Acute Aortic Insufficiency Associated With Wegener Granulomatosis

Leff, Robert D.; Hellman, Richard; Mullany, Charles J.

doi:10.4065/74.9.897

Cited by 26 publications

(13 citation statements)

References 9 publications

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“…In the study of Penmetcha et al [10], severe mitral valve regurgitation in a case of scleroderma/systemic lupus overlap was reported. And in a report by Leff et al [11], acute aortic insufficiency in a 17-year-old patient with Wegener's granulomatosis was reported.…”

Section: Discussionmentioning

confidence: 99%

Rheumatological Findings in Candidates for Valvular Heart Surgery

et al. 2012

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Background and Objectives. Valvular heart diseases are among the frequent causes of cardiac surgery. Some patients have a well-known rheumatic condition. Heart valves are fragile connective tissues which are vulnerable to any systemic autoimmune diseases. This study was designed to evaluate the frequency of rheumatological background in patients candidate for valvular heart surgery in Afshar Cardiovascular Center, Yazd, Iran. Methods. One hundred and twenty (120) patients candidate for valvular heart surgery were selected for this study. Careful history and physical examination were undertaken from rheumatological stand points. The most sensitive screening serologic tests were also assayed. Results. The result of this study showed that 53.3% were male and 46.6% were female with mean age of 48.18 ± 17.65 years old. 45.8% of the patients had history of nonmechanical joint disease, 14.2% had history of rheumatological conditions in their family, and 30% had history of constitutional symptoms. 29.8% had positive joint dysfunction findings in their physical examination while 25.8% had anemia of chronic disease. Positive Rheumatoid factor (RF), anticyclic citrullinated peptide (CCP, ACPA), C-reactive protein (CRP), antinuclear antibody (ANA), abnormal urine and elevated erythrocyte sedimentation rate (ESR) were 34, 2.5, 26.7, 4.2, 5, and 36.7%, respectively. Antineutrophil cytoplasmic antibody (ANCA) and antiphospholipid (APL) were positive in a few cases. Conclusion. The findings of this study show immunologic bases for most patients with valvular heart diseases candidate for surgery. Undifferentiated connective tissue diseases may play an important role in the pathophysiology of valvular damage.

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Section: Discussionmentioning

confidence: 99%

Rheumatological Findings in Candidates for Valvular Heart Surgery

et al. 2012

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“…The involvement of the heart or great vessels in ANCA‐associated vasculitis syndromes is exceptional 3,4 . The accepted pathogenesis of the disease is ANCA's induction of vasa vasorum vasculitis of large vessels, such as the aorta, and the pathologic progress of aortitis and valvitis syndromes.…”

Section: Discussionmentioning

confidence: 99%

“…1 The involvement of the heart or great vessels in ANCA-associated vasculitis syndromes is exceptional. 3,4 The accepted pathogenesis of the disease is ANCA's induction of vasa vasorum vasculitis of large vessels, such as the aorta, and the pathologic progress of aortitis and valvitis syndromes. The inflammatory process causes aortic root dilatation and cusp retraction, leading to valvular incompetence and hemodynamic compromise.…”

Section: Discussionmentioning

confidence: 99%

Necrotizing Vasculitis: A Cause of Aortic Insufficiency and Conduction System Disturbance

Pérez

García²,

Marquès

et al. 2003

Echocardiography

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Cardiac involvement in vasculitis syndromes is uncommon. We describe a 50-year-old male who presented with progressive dyspnea and myalgies. Echocardiogram revealed significant thickening of aortic root, aortic cusps, and anterior mitral valve leaflet, with severe aortic regurgitation that required aortic valve replacement. Furthermore, this patient suffered progressive atrioventricular block that needed implantation of a pacemaker. The study performed disclosed the presence of necrotizing vasculitis positive for perinuclear antineutrophil cytoplasmic antibody.

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“…A transthoracic echocardiogram was performed in the workup revealing grade 2 aortic insufficiency. Blood cultures were negative, but with strong clinical suspicion, the diagnosis of WG was made with an ANCA titer of 1:512 [6].…”

Section: Review Of the Literaturementioning

confidence: 99%

Cardiac manifestations of Wegener's granulomatosis: Case report and review of the literature

Ruisi

Finkielstein

2010

Journal of Cardiology Cases

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Wegener's granulomatosis, first characterized as a clinical syndrome in 1936, is a rare form of vasculitis of the small-and medium-sized blood vessels affecting mainly the upper and lower respiratory tracts as well as the kidneys. This review article describes the case of a 45-year-old man who developed aortic regurgitation and third degree heart block secondary to Wegener's granulomatosis. He subsequently presented to our institution 3 years later with pulmonary hemorrhage. The ensuing literature review reveals that historically clinical cardiac involvement in Wegener's has been rare, particularly with valvular lesions. However, recent literature has shown an increase in reported cases of cardiac valvular lesions in Wegener's. The presented case report along with recently reported case reports highlight the morbidity and mortality associated with such cardiac lesions. We believe routine cardiovascular supervision including echocardiography and electrocardiograms are important for the screening and monitoring of patients with Wegener's granulomatosis.

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Acute Aortic Insufficiency Associated With Wegener Granulomatosis

Cited by 26 publications

References 9 publications

Rheumatological Findings in Candidates for Valvular Heart Surgery

Rheumatological Findings in Candidates for Valvular Heart Surgery

Necrotizing Vasculitis: A Cause of Aortic Insufficiency and Conduction System Disturbance

Cardiac manifestations of Wegener's granulomatosis: Case report and review of the literature

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