Right Atrial Thrombus Mimicking Myxoma and Bilateral Pulmonary Artery Aneurysms in a Patient with Behçet's Disease—A Case Report

Ag, Sayin; Fs, Vural; Ak, Bozkurt; B, Öz; Uygun, Nesrin

doi:10.1177/000331979304401111

Cited by 38 publications

(14 citation statements)

References 4 publications

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“…109 It was reported that the largest aneurysm was 7 cm, 109 and the mean diameter of aneurysms was 2.3±1.1 cm. 122 The present study, however, revealed much larger dimensions of PAAs with a mean diameter of 4.0 cm.…”

Section: Discussioncontrasting

confidence: 70%

Pulmonary artery aneurysms in Behçet disease

Yuan

2014

J. vasc. bras.

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Pulmonary artery aneurysms (PAAs) are the most common type of pulmonary involvement in Behçet' s disease. However, the relationships between clinical features and prognosis have not been sufficiently evaluated. This article describes the results of a comprehensive review, revealing that PAAs have a predilection for hemoptysis manifestations, increased dimensions, right lower lobar location, multiplicity and concurrent intramural thrombus formation. Surgical intervention was needed in one third of patients. Patients with massive hemoptysis and PAA rupture warranted emergency operations. Conservatively treated patients were prone to PAA progression; interventional embolization was associated with higher risks of recurrence and reintervention for PAAs; and surgically treated patients exhibited the highest mortality rates. In conclusion, PAAs in Behçet' s disease are characterized by a predilection for hemoptysis manifestations, right lower lobar location, multiplicity, and concurrent intramural thrombus formation. Both the condition itself and the surgical operations it warrants are linked with high mortality due to PAA hemorrhage.Keywords: hemoptysis; therapeutic embolization; vasculitis. ResumoOs aneurismas das artérias pulmonares (PAA) são as manifestações mais comuns dos pulmões na doença de Behçet. No entanto, as relações entre as características clínicas e o prognóstico ainda não foram devidamente explicadas. O objetivo do presente artigo foi fazer uma ampla revisão da literatura sobre esta questão. As fontes de dados contaram com uma ampla revisão bibliográfica dos anos de 1990 a 2013, sobre os seguintes temas: doença de Behçet, síndrome de HughesStovin, aneurisma de artéria pulmonar e pseudoaneurisma da artéria pulmonar. Os PAA evoluíram com predileção por hemoptise, aumento de dimensões, localização no lobo inferior direito, multiplicidade e formação de trombo intramural. A intervenção cirúrgica foi necessária em um terço dos pacientes. O tratamento cirúrgico emergencial foi indicado na vigência de ruptura do PAA e de hemoptise maciça. Os pacientes tratados conservadoramente evoluíram com propensão para a progressão do PAA. A embolização dos PAA foi associada a uma taxa maior de recidiva e de reintervenção. Houve diferença significativa entre os grupos quanto às taxas de mortalidade, tendo o grupo do tratamento cirúrgico apresentado a maior taxa. Dentre as variáveis citadas, a hemoptise, o envolvimento da artéria lobar e a ruptura do PAA foram fatores preditivos de maior risco de mortalidade. Houve diferenças significativas nas taxas de mortalidade entre os pacientes cirúrgicos e intervencionistas, e entre os três grupos de pacientes: cirúrgico, intervencionista e conservador. Os pacientes tiveram uma sobrevida global de 61,7% em um seguimento médio de 22,5 meses. Os PAA, na doença de Behçet, apresentaram as seguintes predileções: tendência a hemoptise, multiplicidade, localização no lobo inferior direito e presença de trombos intramurais. As rupturas e hemorragias dos PAA, aliadas ao necessário tr...

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Section: Discussioncontrasting

confidence: 70%

Pulmonary artery aneurysms in Behçet disease

Yuan

2014

J. vasc. bras.

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“…Ozkan et al (6) demonstrated that there is no difference in the prevalence of echocardiographic abnormalities between patients with BD and control subjects. Since the first description by Buge in 1977 (7) up to date, only about 50 cases have been reported (1,2,(8)(9)(10)(11)(12)(13)(14)(15)(16)(17).…”

Section: Discussionmentioning

confidence: 99%

“…The main differential diagnoses are primary heart tumour and infectious endocarditis. The differentiation from atrial myxoma is sometimes difficult based on the fact that the appearances of the myxoma may be atypical and the surface may be covered by thrombus (12,17). Imaging tests as CT chest, and MRN could be helpful in the assessment of thoracic manifestations of BD including thrombus of the systemic veins, heart and pulmonary arteries.…”

Section: Discussionmentioning

confidence: 99%

Intracardiac Thrombus in a Young Man: Don't Forget Behçet's Disease!

et al. 2012

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Intracardiac thrombosis is an exceptional complication of Behçet's disease. The management of this involvement is difficult due to the risk of recurrence. We present the case of a young man admitted to our hospital for intermittent fever. The microbiologic investigations did not show any causative germ. We discovered a right ventricle thrombus on echocardiography. We confirmed the diagnosis of pulmonary embolism on CT angiogram. The patient developed oral and genital ulcerations which were consistent with Behçet's syndrome. The thrombus had disappeared after treatment with anticoagulant, corticosteroid and immunosuppressors. Intracardiac thrombosis can reveal Behçet's disease. An exhaustive examination and close monitoring should be performed in order to reveal pathognomonic signs as soon as possible and to promptly start the appropriate treatment.

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“…Only 10 cases have been reported to date in the English literature. [3][4][5][6][7][8][9][10][11] As well, pulmonary involvement with this disease is rare, with the usual clinical presentation of pulmonary involvement being recurrent massive hemoptysis. 10,12 Repetitive pulmonary embolism has been reported in only 3 cases.…”

Section: Discussionmentioning

confidence: 99%