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Predictors of life-threatening events in adult patients with left ventricular noncompaction
Olga V Blagova1, Nadezhda V Varionchik1, Ekaterina V Pavlenko1
1Division of Internal Medicine No. 1, Department of Cardiology, I.M. Sechenov First Moscow State Medical University (Sechenov University), Moscow 119048, Russia.
Insights
Frequent premature ventricular contractions (PVCs), non-sustained ventricular tachycardia (VT), and low QRS voltage predict life-threatening events in left ventricular noncompaction (LVNC) patients. Echocardiogram signs of systolic dysfunction also indicate increased risk.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Left ventricular noncompaction (LVNC) is a genetic cardiomyopathy characterized by excessive ventricular trabeculae and deep intertrabecular spaces.
- LVNC presents with arrhythmias, heart failure, and a heightened risk of thrombus formation.
Purpose of the Study:
- To identify predictors of arrhythmic events, thromboembolism, and adverse outcomes (death/transplantation) in adult LVNC patients.
- To establish prognostic markers for severe complications in LVNC.
Main Methods:
- A cohort of 125 adult LVNC patients underwent electrocardiography, echocardiography, and 24-hour Holter monitoring.
- Additional investigations included cardiac imaging (MRI, CT), genetic testing, and myocardial pathology.
- Primary endpoints: death, heart transplantation, combined death/transplantation, and sudden cardiac death. Secondary endpoints: thrombosis, embolism, MI, sustained VT, and ICD interventions.
Main Results:
- Non-sustained VT correlated with NYHA CHF class, poor R-wave progression, myocarditis, and mortality.
- Thrombosis/embolism associated with advanced NYHA class, enlarged right atrium/ventricle, and specific LV dimensions/velocities.
- Sustained VT linked to PVCs and low QRS voltage; >500 PVCs/day predicted ICD intervention. Fatal outcomes associated with impaired LV ejection fraction, NYHA class ≥3, VT, and myocarditis.
Conclusions:
- Frequent PVCs, non-sustained VT, low QRS voltage, and echocardiographic systolic dysfunction are key predictors of life-threatening events in LVNC.
- These findings aid in risk stratification and management of patients with LVNC.
Background:
Left ventricular noncompaction (LVNC) is a genetic cardiomyopathy. It is characterized by intensely developed trabeculae in the ventricles with deep intertrabecular lacunae. LVNC manifests as arrhythmias and heart failure with a predisposition for thrombus formation.
Aim:
To study predictors of arrhythmic, thromboembolic events and adverse outcomes (death/transplantation) in adult patients with LVNC.
Methods:
Adult patients with LVNC were included (n = 125; mean follow-up: 14 months). Electrocardiography, echocardiography, and 24-hour electrocardiography monitoring were performed. Other procedures were conducted for some patients including: Coronary angiography; cardiac magnetic resonance imaging; cardiac computed tomography; genetic testing; myocardial pathological examination; and anti-cardiac antibody level estimation. Primary endpoints were death, heart transplantation, combined endpoint (death + transplantation), and sudden cardiac death. Secondary endpoints were intracardiac thrombosis, embolic events, myocardial infarction, sustained ventricular tachycardia (VT), and implantable cardioverter-defibrillator intervention.
Results:
LVNC manifestations included non-sustained VT, thrombosis/embolism, sustained VT, and sudden cardiac death. Non-sustained VT was associated with the New York Heart Association (NYHA) chronic heart failure (CHF) class, poor R-wave progression, superimposed myocarditis, and higher mortality. Thrombosis/embolism was associated with NYHA CHF class ≥ 3, right ventricular end-diastolic diameter ≥ 3 cm, right atrium volume ≥ 67 mL, left ventricle end-diastolic diameter ≥ 6.3 cm, and velocity time integral ≤ 11.2 cm. Sustained VT was associated with premature ventricular contractions (PVCs), low QRS voltage, and atrioventricular block. PVCs > 500/day were predictive of defibrillator intervention. Fatal outcomes were associated with E wave/A wave ratio > 1.9, left ventricle ejection fraction < 35%, NYHA CHF class ≥ 3, VT, and myocarditis.
Conclusion:
Frequent PVCs, non-sustained VT, low QRS voltage, and signs of systolic dysfunction on echocardiogram are predictors of life-threatening events in patients with LVNC.
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