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Congenital multiple coronary arteriovenous fistulas.

ABSTRACT: Coronary arteriovenous fistulas are congenital or acquired abnormalities characterized by abnormal communication between the coronary circulation and cardiac chambers or other vessels. Frequently, patients are asymptomatic and their diagnosis can be carried out incidentally by echocardiography. Knowing the echocardiographic findings characteristic of this malformation will prevent the diagnosis from going unnoticed.

SUBMITTER: Lacalzada-Almeida J

PROVIDER: S-EPMC6132164 | biostudies-literature | 2018 Sep

REPOSITORIES: biostudies-literature

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Congenital multiple coronary arteriovenous fistulas.

Lacalzada-Almeida Juan J García-Niebla Javier J Izquierdo-Gómez María M MM Marí-López Belén B Laynez-Cerdeña Ignacio I Bosa-Ojeda Francisco F

Clinical case reports 20180713 9

Coronary arteriovenous fistulas are congenital or acquired abnormalities characterized by abnormal communication between the coronary circulation and cardiac chambers or other vessels. Frequently, patients are asymptomatic and their diagnosis can be carried out incidentally by echocardiography. Knowing the echocardiographic findings characteristic of this malformation will prevent the diagnosis from going unnoticed. ...[more]

PMID: 30214786

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Project description:BACKGROUND:Dural arteriovenous fistulas are intracranial vascular malformations, fed by dural arteries and draining venous sinuses or meningeal veins. Clinical course varies widely and ranges from benign with spontaneous remission to fatal, due to cerebral hemorrhage. In a 10-year single institution experience, clinical presentation of dural arteriovenous fistulas, and in particular headache and angiographic features, as well as long-term outcome were analyzed. METHODS:Data of 42 intracranial dural arteriovenous fistulas of 40 patients concerning demographic characteristics, medical history and risk factors, clinical presentation and headache features, location and neuroimaging findings, as well as treatment and outcome, were collected. Furthermore, we used the modified-Rankin Scale to assess the long-term outcome, by telephone contact with patients and/or their relatives. RESULTS:Patients aged between 25 and 89?years (mean age 55.8?±?15.5). According to different clinical presentation and evolution, related to their unique drainage pattern into the cavernous sinus, we examined the carotid-cavernous fistulas separately from other dural arteriovenous fistulas. Interestingly, we found that the migraine-like headache was the major onset symptom of dural arteriovenous fistulas different from carotid-cavernous fistulas (p?=?0.036). On the other hand, non-migraine-like headache was a typical characteristic of carotid-cavernous fistulas (p?=?0.003). Moreover, ocular symptoms were more frequently observed in carotid-cavernous fistulas (92.9% p?< 0.001). Seventy percent of patients did not report any impact on quality of life (mRS 0 or 1) at follow-up. CONCLUSIONS:These findings suggest a link between the site of lesion and clinical features of the headache, a symptom that usually leads to hospitalization. In particular, ocular symptoms accompanying non-migraine-like headache should be promptly recognized and raise the suspicion of a carotid-cavernous fistula, while migraine-like headache may suggests other dural arteriovenous fistulas. This study provides new significant insights on headache and its characteristics as a presentation symptom in dural arteriovenous fistulas.

Project description:Background and purposeA minority of intracranial dural arteriovenous fistulas progress with time. We sought to determine features that predict progression and define outcomes of patients with progressive dural arteriovenous fistulas.Materials and methodsWe performed a retrospective imaging and clinical record review of patients with intracranial dural arteriovenous fistula evaluated at our hospital.ResultsOf 579 patients with intracranial dural arteriovenous fistulas, 545 had 1 fistula (mean age, 45 ± 23 years) and 34 (5.9%) had enlarging, de novo, multiple, or recurrent fistulas (mean age, 53 ± 20 years; P = .11). Among these 34 patients, 19 had progressive dural arteriovenous fistulas with de novo fistulas or fistula enlargement with time (mean age, 36 ± 25 years; progressive group) and 15 had multiple or recurrent but nonprogressive fistulas (mean age, 57 ± 13 years; P = .0059, nonprogressive group). Whereas all 6 children had fistula progression, only 13/28 adults (P = .020) progressed. Angioarchitectural correlates to chronically elevated intracranial venous pressures, including venous sinus dilation (41% versus 7%, P = .045) and pseudophlebitic cortical venous pattern (P = .048), were more common in patients with progressive disease than in those without progression. Patients with progressive disease received more treatments than those without progression (median, 5 versus 3; P = .0068), but as a group, they did not demonstrate worse clinical outcomes (median mRS, 1 and 1; P = .39). However, 3 young patients died from intracranial venous hypertension and intracranial hemorrhage related to progression of their fistulas despite extensive endovascular, surgical, and radiosurgical treatments.ConclusionsFew patients with dural arteriovenous fistulas follow an aggressive, progressive clinical course despite treatment. Younger age at initial presentation and angioarchitectural correlates to venous hypertension may help identify these patients prospectively.

Dataset Information

Congenital multiple coronary arteriovenous fistulas.

Publications

Congenital multiple coronary arteriovenous fistulas.

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