Abstract
Intracranial AVMs (iAVMs) are high-pressure, low-resistance vascular networks of feeding arteries and draining veins that encompass complex physiologic and molecular processes. AVM biology may contribute to their growth, regression, remodeling, and de novo recurrence after successful resection. Over the past decades, mouse models and human AVM studies have provided insights into molecular signaling in iAVMs. Although a complete understanding of AVM biology remains elusive, this chapter reviews the well-established literature in AVM physiology and introduces novel AVM signaling research. Furthermore, it provides an overview of syndromes associated with AVM formation and their corresponding genetic defects. Lastly, novel therapeutic approaches to hereditary and syndromic iAVMs are discussed..