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Non-aortic cardiovascular disease in Marfan syndrome: a nationwide epidemiological study

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Clinical Research in Cardiology Aims and scope Submit manuscript

Abstract

Objectives

Studies indicate that other cardiovascular problems than aortic disease are a burden for patients with Marfan syndrome (MFS). The aim of the study was to assess the extent of this issue.

Methods

A registry-based population study of patients with a Ghent II verified MFS diagnosis. Each patient was matched with up to 100 controls on age and sex. From the Danish healthcare system, we identified 407 MFS patients (from 1977 to 2014) and their cardiovascular events and compared them with those in 40,700 controls. Total follow-up time was 16,439 person years.

Results

Mitral valve disease was significantly more common in MFS [HR: 58.9 (CI 38.1–91.1)] and happened earlier and more often in women than men with MFS [age at first registration: 22 vs. 38 years, HR: 2.1 (CI 1.0–4.4)]. Heart failure/cardiomyopathy was also more common in MFS [HR: 8.7 (CI 5.7–13.4)] and men were more affected than women, and at younger age [39 vs. 64 years, HR: 0.18 (CI 0.06–0.55)].

In all cases, atrioventricular block [HR: 4.9 (1.5–15.6)] was related to heart surgery. Supraventricular [HR: 9.7 (CI 7.5–12.7)] and ventricular tachycardia [HR: 7.7 (CI 4.2–14.3)] also occurred more often than in the control group. The risk of sudden cardiac death was increased [HR: 8.3 (CI 3.8–18.0)] but the etiology was unclear due to lack of autopsies.

Conclusion

Non-aortic cardiovascular disease in patients with MFS is exceptionally prevalent and the range of diseases varies between women and men. Physicians caring for MFS patients must be aware of this large spectrum of cardiovascular diseases.

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Availability of data and materials and code availability

The information used in the analyses combine Danish administrative registers (as described in the paper). The data use is subject to the European Union’s General Data Protection Regulation (GDPR). The data are physically stored on computers at Statistics Denmark and the data may not be transferred to computers outside Statistics Denmark. Researchers interested in obtaining access to the register data employed in this paper are required to submit a written application to gain approval from Statistics Denmark. The application must include a detailed description of the proposed project, its purpose, as well as a description of the required datasets, variables, and analysis population. Applications can be submitted by researchers who are affiliated with Danish institutions accepted by Statistics Denmark, or by researchers outside of Denmark who collaborate with researchers affiliated with these institutions.

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Funding

The study did not receive any specific funding.

Author information

Authors and Affiliations

  1. Department of Cardiology, Aalborg University Hospital, Hobrovej 18-22, 9000, Aalborg, Denmark

    Niels H. Andersen & Kristian A. Groth

  2. Department of Clinical Genetics, Aarhus University Hospital, Palle Juul-Jensens Boulevard, 8200, Aarhus, Denmark

    Agnethe Berglund

  3. Center for Rare Diseases, Department of Pediatrics, Copenhagen University Hospital, Rigshospitalet, 2100, Copenhagen , Denmark

    Hanne Hove

  4. The RAREDIS Database, Section of Rare Diseases, Department of Clinical Genetics, Copenhagen University Hospital, Rigshospitalet, 2100, Copenhagen, Denmark

    Hanne Hove

  5. Department of Endocrinology, Aarhus University Hospital, Palle Juul-Jensens Boulevard, 8200, Aarhus, Denmark

    Claus H. Gravholt & Kirstine Stochholm

  6. Department of Molecular Medicine, Aarhus University Hospital, Palle Juul-Jensens Boulevard, 8200, Aarhus, Denmark

    Agnethe Berglund & Claus H. Gravholt

  7. Center for Rare Diseases, Aarhus University Hospital, Palle Juul-Jensens Boulevard, 8200, Aarhus, Denmark

    Kirstine Stochholm

Authors
  1. Niels H. Andersen
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  2. Kristian A. Groth
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  4. Hanne Hove
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Contributions

All authors conceptualized and designed the study and supported the data collection. Analyses and calculations were done by Kirstine Stochholm. Niels Holmark Andersen drafted the initial manuscript. All authors reviewed and revised the manuscript.

Corresponding author

Correspondence to Niels H. Andersen.

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Conflict of interest

None to declare.

Ethical approval

The study was approved by the Scientific Ethical Committee (31422) and the Danish Data Protection Agency (2011–41-6986).

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All authors approved the final manuscript as submitted and agreed to be accountable for all aspects of the work.

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Andersen, N.H., Groth, K.A., Berglund, A. et al. Non-aortic cardiovascular disease in Marfan syndrome: a nationwide epidemiological study. Clin Res Cardiol 110, 1106–1115 (2021). https://doi.org/10.1007/s00392-021-01858-3

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  • DOI: https://doi.org/10.1007/s00392-021-01858-3

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