Teodora Paunic1, Stojan Peric2, Edita Cvitan2, Srdjan Raspopovic3, Marina Peric4, Gorana Mandic Stojmenovic2, Vidosava Rakocevic Stojanovic5. 1. General Hospital Djordje Joanovic, Zrenjanin, Serbia. 2. Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia. 3. Cardiology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia. 4. Mother and Child Health Care Institute, Belgrade, Serbia. 5. Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia. Electronic address: vidosava_r@yahoo.co.uk.
Abstract
BACKGROUND: Myotonic dystrophy type 1 (DM1) is an autosomal-dominant disease. One third of DM1 patients die suddenly, most of them due to the heart conduction abnormalities and arrhythmias. The aim of this study was to analyze echocardiographic findings in a large cohort of DM1 patients. METHODS: This retrospective study comprised 111 patients and 71 healthy controls (HCs) matched for gender and age. RESULTS: Mitral valve (MV) prolapse was observed in 23% of our DM1 patients vs. 8.5% of HCs (p < 0.05). Left ventricle (LV) systolic dysfunction was observed in 6% of patients and none of the HCs (p < 0.05). Frequency of diastolic dysfunction showed no significant difference between DM1 patients and HCs (8.1% vs. 15.5%, p > 0.05). Systolic dysfunction was more common in patients with severe electrocardiographic (ECG) abnormality (18.8% vs. 2.7%, p < 0.01). CONCLUSION: One fourth of DM1 patients have MV prolapse. Approximately 15% of DM1 patients have systolic or diastolic LV dysfunction. These patients should have benefit from medical therapy. Furthermore, it seems that treatment of conduction defects might prevent development of the heart failure (HF).
BACKGROUND:Myotonic dystrophy type 1 (DM1) is an autosomal-dominant disease. One third of DM1patients die suddenly, most of them due to the heart conduction abnormalities and arrhythmias. The aim of this study was to analyze echocardiographic findings in a large cohort of DM1patients. METHODS: This retrospective study comprised 111 patients and 71 healthy controls (HCs) matched for gender and age. RESULTS:Mitral valve (MV) prolapse was observed in 23% of our DM1patients vs. 8.5% of HCs (p < 0.05). Left ventricle (LV) systolic dysfunction was observed in 6% of patients and none of the HCs (p < 0.05). Frequency of diastolic dysfunction showed no significant difference between DM1patients and HCs (8.1% vs. 15.5%, p > 0.05). Systolic dysfunction was more common in patients with severe electrocardiographic (ECG) abnormality (18.8% vs. 2.7%, p < 0.01). CONCLUSION: One fourth of DM1patients have MV prolapse. Approximately 15% of DM1patients have systolic or diastolic LV dysfunction. These patients should have benefit from medical therapy. Furthermore, it seems that treatment of conduction defects might prevent development of the heart failure (HF).