Premium
Apical left ventricular myocardial dysfunction is an early feature of cardiac involvement in myotonic dystrophy type 1
Author(s) -
Garcia Rodrigue,
Labarre Quentin,
Degand Bruno,
Ingrand Pierre,
Le Gal François,
Bonnet Benjamin,
Delaubier Anne,
Guillou Claire,
Gellen Barnabas,
Coisne Damien,
Bouleti Claire,
Christiaens Luc
Publication year - 2017
Publication title -
echocardiography
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.404
H-Index - 62
eISSN - 1540-8175
pISSN - 0742-2822
DOI - 10.1111/echo.13426
Subject(s) - medicine , ejection fraction , cardiology , myotonic dystrophy , qrs complex , prospective cohort study , heart failure
Left ventricular ( LV ) dysfunction is a major prognostic determinant in myotonic dystrophy type 1 ( DM 1). Therefore, markers of early‐stage LV impairment may be useful. The aim of this study was to evaluate 2D echocardiographic LV strain in a cohort of DM 1 patients with preserved left ventricular ejection fraction ( LVEF ) and to compare the results with matched controls. Methods This prospective single‐center study included 33 consecutive DM 1 patients between February 2014 and February 2015. Mean age was 38.2±12.9 years, and 17 (52%) were males. Exclusion criteria were LVEF <55%, QRS >120 milliseconds, history of atrial fibrillation, and presence of a pacemaker with ventricular pacing. DM 1 patients were matched to healthy controls according to sex and age. Results DM 1 patients showed significant impairment of global longitudinal strain ( GLS ) as compared to controls (−18.0±1.9 vs −19.1±2.4; P =.03), characterized by a marked alteration at the apex (−20.0±3.3 vs −22.7±3.1; P <.001). DM 1 patients had also global radial strain impairment (20.0±9.8 vs 27.5±14.9; P =.024) compared to controls while global circumferential strain was not statistically different between groups ( P =.94). Intra‐ and inter‐observer analysis showed good reproducibility of GLS . Conclusion Despite preserved LVEF , DM 1 patients exhibited significantly altered LV GLS , particularly at the apex, as compared with controls. The detection of impaired myocardial deformation at early stages of the disease might help to screen high‐risk patients who need closer follow‐up.