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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