4091Mechanical dispersion by speckle tracking echocardiography in fabry disease

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
T F Cianciulli ◽  
M C Saccheri ◽  
A M Risolo ◽  
J A Lax ◽  
R J Mendez ◽  
...  
Keyword(s):  
Fabry Disease ◽  
Speckle Tracking ◽  
Heart Diseases ◽  
Systolic Dysfunction ◽  
Cross Sectional Study ◽  
Left Ventricular ◽  
Peak Strain ◽  
Cross Sectional ◽  
Mechanical Dispersion ◽  
Group I

Abstract Background Fabry disease is a rare X-linked storage disorder caused by a deficiency of the lysosomal enzyme α-galactosidase A and generally causes multi-organ dysfunction. Heart disease is the main cause of death, due to severe left ventricular (LV) systolic dysfunction and sudden death. In several heart diseases, the LV systolic dysfunction and ventricular arrhythmias are associated with mechanical dispersion (MD). The presence of MD in patients with FD has not been studied yet. In this cross-sectional study, we investigated the prevalence of MD in patients with FD. Methods Complete echocardiographic and speckle tracking echocardiographic (STE) data were collected. MD is an index of inter-segmental discoordination of contraction which has been used to quantify LV dyssynchrony and was defined as the standard deviation (SD) of time to peak negative strain in 17 left ventricular segments. Patients were divided into two groups according to whether or not they had left ventricular hypertrophy (LVH). MD was defined as an SD >49 msec. Results We studied 108 patients with FD, 24 patients (22%) were excluded due to inadequate imaging quality or presence of comorbidities, so the final study population consisted of 84 patients (mean age 33.3±14.6 years, 60.7% women). LVH in FD appears at older ages than in patients without LVH (48±12.5 y/o vs 27.8±11.1 y/o, p<0.0001). Patients with FD without LVH (Group I) showed normal global longitudinal peak strain (GLPS) (21.2±2.5%) and no MD (32.7±8.8 msec). In Group II (n=23) patients with FD with LVH, 17 (73.9%) had MD >49 msec prolonged mechanical dispersion (73.3±20.7 msec) and reduced GLPS (13.6±4.0%). MD was more pronounced in Fabry patients with LVH than in patients without LVH (63.4±24.7 msec vs. 32.7±8.8 msec, p<0.0001). GLPS was lower in Fabry patients with LVH than in patients without LVH (15.3±4.7% vs 21.2±2.5%, p<0.0001). Figure 1 Conclusions To our knowledge, this is the first study to demonstrate the prevalence of mechanical dispersion in patients with FD. Mechanical dispersion was seen in 73.9% of patients with FD with LVH. This dyssynchrony should be taken into account in patients who develop heart failure or life-threatening ventricular tachyarrhythmias.

scholarly journals GH Receptor Exon 3 Genotype and Echocardiographic Abnormalities in Patients With Active Acromegaly

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A643-A644
Author(s):  
Karla Serrano ◽  
Etual Espinosa ◽  
Daniel Marrero-Rodríguez ◽  
Eduardo Almeida ◽  
Gloria Silva-Roman ◽  
...  
Keyword(s):  
Systolic Dysfunction ◽  
Cross Sectional Study ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Genotype 3 ◽  
Cross Sectional ◽  
Ventricular Ejection Fraction ◽  
Gh Receptor ◽  
Active Acromegaly

