Chronic thromboembolic pulmonary hypertension: A 10-year analysis from a Portuguese referral center

Gonçalo Samouco; Marta Fonseca; Joana Batista Correia; Cátia Santos-Ferreira; Patrícia Marques-Alves; Rui Baptista; Graça Castro; Lino Gonçalves

doi:10.1016/j.repc.2021.05.018

Chronic thromboembolic pulmonary hypertension: A 10-year analysis from a Portuguese referral center

Rev Port Cardiol. 2022 Sep;41(9):741-748. doi: 10.1016/j.repc.2021.05.018. Epub 2022 May 25.

[Article in English, Portuguese]

Authors

Gonçalo Samouco¹, Marta Fonseca², Joana Batista Correia³, Cátia Santos-Ferreira⁴, Patrícia Marques-Alves⁴, Rui Baptista⁵, Graça Castro⁴, Lino Gonçalves⁶

Affiliations

¹ Pulmonology Department, Unidade Local de Saúde da Guarda, Portugal.
² Cardiology Department, Centro Hospitalar de Setúbal, Portugal.
³ Pulmonology Department, Centro Hospitalar de Tondela-Viseu, Portugal.
⁴ Cardiology Department, Centro Hospitalar e Universitário de Coimbra, Portugal.
⁵ Cardiology Department, Centro Hospitalar de Entre o Douro e Vouga, Santa Maria da Feira, Portugal; Univ Coimbra, Coimbra Institute for Clinical and Biomedical Research (iCBR), Faculty of Medicine, Coimbra, Portugal; Univ Coimbra, Center for Innovative Biomedicine and Biotechnology (CIBB), Coimbra, Portugal; Clinical Academic Centre of Coimbra (CACC), Coimbra, Portugal. Electronic address: rui.baptista@fmed.uc.pt.
⁶ Cardiology Department, Centro Hospitalar e Universitário de Coimbra, Portugal; Univ Coimbra, Coimbra Institute for Clinical and Biomedical Research (iCBR), Faculty of Medicine, Coimbra, Portugal; Univ Coimbra, Center for Innovative Biomedicine and Biotechnology (CIBB), Coimbra, Portugal; Clinical Academic Centre of Coimbra (CACC), Coimbra, Portugal.

PMID: 36066267
DOI: 10.1016/j.repc.2021.05.018

Abstract

Introduction and objectives: Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive, but potentially curable, form of pulmonary hypertension. Pulmonary endarterectomy (PEA) is a complex surgery that frequently achieves hemodynamic normalization and symptom resolution, although not all patients are suitable for the procedure. We aimed to assess long-term outcomes of CTEPH, namely all-cause mortality and hospital admission for decompensated heart failure, according to treatment modalities in patients who underwent PEA or non-surgical therapy.

Methods: A 10-year retrospective study of patients with CTEPH at a referral center was conducted. Forty-five patients were included and median follow-up time was 57 (IQR 24-93) months. Survival analysis was performed and a multivariate Cox regression model was used to identify independent predictors of outcomes.

Results: Patients were mostly female (59%) and mean age was 63±16 years. Two-thirds were severely symptomatic at diagnosis, with 62.2% of patients presenting in WHO functional class (WHO FC) III or IV. One-, two- and three-year survival was 93.3%, 82.4% and 75.9%, respectively. Serum BNP (HR 1.003; 95% CI: 1.001-1.005; p=0.003) and creatinine (HR 12.092; 95% CI: 1.121-130.390; p=0.040) were predictors of death. Mortality was numerically lower in those who underwent PEA (p=0.135). PEA was associated with decreased risk of the combined endpoint of all-cause mortality and hospital admission for decompensated heart failure (HR 0.198; 95% CI: 0.040-0.982; p=0.047), as were lower serum BNP (HR 1.003; 95% CI: 1.001-1.005; p=0.008) and mPAP (HR 1.073; 95% CI: 1.022-1.128; p=0.005) at diagnosis. Most patients who underwent PEA presented improved WHO FC (92.9%) and post-surgical residual pulmonary hypertension was identified in only 21.4%.

Conclusion: PEA provided a better overall prognosis than non-surgical therapy, improving symptoms and frequently achieving hemodynamic normalization, with a numerical trend for lower mortality. Higher serum BNP, creatinine and mPAP at diagnosis were independently associated with worse outcomes.

Keywords: Chronic thromboembolic pulmonary hypertension; Hipertensão pulmonar tromboembólica crónica; Prognosis; Prognóstico; Sobrevivência; Survival.