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[Systolic time intervals and echocardiographic parameters in Hegglin syndrome (author's transl)]
Insights
Patients with Hegglin syndrome exhibit a hypodynamic cardiac function, characterized by impaired systolic time intervals and reduced echocardiographic parameters, suggesting heart failure. This study clarifies cardiac function in Hegglin syndrome.
Area of Science:
- Cardiology
- Cardiac Electrophysiology
- Medical Diagnostics
Context:
- Hegglin syndrome (HS) is characterized by a prolonged QT interval and shortened QS2, but its impact on cardiac function is not well understood.
- Understanding cardiac function in HS is crucial for patient management and risk stratification.
- Previous studies have not comprehensively evaluated the cardiac hemodynamics in Hegglin syndrome patients.
Purpose:
- To estimate the cardiac function in patients with Hegglin syndrome using left ventricular echocardiographic parameters and systolic time intervals (STI).
- To compare these parameters between Hegglin syndrome patients and a control group of normal subjects.
- To elucidate the underlying mechanisms of cardiac dysfunction in Hegglin syndrome.
Summary:
- Patients with Hegglin syndrome demonstrated significantly prolonged corrected pre-ejection period (PEPc) and PEP/ET, and a shortened corrected ejection time (ETc) compared to normal subjects.
- Echocardiographic parameters including Mitral EF slope (DDR), ejection fraction (EF), and mean ventricular circumferential fiber shortening (mVCF) were significantly decreased in HS patients.
- Despite these findings, cardiac index (C.I.) did not differ between groups, indicating a specific pattern of hypodynamic cardiac function.
Impact:
- The study reveals a heart failure pattern in systolic time intervals and hypodynamic cardiac function in echocardiographic parameters in Hegglin syndrome.
- These findings suggest that the hypodynamic cardiac function in HS results from a combination of reduced pump function and diminished myocardial contractility.
- This research provides critical insights into the pathophysiology of Hegglin syndrome, aiding in the development of targeted therapeutic strategies.
Abstract:
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.