Abstract Background: The GH receptor (GHR) exon 3 polymorphism occurs at a genomic level. Approximately 50-60% of the population is homozygous for the exon-3 containing genotype (+3/+3), 30-40% are heterozygous (+3/-3) and 10-20% are homozygous for the exon-3 lacking genotype (-3/-3). Some studies suggest that children homo- and heterozygous for the GHR exon 3 lacking genotype (-more efficient 3/-3 and +3/-3, respectively) respond better to treatment with exogenous rhGH and there is also in vitro evidence showing a more efficient signal transduction through this exon 3 deleted isoform. Some studies have found that patients with acromegaly harboring the exon 3-deleted genotype may have a higher prevalence of diabetes and hypertension. Hypothesis and Objective: Patients with active acromegaly harboring the exon 3-lacking GHR genotype may have more echocardiographic abnormalities than those who are homozygous for the exon 3 containing genotype. Patients and Methods: This is a cross-sectional study of patients with active acromegaly, defined by an IGF-1 level &gt; 1.3 times the upper limit of normal (x ULN), who underwent transthoracic echocardiography. Exon-3 GHR genotype was determined by PCR using previously described sense and antisense primers. Results: The cohort consisted of 28 patients, 54% female, with a mean age of 51 ± 12 years. Mean disease duration at the time of echocardiographic examination was 4.48 ± 4.7 years; median basal GH and IGF-1 were 12 ± 26 ng/mL and 2.4 ± 1.04 x ULN. The prevalence of hypertension and diabetes were 43% and 36%, respectively. Fifty three percent of the patients were homozygous for the exon 3-containaing genotype (+3/+3), 18% were homozygous for the exon 3-lacking genotype (-3/-3) and 29% were heterozygous (+3/-3). Clinical and biochemical features did not differ between patients with the different GHR genotypes, except for hypertension that was more prevalent in the +3/+3 genotype group (60% vs 23%, p= 0.04). The frequency of the different echocardiographic parameters was similar among groups (left ventricular hypertrophy 33% vs 15%, p= 0.27; diastolic dysfunction 47% vs 31%, p= 0.39; subclinical systolic dysfunction 42% vs 54%, p= 0.54; left ventricular ejection fraction 59±10% vs 60±16%, p= 0.83); aortic valve abnormalities 19% vs 15%, p=0.63; mitral valve abnormalities 46% vs 15%, p=0.07). Conclusions: Echocardiographic abnormalities in patients with active acromegaly do not differ among patients with the different GHR exon 3 genotypes. The clinical spectrum of acromegaly varies considerably. Although such variability is usually related to the severity of the hypersomatotropinemia, in many patients this is not the case.

scholarly journals A tertiary care centre profile of heart diseases among diagnosed cases of chronic kidney disease

2020 ◽  
Vol 8 (2) ◽  
pp. 652
Author(s):  
Vijay Bakhtar ◽  
Niyati Bakhtar ◽  
Neha Pandey ◽  
Nikhil Bakhtar
Keyword(s):  
Chronic Kidney Disease ◽  
Cardiovascular Diseases ◽  
Kidney Disease ◽  
Heart Diseases ◽  
Tertiary Care ◽  
Cross Sectional Study ◽  
Left Ventricular ◽  
Tertiary Care Centre ◽  
Cross Sectional ◽  
Cardiac Evaluation

Background: Chronic Kidney Disease (CKD) is a risk factor for development of cardiovascular diseases. Cardiovascular diseases are the predominant cause of morbidity and mortality in patients with CKD. There is limited data on cardiovascular diseases among CKD patients from developing countries including India. With the present study, the prevalence and patterns of cardiac diseases among patients with CKD were profiled.Methods: This was a cross sectional study in which 217 patients with CKD were studied over a period of two years and six months. Data on demographic characteristics and risk factors for cardiovascular diseases were collected using a standardized questionnaire. Cardiac evaluation was done using resting ECG and echocardiography.Results: One hundred eighteen (54.4%) patients had either eccentric or concentric LVH. Patients with LVH were more likely to be hypertensive (p<0.001) or anemic (p=0.034). Up to 9.2% of study subjects had valvular heart disease (rheumatic or degenerative) and 22% had pericarditis. Patients with pericarditis were more likely to have a serum urea concentration greater than 60mg/dl (p=0.327). Forty-one patients (18.9%) had left ventricular systolic failure (EF<50%). There was a statistically insignificant higher prevalence of systolic failure in patients with LVH (21% vs. 16%), (p=0.346). Thirty-eight participants (17.5%) had diastolic failure while 2% had cardiac rhythm abnormalities.Conclusions: Cardiac abnormalities are common in a relatively young Indian population with CKD. Clinicians should routinely screen and manage cardiovascular disease in CKD patients.

scholarly journals Myonecrosis in Aortic Valvular Heart Diseases

2009 ◽  
Vol 13 (Number 2) ◽  
pp. 9-12
Author(s):  
Md. Khalequzzaman ◽  
M A Islam ◽  
Md. O Hoque ◽  
M Ferdous ◽  
A H K Chowdhury ◽  
...  
Keyword(s):  
Aortic Stenosis ◽  
Heart Diseases ◽  
Cross Sectional Study ◽  
Left Ventricular ◽  
Definitive Treatment ◽  
Control Group ◽  
Cross Sectional ◽  
Valvular Heart Diseases ◽  
Significant Difference ◽  
Venous Sample

This cross sectional study was done among 20 patients with aortic stenosis and 20 healthy controls to evaluate the association of cardiac specific troponin 1 (ant) and sonic valvular heart diseases. The study was conducted in °militant, department in National laminae of Cardiovascular Diseases (N1CVD.)A structured queslionilaire and checklist was used to collect data through face to face interview. Color dapple, echocarchiognsphy was done and 5 ml of venous sample was dmwo from each subjects and laboratory estimation of an, was done. The arid in control group and sonic stenosis patients showed significant difference in mean (<0.001). ant level in aortic stenosis patients increases in the absence of heart failure indicating that it can expose the cardiotnyocnes to injury prior to development of oven left ventricular dysftinction. So. serial monitoring of aid may help clinicians to give definitive treatment (reface development af complications.

