Distinct Subgroups in Hypertrophic Cardiomyopathy in the NHLBI HCM Registry

Neubauer, Stefan; Kolm, Paul; Ho, Carolyn Y.; Kwong, Raymond Y.; Desai, Milind Y.; Dolman, Sarahfaye; Appelbaum, Evan; Desvigne‐Nickens, Patrice; DiMarco, John P.; Friedrich, Matthias G.; Geller, Nancy L.; Harper, Andrew R.; Jarolím, Petr; Jerosch‐Herold, Michael; Kim, Dong-Yun; Maron, Martin S.; Schulz‐Menger, Jeanette; Piechnik, Stefan K.; Thomson, Kate; Zhang, Cheng; Watkins, Hugh; Weintraub, William S.; Kramer, Christopher M.; Mahmod, Masliza; Jacoby, Daniel; White, James A.; Chiribiri, Amedeo; Helms, Adam S.; Choudhury, Lubna; Michels, Michelle; Bradlow, William; Salerno, Michael; Heitner, Stephen B.; Prasad, Sanjay K.; Mohiddin, Saidi A; Swoboda, Peter; Mahrholdt, Heiko; Bucciarelli-Ducci, Chiara; Weinsaft, Jonathan W.; Kim, Han; Gulsin, Gaurav S.; Rossum, Albert C. van; Williamson, Eric E.; Flett, Andrew; Dawson, Dana; Mongeon, François‐Pierre; Olivotto, Iacopo; Crean, Andrew M.; Owens, Anjali Tiku; Anderson, Lisa; Biagini, Elena; Newby, David; Berry, Colin; Kim, Bette; Larose, Éric; Abraham, Theodore P.; Sherrid, Mark V.; Nagueh, Sherif F.; Rimoldi, Ornella; Elstein, Eleanor; Autore, Camillo

doi:10.1016/j.jacc.2019.08.1057

Cited by 163 publications

(110 citation statements)

References 32 publications

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“…Dimitrow et al [35] enrolled 18 HOCM patients with a DDD pacemaker and found that the LVOT gradient reductions both at acute DDD pacing and at midterm follow-up (at least 6 months) were significantly greater in the patients with nonreversed septal curvature than those with reversed septal curvature. e latest article [36] published in the Journal of the American College of Cardiology reported that patients with the reversed curvature form were younger and less commonly had hypertension. In our study, the distributions of age and hypertension in different subgroups were similar.…”

Section: Discussionmentioning

confidence: 99%

“…In our study, the distributions of age and hypertension in different subgroups were similar. It has been unclear whether the reversed septal curvature is a risk factor for outcome events [36], let alone for the all-cause mortality. Another reason for no data on the reversed septal curvature in our study was due to the absence of uncommonly related echocardiography data.…”

Section: Discussionmentioning

confidence: 99%

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Echocardiographic Predictors of All-Cause Mortality in Patients with Hypertrophic Cardiomyopathy following Pacemaker Implantation

Zhang

Hua

et al. 2020

Cardiology Research and Practice

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Objectives. To examine the association between the echocardiographic parameters measured as left atrial diameter (LAD) and left ventricular end-diastolic diameter (LVEDD) and long-term risk of all-cause mortality in adults with hypertrophic cardiomyopathy (HCM) following pacemaker implantation. Methods. A total of 94 adult patients with HCM who underwent pacemaker implantation from November 2002 to June 2013 in our Arrhythmia Center for symptomatic bradycardia and did not receive an implantable cardiac defibrillator (ICD) or cardiac resynchronization therapy (CRT) during follow-up were retrospectively extracted. Results. After careful examination of the medical records, we retrospectively evaluated the clinical characteristics of 74 patients with LAD records (58.1 ± 14.9 years) and 76 patients with LVEDD records (57.6 ± 15.2 years). Based on the receiver-operating characteristic (ROC) curve, the values of LAD = 44 mm and LVEDD = 43 mm were identified to predict the all-cause mortality. In the Kaplan–Meier survival, LAD ≥44 mm and LVEDD ≥43 mm were both significantly associated with all-cause mortality (log-rank test P<0.05). Cox regression analysis indicated that LAD ≥44 mm (HR 3.580; 95% CI = 1.055–12.148; P=0.041) was an independent predictor of all-cause mortality, while LVEDD ≥43 mm was not significantly associated with all-cause mortality. LVOTO was also significantly associated with all-cause mortality (HR = 0.166; 95% CI = 0.036–0.771; P=0.022). Conclusions. In HCM patients with pacemaker implantation, LAD ≥44 mm was an independent predictor of all-cause mortality.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Echocardiographic Predictors of All-Cause Mortality in Patients with Hypertrophic Cardiomyopathy following Pacemaker Implantation

