Mariusz Klopotowski1, Krzysztof Kukula2, Lukasz A Malek3, Mateusz Spiewak4, Magdalena Polanska-Skrzypczyk2, Jacek Jamiolkowski5, Maciej Dabrowski2, Rafal Baranowski6, Anna Klisiewicz7, Mariusz Kusmierczyk8, Anna Jasinska2, Ewelina Jarmus2, Mariusz Kruk9, Witold Ruzyllo9, Adam Witkowski2, Lidia Chojnowska2. 1. Department of Interventional Cardiology and Angiology, Institute of Cardiology, Warsaw, Poland. Electronic address: mklopotowski@ikard.pl. 2. Department of Interventional Cardiology and Angiology, Institute of Cardiology, Warsaw, Poland. 3. Department of Interventional Cardiology and Angiology, Institute of Cardiology, Warsaw, Poland; Magnetic Resonance Unit, Institute of Cardiology, Warsaw, Poland. 4. Magnetic Resonance Unit, Institute of Cardiology, Warsaw, Poland; Department of Coronary Disease and Structural Heart Diseases, Institute of Cardiology, Warsaw, Poland. 5. Public Health Department, Medical University of Bialystok, Bialystok, Poland. 6. Department of Cardiac Arrhythmias, Institute of Cardiology, Warsaw, Poland. 7. Department of Congenital Heart Diseases, Institute of Cardiology, Warsaw, Poland. 8. Department of Cardiac Surgery and Transplantation, Institute of Cardiology, Warsaw, Poland. 9. Department of Coronary Disease and Structural Heart Diseases, Institute of Cardiology, Warsaw, Poland.
Abstract
BACKGROUND: The presence of late gadolinium enhancement (LGE) in hypertrophic cardiomyopathy (HCM) is associated with worse clinical outcome and the extent of LGE predicts the increased risk of sudden cardiac death (SCD). Limited data exist regarding the distribution of LGE. We attempted to verify whether the presence of LGE outside the interventricular insertion points carries additional risk for patients with HCM. METHODS: In this prospective study, 328 patients with HCM, who underwent cardiac magnetic resonance (CMR) were enrolled. Five major risk factors for SCD were assessed in all patients. The median follow-up was 37 months. RESULTS: LGE was detected in 226 (68.9%) patients. In 70 (21.3%) patients it was present only at the interventricular insertion points - LGE (+) group, while in 156 (47.6%) it was noted in other locations - LGE (++) group. Primary endpoint defined as SCD or appropriate implantable cardioverter-defibrillator intervention occurred in 14 (4.3%) patients, one in LGE (+) and 13 in LGE (++). In multivariable analysis including five traditional risk factors and left ventricular ejection fraction <50%, only the presence of LGE outside the insertion points was a significant predictor of SCD/aborted SCD (HR 10.01, 95% CI 1.21-83.86, p=0.033). The performance of the multivariable sudden cardiac death risk model was improved by the addition of LGE (++) to the traditional risk factors (likelihood ratio p=0.005). The Kaplan-Meier curves showed better event-free survival in the LGE (-) and LGE (+) patient groups compared to the LGE (++) group. CONCLUSIONS: In HCM patients, presence of LGE outside interventricular insertion points is associated with increased risk of sudden cardiac death or its equivalent as well as overall mortality. Cardiac fibrosis as a substrate for SCD in HCM may be identified on CMR and serve as an imaging biomarker of increased risk.
BACKGROUND: The presence of late gadolinium enhancement (LGE) in hypertrophic cardiomyopathy (HCM) is associated with worse clinical outcome and the extent of LGE predicts the increased risk of sudden cardiac death (SCD). Limited data exist regarding the distribution of LGE. We attempted to verify whether the presence of LGE outside the interventricular insertion points carries additional risk for patients with HCM. METHODS: In this prospective study, 328 patients with HCM, who underwent cardiac magnetic resonance (CMR) were enrolled. Five major risk factors for SCD were assessed in all patients. The median follow-up was 37 months. RESULTS: LGE was detected in 226 (68.9%) patients. In 70 (21.3%) patients it was present only at the interventricular insertion points - LGE (+) group, while in 156 (47.6%) it was noted in other locations - LGE (++) group. Primary endpoint defined as SCD or appropriate implantable cardioverter-defibrillator intervention occurred in 14 (4.3%) patients, one in LGE (+) and 13 in LGE (++). In multivariable analysis including five traditional risk factors and left ventricular ejection fraction <50%, only the presence of LGE outside the insertion points was a significant predictor of SCD/aborted SCD (HR 10.01, 95% CI 1.21-83.86, p=0.033). The performance of the multivariable sudden cardiac death risk model was improved by the addition of LGE (++) to the traditional risk factors (likelihood ratio p=0.005). The Kaplan-Meier curves showed better event-free survival in the LGE (-) and LGE (+) patient groups compared to the LGE (++) group. CONCLUSIONS: In HCM patients, presence of LGE outside interventricular insertion points is associated with increased risk of sudden cardiac death or its equivalent as well as overall mortality. Cardiac fibrosis as a substrate for SCD in HCM may be identified on CMR and serve as an imaging biomarker of increased risk.
Authors: Mariusz Klopotowski; Aleksandra Kwapiszewska; Krzysztof Kukula; Jacek Jamiolkowski; Maciej Dabrowski; Pawel Derejko; Artur Oreziak; Rafal Baranowski; Mateusz Spiewak; Magdalena Marczak; Anna Klisiewicz; Barbara Szepietowska; Zbigniew Chmielak; Adam Witkowski Journal: Clin Cardiol Date: 2018-10-16 Impact factor: 2.882
Authors: Anna Axelsson Raja; Hiroko Wakimoto; Daniel M DeLaughter; Daniel Reichart; Joshua Gorham; David A Conner; Mingyue Lun; Clemens K Probst; Norihiko Sakai; Rachel S Knipe; Sydney B Montesi; Barry Shea; Leonard P Adam; Leslie A Leinwand; William Wan; Esther Sue Choi; Eric L Lindberg; Giannino Patone; Michela Noseda; Norbert Hübner; Christine E Seidman; Andrew M Tager; J G Seidman; Carolyn Y Ho Journal: Proc Natl Acad Sci U S A Date: 2022-07-05 Impact factor: 12.779
Authors: Marcelo Imbroinise Bittencourt; Samária Ali Cader; Denizar Vianna Araújo; Ana Luiza Ferreira Salles; Felipe Neves de Albuquerque; Pedro Pimenta de Mello Spineti; Denilson Campos de Albuquerque; Ricardo Mourilhe-Rocha Journal: Arq Bras Cardiol Date: 2019-03 Impact factor: 2.000