Lipoedema: detailed left atrial volumetric and strain analysis by three-dimensional speckle-tracking echocardiography from the MAGYAR-Path Study.

Introduction: Lipoedema is a chronic feminine disease with disproportional painful fat deposition of the lower body half and occassional arm affection. Aim: The objective of the present study was to assess LA volumetric and functional properties by three-dimensional speckle-tracking echocardiography (3DSTE) in patients with lipoedema and to compare their results to age- and gender-matched healthy controls. It was also aimed to examine whether one-hour use of medical compression stockings (MCS) has any effect on LA volumes and functional properties. Material and methods: The study comprised 25 female patients with stage 2 lipoedema (mean age: 42.5 ±12.2 years). Their results were compared to those of the control group consisting of 54 age- and gender-matched healthy female subjects (mean age: 42.7 ±10.6 years) without classic risk factors. Results: Increased LA volumes could be measured in lipoedema patients as compared to controls regardless of the phase of the cardiac cycle it was measured in, and the LA volumes showed further increase after 1-hour use of MCS. Similarly, LA total and active stroke volumes and LA passive emptying fraction were increased in lipoedema patients, and these parameters were further increased in systole and early-diastole. Peak global radial and 3D strains were increased in lipoedema patients. Peak global longitudinal strain increased in lipoedema patients after one-hour use of MCS compared to data measured at rest. Conclusions: Lipoedema is associated with increased LA volumes and changes in LA functional properties. The use of MCS further increases some of the LA volumetric parameters.

The effects of lower body compression on left ventricular rotational mechanics in lymphoedema (from the MAGYAR-Path Study).

AIMS: Lower body half compression of bilateral secondary leg lymphoedema (LE) without relevant cardiac insufficiency gives rise to whether external leg compression may influence left ventricular (LV) function. Patients with LE were subjected to baseline two-dimensional transthoracic echocardiography (2DTTE) for general assessment then three-dimensional speckle-tracking echocardiography (3DSTE) before and 1 h after lower body half external compression for LV torsion analysis. METHODS AND RESULTS: Baseline 2DTTE was performed in the cohort of 25 LE patients, and the results were compared with those of age- and gender-matched 52 healthy controls (mean age: 47.8 ± 12.8 vs. 40.7 ± 14.0 years, 24 women/1 man vs. 49 women/3 men, respectively). 3DSTE was conducted for the assessment of LV rotational mechanics where apical (AR), and basal rotations (BR) were measured before and 1 h after the use of compression class 2 (ccl 2) flat-knitted medical compression pantyhoses (pressure range: 23-32 mmHg). 2DTTE showed significantly larger LV end-diastolic volume and ejection fraction among LE patients compared with control subjects (108.3 ± 20.1 vs. 98.5 ± 21.7 mL, 69.8 ± 4.8 vs. 65.5 ± 4.3%, respectively) and notably smaller LV end-systolic diameter and posterior wall thickness (28.9 ± 3.5 vs. 31.2 ± 3.4 mm, 8.1 ± 1.0 vs. 9.0 ± 1.7 mm, respectively). The results of 20 patients with LE were considered in 3DSTE examinations due to the drop-out of five probands with technical failures. The data of four LE patients showing significant LV rotational abnormalities were managed separately, and the rotational parameters of the remaining sixteen patients did not differ significantly from those of matched controls except significant reduction of LV BR following the application of medical compression stockings (MCS) (-2.70 ± 1.26 degrees after 1 h use of pantyhose in patient group vs. -4.28 ± 2.18 degrees of the control group; P < 0.05). CONCLUSIONS: The application of compression pantyhoses moderately but significantly decreased LV BR without a remarkable impact on twisting mechanism in LE patients in the absence of LV rotational abnormalities.

Systemic Screening for 22q11.2 Copy Number Variations in Hungarian Pediatric and Adult Patients With Congenital Heart Diseases Identified Rare Pathogenic Patterns in the Region.

