Objective: To determine if there is an association between migraines and intrapulmonary right-to-left shunt.
Background: Several studies have described an association between migraines and intracardiac right-to-left shunt.
Methods: Patients with hereditary hemorrhagic telangiectasia (HHT) were retrospectively recruited from the Toronto Hereditary Hemorrhagic Telangiectasia Center Clinical Database. All patients had been prospectively, systematically asked about a history of migraines and systematically screened for pulmonary and cerebral arteriovenous malformations (AVMs). All patients with a definite diagnosis of HHT, assessed during a 2-year period (February 1997 to April 1999), were included. Univariate analyses and logistic regression were performed, for migraine as the dependent variable and the following independent variables: age, sex, pulmonary AVMs, and cerebral AVMs.
Results: Of the 200 patients assessed during the 2-year period, 124 (62%) had a definite diagnosis of HHT and were included in the analysis. Eighty (65%) were females. Forty-seven (38%) of the HHT patients had a history of migraine, of whom 38 (81%) had migraine with aura. The prevalence of migraine was greater in patients with pulmonary AVMs (46%) compared to patients without pulmonary AVMs (33%), OR = 1.7 (0.8 to 3.6), though this did not reach statistical significance (P = .14). Pulmonary AVMs were significantly associated with migraine (OR = 2.4, 95% CI = 1.1 to 5.5, P = .04), after adjustment for age and sex, using logistic regression.
Conclusions: There is a significant association between intrapulmonary right-to-left shunt and migraine.
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http://dx.doi.org/10.1111/j.1526-4610.2006.00291.x | DOI Listing |
Acta Neurol Belg
January 2025
Intensive Care Department, Cliniques Universitaire Saint-Luc (CUSL), Université Catholique de Louvain (UCL), Brussels, Belgium.
Osler-Weber-Rendu syndrome, or hereditary hemorrhagic telangiectasia (HHT), is a rare vascular disorder characterized by arteriovenous malformations (AVMs) in various organs, including the lungs. Pulmonary AVMs (PAVMs) are especially worrisome due to their potential to form right-to-left shunts, resulting in life-threatening complications such as paradoxical embolism and stroke . We present a case of fatal air embolism in a young patient with a known history of HHT and recurring hemoptysis.
View Article and Find Full Text PDFbioRxiv
October 2024
Laboratory of Stem Cell and Neuro-Vascular Biology, Cell and Developmental Biology Center, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA.
The central nervous system (CNS) parenchyma has conventionally been believed to lack lymphatic vasculature, likely due to a non-permissive microenvironment that hinders the formation and growth of lymphatic endothelial cells (LECs). Recent findings of ectopic expression of LEC markers including Prospero Homeobox 1 (PROX1), a master regulator of lymphatic differentiation, and the vascular permeability marker Plasmalemma Vesicle Associated Protein (PLVAP), in certain glioblastoma and brain arteriovenous malformations (AVMs), has prompted investigation into their roles in cerebrovascular malformations, tumor environments, and blood-brain barrier (BBB) abnormalities. To explore the relationship between ectopic LEC properties and BBB disruption, we utilized endothelial cell-specific overexpression mutants.
View Article and Find Full Text PDFNat Rev Dis Primers
January 2025
European Reference Network for Rare Multisystemic Vascular Disease (VASCERN), HHT Rare Disease Working Group, Paris, France.
Hereditary haemorrhagic telangiectasia (HHT) is a vascular dysplasia inherited as an autosomal dominant trait and caused by loss-of-function pathogenic variants in genes encoding proteins of the BMP signalling pathway. Up to 90% of disease-causal variants are observed in ENG and ACVRL1, with SMAD4 and GDF2 less frequently responsible for HHT. In adults, the most frequent HHT manifestations relate to iron deficiency and anaemia owing to recurrent epistaxis (nosebleeds) or bleeding from gastrointestinal telangiectases.
View Article and Find Full Text PDFGen Thorac Cardiovasc Surg Cases
December 2024
Department of Thoracic Surgery, Kyoto University Graduate School of Medicine, Kyoto, 606-8507, Japan.
Background: Lung transplantation is a viable lifesaving option for patients with diffuse pulmonary arteriovenous malformations (AVMs). We present a case of diffuse pulmonary AVMs associated with juvenile polyposis and hereditary hemorrhagic telangiectasia (JP-HHT) that was successfully managed by lung transplantation.
Case Presentation: A 19-year-old woman developed severe hypoxemia due to pulmonary AVMs diagnosed at 4 years of age.
Semin Intervent Radiol
August 2024
Department of Radiology, University of Arkansas for Medical Sciences College of Medicine, Little Rock, Arkansas.
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder of the blood vessels that leads to the formation of telangiectasias and arteriovenous malformations (AVMs). HHT affects ∼1/5,000 people, but this varies significantly by geography and ancestry. The Curaçao criteria for HHT consist of four diagnostic criteria: spontaneous epistaxis, first-degree relative with HHT, AVMs in characteristic location (liver, lung, brain), and telangiectasias.
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