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Semin intervent Radiol 2024; 41(04): 325-335
DOI: 10.1055/s-0044-1791186
Review Article

Medical and Interventional Management of Hereditary Hemorrhagic Telangiectasia

Jeffrey M. Lynch 
1   Department of Radiology, University of Arkansas for Medical Sciences College of Medicine, Little Rock, Arkansas
,
Elizabeth Stevens
1   Department of Radiology, University of Arkansas for Medical Sciences College of Medicine, Little Rock, Arkansas
,
Mary E. Meek 
1   Department of Radiology, University of Arkansas for Medical Sciences College of Medicine, Little Rock, Arkansas
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Abstract

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder of the blood vessels that leads to the formation of telangiectasias and arteriovenous malformations (AVMs). HHT affects ∼1/5,000 people, but this varies significantly by geography and ancestry. The Curaçao criteria for HHT consist of four diagnostic criteria: spontaneous epistaxis, first-degree relative with HHT, AVMs in characteristic location (liver, lung, brain), and telangiectasias. Sequelae and major symptomology include recurrent epistaxis, dyspnea, heart failure, and stroke from paradoxical emboli among others. HHT patients are best cared for by a multidisciplinary team, ideally all with HHT-specific experience, but in this review, we will discuss the major aspects of the disease including etiology, diagnosis, and treatment recommendations.

Keywords

hereditary hemorrhagic telangiectasia - vascular malformations - interventional radiology


Publication History

Article published online:
07 November 2024

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