P2485Cardiac abnormalities and vascular damage in young adults with type 1 diabetes mellitus: incidence and associations with kidney dysfunction

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
D Medvedev ◽  
K Mahamat ◽  
N Soseliya ◽  
V Efimova ◽  
A Safarova ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Arterial Stiffness ◽  
Cardiac Remodeling ◽  
Systolic Dysfunction ◽  
Global Longitudinal Strain ◽  
Cross Sectional Study ◽  
Left Ventricular ◽  
Applanation Tonometry ◽  
Cross Sectional

Abstract Background In contrast to type 2 diabetes mellitus (DM), cardiac and vascular abnormalities in type 1 DM (T1DM) are not well investigated. We aimed to evaluate occurrence of cardiac remodeling, arterial stiffness and blood pressure (BP) phenotypes in T1DM patients. Methods The cross-sectional study consecutively included T1DM patients 18–44 y.o. without known CVD, in whom 24-hour monitoring of peripheral and central BP (ABPM) with BPLab Vasotens, applanation tonometry and conventional and speckle tracking echo were performed. BP phenotypes were determined according to current guidelines, PWV and CBP - according to individual reference values. Presence of systolic dysfunction was defined as global longitudinal strain (GLS) <20%, left ventricular hypertrophy (LVH) as LV myocardial mass index (LVMI) >95/>115 g/m2 for women/men, LV remodeling (LVR) as RWT ≥0.43. P<0.05 was considered significant. Results A total of 125 patients with T1DM (mean age 29,2±7,6 years, 60% male, median duration of DM 6,9 [2; 11] years, HbA1c 9.9 [6; 12] %, mean BMI 23±3 kg/m2, smoking 39%, median GFR 100 [86; 117] ml/min/1.73 m2, GFR <60 ml/min/1.73 m2 – in 8.8%, median albuminuria 19 [8; 24] mg/g (moderate and high albuminuria in 14.6% and 2.2%) were investigated. According to office BP and ABPM hypertension (HTN) was diagnosed in 28% patients (true and masked in 4.8 and 23.2%, respectively) and true normotension in 72%. Isolated nocturnal HTN was observed in 14.4%. Majority of the patients were dippers (51.2%), non-dippers and night-peakers profiles were registered in and 43.2% and 5.6%, respectively. Central SBP and PWV elevation were observed in 17.6% and 57.6% (PWV >10 m/s - only in 2.4%). Cardiac abnormalities were revealed in 72.4% of patients: GLS<20%, LVH, LVR and diastolic dysfunction (DD) in 71.2, 12, 39.2 and 16.8% patients, respectively. Isolated GLS <20% was detected in 30%, combination of GLS<20% with LVH (or LVR) and DD in 47.2%. Patients with vs without HTN were characterized by higher PWV (7.8±1.5 vs 6.9±1.2, p<0.001), LVMI (89.9 [75; 96] vs 71.5 [64; 77] p<0.001), incidence of DD (29.6 vs 12.2%, p=0.03), LVH (28 vs 6%, p=0.002), trend towards higher rate of central SBP increase (32.7% vs 17.4%, p=0.08), lower incidence of LVR (26 vs 44%, p=0.002) and similar GLS (p=0.16). Groups with vs without nocturnal HTN did not differ by PWV, central SBP, GLS and LVMI. PWV increase was associated only with higher LVMI (88.2 [69; 95] vs 77.6 [68; 83], p=0.042). Correlations (p<0.05) with albuminuria were observed for GLS (r=−0.26), DD (r=0.22) and non-dipping state (r=−0.34). GFR correlated (p<0.05) with GLS (r=−0.32) and PWV (r=−0.32). Conclusion Incidence of prognostically unfavourable phenotypes of HTN, cardiac remodeling and arterial stiffness (even in patients without HTN) were relatively high in T1DM population. GLS and non-dipping state correlated with albuminuria, GLS and PWV with GFR

Heart Failure and Asymptomatic Left Ventricular Systolic Dysfunction in Lymphoma Survivors Treated With Autologous Stem-Cell Transplantation: A National Cross-Sectional Study

