scholarly journals Echocardiographic Features of Cardiomyopathy in Emery-Dreifuss Muscular Dystrophy

2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Michał Marchel ◽  
Agnieszka Madej-Pilarczyk ◽  
Agata Tymińska ◽  
Roman Steckiewicz ◽  
Janusz Kochanowski ◽  
...  

Background. Emery-Dreifuss muscular dystrophy (EDMD) is a very rare type of muscular dystrophy characterized by musculoskeletal abnormalities accompanied by cardiac defects. Two most common genetic subtypes are EDMD1 due to EMD and EDMD2 caused by LMNA gene mutations. The aim of the study was to characterize and compare the cardiac morphology and function in the two main genetic subgroups of EDMD with the use of echocardiography. Methods. 41 patients with EDMD (29 EDMD1 and 12 EDMD2) as well as 25 healthy controls were enrolled in our study. Transthoracic echo with the use of a prescribed protocol was performed. Results. Highly statistically significant differences with regard to left ventricle (LV) volumes between the EDMD and the control group were found. 51% of EDMD patients had an enlarged left atrium and as many as 71% had an enlarged right atrium. The LV ejection fraction (LVEF) was significantly lower in EDMD patients than in the control group which corresponded also with a lower systolic velocity of the mitral annulus. 43% of EDMD patients had LVEF below the normal limit. Diastolic dysfunction was detected in 17% of EDMD patients. There were no significant differences between the two types of EDMD in terms of diameters and volumes of any chamber, as well as the systolic function of both left and right ventricles. Conclusions. A significant number of EDMD patients present LV dilatation and different degrees of systolic dysfunction. Dilatation of the atria dominates over ventricle dilatation. We did not present any significant differences between EDMD1 and EDMD2 in terms of the morphology and the function of the heart.

Author(s):  
L. M. Strilchuk

According to the literature data, gallbladder (GB) condition influences the course of coronary heart disease (CHD) and parameters of heart structure and function. The aim of this work was to estimate the peculiarities of heart condition in patients with CHD (acute myocardial infarction) in dependence of GB condition. We held a retrospective analysis of data of 142 patients. Results. It was revealed that in 83.7 % patients GB was changed: cholelithiasis (34.5 %), past cholecystectomy due to cholelithiasis (7.0 %), sludge and poliposis (17.6 %), bent GB body (13.4 %), neck deformations and signs of past cholecystitis (14.8 %). GB changes were accompanied by significant increase of heart rate, which was the most prominent in case of cholelithiasis, neck deformations and past cholecystitis signs. Conclusions. Pathological conditions of GB were accompanied by left ventricle dilatation, aortic distension, significant decrease of ejection fraction and systolic dysfunction, whereas after GB removal sizes of heart chambers were close to optimal values, although the systolic function did not normalize. Keywords: gallbladder, coronary heart disease, sludge, cholecystitis, heart structure.


2018 ◽  
Vol 2018 ◽  
pp. 1-3
Author(s):  
C. Henriquez ◽  
R. Landau ◽  
N. Sabharwal ◽  
D. Rodriguez ◽  
V. Virparia ◽  
...  

A 73-year-old female with multiple comorbidities including coronary artery disease was admitted for an elective PCI of a lesion detected in the RCA. On the day of the planned PCI, shortly after right femoral artery cannulation, the patient developed a sudden complete heart block requiring the administration atropine and insertion of a temporary pacemaker. Concomitantly, the patient developed acute pulmonary edema, hypotension, and hypoxia requiring intubation for mechanical ventilation. Vasopressors were administered. A coronary angiogram showed patent left and right coronary arteries, unchanged when compared to the previous angiogram. An echocardiogram performed in the cardiac catheterization lab revealed global hypokinesis of the left and right ventricles, with severe LV systolic dysfunction (EF<20%). Following an insertion of an intra-aortic balloon pump, the patient was transferred to the CICU. A repeat echocardiogram in the CICU two hours later revealed a classical echocardiographic presentation of Takotsubo syndrome, apical hypokinesis. By the next morning the patient’s hemodynamic status significantly improved, the balloon pump was removed, and vasopressors were discontinued. Another echocardiogram was performed 24 hours after the event occurred and revealed a marked improvement in LV systolic function (EF 60%), with complete resolution of apical and septal wall motion abnormalities. Three days after the event, the patient was successfully discharged and asymptomatic at two-month follow-up. This case illustrates an atypical presentation of Takotsubo syndrome that was witnessed from onset to its complete resolution during the patient’s hospital stay.


