[Systolic time intervals and echocardiographic parameters in Hegglin syndrome (author's transl)].
The cardiac function in Hegglin syndrome (HS; prolonged QT interval and shortened QS2) remains unclear. In order to estimate cardiac function of HS, left ventricular echocardiographic parameters and systolic time intervals (STI) were analyzed, and compared with those of normal subjects (N) (n = 20). Forty-six patients (pts) of HS are constituted of 23 pts with chronic renal failure, 7 with cardiomyopathy, 5 with ischemic heart disease, 5 with essential hypertension, 4 with acquired valvular disease, 1 with effusive pericarditis and 1 with Romano-Ward syndrome. Corrected preejection period (PEPc) and PEP/ET were significantly larger (0.15 +/- 0.02 vs 0.13 +/- 0.01, p less than 0.001; 0.48 +/- 0.13 vs 0.35 +/- 0.04, p less than 0.001, respectively) in HS. Corrected ejection time (ETc) was significantly smaller (0.37 +/- 0.02 vs 0.41 +/- 0.01, p less than 0.001) in HS. Mitral EF slope (DDR), ejection fraction (EF), and mean ventricular circumferential fiber shortening (mVCF) were significantly decreased (58 +/- 29 vs 92 +/- 25, p less than 0.001; 0.52 +/- 0.15 vs 0.62 +/- 0.07; p less than 0.005; 0.98 +/- 0.33 vs 1.18 +/- 0.20; p less than 0.05, respectively) in HS, but cardiac index (C.I.) did not differ. Thus, patients with Hegglin syndrome showed heart failure pattern in STI and hypodynamic cardiac function in echocardiographic parameters, and our data suggest that hypodynamic cardiac function of HS is caused by both lowered pump function and decreased myocardial contractility.
D000328:Adult; D000368:Aged; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D006321:Heart / Q000503:physiopathology*; D006326:Heart Auscultation; D006331:Heart Diseases / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009200:Myocardial Contraction; D013577:Syndrome; D013599:Systole