ArticleEuropean heart journal. Case reports2020
Severe biventricular thrombosis in eosinophilic granulomatosis with polyangiitis: a case report.
Article in European heart journal. Case reports, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.
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Who cites it
3 citing papers in PubMed, 4 citations in OpenAlex.
- ANCA negative eosinophilic granulomatosis with polyangiitis (EGPA) presenting with left ventricular thrombus: An appreciation of distinct phenotypes and eosinophilic driven pathogenesis.Respirology case reports · 2022Article
- A Rare Presentation of Granulomatosis With Polyangiitis with Multiple Cardiac Valvular Insufficiencies.Cureus · 2022Article
- Cardiac Magnetic Resonance in Rheumatology to Detect Cardiac Involvement Since Early and Pre-clinical Stages of the Autoimmune Diseases: A Narrative Review.Frontiers in cardiovascular medicine · 2022Review
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundEosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare multisystem disease characterized by asthma, rhinosinusitis, and eosinophilia. Cardiac involvement, present in half the patients, may be life threatening. CASE SUMMARY: A young woman with long-standing asthma and nasal polyposis was admitted with new-onset dyspnoea, sinus tachycardia, and eosinophilia. She had severe biventricular thrombosis and severe tricuspid regurgitation (TR) on echocardiography, with preserved ejection fraction of both ventricles. Cardiac magnetic resonance (CMR) imaging showed diffuse subendocardial late gadolinium enhancement (LGE). She had a positive test for perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) confirming the diagnosis of ANCA positive EGPA. She was treated with anticoagulation, high-dose corticosteroids, cyclophosphamide, and rituximab with gradual resolution of her symptoms. Follow-up echocardiography showed significant improvement in ventricular thrombi and TR but could not reliably exclude residual ventricular thrombus. Repeat CMR at 11 months confirmed complete resolution of both ventricular thrombi and near complete resolution of LGE. DISCUSSION: Cardiac involvement in EGPA, a rare cause of heart failure, can manifest as severe biventricular thrombosis and severe TR, resulting in heart failure with preserved ejection fraction. Combined immunosuppression and anticoagulation can lead to complete remission within a year. CMR is instrumental for both diagnosis and follow-up of EGPA, allowing for safe discontinuation of oral anticoagulation.
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