Late Gadolinium Enhancement in Patients With Hypertrophic Cardiomyopathy and Preserved Systolic Function

Mentias, Amgad; Raeisi‐Giglou, Pejman; Smedira, Nicholas G.; Feng, Ke; Sato, Kimi; Wazni, Oussama; Kanj, Mohamad; Flamm, Scott D.; Thamilarasan, Maran; Lever, Harry M.; Desai, Milind Y.

doi:10.1016/j.jacc.2018.05.060

Cited by 176 publications

(112 citation statements)

References 43 publications

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“…However, given the low incidence of events in this population and the importance of LGE in other larger cohorts it is likely that our study was not sufficiently powered to demonstrate an association. [38] [39,40]…”

Section: Correlation Between Cmr Characteristics and Clinical Outcomesmentioning

confidence: 99%

Defining genotype-phenotype relationships in patients with hypertrophic cardiomyopathy using cardiovascular magnetic resonance imaging

et al. 2019

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HCM is the most common inherited cardiomyopathy. Historically, there has been poor correlation between genotype and phenotype. However, CMR has the potential to more accurately assess disease phenotype. We characterized phenotype with CMR in a cohort of patients with confirmed HCM and high prevalence of genetic testing. Methods Patients with a diagnosis of HCM, who had undergone contrast-enhanced CMR were identified. Left ventricular mass index (LVMI) and volumes were measured from steady-state free precession sequences. Late gadolinium enhancement (LGE) was quantified using the full width, half maximum method. All patients were prospectively followed for the development of septal reduction therapy, arrhythmia or death. Results We included 273 patients, mean age 51.2 ± 15.5, 62.9% male. Of those patients 202 (74.0%) underwent genetic testing with 90 pathogenic, likely pathogenic, or rare variants and 13 variants of uncertain significance identified. Median follow-up was 1138 days. Mean LVMI was 82.7 ± 30.6 and 145 patients had late gadolinium enhancement (LGE). Patients with beta-myosin heavy chain (MYH7) mutations had higher LV ejection fraction (68.8 vs 59.1, p<0.001) than those with cardiac myosin binding protein C (MYBPC3) mutations. Patients with MYBPC3 mutations were more likely to have LVEF < 55% (29.7% vs 4.9%, p = 0.005) or receive a defibrillator than those with MYH7 mutations (54.1% vs 26.8%, p = 0.020).

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Section: Correlation Between Cmr Characteristics and Clinical Outcomesmentioning

confidence: 99%

Defining genotype-phenotype relationships in patients with hypertrophic cardiomyopathy using cardiovascular magnetic resonance imaging

et al. 2019

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“…LGE is a robust, validated technique and its presence carries important prognostic information [49][50][51][52][53]. However, a gap remained: the LGE technique could not detect global myocardial changes such as those occurring in diffuse fibrosis [54] or in some clinical patterns of myocarditis [55].…”

Section: Beyond Ejection Fraction and Its Limits: Advanced Imagingmentioning

confidence: 99%

Structural and myocardial dysfunction in heart failure beyond ejection fraction

et al. 2019

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Heart failure is a multifaceted syndrome addressing for a high rate of death among the general population. The common approach to this disease has been always based on the evaluation of the left ventricular ejection fraction by two-dimensional echocardiography with Simpson's method. Mounting evidences have demonstrated the pitfalls of this method and have suggested that the management of heart failure requires a deep knowledge of the pathophysiological insights of the disease and cannot rely only on the evaluation of the left ventricular ejection fraction. Several advanced imaging technologies overwhelm the evaluation of ejection fraction and could provide a better understanding of the myocardial abnormalities underlying heart failure. Considering the limitation of left ventricular ejection fraction and the systemic involvement of heart failure, classifications of heart failure based on ejection fraction should be substituted with a comprehensive "staging" of multiorgan damage, not only considering the heart but also the lungs, kidneys, and liver, such as the HLM staging system. Such a holistic approach based on the HLM staging system and multimodality imaging can provide a global assessment of patient features allowing for targeted therapies and better heart failure management.

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“…Pozdní sycení myokardu gadoliniem není typické pro atletické srdce a favorizuje diagnózu HKMP, nemusí být však vyjádřeno u jejích mírnějších forem. 4,26,27 Nedávno publikovaná studie z Velké Británie 28 také naznačuje, že signifi kantní regrese kalkulované hmotnosti myokardu může být pomocí MR detekovatelná již po měsíční tréninkové pauze, což by například pro vrcholové sportovce mohla být zajímavá alternativa tříměsíčního echokardiografi ckého dekondičního protokolu.…”

Section: Další Zobrazovací Metodyunclassified

(Athlete's heart vs hypertrophic cardiomyopathy - differential diagnostics according to current guidelines)

Bláha¹

2020

Cor Vasa

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Tento článek prosím citujte takto: Bláha M. Atletické srdce vs. hypertrofická kardiomyopatie -diferenciální diagnostika podle aktuálních doporučení. Cor Vasa 2020;62:50-55. SOUHRNHypertrofi cká kardiomyopatie (HKMP) je jednou z nejčastějších příčin náhlé smrti mladých sportovců do 35 let. Její fenotypický projev se do značné míry může podobat fyziologické reakci myokardu na vysokou tréninkovou zátěž, kdy hovoříme o takzvaném atletickém (sportovním) srdci. Tyto dva stavy je třeba od sebe rozlišit, abychom neohrozili život sportovce či sportovkyně s nerozpoznanou HKMP a také abychom na druhou stranu zbytečně nezastavili jejich sportovní kariéru. ABSTRACTHypertrophic cardiomyopathy (HCM) is one of the most common causes of sudden death of young athletes under 35 years of age. Its phenotypic manifestation may resemble the physiological response of the myocardium to a high training load in case of so-called athlete's heart. These two modalities must be distinguished from each other in order to preserve the life of athletes with yet unrecognized HCM, and also on the other side, to avoid unnecessary stopping the career of the healthy ones.

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Late Gadolinium Enhancement in Patients With Hypertrophic Cardiomyopathy and Preserved Systolic Function

Abstract: In low-/intermediate-risk adult patients with HCM (obstructive, myectomy, and nonobstructive subgroups) with preserved systolic function, %LGE was significantly associated with a higher rate of composite endpoint, providing incremental prognostic utility.

Cited by 176 publications

References 43 publications

Defining genotype-phenotype relationships in patients with hypertrophic cardiomyopathy using cardiovascular magnetic resonance imaging

Defining genotype-phenotype relationships in patients with hypertrophic cardiomyopathy using cardiovascular magnetic resonance imaging

Structural and myocardial dysfunction in heart failure beyond ejection fraction

(Athlete's heart vs hypertrophic cardiomyopathy - differential diagnostics according to current guidelines)

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