2021 ◽  
Vol 11 ◽  
Author(s):  
Yandong Liu ◽  
Jiawei Cai ◽  
Lefeng Qu

Background: Carotid atherosclerotic disease is associated with aortic stenosis and reduced cardiac function. The causality between carotid and cardiac pathologies is unknown. We aim to explore the effects of carotid stenosis or occlusion on cardiac pathology and function.Methods and Results: We produced carotid obstruction or stenosis in 36 atherogenic mice with 150- or 300-μm tandem surgery or sham surgery. The structure and function of the heart were assessed by histology and animal ultrasound. The 150-μm group had larger plaque burden and thicker valve leaflets in the aortic root than did the control group. Also, the two surgery groups had a thicker left ventricular posterior wall and smaller internal diameter compared with controls. Increased myocardial fibrosis was also found in the 150-μm group compared with controls, although the surgery groups had preserved systolic function compared with that of controls.Conclusions: In a mouse model, carotid occlusion accentuated the formation of aortic stenosis and promoted ventricular remodeling without impairing systolic function. Carotid atherosclerotic plaque may be a pathogenic factor for aortic stenosis and ventricular remodeling.


2019 ◽  
Vol 21 (1) ◽  
pp. 62
Author(s):  
Gabriela Corina Zaharie ◽  
Monica Hasmasanu ◽  
Ligia Blaga ◽  
Melinda Matyas ◽  
Daniel Muresan ◽  
...  

Aim: To asses the cardiac morphology and functional changes specific for newborns from intrauterine growth restriction (IUGR) pregnancies.Material and method: A cohort of IUGR infants were evaluated by serial echocardiographies at delivery and at the first and six months follow-ups. IUGR newborn delivery status was compared to that of newborns in the control group according to gestational age (AGA).Results: Left heart measurements were significantly lower in IUGR newborns compared to AGA babies. Left ventricular size increased at follow-up inthe IUGR group (p<0.05). Systolic dysfunction (the myocardial performance index (MPI)> 0.47) was identified in 40% of the neonates in the IUGR group (16/40), respectively 4.76% in the control group. IUGR neonates had a significantly increased proportion of systolic malfunction (p=0.004).Conclusion: IUGR patients had reduced left ventricle dimensions compared to AGA babies. The MPI stands out as a marker of leftheart function in newborns. Systolic dysfunction was a hallmark of the cardiac adaptation in IUGR neonates. 


2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Xing Xing ◽  
Dan Li ◽  
Shaomin Chen ◽  
Lingli Wang ◽  
Zhaoping Li ◽  
...  

Abstract Background The purpose of this study was to evaluate left ventricular systolic function in patients with different types of ischemic heart disease using two-dimensional speckle tracking imaging (2D-STI). Methods We retrospectively studied patients who were admitted to Peking University Third Hospital from January 2011 to December 2017 due to chest tightness and chest pain. Two hundred forty-two patients were divided into control group, CMD group and obstructive CAD group. The main coronary artery stenosis was confirmed by coronary angiography or coronary computed tomography and coronary flow reserve (CFR) in patients was measured by transthoracic Doppler echocardiography. Left ventricular strain and strain rate (SR) measured by 2D-STI. Cardiac structure and function were measured by conventional echocardiography. Results Conventional echocardiography showed that there was no significant difference in cardiac structure and function among the three groups (P > 0.05). Moreover, the longitudinal strain (LS) of each ventricular wall in CMD group was notably lower than that in control group (P < 0.01). In addition, global longitudinal SR and longitudinal SR in CMD group and obstructive CAD group were obviously lower than those in control group (P < 0.01). GLS, endocardial LS and epicardial LS were negatively correlated with CFR (P < 0.01). Conclusions Early left ventricular systolic dysfunction was found in patients with CMD and patients with obstructive CAD, with similar degree. CFR is an independent influencing factor of GLS. GLS and stratified LS have certain diagnostic value for CMD.


