Left ventricle dyssincrony is a common finding in patients with myotonic dystrophy

• Type: Summary of Presentation in an International Conference
• Date: 2012

Authors

Sousa C (Author) Other; Rangel I (Author) Other; Martins E (Author) Other; Correia AS (Author) Other; Nadais G (Author) Other; Silveira F (Author) Other; Goncalves A (Author) Other; Silva Cardoso JC (Author) Other; Macedo F (Author) Other; Maciel MJ (Author) FMUP

Conference proceedings International

Title: European Journal of Heart Failure Supplements

Initial page: P1298

Heart Failure Congress 2012

Belgrade, 2012

Scientific classification

FOS: Medical and Health sciences > Clinical medicine

Other information

Abstract (EN): Background: Myotonic dystrophy type 1 (MD1) is a systemic disease that may cause sudden death. Conduction disturbances, including left bundle branch block (LBBB), are frequently seen. However the prevalence of mechanical dyssincrony in unknown. The purpose of this study was to evaluate the presence of electrical and mechanical cardiac dyssincrony in DM1 patients. Methods and results: 20 patients with miotonic dystrophy and without cardiovascular symptoms were evaluated through ECG and echocardiography. Interventricular and intraventricular dyssincrony were assessed according to current guidelines criteria: difference between left and right ventricle pre-ejection intervals > 40 ms for interventricular dyssincrony and M-mode septal-to-posterior wall motion delay> 130 ms or QRS to S' wave onset delay between opposite LV walls > 40 ms by tissue Doppler imaging for intraventricular dyssincrony. Eighteen patients were analyzed (2 patients excluded for pacemaker rhythm), 50% were male, with mean age of 40.2±13.0 years. Mean left ventricle ejection fraction (LVEF) was 56.8±8.8% (2 patients had asymptomatic left ventricle systolic dysfunction -- LVEF of 44 and 31%). All patients had preserved right ventricular function. Sixteen patients (88.9%) were in sinus rhythm. Three patients had first degree atrioventricular block (PR 256±35.6 ms). Electrocardiographic criteria identified LV dyssincrony in 5 patients: 3 LBBB (QRS 171.7±27.7 ms) and 2 non specific intraventricular conduction delays (QRS 138±17.0 ms). Echocardiographic intraventricular dyssincrony criteria were present in 8 patients (44.4%). Among these, only 3 presented concomitant electrocardiographic signs of dyssincrony (1 LBBB and 2 non specific intraventricular conduction delay). Patients with mechanical dyssincrony had similar mean ejection fraction to those without dyssincrony (57.5±12.0 vs 58.0±3.2%), as well as tissue Doppler lateral S wave velocity (10.4±1.4 vs 10.0±2.8 cm/s). Additionally, interventricular echocardiographic dyssincrony criteria were met in 2 patients, both carriers of LBBB. No differences were found between patients with and without mechanical dyssincrony relative to demographic, clinical or echocardiographic diastolic function evaluation. Conclusions: Mechanical dyssincrony was a common finding among MD1 patients and not necessarily associated with electrical dyssincrony. This suggests that there might be a myocardial cause of asynchrony rather than electrical in this disease. Early identification of such abnormalities may guide towards the need for additional therapies which could improve survival in this particular population.

Language: English

Type (Professor's evaluation): Scientific

Notes: Sousa C, Rangel I, Martins E, Correia AS, Nadais G, Silveira F, Goncalves A, Silva Cardoso JC, Macedo F, Maciel MJ. Left ventricle dyssincrony is a common finding in patients with myotonic dystrophy. Eur J Heart Fail Suppl 2012; 11(Suppl 1): P1298. ISSN 1567-4215. Heart Failure Congress 2012, Belgrade, 2012