title: Multimodality imaging for pre-clinical assessment of Fabry's cardiomyopathy.
abstract: Anderson Fabry's disease (AFD) is a rare but underdiagnosed intracellular lipid disorder which can cause left ventricular hypertrophy (LVH). Pre-clinical diagnosis of Fabry's disease is important as it permits early stratification for enzyme replacement therapy, improving the patient's long-term prognosis, avoiding progression to irreversible fibrosis, and preventing cardiovascular complications. Combinations of imaging modalities that integrate the strengths of each modality and at the same time eliminate weaknesses of an individual modality can offer improved diagnostics, therapeutic monitoring, and pre-clinical assessment of Fabry's disease. This review discusses the advantages and challenges in developing multimodality imaging systems of Fabry's cardiomyopathy, highlights some successful combinations that are now routinely used in the clinic and in research, and discusses recent advances in multimodality instrumentation that may offer new opportunities for pre-clinical assessment of this disease.
authors.affiliation: Texas Heart Institute and St Luke's Episcopal Hospital, Houston, TX, USA Institute of Cardiology, 'G. D'Annunzio' University, Chieti, C/o Ospedale SS. Annunziata, Via dei Vestini, 66013 Chieti, Italy rmadonna@unich.it rmadonna@heart.thi.tmc.edu.
authors.forename: Rosalinda
authors.initials: R
authors.lastname: Madonna
authors.affiliation: Texas Heart Institute and St Luke's Episcopal Hospital, Houston, TX, USA.
authors.forename: Cihan
authors.initials: C
authors.lastname: Cevik
authors.affiliation: Department Cuore e Grossi Vasi Attilio Reale, Sapienza University in Rome, Policlinico Umberto I, Rome, Italy.
authors.forename: Nino
authors.initials: N
authors.lastname: Cocco
mesh_terms: D009202:Cardiomyopathies / Q000175:diagnosis* / Q000209:etiology*; D042241:Early Diagnosis; D000795:Fabry Disease / Q000150:complications* / Q000175:diagnosis*; D006801:Humans; D064847:Multimodal Imaging
keywords: Fabry's disease; Molecular imaging; Multimodality imaging