2009 ◽  
Vol 150 (45) ◽  
pp. 2060-2067 ◽  
Author(s):  
András Nagy ◽  
Zsuzsanna Cserép

Diabetes mellitus, a disease that has been reaching epidemic proportions, is an important risk factor to the development of cardiovascular complication. The left ventricular diastolic dysfunction represents the earliest pre-clinical manifestation of diabetic cardiomyopathy, preceding systolic dysfunction and being able to evolve to symptomatic heart failure. In early stages, these changes appear reversible with tight metabolic control, but as pathologic processes become organized, the changes are irreversible and contribute to an excess risk of heart failure among diabetic patients. Doppler echocardiography provides reliable data in the stages of diastolic function, as well as for systolic function. Combination of pulsed tissue Doppler study of mitral annulus with transmitral inflow may be clinically valuable for obtaining information about left ventricular filling pressure and unmasking Doppler inflow pseudonormal pattern, a hinge point for the progression toward advanced heart failure. Subsequently we give an overview about diabetes and its complications, their clinical relevance and the role of echocardiography in detection of diastolic heart failure in diabetes.


2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
Y K Tse ◽  
Y J Yu ◽  
H L Li ◽  
M Z Wu ◽  
Q W Ren ◽  
...  

Abstract Introduction Multiple valvular heart disease, a combination of stenotic and regurgitant lesions occurring on two or more valves, is a highly prevalent condition. For these patients, surgical correction is the only definitive treatment to improve prognosis, yet concomitant aortic and mitral (double) valve surgery is associated with poor post-operative outcomes. While current guidelines outline left ventricular dimensions and function as surgical triggers, little is known regarding the importance of right ventricular (RV) remodelling in these patients. Purpose We sought to evaluate the prognostic value of RV remodelling in patients undergoing double valve surgery. Methods RV remodelling was characterised by transthoracic echocardiography in 152 patients undergoing concomitant aortic and mitral valve replacement (n=118) or aortic valve replacement and mitral valve repair (n=34). Four patterns of RV remodelling were defined according to the presence of RV dilation (tricuspid annulus diameter&gt;35mm) and RV systolic dysfunction (percentage RV fractional area change &lt;35%): normal RV size and systolic function (pattern 1); dilated RV with normal systolic function (pattern 2); RV systolic dysfunction with normal RV size (pattern 3); and dilated RV with systolic dysfunction (pattern 4). Adverse events were defined as the composite of all-cause mortality and hospitalisation for heart failure. Results Overall, 62 (41%), 31 (20%), 35 (23%), and 24 (16%) patients were classified as RV remodelling patterns 1, 2, 3, and 4, respectively. Patients with advanced RV remodelling patterns were more frequently male, had worse renal function, and a higher EuroSCORE II. During a median follow-up of 43 months, 41 adverse events (22 heart failure hospitalisation and 19 deaths) occurred. Patients with patterns 3 and 4 RV remodelling had an increased risk of adverse events compared to pattern 1 (log-rank χ2 27.42; p&lt;0.001; Figure 1). After adjustments for EuroSCORE II and significant tricuspid regurgitation, RV remodelling patterns 3 (Hazard Ratio [HR] 3.24, 95% Confidence Interval [CI] 1.27–8.24, p=0.014) and 4 (HR 6.18, 95% CI 2.49–15.32, p&lt;0.001) were independently associated with poor post-operative outcomes. Importantly, RV remodelling patterns provided incremental prognostic value to EuroSCORE II (χ2 increased from 18 to 38, p&lt;0.001). Conclusion In patients with concomitant aortic and mitral valve disease, RV remodelling is frequent and associated with poorer outcomes. Our study highlights the involvement of the RV in left-sided valvular heart disease and underlines the importance of preoperative assessment of RV geometry and function in patients undergoing double valve surgery. FUNDunding Acknowledgement Type of funding sources: None. Figure 1


2020 ◽  
Vol 25 (2) ◽  
pp. 45-51
Author(s):  
A. V. Osokina ◽  
V. N. Karetnikova ◽  
O. V. Polikutina ◽  
A. V. Ivanova ◽  
O. V. Gruzdeva ◽  
...  