2015 ◽  
Vol 33 (24) ◽  
pp. 2683-2691 ◽  
Author(s):  
Klaus Murbraech ◽  
Knut B. Smeland ◽  
Harald Holte ◽  
Jon Håvard Loge ◽  
May Brit Lund ◽  
...  
Keyword(s):  
Risk Factors ◽  
Left Ventricular Systolic Dysfunction ◽  
Systolic Dysfunction ◽  
Cross Sectional Study ◽  
Left Ventricular ◽  
Sectional Study ◽  
Ventricular Systolic Dysfunction ◽  
Cross Sectional ◽  
The Mean ◽  
Lymphoma Survivors

Purpose We aimed to determine the prevalence of left ventricular systolic dysfunction (LVSD), including symptomatic (ie, heart failure [HF]) and asymptomatic LVSD in adult lymphoma survivors (LSs) after autologous hematopoietic stem-cell transplantation (auto-HCT) and to identify risk factors for LVSD in this population. Patients and Methods All LSs treated with auto-HCT as adults in Norway from 1987 to 2008 were eligible for this national cross-sectional study. Asymptomatic LVSD was defined as left ventricular ejection fraction less than 50% by echocardiography, and HF was defined according to current recommendations. The results in LSs were compared with those found in an age- and sex-matched (1:1) control group. Results We examined 274 LSs (69% of all eligible survivors); 62% were men, the mean (± standard deviation) age was 56 ± 12 years, and mean follow-up time from lymphoma diagnosis was 13 ± 6 years. The mean cumulative doxorubicin dose was 316 ± 111 mg/m2, and 35% of LSs had received additional radiation therapy involving the heart. We found LVSD in 15.7% of the LSs, of whom 5.1% were asymptomatic. HF patients were symptomatically mildly affected, with 8.8% of all LSs classified as New York Heart Association class II, whereas more severe HF was rare (1.8%). Compared with controls, LSs had a substantially increased LVSD risk (odds ratio, 6.6; 95% CI, 2.5 to 17.6; P < .001). A doxorubicin dose ≥ 300 mg/m2 and cardiac radiation therapy dose greater than 30 Gy were independent risk factors for LVSD. Conclusion LVSD was frequent and HF more prevalent than previously reported in LSs after auto-HCT. Our results may help to identify LSs at increased LVSD risk and can serve as a basis for targeted surveillance strategies.

scholarly journals The Echocardiographic Findings in Patients with Atrial Fibrillation in a Tertiary Care Center of Nepal: A Descriptive Cross-sectional Study

2021 ◽  
Vol 59 (233) ◽  
Author(s):  
Smriti Shakya ◽  
Ratna Mani Gajurel ◽  
Chandra Mani Poudel ◽  
Hemant Shrestha ◽  
Surya Devkota ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Left Atrium ◽  
Left Ventricular Systolic Dysfunction ◽  
Care Center ◽  
Systolic Dysfunction ◽  
Tertiary Care ◽  
Cross Sectional Study ◽  
Left Ventricular ◽  
Institute Of Medicine ◽  
Cross Sectional

Introduction: Atrial fibrillation is the most prevalent supraventricular arrhythmia responsible forthe large morbidity and mortality burden worldwide. There are various causes of atrial fibrillationthat may affect the prognosis of patients. This study was intended to determine different echocardiographic findings in patients with atrial fibrillation in a tertiary care center. Methods: A descriptive cross-sectional study was conducted at Manmohan Cardiothoracic Vascularand Transplant Center, Institute of Medicine, among 175 patients with atrial fibrillation admittedin the cardiology department from June 2017 to October 2018. It was approved by the InstitutionalReview Board of the Institute of Medicine (Ref.:411(6-11-E)2/073/074). Convenience sampling wasused. Statistical analysis was done using Statistical Package for Social Sciences version 21.0. Results: A total of 175 patients with atrial fibrillation were enrolled where Rheumatic heart disease 68 (38.9%) was the leading cause in which 54 (79.4%) had mitral valve lesion, 1 (1.5%) hadaortic valve lesion and rest had a combination of both. The mixed lesion of mitral stenosis and mitral regurgitation was the commonest. The left atrium size was larger in valvular atrial fibrillation(47.29±6.651mm). The left ventricular systolic dysfunction was seen more in non-valvular atrialfibrillation. The commonest site of thrombus formation was left atrium 7 (63.6%). Conclusions: Atrial fibrillation was common in rheumatic heart disease, especially mixed lesions ofmitral stenosis and regurgitation. Valvular atrial fibrillation had a larger left atrium. The thrombuswas seen in mitral stenosis and left ventricular systolic dysfunction. The left atrium size and left ventricular ejection fraction were associated with the occurrence of atrial fibrillation.

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