Zhang

Hua

et al. 2020

Cardiology Research and Practice

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“…Its presence makes physiologic or athletic remodelling unlikely and is more suggestive of underlying pathology [ 43 , 44 ]. The presence and extent of LGE is also independently associated with an increased risk of SCD and adverse outcomes (including development of heart failure), particularly if > 15% of total LV mass [ 43 , 45 •, 46 – 48 ]. Although not yet incorporated into risk calculators, it is clear that LGE in HCM is prognostic and may even outperform and increase the discriminative power of current SCD risk scores [ 49 ].…”

Section: Hypertrophic Cardiomyopathymentioning

confidence: 99%

Cardiovascular Magnetic Resonance for the Differentiation of Left Ventricular Hypertrophy

Burrage

Ferreira

2020

Curr Heart Fail Rep

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Purpose of Review Left ventricular hypertrophy (LVH) is a common presentation encountered in clinical practice with a diverse range of potential aetiologies. Differentiation of pathological from physiological hypertrophy can be challenging but is crucial for further management and prognostication. Cardiovascular magnetic resonance (CMR) with advanced myocardial tissue characterisation is a powerful tool that may help to differentiate these aetiologies in the assessment of LVH. Recent Findings The use of CMR for detailed morphological assessment of LVH is well described. More recently, advanced CMR techniques (late gadolinium enhancement, parametric mapping, diffusion tensor imaging, and myocardial strain) have been used. These techniques are highly promising in helping to differentiate key aetiologies of LVH and provide valuable prognostic information. Summary Recent advancements in CMR tissue characterisation, such as parametric mapping, in combination with detailed morphological assessment and late gadolinium enhancement, provide a powerful resource that may help assess and differentiate important causes of LVH.

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“…In HCM, LGE (figure 5A) is present in approximately 50% of individuals,18–20 typically in mid-wall of the hypertrophied myocardium. Small, focal areas of LGE confined to the RV insertion point do not reflect replacement fibrosis or scarring and is associated with a low risk of adverse events (figure 5C).…”

Section: Introductionmentioning

confidence: 99%

“…Small, focal areas of LGE confined to the RV insertion point do not reflect replacement fibrosis or scarring and is associated with a low risk of adverse events (figure 5C). Recent findings from the Hypertrophic Cardiomyopathy Registry comprising 2755 prospectively recruited patients with HCM demonstrate that individuals with LGE had thicker walls, more baseline arrhythmias and were more likely to be sarcomere mutation positive 20…”

Section: Introductionmentioning

confidence: 99%

Advanced imaging for risk stratification of sudden death in hypertrophic cardiomyopathy

Ramchand

Fava

Chetrit

et al. 2020

Heart

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Hypertrophic cardiomyopathy (HCM) is a common inherited cardiac condition, which typically manifests as left ventricular hypertrophy. A small subset of patients with HCM have an increased risk of sudden cardiac death (SCD) from ventricular arrhythmias. Risk of SCD can be effectively reduced following implantation of implantable cardiac defibrillators (ICD), although this treatment carries a risk of complications such as inappropriate shocks. With this in mind, we turn to advances in cardiac imaging to guide risk stratification for SCD and to select the appropriate individual who may benefit from ICD implantation. In this review, we have taken the opportunity to briefly summarise the role of imaging in the diagnosis of HCM before focusing on how specific imaging features influence risk of SCD in patients with HCM.

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Distinct Subgroups in Hypertrophic Cardiomyopathy in the NHLBI HCM Registry

Cited by 163 publications

References 32 publications

Echocardiographic Predictors of All-Cause Mortality in Patients with Hypertrophic Cardiomyopathy following Pacemaker Implantation

Echocardiographic Predictors of All-Cause Mortality in Patients with Hypertrophic Cardiomyopathy following Pacemaker Implantation

Cardiovascular Magnetic Resonance for the Differentiation of Left Ventricular Hypertrophy

Advanced imaging for risk stratification of sudden death in hypertrophic cardiomyopathy

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