Pulmonary arteriovenous malformations: techniques and long-term outcome of embolotherapy.

White, Robert I.; Lynch-Nyhan, Alma; Terry, Peter B.; Buescher, Philip; Farmlett, E J; Charnas, Lawrence; Shuman, K; Kim, W; Kinnison, M L; Mitchell, S E

doi:10.1148/radiology.169.3.3186989

Cited by 465 publications

(341 citation statements)

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“…43,44 In a recent study, a higher prevalence of PAVMs was found in females, but only in the HHT1 group, whereas HAVMs were found to be more frequent in females from both HHT1 and 2. 27 Although we did not confirm the influence of sex on the occurrence of PAVMs, we found a strong and significant predominance of HAVMs in females, both for symptomatic and asymptomatic lesions.…”

Section: Discussionmentioning

confidence: 89%

Genotype-phenotype correlations in hereditary hemorrhagic telangiectasia: Data from the French-Italian HHT network

Lesca

Olivieri

Burnichon³

et al. 2007

Genetics in Medicine

203

187

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Purpose: Hereditary hemorrhagic telangiectasia is an autosomal dominant disorder characterized by arteriovenous malformations (AVM), mostly cutaneous and mucous (telangiectases), but also involving the lungs (PAVM), liver (HAVM) and brain (CAVM). We studied the relationship between the phenotype and genotype in patients with a proven mutation in either ENG (HHT1) or ACVRL1 (HHT2). Methods: Clinical features and their age of onset were compared between HHT1 and HHT2. The type of mutation was also analyzed. Clinical manifestations were distinguished from lesions found by screening. Results: Ninety-three HHT1 patients and 250 HHT2 patients were included. Epistaxis occurred later in HHT2, with incomplete penetrance (P Ͻ 0.0001). Symptomatic PAVMs were more frequent in HHT1 (34.4 vs. 5.2%, P Ͻ 0.001), as were cerebral abscesses (7.5 vs. 0.8%, P ϭ 0.002).Gastrointestinal bleeding occurred more frequently in HHT2 (16.4 vs. 6.5%, P ϭ 0.017). Symptomatic hepatic involvement was only seen in HHT2 patients. PAVMs were more frequently detected in asymptomatic HHT1 patients (54 vs. 12.8%, P Ͻ 0.0001). PAVMs and HAVMs were often family clustered in HHT1 and HHT2, respectively. Truncating mutations were associated with a higher frequency of epistaxis and telangiectasis, in HHT2. Conclusion: This study shows major differences between HHT1 and HHT2 phenotypes, which should be taken into account for future clinical studies. Genet Med 2007:9(1):14-22.

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Section: Discussionmentioning

confidence: 89%

Genotype-phenotype correlations in hereditary hemorrhagic telangiectasia: Data from the French-Italian HHT network

Lesca

Olivieri

Burnichon³

et al. 2007

Genetics in Medicine

203

187

View full text Add to dashboard Cite

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“…It is possible that a thrombus may also form in-situ within a PAVM, and this can dislodge and embolize into the cerebral circulation. The radiological literature currently advocates the occlusion of all such malformations having feeding vessels of 3 mm or greater (5).…”

Section: Discussionmentioning

confidence: 99%

Treatment of Pulmonary Arteriovenous Malformation Using Platinum Coils: Case Report

