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87th Annual Meeting of the German Society of Oto-Rhino-Laryngology, Head and Neck Surgery

German Society of Oto-Rhino-Laryngology, Head and Neck Surgery

04.05. - 07.05.2016, Düsseldorf

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Complications and causes of death in patients with hereditary haemorrhagic telangiectasia (HHT)

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  • corresponding author Freya Droege - Klinik für Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie, Universitätsklinikum Essen, Essen, Germany
  • author Stephan Lang - Klinik für Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie, Universitätsklinikum Essen, Essen, Germany
  • author Urban Geisthoff - Klinik für Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie, Universitätsklinikum Essen, Essen, Germany

German Society of Oto-Rhino-Laryngology, Head and Neck Surgery. 87th Annual Meeting of the German Society of Oto-Rhino-Laryngology, Head and Neck Surgery. Düsseldorf, 04.-07.05.2016. Düsseldorf: German Medical Science GMS Publishing House; 2016. Doc16hno27

doi: 10.3205/16hno27, urn:nbn:de:0183-16hno278

Published: September 7, 2016

© 2016 Droege et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 License. See license information at http://creativecommons.org/licenses/by/4.0/.

Outline

Text

Background: Few studies reported a reduced life expectancy in patients with hereditary haemorrhagic telangiectasia (HHT) due to severe comorbidities as well as complications [1], [2], [3], [4].

Methods: A total of 70 relatives of deceased patients with diagnosed hereditary haemorrhagic telangiectasia (HHT) completed a questionnaire about causes of death, symptoms and comorbidities patients developed. The data was compared to the general population.

Results: The mean age at death in patients with hereditary haemorrhagic telangiectasia (HHT) was lower than in the general population (64.0 years vs. 80.3 years respectively, T-Test: 34.9, p < 0.000). In many cases arteriovenous malformations led to severe comorbidities or even death of the patients. Unexpectedly the main causes of death were cardiac failure and infectious diseases causing a septic shock. Surprisingly just 11.5% of the patients with HHT died from a severe bleeding such as epistaxis and gastrointestinal bleeding.

Discussion: Patients with hereditary haemorrhagic telangiectasia (HHT) seem to have a lower life expectancy than the general population. Diagnosing patients at an early stage could prevent comorbidities and improve survival rates.

Outline

References

1.
Donaldson JW, McKeever TM, Hall IP, Hubbard RB, Fogarty AW. Complications and mortality in hereditary hemorrhagic telangiectasia: A population-based study. Neurology. 2015;84(18):1886-93. DOI: 10.1212/WNL.0000000000001538 External link
2.
Sabbà C, Pasculli G, Suppressa P, D'Ovidio F, Lenato GM, Resta F, Assennato G, Guanti G. Life expectancy in patients with hereditary haemorrhagic telangiectasia. QJM. 2006;99(5):327-34.
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Kjeldsen AD, Vase P, Green A. Hereditary hemorrhagic telangiectasia. A population-based study on prevalence and mortality among Danish HHT patients. Ugeskr Laeger. 2000;162(25):3597-601.
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Edwards CP, de Gussem EM, Mager JJ, Westermann CJ, Faughnan ME. Life expectancy of parents with hereditary hemorrhagic telangiectasia in a Canadian population. Can Respir J. 2010;17:49A.