Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2020 Dec;28(12):645-655.
doi: 10.1007/s12471-020-01467-1.

The outcome of pulmonary hypertension and its association with pulmonary artery dilatation

A L Duijnhouwer  1 , J Lemmers  2 , J Smit  3 , J van Haren-Willems  4 , H Knaapen-Hans  2 , T Ten Cate  3 , W Hagmolen Of Ten Have  4 , M-J de Boer  3 , J Roos-Hesselink  5 , M Vonk  2 , A van Dijk  3
Affiliations

The outcome of pulmonary hypertension and its association with pulmonary artery dilatation

A L Duijnhouwer et al. Neth Heart J. 2020 Dec.

Abstract

Background: Pulmonary artery (PA) dilatation is often seen in pulmonary hypertension (PH) and is considered a long-term consequence of elevated pressure. The PA dilates over time and therefore may reflect disease severity and duration. Survival is related to the stage of the disease at the time of diagnosis and therefore PA diameter might be used to predict prognosis. This study evaluates the outcome of patients with pulmonary arterial hypertension (PAH) and chronic thrombo-embolic pulmonary hypertension (CTEPH) and investigates whether PA diameter at the time of diagnosis is associated with mortality.

Methods: Patients visiting an outpatient clinic of a tertiary centre between 2004 and 2018 with a cardiac catheterisation confirmed diagnosis of PAH or CTEPH and a CT scan available for PA diameter measurement were included. PA diameter and established predictors of survival were collected (New York Heart Association (NYHA) class, N‑terminal pro-brain natriuretic peptide (NT-proBNP) level and 6‑min walking distance (6MWD)).

Results: In total 217 patients were included (69% female, 71% NYHA class ≥III). During a median follow-up of 50 (22-92) months, 54% of the patients died. Overall survival was 87% at 1 year, 70% at 3 years and 58% at 5 years. The mean PA diameter was 34.2 ± 6.2 mm and was not significantly different among all the diagnosis groups. We found a weak correlation between PA diameter and mean PA pressure ( r = 0.23, p < 0.001). Male sex, higher age, shorter 6MWD and higher NT-proBNP level were independently associated with mortality, but PA diameter was not.

Conclusion: The prognosis of PAH and CTEPH is still poor. Known predictors of survival were confirmed, but PA diameter at diagnosis was not associated with survival in PAH or CTEPH patients.

Keywords: Dilatation; Hypertension; Pulmonary; Pulmonary artery.

PubMed Disclaimer

Conflict of interest statement

A.L. Duijnhouwer, J. Lemmers, J. Smit, J. van Haren-Willems, H. Knaapen-Hans, T. ten Cate, W. Hagmolen of ten Have, M.-J. de Boer, J. Roos-Hesselink, M. Vonk and A. van Dijk declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Example of a measurement of the main pulmonary artery in the transverse plane at the level of the main pulmonary artery on CT scan
Fig. 2
Fig. 2
Survival curves of the four investigated pulmonary hypertension groups. IPAH idiopathic pulmonary arterial hypertension, CTD-PH pulmonary hypertension associated with connective tissue disease, CHD-PH pulmonary hypertension associated with congenital heart disease, CTEPH chronic thromboembolic pulmonary hypertension
Fig. 3
Fig. 3
Comparison of survival curves of patients diagnosed in the periods 2008–2013 and 2013–2018
Fig. 4
Fig. 4
Forest plot showing the odds ratios for the association between the presence of a risk factor and the likelihood of dying. BSA body surface area, NYHA FC New York Heart Association functional class, PA pulmonary artery, PAP pulmonary artery pressure, NT-proBNP N-terminal pro-brain natriuretic peptide, 6MWD 6-min walking distance
See this image and copyright information in PMC

References

    1. Badesch DB, Raskob GE, Elliott CG, Krichman AM, Farber HW, Frost AE, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL registry. Chest. 2010;137(2):376–387. doi: 10.1378/chest.09-1140. - DOI - PubMed
    1. Simonneau G, Gatzoulis MA, Adatia I, Celermajer D, Denton C, Ghofrani A, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25, suppl D):34–41. doi: 10.1016/j.jacc.2013.10.029. - DOI - PubMed
    1. Peacock AJ, Murphy NF, McMurrey JJV, Caballero L, Stewart S. An epidemiological study of pulmonary arterial hypertension. Eur Respir J. 2007;30(1):104–109. doi: 10.1183/09031936.00092306. - DOI - PubMed
    1. Humbert M, Sitbon O, Chaouat A, Bertocchi M, Habib G, Gressin V, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006;173(9):1023–1030. doi: 10.1164/rccm.200510-1668OC. - DOI - PubMed
    1. Boucly A, Weatherald J, Savale L, Jaïs X, Cottin V, Prevot G, et al. Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension. Eur Respir J. 2017;50(2):1–10. doi: 10.1183/13993003.00889-2017. - DOI - PubMed