Definition of HHT
Understanding HHT | The facts
NOSEBLEEDS
90-95%
Of individuals with HHT will have nosebleeds by adulthood, but severity varies from infrequent and minor to daily and severe.
TELANGIECTASES
90-95%
Develop at least a few telangiectases (small red or purple spots) on the skin of the face and/or hands by middle age.
GASTRO-INTESTINAL BLEEDING
20-25%
Develop significant gastric or intestinal bleeding, but rarely before 50 years of age unless affected with juvenile polyposis in conjunction with HHT.
BRAIN AVMs
5-20%
Have at least one brain AVM. These can be present at any time in life, even at birth, and can cause serious complications. Routine screening is recommended.
LIVER AVMs
10%
Liver AVMs are relatively common, but only approximately 10% cause symptoms.
Abnormal blood vessels in the liver are relatively common. Most cause no symptoms, however high cardiac output resulting in possible cardiac failure has been linked to liver HHT. Biopsy or treatment should rarely be done; specifically, embolisation should not be done.
An Overview of HHT | By Prof Claire Shovlin
VASCERN, the European Reference Network for Rare Multisystemic Vascular Diseases is dedicated to improving the lives of patients with rare vascular diseases across Europe.
Our main goal is to provide highly specialized and integrated healthcare to patients with rare vascular diseases. We bring together a multidisciplinary team of experts from across Europe with extensive experience in rare vascular diseases, including specialists in cardiology, genetics, otorhinolaryngology, pediatrics, and many others.
Pro. Claire L. Shovlin is Chair of VASCERN HHT and Lead Clinician VASCERN HHT in London.
Document for your doctor
Should your doctor not understand HHT, we recommend you download the following fact sheet and bring with you to your next visit.
This document offers a quick view of the most common facts of HHT.
Download the fact sheet here: Fact-Sheet-2020
