The Heart Muscle and Valve Involvement in Marfan Syndrome, Loeys-Dietz Syndromes, and Collagenopathies.

Antonello Credendino, Guglielmina Pepe, Giuseppe Limongelli, Maria Giovanna Russo, Stefano Nistri, Alessandro Della Corte, Gioacchino Scarano, Flavia Chiosi, Francesco Natale, Giovanni Signore, Adelaide Fusco, Emanuele Monda, Martina Caiazza, Annapaola Cirillo, Chiara Granato, Fiorella Fratta, Giuseppe Palmiero, Michele Lioncino, Alfredo Mauriello

Journal: Heart failure clinics 2021;18(1):165-175

PMID: 34776077

Full Text on Elsevier Science View on PubMed

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.

Address: Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli", Naples 80131, Italy.; Grupo de Enfermedades Cardiovasculares, Vall d'Hebron Institut de Recerca (VHIR), Barcelona, Spain.; Department of Orthopaedics, AORN dei Colli, Monaldi Hospital.; Department of Ophthalmology, Azienda Ospedaliera dei Colli AORN Monaldi, Naples 80100, Italy.; Cardiology Service, CMSR Veneto Medica, Altavilla Vicentina, Italy.; Department of Experimental and Clinical Medicine, University of Florence, CRR Tuscany Marfan Center, Florence, Italy. Electronic address: [email protected].

DOI: 10.1016/j.hfc.2021.07.007

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MeSH Terms: Humans, Loeys-Dietz Syndrome, Marfan Syndrome, Myocardium