Multimodal treatment strategies for chronic thromboembolic pulmonary hypertension: a tertiary referral center perspective

Introduction

Chronic thromboembolic pulmonary hypertension (CTEPH) is a severe and potentially curable subtype of pulmonary hypertension (PH), causing by persistent pulmonary vascular obstruction by organized thromboembolic material. Tertiary referral centers play a crucial role in advanced diagnostic evaluation and selection of targeted therapeutic strategies.

Aim

To describe the clinical profile and therapeutic management of patients with CTEPH followed at a tertiary referral center.

Methods

Retrospective analysis of consecutive patients with established CTEPH under regular follow-up at a specialized PH center. Demographic characteristics, risk factors for chronic thromboembolic disease, NT-proBNP, hemodynamic parameters, and imaging findings were collected. Pharmacological therapy, interventional procedures such as balloon pulmonary angioplasty (BPA) and pulmonary endarterectomy (PEA) were evaluated.

Results

Fifty patients were evaluated, of which 68% were female. The mean age was 66.7 ± 13.9 years. Five patients died (mean age 69.6 ± 8.4 years), with one death related to a PEA complication.

Acute PE was the most prevalent risk factor (78%), followed by deep vein thrombosis (DVT) (32%) and 26% of patients had a history of both. Other prevalent risk factors were cancer (18%) and coagulopathy (18%), with the antiphospholipid syndrome (APS) being the most frequent coagulopathy.

64% of patients were on medical therapy (MT): 34% on single MT, 26% on double MT and 2% under triple MT. Riociguat was the most used. 34% underwent BPA and 40% underwent PEA. Five patients underwent to both PEA and BPA and were also underTM.

At baseline, most patients presented with elevated NT-proBNP, evidence of right ventricular (RV) overload – 51% RV dilatation, 38% D-shape left, ventricle, mean VD-AD 53±28.7 cm2 - and elevated pulmonary pressures - mean PSAP 57.3±29.9 mmHg, mean mPAP 41.2±13.6 mmHg.

During follow-up, significant improvements were observed in all echocardiographic and invasive hemodynamic parameters previous mentioned, with statistical significance (p< 0.05).

Conclusion

Patients with CTEPH at a tertiary referral center frequently present with advanced disease, requiring comprehensive assessment and access to specialized therapeutic options. These findings highlight the importance of early diagnosis and referral to experienced CTEPH centers to optimize outcomes.For image description, please refer to the figure legend and surrounding text.