ABSTRACT
Cardiac angiosarcoma (CA) is the most common primary malignant heart tumour. Its atypical symptoms and rapidly progressive nature contribute to delayed diagnosis and poor outcome. We report the case of a 52-year-old woman admitted with a large pericardial effusion. An extensive study of the aetiology of the pericardial effusion was inconclusive. Two months later the patient returned with ischaemic stroke. An echocardiogram revealed a probable right atrium contained rupture. The patient was submitted to surgical correction but died 9 days later. Histology revealed an angiosarcoma. This case exemplifies the atypical presentation of CA and highlights the importance of a multimodal diagnostic work-up in patients with idiopathic pericardial effusion. LEARNING POINTS: Cardiac angiosarcoma is often overlooked as an initial diagnosis because of its rarity and atypical symptoms, which, in association with its aggressiveness, contribute to delayed diagnosis and fatal outcome.Pericardial biopsy is an important technique that may help to disclose the aetiology of pericardial effusion and should be considered for the confirmation of malignant pericardial disease.Patients presenting with pericardial effusion with cardiac tamponade with an unclear cause after diagnostic work-up should be followed closely.
ABSTRACT
Tricuspid stenosis is an uncommon valvular abnormality commonly associated with other valvular lesions. Ebstein's anomaly is a rare congenital heart malformation characterized primarily by abnormalities of the tricuspid valve and right ventricle. Endomyocardial fibrosis is a restrictive cardiomyopathy observed in tropical and subtropical regions. It may cause right ventricular distortion with apparent apical displacement of the tricuspid valve, mimicking Ebstein's anomaly. Eosinophilia is the most commonly cited aetiological link in endomyocardial fibrosis. Here we report the case of 42-year-old male patient who presented with heart failure and severe tricuspid stenosis where a diagnosis of hypereosinophilic syndrome was also established. This case represented a diagnostic challenge in the search for the definitive cause of the tricuspid stenosis. LEARNING POINTS: Ebstein's anomaly is a rare congenital heart malformation characterized primarily by abnormalities of the tricuspid valve and right ventricle. The tricuspid valve is usually incompetent, and very rarely stenotic.Hypereosinophilic syndromes can be associated with heart damage. The fibrotic stage of eosinophil-mediated heart damage is characterized by altered cardiac function due to either compromise/entrapment of the cordae tendineae and/or restrictive cardiomyopathy.Endomyocardial fibrosis is a restrictive cardiomyopathy observed in tropical and subtropical regions that may be indistinguishable from the Loeffler's endocarditis observed in temperate climates. It may cause right ventricle distortion and apical displacement of the tricuspid valve, mimicking Ebstein's anomaly.
