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  • Review Article
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Imaging modalities for the diagnosis of pulmonary hypertension in systemic sclerosis

Nature Reviews Rheumatology volume 8, pages 203–213 (2012)Cite this article

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Abstract

Patients with systemic sclerosis (SSc) are at considerable risk of developing pulmonary arterial hypertension (PAH). PAH has a dramatic impact on the natural history of the disease and overall survival of the patient. Despite progress made in elucidating the pathogenesis of PAH and introduction of novel therapies, SSc-related PAH (SScPAH) remains a devastating disease that responds poorly to therapy. Although early diagnosis is of paramount importance, there are no available validated strategies for assessing SScPAH because reliable evaluation of the structure and function of the right ventricle is difficult owing to its complex geometry. Additionally, myocardial fibrosis might affect cardiac contractility and contribute to heart failure. Modern imaging modalities, such as novel echocardiographic techniques and cardiac MRI, are highly sensitive, quantitative and reproducible methods that allow noninvasive assessment of regional and global myocardial performance without relying on geometric assumptions. In this Review, we examine the imaging modalities currently available, focusing on evolving diagnostic imaging methodologies and their possible clinical implications in the SScPAH setting.

Key Points

  • Systemic sclerosis-related pulmonary arterial hypertension (SScPAH) is a devastating complication of systemic sclerosis (SSc) that increases morbidity and mortality

  • The accurate and reliable assessment of right ventricular function and morphology with noninvasive modalities is important for the diagnosis and the overall management of the disease

  • Echocardiography is a simple, safe, cheap and easily reproducible diagnostic tool for screening patients with SSc who are at high risk of developing PAH

  • Tissue Doppler and strain rate imaging can detect abnormalities of systolic and diastolic ventricular function in asymptomatic patients with SSc

  • Cardiac MRI offers a quantitative and comprehensive approach to assess the function and extent of right ventricular remodeling in patients with SScPAH, with potential future clinical applications in early diagnosis and patient monitoring

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Figure 1: Conventional and tissue Doppler echocardiography for the assessment of SScPAH.
Figure 2: Four-chamber steady state free precession cardiac MRI sequence for the assessment of biventricular function in a patient with systemic sclerosis-related pulmonary arterial hypertension.
Figure 3: Schematic summary of pathogenetic mechanisms involved in SScPAH and the imaging modalities used for their diagnostic assessment.

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Acknowledgements

The authors thank Drs George Giannakoulas and Vasilis Kamperidis for ultrasound images from their archives. T. Dimitroulas is supported by the Rheumatology Society of Northern Greece and Hellenic Society for Rheumatology.

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Authors and Affiliations

  1. Department of Rheumatology, Dudley Group NHS Foundation Trust, Russells Hall Hospital, DY1 2HQ, Dudley, UK

    Theodoros Dimitroulas

  2. Onassis Cardiac Surgery Center, 50 Esperou Street, P. Faliro, 175-61, Athens, Greece

    Sophie Mavrogeni

  3. Arthritis Research UK Epidemiology Unit, School of Translational Medicine, University of Manchester, Rutherford House, Manchester Science Park, 40 Pencroft Way, M15 6SZ, Manchester, UK

    George D. Kitas

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T. Dimitroulas was responsible for researching the data and writing the article. S. Mavrogeni was responsible for researching the data and writing the article and contributed to the discussion of content. G. D. Kitas contributed to the discussion of content and was responsible for reviewing the manuscript before submission.

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Correspondence to Sophie Mavrogeni.

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Dimitroulas, T., Mavrogeni, S. & Kitas, G. Imaging modalities for the diagnosis of pulmonary hypertension in systemic sclerosis. Nat Rev Rheumatol 8, 203–213 (2012). https://doi.org/10.1038/nrrheum.2012.2

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