Congenital heart defects (CHD) are the most common developmental abnormalities, affecting approximately 0.9% of livebirths. Genetic factors, including copy number variations (CNVs), play an important role in their development. The most common CNVs are found on chromosome 22q11.2. The genomic instability of this region, caused by the eight low copy repeats (LCR A-H), may result in several recurrent and/or rare microdeletions and duplications, including the most common, ∼3 Mb large LCR A-D deletion (classical 22q.11.2 deletion syndrome). We aimed to screen 22q11.2 CNVs in a large Hungarian pediatric and adult CHD cohort, regardless of the type of their CHDs. All the enrolled participants were cardiologically diagnosed with non-syndromic CHDs. A combination of multiplex ligation-dependent probe amplification (MLPA), chromosomal microarray analysis and droplet digital PCR methods were used to comprehensively assess the detected 22q11.2 CNVs in 212 CHD-patients. Additionally, capillary sequencing was performed to detect variants in the TBX1 gene, a cardinal gene located in 22q11.2. Pathogenic CNVs were detected in 5.2% (11/212), VUS in 0.9% and benign CNVs in 1.8% of the overall CHD cohort. In patients with tetralogy of Fallot the rate of pathogenic CNVs was 17% (5/30). Fifty-four percent of all CNVs were typical proximal deletions (LCR A-D). However, nested (LCR A-B) and central deletions (LCR C-D), proximal (LCR A-D) and distal duplications (LCR D-E, LCR D-H, LCR E-H, LCR F-H) and rare combinations of deletions and duplications were also identified. Segregation analysis detected familial occurrence in 18% (2/11) of the pathogenic variants. Based on in-depth clinical information, a detailed phenotype-genotype comparison was performed. No pathogenic variant was identified in the TBX1 gene. Our findings confirmed the previously described large phenotypic diversity in the 22q11.2 CNVs. MLPA proved to be a highly efficient genetic screening method for our CHD-cohort. Our results highlight the necessity for large-scale genetic screening of CHD-patients and the importance of early genetic diagnosis in their clinical management.

Left ventricular rotational abnormalities in adult patients with corrected tetralogy of Fallot following different surgical procedures (Results from the CSONGRAD Registry and MAGYAR-Path Study).

BACKGROUND: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease (CHD). Abnormal aortic dimensions and elasticity parameters have been long described for corrected TOF (cTOF) together with left ventricular (LV) rotational abnormalities, but results are conflicting. The present study focuses on investigating LV rotational mechanics in cTOF, and possible correlation of these parameters with aortic elasticity. It was also aimed to be examined whether different surgical strategies have any effect on these results. METHODS: The study involved 26 adult cTOF patients, from which 14 had palliative surgery first and a late total correction (pcTOF), while early total correction was the treatment of choice in 12 patients (etrTOF). Their results were compared to those of 37 age- and gender-matched healthy adults. Routine transthoracic two-dimensional Doppler echocardiography extended with assessment of aortic elastic properties and three-dimensional speckle-tracking echocardiography (3DSTE) was performed in all cTOF patients and controls. RESULTS: Sixteen out of 26 cTOF patients showed normally directed LV rotational mechanics, while apical or basal LV rotations were in the same clockwise or counterclockwise directions in 7 and 3 cTOF cases, respectively (LV 'rigid body rotation', RBR). Significantly reduced LV apical rotation and twist could be demonstrated in all cTOF patients with preserved LV basal rotation regardless of previous procedure. pcTOF patients showed significantly reduced LV apical rotation as compared to that of etrTOF cases. Significant correlations could be demonstrated between LV apical rotation and aortic stiffness index (r=-0.55, P=0.03) and aortic distensibility (r=0.52, P=0.04). CONCLUSIONS: Significant LV rotational abnormalities could be demonstrated in cTOF with the high prevalence of LV-RBR. pcTOF patients showed significantly reduced LV apical rotation as compared to that of etrTOF cases. Unexpected abnormal physiologic response of LV rotational mechanics to increased aortic stiffness can be detected in cTOF patients without LV-RBR.

Left ventricular strains correlate with aortic elastic properties in adult patients with corrected tetralogy of Fallot (Results from the CSONGRAD Registry and MAGYAR-Path Study).

