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AIMS To assess the natural history, risk factors for death and deterioration of patients with Eisenmenger Syndrome. METHODS The clinical course of 188 patients from three different cardiac centres specialized in adult congenital heart defects, followed for a median period of 31 years, was retrospectively analysed. According to the diagnosis, 64 males and(More)
OBJECTIVE This multicenter study retrospectively analyzes the data on 114 patients with protein-losing enteropathy after Fontan-type surgery. Special attention was given to the different treatment strategies used and their effect on outcome. METHODS AND RESULTS In 35 participating centers 3029 Fontan operations were performed. The incidence of(More)
BACKGROUND There is a high incidence of anomalous coronary arteries in subjects with congenital heart disease. These abnormalities can be responsible for myocardial ischemia and sudden death or be damaged during surgical intervention. It can be difficult to define the proximal course of anomalous coronary arteries with the use of conventional x-ray coronary(More)
BACKGROUND Life-threatening ventricular arrhythmia and sudden death remain serious late complications after tetralogy of Fallot repair. Nevertheless, there remains no clear way of predicting which patients are at risk. METHODS AND RESULTS The study population included a total of 178 adult survivors (mean follow-up, 21.4 years) of tetralogy of Fallot(More)
AIMS Infective endocarditis accounts for 4% of admissions to a specialized unit for grown-up congenital heart patients. This study defines lesions susceptible to infection, antecedent events, organisms, outcome and surgical treatment in a group of such patients. METHODS AND RESULTS The grown-up congenital heart disease database was searched for all(More)
OBJECTIVES This study was conducted to investigate the presentation and outcome of patients with Ebstein's anomaly of the tricuspid valve. BACKGROUND Ebstin's anomaly may present at any age and has a highly variable clinical course. Previous natural history studies have been based on clinical and angiographic diagnosis and have included mainly older(More)
BACKGROUND Congenital heart defects are generally assumed to have a multifactorial aetiology. We have tested this hypothesis by studying adults with heart defects and their families. METHODS We identified 1094 patients who survived surgery for major cardiac defects before 1970. We chose individuals with disturbance of situs or segmental connection, with(More)
BACKGROUND The inability to obtain complete diagnoses with transthoracic echocardiography in many adults with congenital heart disease provided the incentive to evaluate prospectively the individual and combined roles of magnetic resonance imaging (MRI) and transesophageal echocardiography (TEE) as "second-line" techniques for unresolved diagnostic(More)
BACKGROUND Pulmonary autograft replacement of the diseased aortic valve has not been widely practiced due to concerns regarding late autograft competence and the consequences of creating pulmonary valve disease. To investigate this, the fate of the pioneering series of patients has been determined. METHODS AND RESULTS The 131 hospital survivors of the(More)