Congenital pulmonary arteriovenous malformation: a rare cause of cyanosis in childhood

Abstract

Pulmonary arteriovenous malformation (PAVM) is a rare condition in which there is abnormal connection between pulmonary arteries and veins. The disorder usually appears in late childhood or early adult life, with dyspnea on exertion, clubbing or cyanosis. We present two patients with severe cyanosis and their work-up to diagnosis of PAVM, as a rare cause of cyanosis in childhood.

Keywords: Cyanosis; Pulmonary arteriovenous malformation; Right to left shunting.

Figure 1:. Patient 1: Selective angiography of the left lower lobe PAVM, showing a dilated feeding artery draining into a large vein, with rapid filling of the left atrium. Note the clips for PDA closure

Figure 2:. Patient 2: Anteroposterior projection of the left pulmonary artery injection, demonstrates simultaneous filling of the pulmonary artery, pulmonary capillary bed and pulmonary veins of the left lung. Note haziness of PAVM in superior lingula.