Associazione Nazionale Medici Cardiologi Ospedalieri

CONGRESS ABSTRACT

CONGRESS ABSTRACT

Multimodal therapy with drug combination in recurrent Chronic Thromboembolic Pulmonary Hypertension candidate for BPA

FORTUNATO FABRIZIO ACQUAVIVA DELLE FONTI (BARI) – OSPEDALE GENERALE REGIONALE MIULLI | GALGANO GIUSEPPE ACQUAVIVA DELLE FONTI (BARI) – OSPEDALE GENERALE REGIONALE MIULLI | D’ARMINI ANDREA MARIA PAVIA (PAVIA) – POLICLINICO SAN MATTEO | DI MARINO SERENA TARANTO (TA) – VILLA VERDE | PINTO LUIGI ACQUAVIVA DELLE FONTI (BARI) – OSPEDALE GENERALE REGIONALE MIULLI | CACCIAPAGLIA FABIO ACQUAVIVA DELLE FONTI (BARI) – OSPEDALE GENERALE REGIONALE MIULLI | MUSCI RITA ACQUAVIVA DELLE FONTI (BARI) – OSPEDALE GENERALE REGIONALE MIULLI | SPINELLI GIOVANNA ACQUAVIVA DELLE FONTI (BARI) – OSPEDALE GENERALE REGIONALE MIULLI | GRIMALDI MASSIMO GRIMALDI ACQUAVIVA DELLE FONTI (BARI) – OSPEDALE GENERALE REGIONALE MIULLI

One treatable cause of pulmonary hypertension (PH) is chronic thromboembolic pulmonary hypertension (CTEPH). In 2022, the ESC Guidelines outlined the treatment algorithm for CTEPH, including multimodal therapy with pulmonary endarterectomy (PEA), PH drugs, and balloon pulmonary angioplasty (BPA). Pretreatment with Riociguat before BPA reduced the frequency of significant BPA-related adverse events by optimizing pre-BPA pulmonary hemodynamics. Nevertheless, the potential effects of subcutaneous Treprostinil pretreatmen has not yet documented. We describe the example of a 40-year-old patient with CTEPH who had a right PEA at the age of 20, which completely resolved the PH and caused it to disappear at follow-ups. In 2024 he was hospitalized due to severe dyspnea. Echocardiography displayed a picture of right heart failure with a dilated right ventricle, severely hypokinetic with massive tricuspid regurgitation (TAPSE/PAPs 0,12 mm/mmHg) (Figure 1). Thus, he underwent RHC, which confirmed the recurrence of severe precapillary PH (Figure 2). Riociguat and Treprostinil were started in a double combination. During follow-up, the patient showed clinical improvement on double combination therapy, with Treprostinil progressively increased to high dosages (30 ng/kg/min). In April, despite clinical stability (NYHA II, 6MWT 400 m, NT-pro-BNP 630 ng/ml), the echocardiogram remained substantially unchanged, and RHC confirmed the persistence of severe PH (Figure 2). The recurrence of PH in a patient who had previously had PEA and was on optimized medical treatment led to a referral for BPA. Following a three-stage BPA, the patient was discharged after a few days without experiencing any periprocedural issues. In October the patient was in excellent clinical condition with Riociguat (NYHA I, 6MWT 520 m, NT-pro-BNP 460 ng/ml). Furthermore, the cardiac ultrasound was improved, showing a dilated right ventricle with improved functional parameters resulting in a better RV-PA coupling (TAPSE/PAPs 0.30 mm/mmHg)  (Figure 3). Except for infusion site reaction, we found that subcutaneous Treprostinil at high dosages was safe and improved exercise capacity in individuals with severe CTEPH. The combined therapy with subcutaneous Treprostinil ensures hemodynamic and clinical stability and performs effectively as a background treatment before BPA, reducing the likelihood of side effects and fostering operational success.