data set from the article Camporeale A, Pieroni M, Pieruzzi F, Lusardi P, Pica S, Spada M, Mignani R, Burlina A, Bandera F, Guazzi M, Graziani F, Crea F, Greiser A, Boveri S, Ambrogi F, Lombardi M. Predictors of Clinical Evolution in Prehypertrophic Fabry Disease. Circ Cardiovasc Imaging. 2019 Apr;12(4):e008424. doi: 10.1161/CIRCIMAGING.118.008424. PMID: 30943767.
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This is the abstract:
\n\nBackground: In prehypertrophic Fabry disease, low myocardial T1 values, reflecting sphingolipid storage, are associated with early structural and ECG changes. The correlations between T1 values and functional parameters have not been explored. Furthermore, the potential prognostic role of T1 in predicting disease worsening is still unknown.
\n\nMethods: ECG, 2D echocardiography, cardiopulmonary test, and cardiac magnetic resonance were performed in 44 Fabry patients without left ventricular hypertrophy (35.7±14.5 years, 68.2% females). After a 12-month follow-up, clinical stability was evaluated using Fabry Stabilization Index.
\n\nResults: At baseline, T1 values showed a negative correlation with left ventricular mass ( r=-0.79; P<0.0001), maximum wall thickness ( r=-0.79; P<0.0001), Sokolow-Lyon Index ( r=-0.54; P<0.0001), left atrial volume ( r=-0.49; P<0.0002), and Mainz Severity Score Index ( r=-0.61; P<0.0001). No significant differences in systo-diastolic function and exercise capacity were observed comparing normal and low T1 Fabry patients. Arrhythmias were reported in 2 females with low T1 and late gadolinium enhancement. Five patients (40.0±12.4 years, 2 females) showed clinical worsening (Fabry Stabilization Index >20%) at follow-up. Higher left ventricular wall thickness (odds ratio, 2.61; CI, 1.04-6.57; P=0.04), left atrial volume (odds ratio, 1.24; CI, 1.02-1.51; P=0.03), and lower T1 values (odds ratio, 0.98; CI, 0.96-0.99; P=0.03) at baseline were independently associated with clinical worsening at follow-up.
\n\nConclusions: In prehypertrophic Fabry disease, low T1 values correlate with early electrocardiographic, morphological cardiac changes, and worsening of global disease severity but are not associated with functional abnormalities. The presence of low T1 values is a risk factor for disease worsening, thus representing a potential new tool in prognostic stratification and therapeutic approach.
", "languages": [ { "id": "eng", "title": { "en": "English" } } ], "publication_date": "2020-06-08", "publisher": "Zenodo", "resource_type": { "id": "dataset", "title": { "de": "Datensatz", "en": "Dataset" } }, "subjects": [ { "subject": "Fabry disease;" }, { "subject": "female;" }, { "subject": "gadolinium;" }, { "subject": "hypertrophy, left ventricular" }, { "subject": "magnetic resonance imaging;" }, { "subject": "risk factors." } ], "title": "Data set from the article Camporeale A, Pieroni M, Pieruzzi F, Lusardi P, Pica S, Spada M, Mignani R, Burlina A, Bandera F, Guazzi M, Graziani F, Crea F, Greiser A, Boveri S, Ambrogi F, Lombardi M. Predictors of Clinical Evolution in Prehypertrophic Fabry Disease. Circ Cardiovasc Imaging. 2019 Apr;12(4):e008424. doi: 10.1161/CIRCIMAGING.118.008424. PMID: 30943767." }, "parent": { "access": { "owned_by": { "user": "93055" }, "settings": { "accept_conditions_text": "PdF 3D av PdF 3D available from the corresponding author on reasonable request ailable from the corresponding author on reasonable request
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\r\nOur institute is a didactic center of the Università degli Studi di Milano and it hosts the Faculty of Medicine, the Faculty of Nursing Sciences and some postgraduate medical residency programs. Moreover, our hospital is a medical training center chosen by many foreign students and doctors.
\r\nIn 1988 was established the Cardiovascular Department “Edmondo Malan” comprehensive of every medical and surgical cardiovascular specialties; since then, patients from every region of Italy and from all over the world have been successfully treated.
\r\nThanks to its proven expertise and high quality health care and due to the relevant scientific contribution of its medical staff, Policlinico San Donato was recognized by the Italian Ministry of Health as Institute for Research, Hospitalization and Health Care for the study and treatment of cardiac and great vessel diseases (Istituto di Ricovero e Cura a Carattere Scientifico per lo studio e la cura delle Malattie del cuore e dei grandi vasi nell’adulto e nel bambino – Ministerial Decree, 6 March 2006).
\r\nOur research activities are directly connected with healthcare providing specialized and tailored therapies and treatment to every patient, from fetuses to oldest old people.
\r\nOur research projects are predominantly clinical with a translational approach supported by our biomolecular, bioengineering and advanced-imaging research laboratories. Heart failure, valvular heart diseases, Brugada Syndrome, congenital cardiopathies, aortic diseases and cardiometabolic diseases are the major interests of the lines of research pursued by our IRCCS.