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. 2025 Jun 11;20(1):299.
doi: 10.1186/s13023-025-03825-x.

Patients with suspected pulmonary hypertension based on echocardiography in Behçet's disease: a 5-year follow-up study

Mustafa Ekici  1 , Alper Sarı  2 , Berkan Armağan  3 , Erdinç Ünaldı  4 , Büşra Fırlatan  4 , Gözde Sevgi Kart Bayram  4 , Buğu Bulat  4 , Uğur Nadir Karakulak  5 , Levent Kılıç  4 , Barış Kaya  5 , Ömer Karadağ  4 , Ali Akdoğan  4
Affiliations

Patients with suspected pulmonary hypertension based on echocardiography in Behçet's disease: a 5-year follow-up study

Mustafa Ekici et al. Orphanet J Rare Dis. .

Abstract

Background: This study investigates the long-term functional and transthoracic echocardiographic (TTE) progression in Behcet's disease (BD) patients with pulmonary hypertension (PH), aiming to address knowledge gaps regarding PH in BD.

Methods: This study included 17 BD patients with PH detected by TTE and 6 patients with pulmonary artery involvement but without PH from a previous study at Hacettepe University. PH was defined as an estimated systolic pulmonary artery pressure (sPAP) ≥ 40 mmHg. TTE was conducted by a cardiologist blinded to clinical information, with sPAP calculated using the simplified Bernoulli equation. Right heart catheterization was performed only in two patients who underwent pulmonary endarterectomy operations.

Results: All 23 patients were reached by telephone, and 13 of the 17 with PH attended the clinic for reevaluation and a TTE was performed. After 5 years, all patients were alive. Clinical worsening or FC progression was not observed in any patient with PH except the ones with Group IV PH. Pulmonary endarterectomy (PEA) was performed in 2 of the 4 Group IV PH patients during follow-up. Additional Group II PH diagnosed due to mitral valve disease in a patient with Group IV PH. Other than the ones who underwent PEA there were no increase in sPAP of the BD patients with PH. Among patients with PAI but without PH during first evaluation; only one was mildly symptomatic after 5 years who had a normal sPAP.

Conclusion: Progressive PH was observed only in Group IV PH patients, while other groups remained generally asymptomatic. Although the number of patients enrolled in this study is limited implementation of TEE to the follow-up of BD patients with PAI for screening and monitoring PH can be beneficial for early diagnosis and defining treatment strategy.

Keywords: Behçet's disease; Pulmonary endarterectomy; Pulmonary hypertension; Transthoracic echocardiography.

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Conflict of interest statement

Declarations. Ethics approval and consent to participate: The Ethical Committee of the Hacettepe University approved this study (approval number GO 23/184). Consent for publication: This study contains anonymized patient data. No direct patient identifiers have been provided in this manuscript. Competing interests: The authors have declared no conflicts of interest.

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