UCLA researchers in the Departments of Molecular, Cell, and Development Biology & Surgery have identified Angiopoietin 2 (Ang-2) as a marker and potentially a strong contributing factor to the clinical presentation of pulmonary arteriovenous malformations.
The Fontan procedure is used in pediatric patients who process only a single functional ventricle. After the Fontan circulation is established, some patients may develop pulmonary arteriovenous malformations (PAVMS) due to the unbalanced pulmonary perfusion of hepatic vein blood flow. Some of the symptoms for PAVMS include dyspnea, epistaxis, stroke and brain abscess. PAVMS is mainly diagnosed through radiography and CT scan. PAVMS was historically treated with surgical resection. With the technological advances, embolization has become the mainstay of treatment. Currently, no drug therapy is available for PAVMS.
Researchers at UCLA identified Angiopoietin 2 (Ang-2) as a marker and potentially a strong contributing factor to the clinical presentation of PAVMS. Ang-2 level was significantly elevated (up to 10 fold) in patients who had a Fontan procedure. Ang-2 is an endothelial growth factor and a key regulator of angiogenesis and vessel maturation. It is known that Ang-2 level is significantly higher in patients with acute lung injury. Though antibodies against Angiopoietin 2 have been developed by both Regeneron and Amgen to suppress angiogenesis in cancer, the application of these antibodies to diagnose and treat PAVMS has not been disclosed in the literature.
Diagnosis and treatment of PAVMS: targeting Angiopoietin 2 with antibodies
High levels of Angiopoietin 2 have been identified in patients who had a Fontan surgical procedure. Development is ongoing.
Angiopoietin 2, antibody, Fontan procedure, Hepatic factors, pulmonary arteriovenous malformations, PAVMS