1. The Heart Muscle and Valve Involvement in Marfan Syndrome, Loeys-Dietz Syndromes, and Collagenopathies
- Author
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Gioacchino Scarano, Francesco Natale, Giuseppe Palmiero, Guglielmina Pepe, Antonello Credendino, Adelaide Fusco, Giuseppe Limongelli, Alessandro Della Corte, Martina Caiazza, Giovanni Signore, Alfredo Mauriello, Chiara Granato, Flavia Chiosi, Michele Lioncino, Maria Giovanna Russo, Annapaola Cirillo, Fiorella Fratta, Emanuele Monda, Stefano Nistri, Fusco, A., Mauriello, A., Lioncino, M., Palmiero, G., Fratta, F., Granato, C., Cirillo, A., Caiazza, M., Monda, E., Credendino, A., Signore, G., Natale, F., Chiosi, F., Scarano, G., Della Corte, A., Nistri, S., Russo, M. G., Limongelli, G., and Pepe, G.
- Subjects
Marfan syndrome ,Pathology ,medicine.medical_specialty ,Fibrillinopathie ,Connective tissue ,Loeys–Dietz syndrome ,Cardiomyopathy: mitral valve prolapse ,Marfan Syndrome ,medicine ,Humans ,Mitral valve prolapse ,Ectopia lentis ,Collagenophatie ,Aortic dissection ,Loeys-Dietz Syndrome ,business.industry ,Myocardium ,TGFbetapathies ,General Medicine ,medicine.disease ,medicine.anatomical_structure ,Ehlers–Danlos syndrome ,Heart failure ,Ehlers-Danlos syndrome ,Cardiology and Cardiovascular Medicine ,business - Abstract
The inherited connective tissue disorders (Marfan syndrome, Loeys-Dietz syndrome [LDS], and Ehlers-Danlos syndrome [EDS]) involve connective tissue of various organ systems. These pathologies share many common features, nonetheless compared to Marfan syndrome, LDS' cardiovascular manifestations tend to be more severe. In contrast, no association is reported between LDS and the presence of ectopia lentis. The EDS are currently classified into thirteen subtypes. There is substantial symptoms overlap between the EDS subtypes, and they are associated with an increased incidence of cardiovascular abnormalities, such as mitral valve prolapse and aortic dissection.
- Published
- 2022