Severe biventricular thrombosis in eosinophilic granulomatosis with polyangiitis: a case report
Autor: | Jihad Hamudi, Basheer Karkabi, Devy Zisman, Avinoam Shiran |
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Jazyk: | angličtina |
Rok vydání: | 2020 |
Předmět: |
medicine.medical_specialty
Heart failure Case Reports 03 medical and health sciences 0302 clinical medicine Cardiac magnetic resonance imaging Internal medicine Case report medicine Eosinophilia AcademicSubjects/MED00200 cardiovascular diseases Cardiac thrombosis Anti-neutrophil cytoplasmic antibody 030203 arthritis & rheumatology Cardiac Imaging (Echocardiography / Cardiac MRI / Nuclear Cardiology) Ejection fraction medicine.diagnostic_test business.industry Eosinophilic granulomatosis with polyangiitis medicine.disease Thrombosis Echocardiography Cardiology cardiovascular system medicine.symptom Cardiology and Cardiovascular Medicine Granulomatosis with polyangiitis business Heart failure with preserved ejection fraction 030217 neurology & neurosurgery |
Zdroj: | European Heart Journal: Case Reports |
ISSN: | 2514-2119 |
Popis: | Background Eosinophilic 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. |
Databáze: | OpenAIRE |
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