Hereditary Hemorrhagic Telangiectasia (HHT, Osler-Weber Rendu Syndrome) can be the cause of sudden death. Diagnosis of HHT can be beneficial to relatives with undiagnosed disease.

Causes of sudden death associated with pulmonary disease:

(1) rupture of an arteriovenous malformation with massive hemothorax

(2) rupture of the pulmonary vein in pulmonary hypertension


Causes of sudden death associated with CNS disease:

(1) intracranial hemorrhage

(2) brain abscess

(3) epilepsy secondary to cortical scarring


Other causes of sudden death:

(1) massive gastrointestinal bleeding from a ruptured arteriovenous malformation

(2) rupture of a large hepatic arteriovenous malformation

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