BACKGROUND: Aortopathy is a common phenomenon in tetralogy of Fallot (TOF). The current study was designed to detect left ventricular (LV) deformation abnormalities and its relation to aortic stiffness in corrected TOF (cTOF) using the novel three-dimensional (3D) speckle-tracking echocardiography (3DSTE). Detailed comparative analysis between patients with early palliation-late correction (pcTOF) and early total reconstruction (etrTOF) was also performed. METHODS: The present study consisted of 28 cTOF patients (35.0±15.7 years, 11 males) from which 15 and 13 proved to be pcTOF and etrTOF, respectively. Their clinical parameters were compared to those of 39 matched healthy adults (35.5±6.0 years, 16 males). RESULTS: cTOF patients showed significantly lower global LV longitudinal, circumferential and area strains as compared to controls. In etrTOF patients, global LV 3D strain was higher than in controls. In pcTOF patients, all LV strains proved to be significantly lower as compared to those of etrTOF patients and controls. In all cTOF patients, several moderate correlations could be detected between LV strain parameters and aortic elastic properties. CONCLUSIONS: Significant LV deformational abnormalities could be demonstrated in cTOF patients. etrTOF patients have beneficial LV strain parameters as compared to those of pcTOF patients. LV strains show correlations with aortic elastic properties.

Normal reference values of left ventricular volumetric parameters in healthy adults-real-life single-center experience from the three-dimensional speckle-tracking echocardiographic MAGYAR-Healthy Study.

BACKGROUND: The left ventricle (LV) is the pump of the peripheral circulation, therefore its non-invasive accurate volumetric and functional assessment is essential. Three-dimensional (3D) speckle-tracking echocardiography (STE) is a new tool with capability of quantification of LV volumes and ejection fraction (EF). In the present study, age- and gender-dependency of 3DSTE-derived LV volumetric parameters were aimed to be quantified in healthy adults. METHODS: The present study involved 298 healthy adults. Data acquisition took place over a 6 year period (2011-2017), during which 123 adults had to be excluded due to inferior image quality. The study population was further divided into 4 groups based on age decades. In every case, a complete two-dimensional echocardiography was performed followed by 3DSTE examination. RESULTS: No significant differences were detected between the different age groups regarding 3D end-diastolic volume (EDV) and 3D end-systolic volume (ESV) and their body surface area (BSA)-indexed counterpart. 3DEF differed significantly between the 30-39-year-old males and 40-49-year-old males (P=0.04). Between the youngest and oldest patient group, only 3D left ventricular mass (LVM) was significantly different (P=0.02). The 18-29-year-old females had a significantly different 3DLVM (P<0.001) compared to that of the 50+ year-old females. Between the 40-49-year-old and 50+ year-old females 3DLVM (P=0.02) and BSA-indexed 3DLVM (P=0.05) proved to be significantly different. CONCLUSIONS: 3DSTE seems to be a reasonably viable tool for the quantification of LV volumetric parameters.

Correlations between left ventricular rotational mechanics and parasympathetic autonomic function-results from the three-dimensional speckle-tracking echocardiographic MAGYAR-Healthy Study.

Left ventricular (LV) twist is calculated from the net difference of counterclockwise apical and clockwise basal rotation during systole. The current study was designed to evaluate correlations between autonomic function and LV rotational mechanics in healthy subjects. The present study comprised 18 healthy subjects (mean age: 36±12 years, 12 men). Three-dimensional speckle tracking echocardiography (3DSTE) could be used for non-invasive evaluation of LV rotation and twist. Autonomic function was assessed by means of 5 standard cardiovascular reflex tests. During 3DSTE, basal LV rotation proved to be -3.24±2.02 degree, while apical LV rotation was 9.08±3.04 degree, therefore LV twist was 11.70±6.80 degree. Valsalva test showed significant correlations with LV basal (r=0.529, P=0.019) and apical rotations (r=-0.534, P=0.022), and LV twist (r=-0.467, P=0.044). Heart rate response to deep breathing significantly correlated with LV twist, as well (r=-0.452, P=0.052). The other tests had no any relationship with rotational characteristics. Correlations exist between parasympathetic autonomic function and 3DSTE-derived LV rotation and twist in healthy subjects.

