Left ventricular longitudinal strain impairment predicts cardiovascular events in asymptomatic type 1 myotonic
Rodrigue Garcia1, Michaela Rehman1, Cyril Goujeau1
1CHU Poitiers, Service de Cardiologie, 2 rue de la Milétrie, F-86021 Poitiers, France.
Insights
Global longitudinal strain (GLS) can predict cardiovascular events in patients with myotonic dystrophy type 1 (DM1). Impaired GLS is associated with a higher risk of adverse cardiovascular outcomes, offering a new prognostic tool for DM1 patients.
Area of Science:
- Cardiology
- Genetics
- Medical Diagnostics
Background:
- Type 1 myotonic dystrophy (DM1) has a poor prognosis, with limited predictive factors for cardiovascular events.
- Existing prognostic factors often indicate end-stage disease in DM1 patients.
Purpose of the Study:
- To evaluate the prognostic value of global longitudinal strain (GLS) for cardiovascular events in asymptomatic DM1 patients.
- To identify novel markers for early cardiovascular risk assessment in DM1.
Main Methods:
- Prospective cohort study including DM1 patients from 2011-2015, with follow-up until January 2016.
- Transthoracic echocardiography performed at inclusion to assess GLS.
- Primary endpoint: composite of mortality, advanced atrioventricular blocks, symptomatic sino-atrial block, prolonged HV interval, low left ventricular ejection fraction (LVEF), and new atrial fibrillation.
Main Results:
- 14 out of 46 DM1 patients reached the primary endpoint.
- Significantly impaired GLS was observed in patients who reached the endpoint compared to those who did not (P=0.001).
- GLS independently predicted cardiovascular events (HR 1.4, P=0.002), outperforming LVEF alone.
Conclusions:
- Left ventricular GLS is a potent predictor of cardiovascular events in asymptomatic DM1 patients.
- GLS provides prognostic information independent of LVEF.
- GLS may serve as a valuable tool for early cardiovascular risk stratification in DM1.
Background:
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.
Methods:
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.
Results:
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.
Conclusion:
Left ventricular GLS is a powerful marker to predict cardiovascular events in asymptomatic DM1 patients, independently of LVEF.
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