The Clinicopathologic Spectrum of Hypertrophic Cardiomyopathy. The Experience of the Italian Heart Transplant Program
| Autor: | Antonello Gavazzi, Cesare Bosman, M. Vigano, Edoardo Gronda, G. Catani, U. Livi, Angela Pucci, Edgardo Bonacina, Eloisa Arbustini, Pietro Gallo, R. De Maria, E. Donegani, G. Thiene, Maurizio Porcu, A. Parma, A. Fiocchi, C. Rapezzi, G. Gagliardi, E. Angelini, Giorgio Baroldi, L. De Biase, O. Leone |
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| Rok vydání: | 1998 |
| Předmět: |
Heart transplantation
medicine.medical_specialty business.industry medicine.medical_treatment Cardiomyopathy Restrictive cardiomyopathy Hypertrophic cardiomyopathy Disease medicine.disease Sudden death Natural history Heart failure Internal medicine cardiovascular system medicine Cardiology cardiovascular diseases business |
| Zdroj: | Advances in Cardiomyopathies ISBN: 9788847021815 |
| DOI: | 10.1007/978-88-470-2155-6_9 |
| Popis: | Hypertrophic cardiomyopathy (HCM) is an idiopathic cardiac disease which is predominantly genetic in origin and transmitted as an autosomally dominant trait [1, 2]. The mutated genes code for altered contractile myofibrillar proteins, leading to deformed myocytes and histological evidence of extensive myocell disorganization, namely the disarray typical of hypertrophie cardiomyopathy (HCM). In the natural history of HCM endstage heart failure is relatively infrequent and associated with a pattern of left ventricular systolic impairment with cavity dilatation, which occurrs in about 10% of patients [3, 4], with a yearly incidence of 1.5% [5]. End-stage disease may coexist with premature sudden death in family members carrying the same genetic abnormality [6]. This pattern is characterized by a particularly poor prognosis, so that heart transplantation has been advocated in this subgroup of patients. |
| Databáze: | OpenAIRE |
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