Left ventricular longitudinal strain impairment predicts cardiovascular events in asymptomatic type 1 myotonic dystrophy

Type 1 myotonic dystrophy (DM1) patients' prognosis is very poor. Up until now, only a few prognostic factors for cardiovascular events have been identified, and they are predictive of end-stage disease. The aim was to assess the prognostic value of global longitudinal strain (GLS) for cardiova...

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Published in:International journal of cardiology 2017-09, Vol.243, p.424-430
Main Authors: Garcia, Rodrigue, Rehman, Michaela, Goujeau, Cyril, Degand, Bruno, Le Gal, François, Stordeur, Benjamin, Labarre, Quentin, Christiaens, Luc, Bouleti, Claire
Format: Article
Language:English
Subjects:
Adult
Asymptomatic Diseases - mortality
Echocardiography
Echocardiography - trends
Female
Follow-Up Studies
Humans
Left ventricular function
Male
Middle Aged
Multi-layer speckle tracking
Myotonic Dystrophy - diagnostic imaging
Myotonic Dystrophy - mortality
Myotonic Dystrophy - physiopathology
Myotonic dystrophy type 1
Predictive Value of Tests
Strain
Survival Rate - trends
Ventricular Dysfunction, Left - diagnostic imaging
Ventricular Dysfunction, Left - mortality
Ventricular Dysfunction, Left - physiopathology
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Summary:Type 1 myotonic dystrophy (DM1) patients' prognosis is very poor. Up until now, only a few prognostic factors for cardiovascular events have been identified, and they are predictive of end-stage disease. The aim was to assess the prognostic value of global longitudinal strain (GLS) for cardiovascular events in asymptomatic DM1 patients. DM1 patients were included between 2011 and 2015 and followed up until January 2016. Patients underwent a transthoracic echocardiography at inclusion. The primary endpoint was a composite of all-cause mortality, type 2 Mobitz 2 and type 3 atrioventricular block, symptomatic sino-atrial block, HV interval≥70ms at invasive electrophysiology exploration, left ventricular ejection fraction (LVEF) ≤45% and newly developed atrial fibrillation. Forty-six patients (25 males, mean age 40years old) were included. The primary outcome was reached in 14 patients with a mean follow-up of 38months. GLS of patients who reached the primary endpoint was significantly impaired as compared to those who did not (−15.1 [−16.7; −12.7] vs. −18.2 [−19.2; −16.7] respectively; P=0.001). According to ROC curve analysis, probability of primary outcome occurrence was significantly greater in patients with GLS values≥−17.2% (P=0.001). On multivariate analysis, PR electrocardiogram interval and GLS remained significantly and independently associated with the primary endpoint [hazard ratio (HR) 1.03, 95% confidence interval (CI) 1.01–1.04, P=0.006 for PR interval; HR 1.4, 95% CI 1.1–1.7, P=0.002 for GLS] while LVEF alone was not. Left ventricular GLS is a powerful marker to predict cardiovascular events in asymptomatic DM1 patients, independently of LVEF.
ISSN:0167-5273
1874-1754
DOI:10.1016/j.ijcard.2017.05.061