Lack of Growth of Small (≤2 mm Feeding Artery) Untreated Pulmonary Arteriovenous Malformations in Patients with Hereditary Hemorrhagic Telangiectasia

Curnes, Nicole R.; Desjardins, Benoı̂t; Pyeritz, Reed E.; Chittams, Jesse; Sienko, Danielle; Trerotola, Scott O.

doi:10.1016/j.jvir.2019.04.009

Cited by 4 publications

(6 citation statements)

References 22 publications

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“…Similar findings were reported by Ryan et al in a 2017 study investigating the natural history of small and microscopic untreated PAVMs in adults [112]. The findings from Curnes et al and Ryan et al challenge the guideline of 3-5 year CT follow-up for small untreated PAVMs, suggesting that this interval could be safety extended up to 5-10 years [111,112].…”

Section: Follow-upsupporting

confidence: 80%

“…For each patient in the study, they compared 2 CT exams with the longest interval between them (mean 8.4 years, range 3.1-14.1 years) to assess growth, analyzing a total of 88 PAVMs in 21 patients. They found that untreated PAVMs grew slowly, if at all, and that any demonstrated growth was minimal and clinically inconsequential [111]. Similar findings were reported by Ryan et al in a 2017 study investigating the natural history of small and microscopic untreated PAVMs in adults [112].…”

Section: Follow-upsupporting

confidence: 68%

“…Previous guidelines recommended 3-5 year CT follow-up. However, a 2019 study by Curnes et al has reported a lack of growth over time for small untreated PAVMs in adults [111]. For each patient in the study, they compared 2 CT exams with the longest interval between them (mean 8.4 years, range 3.1-14.1 years) to assess growth, analyzing a total of 88 PAVMs in 21 patients.…”

Section: Follow-upmentioning

confidence: 99%

See 2 more Smart Citations

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: Follow-upsupporting

confidence: 80%

Section: Follow-upsupporting

confidence: 68%

Section: Follow-upmentioning

confidence: 99%

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Approach to Pulmonary Arteriovenous Malformations: A Comprehensive Update

Majumdar

McWilliams

2020

JCM

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“…(12) As diretrizes recomendam acompanhamento a cada 3-5 anos em pacientes com fístulas pequenas (diâmetro < 3 mm). No entanto, dois estudos recentes demonstraram que o crescimento dessas fístulas é lento e pouco frequente (60,61) e recomendam o acompanhamento com TC a cada 5 anos (Figura 6).…”

Section: Medidas Geraisunclassified

Update on pulmonary arteriovenous malformations

2023

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This review aimed to provide an overview of pulmonary arteriovenous malformations, including the major clinical and radiological presentations, investigation, and treatment algorithm of the condition. The primary etiology of pulmonary arteriovenous malformations is hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, with mutations in the ENG gene on chromosome 9 (HHT type 1) or in the ACVRL1/ALK1 complex (HHT type 2). Epistaxis should always be evaluated when repeated, when associated with anemia, and in some cases of hypoxemia. In the investigation, contrast echocardiography and chest CT are essential for evaluating this condition. Embolization is the best treatment choice, especially for correction in cases of hypoxemia or to avoid systemic infections. Finally, disease management was addressed in special conditions such as pregnancy. CT follow-up should be performed every 3-5 years, depending on the size of the afferent and efferent vessels, and antibiotic prophylactic care should always be oriented. Ultimately, knowledge of the disease by health professionals is a crucial point for the early diagnosis of these patients in clinical practice, which can potentially modify the natural course of the disease.

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“…7,10,25 A recent study of patients with follow-up of untreated PAVMs 2 mm showed minimal, if any growth, and no clinical consequences. 73 This raises the question as to the optimal time interval for follow-up on small PAVMs and whether it could be extended to 5 to 10 years. Generally, patients with diffuse PAVMs are followed up more closely, every year, due to the increased risk of complications.…”

Section: Follow-upmentioning

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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Lack of Growth of Small (≤2 mm Feeding Artery) Untreated Pulmonary Arteriovenous Malformations in Patients with Hereditary Hemorrhagic Telangiectasia

Cited by 4 publications

References 22 publications

Approach to Pulmonary Arteriovenous Malformations: A Comprehensive Update

Approach to Pulmonary Arteriovenous Malformations: A Comprehensive Update

Update on pulmonary arteriovenous malformations

Pulmonary Arteriovenous Malformations: What the Interventional Radiologist Should Know

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