Early degeneration of two different tricuspid bioprostheses in restrictive cardiomyopathy
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Abstract:
Early degeneration of tricuspid bioprostheses is a rare, but extremely serious, complication. Several mechanisms have been described, both for surgically implanted and transcatheter bioprosthesis. We report a case of early degeneration of tricuspid porcine bioprosthesis and a subsequent transcatheter valve-in-valve bovine prosthesis due to severe fibrosis with leaflet retraction in a patient with restrictive cardiomyopathy who finally underwent orthotopic heart transplantation.Keywords:
Degeneration (medical)
Restrictive cardiomyopathy
Endomyocardial fibrosis
Idiopathic hypereosinophilic syndrome (HES), a systemic disease that commonly involves the heart leading to progressive endomyocardial fibrosis, frequently manifests as restrictive cardiomyopathy. In this report, we describe the first case of a patient with endomyocardial fibrosis due to HES who underwent orthotopic heart transplantation at our institution. A literature review and discussion are included.
Endomyocardial fibrosis
Restrictive cardiomyopathy
Hypereosinophilic Syndrome
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Endomyocardial fibrosis
Restrictive cardiomyopathy
Peripheral edema
Etiology
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Endomyocardial fibrosis (EMF) was first described in 1948. It is an idiopathic disorder, characterized by the formation of fibrous tissue on the endocardial aspect of one or both ventricles, resulting in a restrictive cardiomyopathy with atrial enlargement. Originally described in Uganda, numerous cases have also been described in the Middle East.
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Restrictive cardiomyopathy
Atrium (architecture)
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Tropical endomyocardial fibrosis is a common cause of restrictive cardiomyopathy worldwide, but is relatively rare in developed countries. We present a case of tropical endomyocardial fibrosis with right ventricular involvement initially mistaken as Ebstein's anomaly. We highlight the need for timely and accurate diagnosis to ensure appropriate management. (Level of Difficulty: Intermediate.)
Endomyocardial fibrosis
Restrictive cardiomyopathy
Endomyocardial Biopsy
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Restrictive cardiomyopathies have been variously classified and interpreted. Although their etiologic and anatomic features may vary, their common denominator is indicated by restrictive hemodynamic behavior of either the left or right ventricle or both. This report describes a case in which such restriction was caused by obliterative endomyocardial fibrosis of unknown etiology. Because of significant symptomatology, including congestive failure and syncope, the patient underwent surgical excision of the endomyocardial overgrowth.
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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.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.
Endomyocardial fibrosis
Restrictive cardiomyopathy
Tricuspid stenosis
Ebstein's anomaly
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Endomyocardial fibrosis
Restrictive cardiomyopathy
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Restrictive cardiomyopathy
Endomyocardial fibrosis
Endocardium
Myocardial fibrosis
Left atrial enlargement
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Eleven patients with a restrictive cardiomyopathy are described. Seven of these had biventricular disease and in them the haemodynamic abnormality resembled that in constrictive pericarditis but the condition was distinguishable because of unequal involvement of the two ventricles, murmurs of atrioventricular valve regurgitation, or characteristic ventricular deformity on angiography. Two of these patients had eosinophilia with the clinical features of Löffer's endocarditis. In 4 patients the disease was apparently confined to the left ventricle; they were investigated on account of atypical chest pain, third heart sound, or abnormal electrocardiogram. Simultaneous measurements of left ventricular pressure and volume throughout diastole were made in 3 patients and showed rapid but abruptly curtailed left ventricular filling. Transvenous endomyocardial biopsy in 2 patients showed fibrous tissue with collagen and irregular elastic fibrils. Surgical biopsy in 3 patients excluded pericardial constriction but was diagnostically unhelpful because of failure to obtain endocardium. Necropsy in one patient showed that the heart had features indistinguishable from tropical endomyocardial fibrosis. It is suggested that the spectrum of ventricular abnormalities in these patients results from endomyocardial fibrosis of varying severity and probably of differing cause. This study has shown that "tropical" endomyocardial fibrosis may occur outside the tropics and suggests that eosinophilia may play a leading role or associated part in the genesis of some cases.
Endomyocardial fibrosis
Restrictive cardiomyopathy
Hypereosinophilic Syndrome
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Endomyocardial fibrosis
Restrictive cardiomyopathy
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