Correlations between left atrial volumes and strains in healthy adults: Detailed analysis from the three-dimensional speckle-tracking echocardiographic MAGYAR-Healthy Study.

INTRODUCTION: Little is known about the relationship between left atrium (LA) volumes and contractility features along the cardiac cycle. The present study aimed to assess, by three-dimensional speckle-tracking echocardiography (3DSTE), correlations between LA volumes, volume-based functional properties, and strains during the cardiac cycle in healthy adults. METHODS: We included 217 healthy adult volunteers (mean age 33.4 ± 12.7 years, 112 males) who underwent complete two-dimensional Doppler echocardiography with 3DSTE. RESULTS: LA stroke volumes were greater in subjects with the greater maximum LA volume (Vmax ) in reservoir, conduit, and booster pump phases of LA function. While LA emptying fraction in LA reservoir phase was not different between subjects depending in their Vmax value, a significantly lower LA emptying fraction could be detected in LA conduit phase in subjects whose Vmax was >50 mL. In booster pump function, LA emptying fraction was not significantly different whatever the Vmax . Only global and mean segmental peak LA radial strain (RS) and 3D strain (3DS) and the same strains at atrial contraction appeared greater in subjects with greater Vmax , whereas the other strain parameters were not different. CONCLUSIONS: In healthy subjects, LA-RS and LA-3DS, objective features of LA contractility, are greater in subjects with greater LA volumes up to a point beyond which this association disappears.

Mitral annulus is dilated with preserved function in acromegaly regardless of its activity: Insights from the three-dimensional speckle-tracking echocardiographic MAGYAR-Path Study.

INTRODUCTION AND OBJECTIVE: Acromegaly is a rare, chronic and slowly developing endocrine disorder caused by hypersecretion of human growth hormone and consequently of insulin-like growth factor-1 during adulthood. The present study was conducted to assess mitral annular (MA) size and function between acromegalic patients and age- and gender-matched healthy controls by three-dimensional speckle-tracking echocardiography (3D-STE). It also aimed to examine whether activity of the disease has any effect on MA parameters. METHODS: This study included 27 patients with acromegaly, three of whom were excluded due to inferior image quality. The mean age of the remaining 24 patients was 55.7±14.0 years and seven were male. Complete two-dimensional Doppler echocardiography and 3D-STE were performed in all cases. RESULTS: Significantly increased end-diastolic and end-systolic MA diameter (2.81±0.36 cm vs. 2.44±0.34 cm and 2.00±0.32 cm vs. 1.65±0.37 cm, respectively), area (9.67±2.33 cm2 vs. 7.38±1.93 cm2 and 5.14±1.62 cm2 vs. 3.74±1.19 cm2, respectively) and perimeter (11.76±1.42 cm vs. 10.27±1.33 cm and 8.61±1.23 cm vs. 7.36±1.10 cm, respectively) were demonstrated in acromegalic patients compared with control subjects. MA functional parameters were not significantly altered compared to those of healthy individuals. CONCLUSIONS: MA dilation could be seen in acromegaly regardless of its activity. Acromegaly is not associated with MA functional impairment.

Diabetes mellitus deteriorates left ventricular deformation in acromegaly-analysis from the three-dimensional speckle-tracking echocardiographic MAGYAR-Path study.