Kim

Lim

2005

J Korean Radiol Soc

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Pulmonary arteriovenous malformation (PAVM) causes right-to-left blood shunting and if it is not treated, it may lead to chronic hypoxemia and other serious clinical manifestations (1 3). Detachable balloons and metallic coils are the embolotherapy devices that have been most commonly used to treat PAVM (4, 5). Platinum coils have recently become available as embolotherapy devices (6, 7). To the best of our knowledge, treatments for PAVM using platinum coils have never been reported in the Korean literature. Therefore, we describe a case of PAVM and its endovascular treatment using platinum coils, and we include a review of the relevant literature. Case ReportA 33-year-old male was admitted to the hospital due to an abnormal mass on the left lower lobe of the lung. Plain chest radiograph and Computed Tomography (CT) revealed a 4.2 3.2 cm sized, ovoid shaped and well demarcated vascular mass with a feeding artery that measured 5 mm and a draining vein, and these findings were compatible with a PAVM (Fig. 1A). The patient had no specific symptoms such as dyspnea or exercise intolerance. Specifically, there was no cutaneous telangiectasia suggestive of hereditary hemorrhagic telangiectasia (HHT). The hemoglobin level determined at the time of catheterization was within the normal range, 16.3 g/dL, with a hematocrit of 48.5%. Endovasular treatment was planned to prevent such serious complications as brain abscess in spite of the absence of clinical symptoms.The right femoral vein was used to access the pulmonary artery. Left pulmonary arteriography was ob- Pulmonary arteriovenous malformation (PAVM) is an abnormal direct communication between the pulmonary arteries and veins without any capillary network. The patients may be completely asymptomatic or may they develop serious complications including hemoptysis and brain abscess. We present here a case of incidentally found PAVM in a 33-year-old male who underwent embolization using platinum coils. Coil embolization is a safe, highly effective procedure that should be considered more often for the treatment of PAVM.

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“…2) The shunting of air, thrombi, and bacteria passing through pulmonary AVMs, bypassing the filtering capabilities of the lungs, may result in transient ischemic attacks, embolic stroke, or cerebral abscess. 4,6,12) Approximately 20% of patients with HHT will develop pulmonary AVM, and 25-33% of such patients will develop cerebral ischemic symptoms. Brain abscesses will occur in 5-9% of these patients.…”

Section: Discussionmentioning

confidence: 99%

Central Nervous System Lesions Associated With Hereditary Hemorrhagic Telangiectasia -Three Case Reports-

Kuwayama

Takase

Kashihara

et al. 2003

Neurol. Med. Chir.(Tokyo)

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Hereditary hemorrhagic telangiectasia (HHT) is easily overlooked in patients with central nervous system (CNS) lesions. Our clinical experience of three patients with CNS lesions associated with HHT stresses the importance of considering HHT. A 23-year-old male presented with consciousness disturbance and right hemiparesis. Emergency cerebral digital subtraction angiography revealed occlusion of the left middle cerebral artery and the left anterior cerebral artery. Pulmonary arteriography showed three pulmonary arteriovenous malformations (AVMs). A 62-year-old male presented with consciousness disturbance and sensory aphasia. Magnetic resonance imaging revealed a ringenhanced lesion in the left temporal lobe which was removed by left frontotemporal craniotomy. The diagnosis was brain abscess. Chest computed tomography (CT) revealed two pulmonary AVMs. A 32-year-old female presented with progressive mild weakness in her left hand. Initial CT showed subcortical hemorrhage in the right frontal lobe. Cerebral angiography revealed no vascular malformations, but chest CT disclosed five pulmonary AVMs. All three patients had a family history of HHT. The possibility of HHT is important to consider in patients with cerebrovascular disease (CVD) or brain abscess to prevent complications, not only in the patients but also their blood relatives. Therefore, the medical and family history of patients with CVD or brain abscess should be investigated and HHT should be considered during the physical examination.

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Pulmonary arteriovenous malformations: techniques and long-term outcome of embolotherapy.

Cited by 465 publications

References 0 publications

Genotype-phenotype correlations in hereditary hemorrhagic telangiectasia: Data from the French-Italian HHT network

Genotype-phenotype correlations in hereditary hemorrhagic telangiectasia: Data from the French-Italian HHT network

Treatment of Pulmonary Arteriovenous Malformation Using Platinum Coils: Case Report

Central Nervous System Lesions Associated With Hereditary Hemorrhagic Telangiectasia -Three Case Reports-

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