Table 3.

Therapeutic approach to HHT according to the different clinical manifestations that may occur [34,37].

Manifestations	Treatment	Comments
Epistaxis	Moisturizing (Topical hydration) Use of oral tranexamic acid if continuous bleeding despite moisturizing topical therapies	Recent evidence shows no superiority on all topical therapy (tranexamic acid, estrogens, propranolol, and bevacizumab) compared to placebo.
	Ablative treatments (laser, radiofrequency ablation, electrosurgery and sclerotherapy). Systemic therapy could be considered: beta blockers, thalidomide, tacrolimus. Antiangiogenics (Bevacizumab)	Consider if epistaxis persists despite topical treatments. Consider systemic therapy before surgery.
	Septodermoplasty	Consider in patients who do not respond to previous treatments.
Digestive bleeding	Endoscopic procedures are diagnostic and therapeutic. Consider capsule endoscopy if endoscopic bleeding is not identified.	Repeat sessions are discouraged to avoid repeated iatrogenic injury to the intestinal mucosa.
	In mild cases, oral antifibrinolytics may be considered.
	If, despite previous treatments, bleeding persists, anemia requiring transfusions, antiangiogenic drugs (bevacizumab) can be initiated.
Anemia	Oral ironIV iron if intolerant or lack of response to oral iron. Red blood cell transfusion.	A usual dose of 35 mg elemental iron tablets daily indicated.
Pulmonary AVMs	Transcatheter embolization: Consider in any AVM with afferent vessel >2 mm in diameter.
	Chest CT is recommended to identify possible recanalization.	Follow-up with CT scan after embolization every 6 months, then every 3–5 years.
Cerebral abscess	If TTCE identifies the presence of a short circuit (although pulmonary AVM is not identified in CT): Antibiotic prophylaxis is recommended prior to dental procedures. Avoid administering air bubbles when cannulating veins.
Pulmonary hypertension	Extend study to identify primary cause and address management (multidisciplinary consultation).
Hepatic VMs	Most patients with symptomatic hepatic AVMs can be managed with medical treatment. Consider bevacizumab for patients who fail medical treatment.
	Refer to referral center to consider liver transplantation in patients with refractory symptomatic hepatic AVMs (HOHF, biliary ischemia, or complicated portal hypertension).	Liver biopsies should be avoided in patients with HHT.
Cerebral AVMs	Treated depending on risk of bleeding and expertise of the neurosurgical team. Embolization or stereotactic radiosurgery depending on the size, location, and symptomatology.

AVMs, arteriovenous malformation; VM, vascular malformation; HOHF, high output heart failure; TTCE, transthoracic contrast echocardiography; CT, contrast tomography.