Recurrence of pulmonary arteriovenous malformations in a female with hereditary hemorrhagic telangiectasia
Pulmonary arteriovenous malformations (AVMs) are an uncommon disorder and may cause life-threatening complications if left untreated. The paucity of good longitudinal data on patients with pulmonary AVMs can be a significant challenge clinically. The authors report a case of recurrence of pulmonary AVMs in a young female with hereditary hemorrhagic telangiectasia (HHT) subjected to transcatheter embolotherapy (TCET) in 1995. Recurrence of pulmonary AVMs was suspected because of marked impairment of oxygenation in 1997 and in 2000, while the patient was pregnant, and later confirmed by imaging studies in early 2003. Despite successful embolization of all visible pulmonary AVMs, contrast echocardiography suggested the presence of intrapulmonary shunt. A shunt of 11.4% was measured using a 100% oxygen test. Loss of flow pattern was the immediate change of pulmonary AVMs after TCET shown by chest sonography. A decrease in the size of the pulmonary AVMs was observed 6 weeks later. This case illustrates the clinical relevance of longitudinal monitoring of arterial blood gases in screening for the recurrence of pulmonary AVMs, particularly in patients with HHT, and the roles of chest sonography and contrast echocardiography in monitoring the efficiency of TCET.