Acromegaly and diabetes mellitus (DM) separately are associated with cardiovascular alterations and modified left ventricular (LV) deformation, which can be quantitatively assessed by three-dimensional speckle-tracking echocardiography (3DSTE). The present study aimed to assess the relationship between LV deformation and twist in the presence of DM in acromegaly. Thirty-seven acromegaly patients were involved in the study, but due to insufficient image quality 13 have to be excluded from analysis. Therefore, the present study comprised 24 acromegaly patients, from which 5 had DM. Their data was compared to 35 age- and gender matched healthy controls. Complete two-dimensional Doppler echocardiography extended with 3DSTE were performed in all patients and controls. Only non-diabetic acromegaly cases had increased global and mean segmental LV radial strain, diabetic acromegaly patients had similar LV radial strain as compared to that of healthy subjects. Other LV strain parameters did not show any difference between diabetic and non-diabetic acromegaly patients. While LV basal rotation was similar among the groups, LV apical rotation was reduced in both diabetic and non-diabetic patients as compared to healthy subjects. However, diabetic acromegaly patients had tendentiously higher values. The ratio of absence of LV twist called as LV rigid body rotation was similar between the groups examined. Diabetes mellitus deteriorates left ventricular deformation in acromegaly.

Left ventricular rotational mechanics in elite athletes doing high dynamic sports. Insights from the 3D speckle-tracking echocardiographic MAGYAR-Sport Study.

BACKGROUND: In normal circumstances while left ventricular (LV) apex rotates counterclockwise, LV base has a clockwise movement at the same time. This sort of towel-wringing-like movement is called LV twist. The present study was designed to test whether differences in LV rotational mechanics could be detected in elite athletes doing high dynamic sports with different static components in their training. METHODS: The subject group comprised 80 elite sportsmen. The following groups were created regarding their physical activity: group CI (high dynamic/low static)(N.=13, mean age: 24.0±5.1 years, group CII (high dynamic/moderate static)(N.=23, mean age: 24.6±7.7 years) and group CIII (high dynamic/high static)(N.=34, mean age: 22.8±6.0 years). Their results were compared to 67 age- and gender-matched non-athletic healthy controls (mean age: 24.0±5.1 years). Three-dimensional speckle-tracking echocardiography was used for the evaluation of LV rotational abnormalities. RESULTS: Reduced LV basal rotation was seen in group CII and CIII subjects compared to the control group (-4.31±1.82 degrees vs. -3.17±2.81 degrees and -2.88±1.88 degrees, P<0.05 and P<0.05, respectively). It was accompanied with LV twist reduction in groups CII and CIII subjects compared to the control group (14.0±3.4 degree vs. 11.3±4.3 degrees and 11.5±4.1 degrees, P<0.05 and P<0.05, respectively). None of the elite athletes showed absence of LV twist called as LV "rigid body rotation." CONCLUSIONS: Significant LV rotational abnormalities including reduced LV basal rotation and twist could be detected in elite athletes doing high dynamic sports with moderate/high static components in their training.

Normal reference values of left atrial volumes and volume-based functional properties using three-dimensional speckle-tracking echocardiography in healthy adults (Insights from the MAGYAR-Healthy Study).

INTRODUCTION: The present study was designed to define normal reference values of three-dimensional speckle-tracking echocardiography (3DSTE)-derived left atrial (LA) volumes, stroke volumes (SVs), and emptying fractions (EFs) with regard to the cardiac cycle. METHODS: The present study involved 256 healthy adult subjects in sinus rhythm who underwent complete two-dimensional Doppler echocardiography and 3DSTE at the same time. However, due to inferior image quality, 87 subjects have been excluded. The remaining population sample comprised of 169 patients who gave informed consent to participate in the study. RESULTS: While systolic maximum LA volume and early diastolic preatrial contraction LA volume did not change over age decades, late-diastolic minimum LA volume decreased and was lowest in subjects aged 40 to 49 years then increased after 50 years. Total atrial EF increased over age decades with a reduction after 50 years. Passive atrial EF showed a significant continuous increase over age decades. Active atrial EF did not change in younger ages and was the highest between ages 40 and 49 years with a significant impairment after 50 years. CONCLUSIONS: This study shows the age- and gender-dependency of normal values of 3DSTE-derived LA volumes, stroke volumes, and emptying fractions with regard to the cardiac cycle in healthy adult subjects.

Normal reference values of three-dimensional speckle-tracking echocardiography-derived mitral annular dimensions and functional properties in healthy adults: Insights from the MAGYAR-Healthy Study.

