Links Between Strokes and Hereditary Hemorrhagic Telangiectasia: A Population-Based Study

Area of Science:

• Vascular Medicine
• Neurology
• Epidemiology

Background:

• Hereditary Hemorrhagic Telangiectasia (HHT) is a genetic disorder characterized by abnormal blood vessel formation.
• HHT patients exhibit a predisposition to various types of strokes.
• The study aimed to evaluate stroke incidence in Albertans with HHT.

Purpose of the Study:

• To assess and compare the yearly stroke incidence in individuals with HHT versus the general population in Alberta.
• To investigate stroke risk factors and subtypes in the HHT population.
• To determine the prevalence of HHT in Alberta.

Main Methods:

• Utilized population-based administrative health data from inpatient and ambulatory admissions over 16 years.
• Employed International Classification of Diseases (ICD)-9 and ICD-10 codes for data extraction.
• Analyzed stroke occurrence by age, gender, stroke subtype, and HHT diagnosis.

Main Results:

• The age-standardized stroke incidence rate in HHT patients was 450 per 100,000, versus 260 per 100,000 in the general population (rate ratio 1.73).
• HHT prevalence in Alberta was found to be 1 in 3800, higher than the global average of 1 in 5000.
• A 3.25:1 female gender preponderance was observed in HHT yearly incidence; acute ischemic strokes and transient ischemic attacks were more common than hemorrhage.

Conclusions:

• HHT patients face a significantly elevated risk of stroke, with quantified risk metrics specific to Alberta.
• Alberta exhibits a higher disease burden of HHT than anticipated, with a notable impact on women.
• Patients diagnosed with HHT should be referred to specialized Centers of Excellence for comprehensive care.