title: Significance of Late Gadolinium Enhancement at Right Ventricular Attachment to Ventricular Septum in Patients With Hypertrophic Cardiomyopathy.
abstract: Cardiovascular magnetic resonance (CMR) with extensive late gadolinium enhancement (LGE) is a novel marker for increased risk for sudden death (SD) in patients with hypertrophic cardiomyopathy (HC). Small focal areas of LGE confined to the region of right ventricular (RV) insertion to ventricular septum (VS) have emerged as a frequent and highly visible CMR imaging pattern of uncertain significance. The aim of this study was to evaluate the prognostic significance of LGE confined to the RV insertion area in patients with HC. CMR was performed in 1,293 consecutive patients with HC from 7 HC centers, followed for 3.4 ± 1.7 years. Of 1,293 patients (47 ± 14 years), 134 (10%) had LGE present only in the anterior and/or inferior areas of the RV insertion to VS, occupying 3.7 ± 2.9% of left ventricular myocardium. Neither the presence nor extent of LGE in these isolated areas was a predictor of adverse HC-related risk, including SD (adjusted hazard ratio 0.82, 95% confidence interval 0.45 to 1.50, p = 0.53; adjusted hazard ratio 1.16/10% increase in LGE, 95% confidence interval 0.29 to 4.65, p = 0.83, respectively). Histopathology in 20 HC hearts show the insertion areas of RV attachment to be composed of a greatly expanded extracellular space characterized predominantly by interstitial-type fibrosis and interspersed disorganized myocyte patterns and architecture. In conclusion, LGE confined to the insertion areas of RV to VS was associated with low risk of adverse events (including SD). Gadolinium pooling in this region of the left ventricle does not reflect myocyte death and repair with replacement fibrosis or scarring.
authors.affiliation: Division of Cardiology, Toronto General Hospital, University Health Network, University of Toronto, Toronto, Ontario, Canada; Cardiovascular Division, Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts. Electronic address: raymond.chan@utoronto.ca.
authors.forename: Raymond H
authors.initials: RH
authors.lastname: Chan
authors.affiliation: The Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, Minnesota.
authors.forename: Barry J
authors.initials: BJ
authors.lastname: Maron
authors.affiliation: Referral Center for Myocardial Diseases, Azienda Ospedaliera Universitaria Careggi, Florence, Italy.
authors.forename: Iacopo
authors.initials: I
authors.lastname: Olivotto
authors.affiliation: Ospedale Sant'Andrea Universita "La Sapienza", Rome, Italy.
authors.forename: Gabriele E
authors.initials: GE
authors.lastname: Assenza
authors.affiliation: The Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, Minnesota.
authors.forename: Tammy S
authors.initials: TS
authors.lastname: Haas
authors.affiliation: The Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, Minneapolis, Minnesota.
authors.forename: John R
authors.initials: JR
authors.lastname: Lesser
authors.affiliation: Division of Cardiology, Toronto General Hospital, University Health Network, University of Toronto, Toronto, Ontario, Canada; Division of Cardiology, Cardiovascular Center, University Hospital, Raemistrasse, Aurich, Switzerland.
authors.forename: Christiane
authors.initials: C
authors.lastname: Gruner
authors.affiliation: Division of Cardiology, Toronto General Hospital, University Health Network, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Andrew M
authors.initials: AM
authors.lastname: Crean
authors.affiliation: Division of Cardiology, Toronto General Hospital, University Health Network, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Harry
authors.initials: H
authors.lastname: Rakowski
authors.affiliation: Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, Massachusetts.
authors.forename: Ethan
authors.initials: E
authors.lastname: Rowin
authors.affiliation: Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, Massachusetts.
authors.forename: James
authors.initials: J
authors.lastname: Udelson
authors.affiliation: Multimodality Cardiac Imaging Section, Policlinico San Donato, Milan, Italy.
authors.forename: Massimo
authors.initials: M
authors.lastname: Lombardi
authors.affiliation: Referral Center for Myocardial Diseases, Azienda Ospedaliera Universitaria Careggi, Florence, Italy.
authors.forename: Benedetta
authors.initials: B
authors.lastname: Tomberli
authors.affiliation: Ente Ospedaliero Ospedali Galliera, Genoa, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Spirito
authors.affiliation: Ente Ospedaliero Ospedali Galliera, Genoa, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Formisano
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Padua, Italy.
authors.forename: Martina P
authors.initials: MP
authors.lastname: Marra
authors.affiliation: Policlinico S. Orsola-Malpighi, Bologna, Italy.
authors.forename: Elena
authors.initials: E
authors.lastname: Biagini
authors.affiliation: Ospedale Sant'Andrea Universita "La Sapienza", Rome, Italy.
authors.forename: Camillo
authors.initials: C
authors.lastname: Autore
authors.affiliation: Cardiovascular Division, Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts.
authors.forename: Warren J
authors.initials: WJ
authors.lastname: Manning
authors.affiliation: Cardiovascular Division, Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts.
authors.forename: Evan
authors.initials: E
authors.lastname: Appelbaum
authors.affiliation: Baylor Heart and Vascular Institute, Baylor University Medical Center, Dallas, Texas.
authors.forename: William C
authors.initials: WC
authors.lastname: Roberts
authors.affiliation: Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Padua, Italy.
authors.forename: Cristina
authors.initials: C
authors.lastname: Basso
authors.affiliation: Hypertrophic Cardiomyopathy Center, Division of Cardiology, Tufts Medical Center, Boston, Massachusetts.
authors.forename: Martin S
authors.initials: MS
authors.lastname: Maron
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002312:Cardiomyopathy, Hypertrophic / Q000175:diagnosis*; D005260:Female; D005500:Follow-Up Studies; D005682:Gadolinium; D006352:Heart Ventricles / Q000473:pathology*; D006801:Humans; D007089:Image Enhancement / Q000379:methods*; D019028:Magnetic Resonance Imaging, Cine / Q000379:methods*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D015203:Reproducibility of Results; D012189:Retrospective Studies; D013997:Time Factors; D054088:Ventricular Septum / Q000473:pathology*; D055815:Young Adult
chemical_list: D005682:Gadolinium