Pulmonary arteriovenous malformations with pulmonary artery hypertension: Therapeutic implication

Abstract

Pulmonary arteriovenous malformations (PAVMs) are rare pulmonary vascular anomalies and represent fistulous connection between arterial and venous branches without customary intervening capillary network that is vital for gas exchange. The incidence is 2–3/100,000. Typically, PAVMs are associated with a normal or low pulmonary vascular resistance as the direct arteriovenous communications are low-resistance circuits. However, pulmonary artery hypertension is uncommon in PAVMs. Treatment of choice for PAVM is embolization. However, it abolishes low-resistance pathways for pulmonary blood flow and therefore can elevate pulmonary artery pressure. For majority of patients with severe PAH, risks of PAVM embolization outweigh potential benefits.

Keywords

• embolization
• pulmonary arteriovenous malformations
• pulmonary hypertension