{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T04:41:42Z","timestamp":1791348102940,"version":"4.3.1"},"reference-count":20,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"4","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Circ: Arrhythmia and Electrophysiology"],"published-print":{"date-parts":[[2015,8]]},"abstract":"<jats:sec>\n            <jats:title>Background\u2014<\/jats:title>\n            <jats:p>Cardiac arrhythmias are common in Fabry disease (FD) and may occur in prehypertrophic cardiomyopathy suggesting an early compromise of conduction tissue (CT). Therefore, FD X-linked and CT may be variously involved in male and female patients with FD cardiomyopathy, affecting CT function.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Methods and Results\u2014<\/jats:title>\n            <jats:p>\n              Among 74 patients with endomyocardial biopsy diagnosis of FD cardiomyopathy, 13 (6 men; 7 women; mean age, 50.1\u00b113.5 years; maximal wall thickness, 16.7\u00b13.7 mm) had CT included in histological specimens and 6 also at electron microscopy. CT glycolipid infiltration was defined as focal, moderate, extensive, or massive, if involved \u226430%, \u226450%, &gt;50%, or 100% of cells; identified as loosely arranged small myocytes positive to HCN4 immunostaining, supplied by a centrally placed thick-walled arteriole. CT involvement was correlated with age, sex, and \u03b1-\n              <jats:italic>Gal<\/jats:italic>\n              gene mutation. CT function was evaluated by electrophysiological study and arrhythmias at Holter registration. CT infiltration was focal\/moderate in 4 women with no arrhythmias and normal electrophysiological study, extensive in 3 women with atrial or ventricular arrhythmias and short HV interval, and massive in 6 men with atrial fibrillation or ventricular arrhythmias and short HV. Short PR\/AH with increased refractoriness was additionally found in 3 patients with extensive\/massive CT infiltration. A male patient with the shortest HV presented infra-Hissian block during decremental atrial stimulation. There was no correlation with age, maximal wall thickness, and type of gene mutation.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusions\u2014<\/jats:title>\n            <jats:p>CT infiltration in FD cardiomyopathy is constant in men and variable in women because of skewed X-chromosome inactivation; its extensive\/massive involvement causes accelerated conduction with prolonged refractoriness and electric instability.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1161\/circep.114.002569","type":"journal-article","created":{"date-parts":[[2015,6,6]],"date-time":"2015-06-06T03:07:51Z","timestamp":1433560071000},"page":"799-805","update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":52,"title":["Pathology and Function of Conduction Tissue in Fabry Disease Cardiomyopathy"],"prefix":"10.1161","volume":"8","author":[{"given":"Andrea","family":"Frustaci","sequence":"first","affiliation":[{"name":"From the University of Rome Sapienza (A.F., E.M., P.F, C.C.), IRCCS L. Spallanzani (E.M., F.S., C.G., R.V., C.C.), and IRCCS S. Raffaele Pisana, Rome, Italy (M.A.R.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emanuela","family":"Morgante","sequence":"additional","affiliation":[{"name":"From the University of Rome Sapienza (A.F., E.M., P.F, C.C.), IRCCS L. Spallanzani (E.M., F.S., C.G., R.V., C.C.), and IRCCS S. Raffaele Pisana, Rome, Italy (M.A.R.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matteo A.","family":"Russo","sequence":"additional","affiliation":[{"name":"From the University of Rome Sapienza (A.F., E.M., P.F, C.C.), IRCCS L. Spallanzani (E.M., F.S., C.G., R.V., C.C.), and IRCCS S. Raffaele Pisana, Rome, Italy (M.A.R.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fernanda","family":"Scopelliti","sequence":"additional","affiliation":[{"name":"From the University of Rome Sapienza (A.F., E.M., P.F, C.C.), IRCCS L. Spallanzani (E.M., F.S., C.G., R.V., C.C.), and IRCCS S. Raffaele Pisana, Rome, Italy (M.A.R.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudia","family":"Grande","sequence":"additional","affiliation":[{"name":"From the University of Rome Sapienza (A.F., E.M., P.F, C.C.), IRCCS L. Spallanzani (E.M., F.S., C.G., R.V., C.C.), and IRCCS S. Raffaele Pisana, Rome, Italy (M.A.R.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Romina","family":"Verardo","sequence":"additional","affiliation":[{"name":"From the University of Rome Sapienza (A.F., E.M., P.F, C.C.), IRCCS L. Spallanzani (E.M., F.S., C.G., R.V., C.C.), and IRCCS S. Raffaele Pisana, Rome, Italy (M.A.R.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pasquale","family":"Franciosa","sequence":"additional","affiliation":[{"name":"From the University of Rome Sapienza (A.F., E.M., P.F, C.C.), IRCCS L. Spallanzani (E.M., F.S., C.G., R.V., C.C.), and IRCCS S. Raffaele Pisana, Rome, Italy (M.A.R.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Chimenti","sequence":"additional","affiliation":[{"name":"From the University of Rome Sapienza (A.F., E.M., P.F, C.C.), IRCCS L. Spallanzani (E.M., F.S., C.G., R.V., C.C.), and IRCCS S. Raffaele Pisana, Rome, Italy (M.A.R.)."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"e_1_3_3_2_2","first-page":"3733","volume-title":"Alpha-Galactosidase A Deficiency. Fabry Disease. The Metabolic and Molecular Bases of Inherited Disease","author":"Desnick RJ","year":"2001","unstructured":"Desnick RJ, Ioannou YA, Eng CM. Alpha-Galactosidase A Deficiency. Fabry Disease. The Metabolic and Molecular Bases of Inherited Disease. 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