Fifteen to fifty percent of patients with hereditary haemorrhagic telangiectasia have pulmonary arteriovenous malformations. The objective of this study was to measure the effect of the presence of pulmonary arteriovenous malformations and of their embolisation on respiratory-related quality of life (QoL). We prospectively recruited patients with a diagnosis of hereditary haemorrhagic telangiec...

The extracardiac conduit Fontan procedure is the last surgical step in the treatment of patients with a functional single ventricle. An acquired pulmonary arteriovenous malformation may appear perioperatively or postoperatively due to an uneven hepatic flow distribution. Here we report a case of a bifurcated Y-graft Fontan operation in a 15-year-old male patient with a unilateral pulmonary arte...

Pulmonary arteriovenous malformations (PAVM) are rare pulmonary vascular anomalies. Although most patients are asymptomatic, PAVMs can cause dyspnoea from right-to-left shunt. Because of paradoxical emboli, various central nervous system complications have been described including stroke and brain abscess. There is a strong association between PAVM and hereditary haemorrhagic telangiectasia. Ch...

A 44-year-old man was admitted with coughing and dyspnea for 3 weeks. Chest radiography and thoracic CT scanning confirmed a huge left lung arteriovenous malformation (Fig. 1). Due to a prohibitively high operative risk, he was investigated with pulmonary angiography in preparation for embolization therapy (Fig. 2). The patient subsequently refused all further therapy. European Journal of Cardi...

A35-year-old white woman with a history of migraine headaches and a grade 2 systolic murmur presented with symptoms of slurred speech, left-sided facial droop, and left hemiparesis after a hysterectomy. These symptoms resolved over several days, and in an effort to identify a potential cardiac source of embolism, a transesophageal echocardiogram was performed. Findings were consistent with a ri...

A35-year-old white woman with a history of migraine headaches and a grade 2 systolic murmur presented with symptoms of slurred speech, left-sided facial droop, and left hemiparesis after a hysterectomy. These symptoms resolved over several days, and in an effort to identify a potential cardiac source of embolism, a transesophageal echocardiogram was performed. Findings were consistent with a ri...

Abstract We report a case of 34-year-old woman who presented with chest pain, shortness breath, and dry cough 1 month duration. Contrast-enhanced computed tomography the revealed an enlarged tortuous right pulmonary vein that could not conclusively exclude arteriovenous malformation. Further evaluation digital subtraction angiography excluded malformation confirmed presence varix. Accordingly, ...

BACKGROUND AND PURPOSE Patients with hereditary hemorrhagic telangiectasia (HHT) are at risk for developing cerebral vascular malformations and pulmonary arteriovenous fistulae. We assessed the risk of neurological dysfunction from these malformations and fistulae. METHODS Three hundred twenty-one consecutive patients with HHT seen at a single institution over a 20-year period were studied. A...