14) mostly due to heart failure. For the specified FUP, only 7 pts were deemed eligible for surgery; the remainder started PH-directed therapy: 2&nbsp;were under monotherapy, 15 had double therapy with PDE5i and endothelin receptor antagonist (ERA)&nbsp;and 6 had triple therapy including selexipag.</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=0.011), 6-minute walk test distance (6MWD) (p= 0.036), right atrial area (p= 0.021) and PSAP (p=0.045) correlated with cardiovascular events defined as a composite endpoint of death and cardiovascular admission. On multivariate analysis, only 6MWD (OR=0.99, 95%CI 0.98-0.99, p=0.029) was an independent predictor of CV outcomes.</span></span></p>

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif">Since 6MWD was the sole strongest prognostic index, we identified 270m as the best cutoff for predicting CV outcomes after ROC curve analysis.&nbsp;Kaplan-Meier analysis showed a significant difference between the two groups (Log Rank 5.45, p=0.02) (Fig.1), emphasizing its prognostic value and better tailoring the therapy for each patient.</span></span></p>

<p>&nbsp;</p>

<p style="text-align:justify"><span style="font-size:11pt"><span style="font-family:Calibri,sans-serif"><strong>Conclusion</strong>: Despite its lower representativeness in clinical trials, CHD remains an important cause for PH with a significant impact in mortality and CV admissions. Physical performance status, as evaluated by 6MWD, was seen as the most significant prognostic factor in our population, and those with less capacity displayed a far worse prognosis, reinforcing its importance in initial evaluation and need for early therapeutic intervention.</span></span></p>