Table 2.

Summary of clinical manifestations and their repercussions in patients with HHT [34,36].

Clinical Manifestations	Prevalence (%)	Comments
Epistaxis	90–95	Most limiting symptom for patients.
Telangiectasias	95	It can produce recurrent bleeding in the bearing areas of the body or those in contact with external surfaces such as the fingertips.
Anemia	50	It is associated with asthenia and chronic fatigue.
Pulmonary AVMs	15–50	Chronic hypoxaemia is only present in case of large pulmonary AVMs. Prevalence 10–20% in HT-II, 60% in HHT-I.
Hepatic AVMs	47–74	Three different types. Depending on their predominance, they increase the risk of HOHF, portal hypertension, hepatic encephalopathy, biliary ischemia, mesenteric ischemia, and hepatic cirrhosis.
Cerebral AVMs	2–20	Nonspecific symptoms (headaches or seizures)
Digestive AVMs	13–30	AVMs predominate in the stomach and duodenum.
Pulmonary hypertension	1–5	Can be caused by different mechanisms including hereditary group 1 PAH, or due to high cardiac output in the setting of liver AVMs (mostly associated with ACVRL1 mutation).

AVMs, arteriovenous malformation; HOHF, high output heart failure; PAH, pulmonary arterial hypertension.