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Special Issue Editor

Prof. Dr. Poul Erik Andersen

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Guest Editor

Clinical Institute - Radiology, University of Southern Denmark, DK 5000 Odense C, Denmark
Interests: radiology- interventional; embolization; pulmonary arteriovenous malformations- embolization; Uterine fibroid- embolization and MR; peripheral arterial interventions- ballons and stents; thorax radiology- diagnostic and interventional; osteochondrodysplasias- diagnostics and epidemiology

Special Issue Information

Dear Colleagues,

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant inherited vascular disease in which patients may develop mucocutaneous telangiectatic lesions and visceral arteriovenous malformation. Especially pulmonary arteriovenous malformations (PAVM) may have severe consequences for the patients, if untreated. PAVM are congenital vascular anomalies of the lung in which abnormally dilated vessels provide a direct capillary-free communication between the pulmonary and systemic circulations with three main clinical consequences: (1 pulmonary arterial blood passing through these right-to-left shunts cannot be oxygenated, which leads to arterial hypoxemia; (2 the absence of normally filtering capillary bed allows thromboembolic material to reach the systemic circulation (paradoxical embolism), which can result in transient ischemic attack, stroke or in brain abscess; (3 rarely rupture of the thin-walled PAVMs which can lead to hemoptysis or hemothorax, particularly during pregnancy.

PAVM may occur sporadically but most often in association with HHT. About 90% of patients with PAVM have underlying HHT. Screening for PAVM in patients with HHT is recommended, and patients with PAVM should be screened for HHT.

The generally accepted treatment strategy of first choice is embolization of the afferent arteries to the PAVM. It is a minimally invasive procedure and at the same time a lung preserving treatment with a very high technical success, high effectiveness and low morbidity and mortality. Embolization is a well-established method of treating PAVM, which prevents cerebral stroke and abscess as well as pulmonary hemorrhage and further raises the functional level significantly. Vascular plugs, coils or in combination are the primary choices as embolization materials.

Prof. Dr. Poul Erik Andersen
Guest Editor

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Keywords

pulmonary artery
pulmonary circulation
pulmonary arteriovenous malformations
hereditary hemorrhagic telangiectasia
embolization, therapeutic
radiology
interventional

Published Papers (2 papers)

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Research

Jump to: Review

8 pages, 9533 KiB

Open AccessArticle

Incidence of Spontaneous Pulmonary AVM Rupture in HHT Patients

by Adam Fish, Katharine Henderson, Alex Moushey, Jeffrey Pollak and Todd Schlachter

J. Clin. Med. 2021, 10(20), 4714; https://0-doi-org.brum.beds.ac.uk/10.3390/jcm10204714 - 14 Oct 2021

Cited by 5 | Viewed by 1697

Abstract

The spontaneous rupture of pulmonary AVMs, resulting in pulmonary hemorrhage and hydrothorax, is a life-threatening complication. While this phenomenon has been previously reported, the true incidence is not yet known. This study retrospectively reviewed records of 801 HHT patients with pulmonary AVMs to identify a single lifetime episode of hemothorax or pulmonary hemorrhage secondary to pulmonary AVM rupture. The lifetime prevalence and incidence of pulmonary AVM rupture in HHT patients was 2.7% and 0.16% respectively. In these patients, AVM rupture represented the initial presentation of HHT in nine (40.9%) cases and was life-threatening in nine (40.9%) cases. All cases occurred in virgin lesions, and subsequent embolization was curative. While a feared complication, pulmonary AVM rupture is rare and is likely effectively prevented by existing embolization techniques and indications. Full article

(This article belongs to the Special Issue Pulmonary Arteriovenous Malformations: Diagnosis and Management)

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Review

Jump to: Research

12 pages, 1255 KiB

Open AccessReview

Hereditary Hemorrhagic Telangiectasia and Arterio-Venous Malformations—From Diagnosis to Therapeutic Challenges

by Mariana Floria, Elena Diana Năfureanu, Diana-Elena Iov, Oana Sîrbu, Mihaela Dranga, Anca Ouatu, Daniela Maria Tănase, Oana Bogdana Bărboi, Vasile Liviu Drug and Mihail Dan Cobzeanu

J. Clin. Med. 2022, 11(9), 2634; https://0-doi-org.brum.beds.ac.uk/10.3390/jcm11092634 - 07 May 2022

Cited by 3 | Viewed by 2095

Abstract

Hereditary hemorrhagic telangiectasia is a rare autosomal dominant vascular disease defined by the presence of mucosal and cutaneous telangiectasia and visceral arterio-venous malformations. The latter are abnormal capillary-free direct communications between the pulmonary and systemic circulations with the following consequences: arterial hypoxemia caused by right-to-left shunts; paradoxical embolism with transient ischemic attack or stroke and brain abscess caused by the absence of the normally filtering capillary bed; and hemoptysis or hemothorax due to the rupture of the thin-walled arterio-venous malformations (particularly during pregnancy). It is frequently underdiagnosed, commonly presenting as complications from shunting through arterio-venous malformations: dyspnea, chronic bleeding, or embolism. Arterio-venous malformations are present not only in the lungs, but can also be found in the liver, central nervous system (mainly in the brain), nasal mucosa, or the gastrointestinal tract. The first choice of therapy is embolization of the afferent arteries of the arterio-venous malformations, a minimally invasive procedure with a high efficacy, a low morbidity, and low mortality. Other therapeutic modalities are surgery (resection) or stereotactic radiosurgery (using radiation). Routine screening for arterio-venous malformations is indicated in patients diagnosed with this condition and can prevent severe complications such as acute hemorrhages, brain abscesses, or strokes. Clinicians should provide a long-term follow-up for patients with arterio-venous malformations, in an effort to detect their growth or reperfusion in case of previously treated malformations. In spite of two experts’ consensuses, it still possesses multiple therapeutic challenges for physicians, as several aspects regarding the screening and management of arterio-venous malformations still remain controversial. Multidisciplinary teams are especially useful in complex cases. Full article

(This article belongs to the Special Issue Pulmonary Arteriovenous Malformations: Diagnosis and Management)

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