Author + information
- Victoria Nicole Parikh,
- Myriam Amsallem,
- Francois Haddad,
- Euan Ashley and
- Matthew Wheeler
Background: Cardiomyopathies caused by Lamin A/C (LMNA) variants have been described as dilated and arrhythmogenic. However, hypokinetic non-dilated cardiomyopathy is increasingly recognized as a potentially inherited phenotype. In this study, our objective was to determine the prevalence of left ventricular (LV) enlargement in 18 patients with LMNA variants; our second objective was to evaluate LV strain in patients with borderline to normal LV ejection fraction (LVEF).
Methods and Results: Sixty percent of patients were male with median age 50 years (IQR 41-59). Prevalence of atrial fibrillation and ventricular tachycardia was 48% and 64% respectively. While median LVEF was 38% (IQR 29%-55%), median left ventricular diastolic diameter (LVIDd) indexed to body surface area was 2.7 cm/m2 (IQR 2.5-3cm/m2), with only 3 patients meeting criteria for abnormal indexed LVIDd (≥3.3 cm/m2, Figure). Further, indexed LVIDd did not correlate with LVEF or LV global longitudinal strain (LVGLS). Interestingly, while LVGLS correlated with LVEF overall (p<0.0001, R2 = 0.69), among patients with EF>50%, LVGLS was variable, and 28% of patients had abnormal LVGLS (p=0.5, R2 = 0.01, N= 7).
Conclusions: These findings underscore the variable phenotype of inherited cardiomyopathies and emphasize the importance of consideration of inherited etiology even in non-dilated presentations. Further, some potentially clinically relevant heterogeneity exists in LVGLS with normal LVEF, requiring cohort expansion and longitudinal data for further examination.
Poster Hall, Hall C
Saturday, March 18, 2017, 9:45 a.m.-10:30 a.m.
Session Title: Advances in HCM, PPCM and Other Cardiomyopathies
Abstract Category: 13. Heart Failure and Cardiomyopathies: Clinical
Presentation Number: 1201-286
- 2017 American College of Cardiology Foundation