Significance of Late Gadolinium Enhancement at Right Ventricular Attachment to Ventricular Septum in Patients With Hypertrophic Cardiomyopathy

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 se...

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Bibliographic Details
Published in:The American journal of cardiology 2015-08, Vol.116 (3), p.436-441
Main Authors: Chan, Raymond H., MD, MPH, Maron, Barry J., MD, Olivotto, Iacopo, MD, Assenza, Gabriele E., MD, Haas, Tammy S., RN, Lesser, John R., MD, Gruner, Christiane, MD, Crean, Andrew M., MD, Rakowski, Harry, MD, Rowin, Ethan, MD, Udelson, James, MD, Lombardi, Massimo, MD, Tomberli, Benedetta, MD, Spirito, Paolo, MD, Formisano, Francesco, MD, Marra, Martina P., MD, Biagini, Elena, MD, Autore, Camillo, MD, Manning, Warren J., MD, Appelbaum, Evan, MD, Roberts, William C., MD, Basso, Cristina, MD, PhD, Maron, Martin S., MD
Format: Article
Language:English
Subjects:
Adolescent
Adult
Age
Aged
Cardiac arrhythmia
Cardiology
Cardiomyopathy
Cardiomyopathy, Hypertrophic - diagnosis
Cardiovascular
Cardiovascular disease
Coronary vessels
Female
Follow-Up Studies
Gadolinium
Heart attacks
Heart failure
Heart Ventricles - pathology
Humans
Image Enhancement - methods
Magnetic Resonance Imaging, Cine - methods
Male
Middle Aged
Prognosis
Reproducibility of Results
Retrospective Studies
Time Factors
Ventricular Septum - pathology
Young Adult
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Summary: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.
ISSN:0002-9149
1879-1913
DOI:10.1016/j.amjcard.2015.04.060