Ehlers-Danlos syndrome.
We have described a patient with type 4 Ehlers-Danlos syndrome who died of a ruptured pulmonary artery. Of the six subtypes of Ehlers-Danlos syndrome, which can usually be clinically differentiated, only types 1 and 4 appear to be associated with a substantial risk of arterial rupture.
D000328:Adult; D004535:Ehlers-Danlos Syndrome / Q000150:complications*; D005260:Female; D006801:Humans; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000209:etiology*; D011651:Pulmonary Artery; D012422:Rupture, Spontaneous