Screening pulmonary arteriovenous malformations in a large cohort of Spanish patients with hemorrhagic hereditary telangiectasia

Parra, José Antonio; Cuesta, Jose María Luzón; Zarrabeitia, Roberto; Fariñas‐Álvarez, Concepción; Bueno, Jesús; Marqués, Sarah; Parra-Fariñas, Carmen; Botella, María Luisa; Bernabéu, Carmelo; Zarauza, Jesús

doi:10.1016/j.ijcard.2016.05.065

Cited by 9 publications

(3 citation statements)

References 37 publications

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“…Moreover, none of the grade 1 shunts subsequently needed embolization, whereas 25.3% of grade 2 shunts and 77.4% of grade 3 shunts required endovascular closure of PAVM. Other studies have reported similar observations, for example that increased shunt grade predicts presence of PAVM on chest CT [24][25][26], and that patients with grade 1 shunts do not receive intervention, whereas those with grade 2 and 3 shunts often do [27][28][29][30].…”

Section: Transthoracic Contrast Echocardiographysupporting

confidence: 53%

Approach to Pulmonary Arteriovenous Malformations: A Comprehensive Update

Majumdar

McWilliams

2020

JCM

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Pulmonary arteriovenous malformations (PAVMs) are abnormal direct vascular communications between pulmonary arteries and veins which create high-flow right-to-left shunts. They are most frequently congenital, usually in the setting of hereditary hemorrhagic telangiectasia (HHT). PAVMs may be asymptomatic or present with a wide variety of clinical manifestations such as dyspnea, hypoxemia, or chest pain. Even when asymptomatic, presence of PAVMs increases patients’ risk of serious, potentially preventable complications including stroke or brain abscess. Transcatheter embolotherapy is considered the gold standard for treatment of PAVMs. Though previous guidelines have been published regarding the management of PAVMs, several aspects of PAVM screening and management remain debated among the experts, suggesting the need for thorough reexamination of the current literature. The authors of this review present an updated approach to the diagnostic workup and management of PAVMs, with an emphasis on areas of controversy, based on the latest literature and our institutional experience.

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Section: Transthoracic Contrast Echocardiographysupporting

confidence: 53%

Approach to Pulmonary Arteriovenous Malformations: A Comprehensive Update

Majumdar

McWilliams

2020

JCM

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“…These schemes have identified a threshold of contrast below which further imaging is not required, as a treatable PAVM is unlikely to be found. 15,[37][38][39] The shunting associated with PAVM differs from that seen with PFO or other intracardiac shunts in that the contrast appears late after initial injection 37 (requiring time to transit from the right ventricle to the lungs and back to the left heart), whereas intracardiac shunt has the potential to appear early after injection (a threshold of three cardiac cycles or less tends to be used to define "early" shunt). Intracardiac shunt is also typically increased by the Valsalva maneuver by reversing the intracardiac left-to-right pressure gradient, where no increase in shunt is seen for PAVM with Valsalva.…”

Section: Saline Contrast Echocardiographymentioning

confidence: 99%

Pulmonary Arteriovenous Malformations: What the Interventional Radiologist Should Know

Kaufman

McDonald

Balch

et al. 2022

Semin intervent Radiol

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Pulmonary arteriovenous malformations (PAVMs) are abnormal connections between the pulmonary artery and pulmonary vein bypassing the normal capillary bed causing a right-to-left shunt. The majority (80–90%) of PAVMs are associated with hereditary hemorrhagic telangiectasia (HHT). PAVMs may be asymptomatic or present with symptoms of hypoxia, shortness of breath, migraines, sequelae of paradoxical embolization, or rupture. Transcatheter embolization has become the standard of care. This article will review the clinical presentation, workup, genetics, imaging findings, embolization, complications, and follow-up for patients with PAVMs.

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“…However, these abnormal communications between pulmonary arteries and veins are commonly seen in patients with hereditary hemorrhagic telangiectasia (HHT) (also known as the Osler-Weber-Rendu syndrome) [2,3]. Aside from the congenital etiology, several conditions may lead to acquired forms of multiple PAVFs-including cirrhosis, chest trauma, metastatic carcinoma, schistosomiasis, and systemic amyloidosis [4,5].…”

Section: Introductionmentioning

confidence: 99%

Multiple Pulmonary Arteriovenous Fistula in a Mother and His Son: A Case Report

Firouzi¹,

Behrooj²,

Pirborj³

et al. 2017

J Cardiovasc Dis Diagn

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Multiple Pulmonary Arteriovenous Fistulas (PAVFs) are rare vascular abnormalities in the general population, with a report on 15000 autopsies revealing only 3 cases of multiple PAVFs. However, these abnormal communications between pulmonary arteries and veins are commonly seen in patients with Hereditary Hemorrhagic Telangiectasia (HHT). Several conditions such as cirrhosis, chest trauma, metastatic carcinoma, schistosomiasis, and systemic amyloidosis are accountable for acquired form PAVF. We describe 2 patients (mother and son) with multiple PAVFs and no apparent signs or symptoms suggestive of HHT. A pulmonary artery injection wes performed for Case 1 (mother) and showed bilateral PAVFs with aneurysmal dilation. Given the diagnosis of PAVFs in the mother, we considered the same pathology for the child's symptoms. Therefore, the boy also underwent pulmonary angiography, which revealed multiple bilateral PAVFs. Both patients underwent interventional angiography and the PAVFs were occulted with stainless coil. In conclusion, the presence of multiple PAVFs should be considered in the differential diagnosis of patients with unexplained exertional dyspnea and cyanosis, even in the absence of diagnostic features suggestive of HHT.

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Screening pulmonary arteriovenous malformations in a large cohort of Spanish patients with hemorrhagic hereditary telangiectasia

Cited by 9 publications

References 37 publications

Approach to Pulmonary Arteriovenous Malformations: A Comprehensive Update

Approach to Pulmonary Arteriovenous Malformations: A Comprehensive Update

Pulmonary Arteriovenous Malformations: What the Interventional Radiologist Should Know

Multiple Pulmonary Arteriovenous Fistula in a Mother and His Son: A Case Report

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