Aim. To study the changes of echocardiographic parameters and their relationship with the procollagen I C-terminal propeptide (PICP) during hospitalization of patients with ST-segment elevation myocardial infarction (STEMI) and preserved left ventricular (LV) systolic function.Material and methods. A total of 120 (100%) patients hospitalized with STEMI were examined. Upon admission, all patients underwent standard examinations to verify myocardial infarction (MI), including coronary angiography and, if necessary, coronary stent implantation. The mean values of LV ejection fraction (LVEF) were 40-49% in 3 patients (2,5%), <40% — in 31 patients (26%), LVEF was. We also analyzed patients with LVEF ≥50%, n=86 (71,6%); mean age was 57,8 years. During the hospitalization, all patients received standard therapy; on the 1st and 12th day of MI, the PICP levels in venous blood serum was determined by enzyme-linked immunosorbent assay. In order to compare PICP values, a control group of healthy volunteers n=20 (100%) was formed, which were comparable by gender and age. In this group, the concentration of PICP was 179,2 [163.5; 194.9] ng/ml.Results. By the 12th day, a significant decrease in the following parameters of the transmitral flow was revealed: DT (p=0,049), dE (0,012), Em (0,029), Em/ Am (p=0,000), Em/PICP (p=0,001). This indicates diastolic function deterioration. At the same time, by the end of hospitalization, systolic function deterioration was recorded in 15,1% of cases. Initially, a higher PICP on the 1st day relative to the control group tended to decrease the concentration by the 12th day, but the differences did not reach statistical significance (p=0,466). Correlation analysis showed a relationship between PICP and echocardiography (Tei index, p=0,026, and mitral annulus velocity, p=0,049).Conclusion. At the hospital stage of treatment of patients with STEMI and preserved LVEF, a negative changes of echocardiography parameters characterizing diastolic dysfunction was revealed. Positive correlation was established between the concentration of PICP with mitral annulus velocity and the Tei index, indicating an association between the myocardial fibrosis and diastolic dysfunction.


2007 ◽  
Vol 51 (2) ◽  
pp. 168-175 ◽  
Author(s):  
Cláudia Maria V. Freire ◽  
Ana Luiza M.T. Moura ◽  
Márcia de Melo Barbosa ◽  
Lucas José de C. Machado ◽  
Anelise Impeliziere Nogueira ◽  
...  

Diabetes mellitus, a disease that has been reaching epidemic proportions, is an important risk factor to the development of cardiovascular complication. Diabetes causes changes within the cardiac structure and function, even in the absence of atherosclerotic disease. The left ventricular diastolic dysfunction (VE) represents the earliest pre-clinical manifestation of diabetic cardiomyopathy, preceding the systolic dysfunction and being able to evolve to symptomatic heart failure. The doppler echocardiography has emerged as an important noninvasive diagnostic tool, providing reliable data in the stages of diastolic function, as well as for systolic function. With the advent of recent echocardiographic techniques, such as tissue Doppler and color M-mode, the accuracy in identifying the moderate diastolic dysfunction, the pseudonormal pattern, has significantly improved. Due to cardiometabolic repercussions of DM, a detailed evaluation of cardiovascular function in diabetic patients is important, and some alterations may be seen even in patients with gestational diabetes.


2006 ◽  
Vol 16 (1) ◽  
pp. 61-66 ◽  
Author(s):  
Canan Ayabakan ◽  
Figen Akalin ◽  
Sami Mengütay ◽  
Birol Çotuk ◽  
İlhan Odabaş ◽  
...  

To determine the cardiac response to intensive endurance training during prepuberty, we studied 22 male prebubertal swimmers who had been trained for at least 3 years, with a mean of 3.91 years and a standard deviation of 1.10 years, and 8 hours per week, the mean being 10.0 hours and the standard deviation 1.7 hours. The control group consisted of 21 boys of similar age, height and weight (p is more than 0.05 for all), who were not participating regularly in sporting activities. Left ventricular dimensions and systolic function were examined with M-Mode; velocities and durations of transmitral flow were measured with pulsed wave Doppler; and tissue Doppler velocities and durations were measured with pulsed wave tissue Doppler echocardiography. We determined the regional velocities of the lateral mitral annulus in four-chamber position, the left ventricular posterolateral wall, and the midseptum in long-axis position. Interventricular septal thickness, left ventricular posterior wall thickness, left ventricular mass and relative wall thickness were increased in swimmers (p is less than 0.05). All the tissue Doppler measurements were similar in both groups, except the septal isovolumic relaxation time. We observed that the left ventricular wall thickness had increased concentrically in prepubertal swimmers compared to controls, without a significant change in the left ventricular diastolic diameter. This finding is contrary to the previous studies on adult swimmers. Whether the structural changes observed in our study reflect the unique cardiac adaptation of the hearts of children to exercise will only be disclosed by longitudinal studies of prepubertal athletes.


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