SPC365

Causes of death in pulmonary arterial hypertension patients: is there something else besides the right ventricle?

Session: Posters - F. Valvular, Myocardial, Pericardial, Pulmonary, Congenital Heart Disease
Speaker: Bárbara Marques Ferreira

Congress: CPC 2021
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: Posters
FP Number: ---
Authors: Bárbara Marques Ferreira; Filipa Ferreira; Sofia Alegria; Ana Rita Pereira; Alexandra Briosa; Débora Repolho; Ângela Manuel; Maria José Loureiro; João Grade Santos; Mariana Martinho; Ana Marques; Daniel Sebaiti; Hélder Pereira

<p><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-family:&quot;Calibri&quot;,sans-serif">Background: Pulmonary arterial hypertension (PAH) has great morbidity and mortality. Despite improvements in the diagnosis and management of PAH over the past 2 decades with the introduction of targeted medical therapies leading to improved survival, the disease continues to have a poor long-term prognosis. It is reasonable to postulate that, with improved survival, not all patients die from right ventricular (RV) failure. The direct cause of death in PAH patients is not well studied.</span></span></span></p>

<p><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-family:&quot;Calibri&quot;,sans-serif">Objective: to identify the main cause that led to the death of patients with PAH in a tertiary care center in the modern era and to investigate clinical context and treatment in this patient population before death.</span></span></span></p>

<p><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-family:&quot;Calibri&quot;,sans-serif">Methods: Retrospective single-center study of consecutive deceased patients (pts) with a diagnosis of PAH confirmed by right heart catheterization who were regularly followed in our center between 2008 and 2020. Data were collected concerning last clinical evaluation before death. The clinical circumstances surrounding the deaths were analyzed. </span></span></span></p>

<p><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-family:&quot;Calibri&quot;,sans-serif">Results: From 82 pts followed in our referral center with PAH, a total of 29 patients died (mean age at diagnosis 48&plusmn;16 years; 62,1% female). Median time from diagnosis to death was 3,0 years (range 0 to 35). Word Health Organization functional class at the time of last clinic visit was either III or IV in 65% patients and more than a half (55%) had physical findings suggestive of right heart failure. Right ventricle was severely dilated in 34,5% with impaired systolic function in 55,2%. Ten patients (34,5%) were on oxygen, all patients were under pulmonary vasodilator therapy (PVT), that included combined therapy in 76% and parental prostacyclin analogues in 46,2%. Two patients died waiting for lung transplant. </span></span></span></p>

<p><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-family:&quot;Calibri&quot;,sans-serif">The causes of death were divided as follow: G1- Directly related to PAH (72,4%): 16 pts with progressive right heart failure and 5 pts with sudden death; G2- related to an intercurrent illness in which PAH contributed to the death (10,3%); G3 &ndash; Unrelated to PAH (13,8%). The final cause of death could not be adequately assessed in 1 patient. </span></span></span></p>

<p><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-family:&quot;Calibri&quot;,sans-serif">As expected, pts in G1 had more severe disease than G3, by multiparametric prognostic factors specified in table 1.</span></span></span></p>

<p><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-family:&quot;Calibri&quot;,sans-serif">Importantly, 79,3% of the patients that died directly from PAH were not in high-risk in last clinical visit assessed by COMPERA score. </span></span></span></p>

<p><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-family:&quot;Calibri&quot;,sans-serif">Conclusion- In the vast majority of our PAH patients, the disease contributed to their death. Right ventricular failure or sudden death was the sole cause of death in 72,4%. </span></span></span><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-family:&quot;Calibri&quot;,sans-serif">Near 80% of these patients were not in high-risk. </span></span></span><span style="font-size:12pt"><span style="font-family:&quot;Times New Roman&quot;,serif"><span style="font-family:&quot;Calibri&quot;,sans-serif">More studies are needed to identify pts at risk of death related to PAH in patients with low or intermediate risk. </span></span></span></p>