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Influence of long-standing pulmonary arterial hypertension and its severity on pulmonary artery aneurysm development

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Abstract

Pulmonary artery aneurysm (PAA) is a common finding in patients with long-term pulmonary arterial hypertension (PAH). The influence of PAH severity in the development of PAA remains unclear. We sought to determine whether PAA development is related to PAH severity and whether treatment optimization based on risk profile estimation is effective to stop pulmonary artery (PA) enlargement. This is a retrospective study of 125 PAH patients who underwent an imaging test (computed tomography or magnetic resonance) combined with a right heart catheterization within a six-month period. A multivariate analysis was performed to identify independent risk factors for PAA. Patients who underwent an additional imaging-test and RHC during follow-up were analyzed to evaluate changes on PA dimensions. PAA was diagnosed in 42 (34%) patients. PAA was more frequent in patients with congenital heart disease and toxic oil syndrome. PAH time-course showed to be an independent risk factor for PAA (HR 1.051, 95% CI 1.013–1.091, p = 0.008) whereas PAH severity did not. Twenty-six patients underwent a follow-up imaging-test and catheterization. After treatment optimization, a non-significant reduction of mean PA pressure was observed (58.5 mmHg [43.5–70.8] vs. 55.5 mmHg [47.5–66.3], p = 0.115) and a higher proportion of patients achieved a low-risk profile (19% vs. 35%, p = 0.157). However, the PA diameter significantly increased (40.4 ± 10.1 mm vs. 42.1 ± 9.6 mm; p = 0.003). PAA is a common condition in long-standing PAH but its development is not necessarily related to PAH severity. Despite stabilization after treatment optimization, a progressive PA dilatation was observed.

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Abbreviations

CT:

Computed tomography

CTD:

Connective tissue disease

CHD:

Congenital heart disease

MR:

Magnetic resonance

PA:

Pulmonary artery

PAA:

Pulmonary artery aneurysm

PAH:

Pulmonary arterial hypertension

RHC:

Right heart catheterization

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Acknowledgements

JN, JMMC and PES contributed to the conception and design of the study, drafting and final approval of the manuscript. AL, CJLG, MTVM, SH, SAC, YRO, JFD and FAY contributed to drafting and final approval of the manuscript. Figure 4b, c was taken from [20] and reproduced with the permission of the copyright holder (Biomed Central). Funding for this study was supplied by the Instituto de Salud Carlos III, Spanish Ministry of Science, Innovation and Universities, through the CIBER de enfermedades CardioVasculares (CIBERCV), Spain (CB16/11/00502). medicidiom.com was responsible for the English language review.

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Authors and Affiliations

  1. CIBER de Enfermedades Cardiovasculars (CIBERCV), Madrid, Spain

    Jorge Nuche, Carmen Jiménez López-Guarch, Juan F. Delgado, Fernando Arribas Ynsaurriaga & Pilar Escribano Subías

  2. Department of Cardiology, Hospital Universitario 12 de Octubre, Instituto de Investigación Sanitaria Hospital 12 de Octubre (imas12), Avenida de Córdoba s/n, 28041, Madrid, Spain

    Jorge Nuche, Ana Lareo, Sergio Huertas, Carmen Jiménez López-Guarch, Maite Velázquez Martín, Juan F. Delgado, Fernando Arribas Ynsaurriaga & Pilar Escribano Subías

  3. Centro Nacional de Investigaciones Cardiovasculares, Madrid, Spain

    Jorge Nuche & Sergio Huertas

  4. Department of Cardiology, Leiden University Medical Center, Leiden, The Netherlands

    José Manuel Montero-Cabezas

  5. Facultad de Medicina, Universidad Complutense de Madrid, Madrid, Spain

    Jorge Nuche, Carmen Jiménez López-Guarch, Juan F. Delgado, Fernando Arribas Ynsaurriaga & Pilar Escribano Subías

  6. Deparment of Radiology, Hospital Universitario 12 de Octubre, Madrid, Spain

    Sergio Alonso Charterina & Yolanda Revilla Ostolaza

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  1. Jorge Nuche
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  2. José Manuel Montero-Cabezas
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  3. Ana Lareo
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  9. Juan F. Delgado
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  11. Pilar Escribano Subías
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Correspondence to Pilar Escribano Subías.

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Nuche, J., Montero-Cabezas, J.M., Lareo, A. et al. Influence of long-standing pulmonary arterial hypertension and its severity on pulmonary artery aneurysm development. Heart Vessels 35, 1290–1298 (2020). https://doi.org/10.1007/s00380-020-01600-5

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