No abstract available.
Telangiectasia, Hereditary Hemorrhagic

No abstract available.
Arteriovenous Fistula* ; Telangiectasia, Hereditary Hemorrhagic*

No abstract available.
Arteriovenous Malformations ; Embolization, Therapeutic ; Telangiectasia, Hereditary Hemorrhagic

No abstract available.
Activins* ; Humans ; Telangiectasia, Hereditary Hemorrhagic* ; Telangiectasis*

No abstract available.
Activins* ; Humans ; Telangiectasia, Hereditary Hemorrhagic* ; Telangiectasis*

High-output cardiac failure is a rare complication of hereditary hemorrhagic telangiectasia (HHT) usually caused by shunting of blood through atriovenous malformations (AVMs) in the liver. We describe two cases of high output heart failure due to large hepatic AVMs. Clinical suspicion of HHT based on detailed history taking and physical examination is essential for early detection and proper management of heart failure associated with HHT.
Heart ; Heart Failure ; Liver ; Physical Examination ; Telangiectasia, Hereditary Hemorrhagic

Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber disease, is a rare autosomal dominant disorder characterized by hemorrhage from nasal, mucocutaneous and gastrointestinal telangiectasia, in addition to vascular anomalies in other organs, particularly in the pulmonary, hepatic and cerebral circulations. The most common clinical manifestations are epistaxis and gastrointestinal bleeding. Endoscopic band ligation is effective for non-variceal, non-ulcer bleeding. It has the advantage of ease of use and is relatively inexpensive. We report a case of endoscopic band ligation therapy for gastric bleeding in patient with Rendu-Osler-Weber disease.
Epistaxis ; Hemorrhage* ; Humans ; Ligation* ; Telangiectasia, Hereditary Hemorrhagic* ; Telangiectasis

Pulmonary arterionevous malformation (PAVMs) are a rare disorder with an incidence of 2-3/1,000,000 population. Approximately 70% of cases are associated with Osler-Weber-Rendu Disease. This intrapulmonary malformation causes hypoxemia and dyspnea largely attributed to the right to left shunting.

We present a case of 25-year-old male who was admitted for fever and headaches with chronic history of epistaxis, hemoptysis, cyanosis and clubbing. Central cyanocis and clubbing were evident with hypoxemia of 65%. Heart sounds and peripheral pulses were normal. There was no systolic bruit noted in the lung bases. Multiple telangiectasias were seen in the truncal area and abdomen. Chest x-ray revealed multi-chambered cardiomegaly with no opacifications. CBC showed erythrocytosis with hematocrits of 0.68-0.78. Transthoracic echocardiography showed intact interatrial and interventricular septum, with contrast study suggestive of intrapulmonary shunting. CT angiography revealed PAVMs in bilateral lung fields. Pulmonary angiography demonstrated diffuse PAVMS in the left lung with 3 large PAVMs with multiple feeders > 7mm and smaller PAVMs in the Right lower lobe. The diagnosis was Multiple Pulmonary Arteriovenous Malformation, Osler-Weber-Rendu Disease, Brain Abscess Left Temporoparietal area. Craniotomy with brain abscess evacuation was done. Left pneumonectomy or Right lobectomy was considered but was deemed unacceptable due to high morbidity and mortality. Endovascular coil embolization was done on the 3 large fistulas on the Left lobe. Post procedure, his arterial oxygenation improved to 96%. He has no recurrence of hemoptysis, no headaches and with less episodes of shortness of breath 2 months on follow-up. Diagnosing the complex diffuse intrapulmonary malformations requires a high index of suspicion among patients with chronic cyanosis, chronic history of bleeding, and brain abscess. Treatment of this condition and its associated complications remain a big challenge and should be highly individualized.

A case of hereditary hemorrhagic telangiectasia was reported. Repeated hemorrhage in nasal and digestive system are main clinical manifestation. Capillary expanded on tongue and finger is the main physical sign. Main clinical manifestation and typical physical signs, combined with family history, can help to establish a diagnosis.
Humans ; Male ; Middle Aged ; Pedigree ; Telangiectasia, Hereditary Hemorrhagic ; genetics

Adult ; Arteriovenous Malformations ; Humans ; Male ; Telangiectasia, Hereditary Hemorrhagic