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New guidelines expand management options for hereditary hemorrhagic telangiectasia

Annals of Internal Medicine
Reuters Health - 09/09/2020 - New guidelines provide updated advice on managing epistaxis and vascular malformations and new recommendations for managing gastrointestinal (GI) bleeding, anemia, pediatric care, and pregnancy and delivery.

The expert panel behind the Second International Hereditary Hemorrhagic Telangiectasia (HHT) Guidelines extended its recommendations for epistaxis treatment to include oral tranexamic acid, ablative therapies, systemic antiangiogenic agents and surgical approaches when epistaxis does not respond to moisturizing topical therapies.

Patients with suspected HHT-related GI bleeding should first undergo esophagogastroduodenoscopy; if this does not reveal significant HHT-related telangiectasia, capsule endoscopy should be considered, say Dr. Marie E. Faughnan of St. Michael's Hospital, in Toronto, Canada and colleagues.

Treatment for HHT-related GI bleeding should depend on the severity of the bleeding and its response to oral iron replacement, according to the authors. Oral antifibrinolytics are appropriate for mild bleeding, whereas intravenous bevacizumab or other systemic antiangiogenic therapy can be considered for moderate to severe bleeding.

All adults should be tested for iron deficiency and anemia, as should children with recurrent bleeding and/or symptoms of anemia. Iron replacement should be the initial treatment of iron deficiency and anemia, and red blood cell transfusions should be reserved for certain defined settings.

HHT is not an absolute contraindication to anticoagulation or antiplatelet therapy, but the expert panel recommends against the use of dual antiplatelet therapy and/or the combination of antiplatelet therapy and anticoagulation where possible.

Diagnostic genetic testing of asymptomatic children of a parent with HHT and screening of those with HHT for pulmonary AVMs (arteriovenous malformations) are recommended, and large pulmonary AVMs associated with reduced oxygen saturation should be treated to avoid serious complications.

Pulmonary AVM screening of asymptomatic children with HHT should be repeated at five-year intervals, according to the guidelines, which appear in the Annals of Internal Medicine.

Asymptomatic children with HHT should also be screened for brain VMs, and brain VMs with high-risk features should be treated.

Clinicians should discuss preconception and prenatal diagnostic options with HHT-affected individuals, and recommendations for pregnant women with HHT depend upon previous screening, treatment, and symptoms.

Epidural anesthesia should not be withheld because of the diagnosis of HHT, and women with known, non-high-risk brain VMs can labor and proceed with vaginal delivery.

Pregnant women with HHT should be managed at a tertiary care center by a multidisciplinary team if they have untreated pulmonary AVMs and/or brain VMs or have not been recently screened for pulmonary AVMs.

Guidelines for the diagnosis of HHT and for the diagnosis and management of liver VMs, brain VMs, and pulmonary AVMs are carried over from the First International HHT Guidelines.

"These recommendations should facilitate implementation of the key components of the HHT care into clinical practice," the authors conclude.

Dr. Faughnan did not respond to a request for comments.

By Reuters Staff

SOURCE: https://bit.ly/329gxsC Annals of Internal Medicine, online September 8, 2020.

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