INTRODUCTION: There is a limited number of echocardiographic studies determining mitral annular (MA) dimensions in healthy subjects. The present study aimed to establish normal reference values of three-dimensional speckle-tracking echocardiography-derived MA dimensions and functional properties in healthy adults in relation with age and gender. METHODS: The present study comprised 298 healthy adult subjects. From this population, 94 subjects were excluded due to inadequate image quality. Therefore the remaining group consisted of 204 subjects with the mean age of 33.88 ± 12.97 years (107 males). The population sample was further divided into age categories: 18-29 years (n = 105; mean age: 24.11 ± 2.98 years, 51 males), 30-39 years (n = 44; mean age: 33.80 ± 2.39 years, 31 males), 40-49 years (n = 19; mean age: 43.47 ± 3.18 years, 11 males) and ≥50 years of age (n = 36, mean age: 57.42 ± 6.11 years, 14 males). RESULTS: End-diastolic MA dimensions did not change significantly during the decades. End-systolic MA diameter, area, and perimeter were larger over the age of 50 years than in the 18-29 year-old group. MA fractional area change was found smaller over the age of 50 years than in 18-29-year-old group. While end-diastolic MA variables did not show gender-differences, end-systolic MA area and perimeter were lower in females in the 18-29-year-old group. CONCLUSIONS: End-systolic MA dimensions change over decades, resulting in a special pattern of MA functional properties with significant reduction over the age of 50 years.

Normal reference values of left ventricular strain parameters in healthy adults: Real-life experience from the single-center three-dimensional speckle-tracking echocardiographic MAGYAR-Healthy Study.

INTRODUCTION: A number of studies defined normal reference values of three-dimensional (3D) speckle-tracking echocardiography (3DSTE)-derived left ventricular (LV) strains. The present study aimed to quantify normal reference values of LV strains in healthy adult population in real clinical world settings in different age groups, and to determine age- and gender-dependence of these variables in a high volume single center. METHODS: The present prospective study included 296 healthy adult subjects. Among them, 124 were excluded due to inferior image quality during the 6-year recruitment period (2011-2017). The remaining population was further divided into four subgroups based on age decades. RESULTS: While global radial (RS) strain showed an increase-decrease-increase pattern with age, circumferential, longitudinal, and area strains (CS, LS, AS, respectively) were nonsignificantly lower in older ages. Only global LV-LS showed gender-dependency with higher values in females. Although moderately higher RS and 3DS and lower LS, and AS were observed in males, clear gender-dependency could not be detected in different age decades. CONCLUSIONS: This study provides normal reference values of 3DSTE-derived global, segmental, mean segmental, and regional LV strains in healthy adult subjects based on real-life clinical experience. Age-, gender-, and functional nonuniformity of LV strains were also defined.

The impact of lower body compression garment on left ventricular rotational mechanics in patients with lipedema-Insights from the three-dimensional speckle tracking echocardiographic MAGYAR-Path Study.

Lipedema is a lymphedema-masquerading symmetrical, bilateral and disproportional obesity. Its conservative maintenance treatment comprises the use of flat-knitted compression pantyhoses. Lipedema is known to be associated with left ventricular morphological and functional alterations. The present study aimed to assess the effects of graduated compression stockings on left ventricular (LV) rotational mechanics measured by three-dimensional speckle-tracking echocardiography (3DSTE) in lipedema patients. The present study comprised twenty lipedema patients (mean age: 45.8 ± 11.0 years, all females) undergoing 3DSTE who were also compared to 51 age- and gender-matched healthy controls (mean age: 39.8 ± 14.1 years, all females). 3DSTE analysis was performed at rest, and subsequent to 1 hour application of compression class 2 made-to-measure flat-knitted pantyhose. Six lipedema patients showed significant LV rotational abnormalities. Of the remaining fourteen lipedema patients LV basal rotation rotation showed significant reduction, while LV apical rotation showed significant increase with unchanged LV twist after a 60-minute use of compression garment. Significant changes in LV rotational mechanics could be detected among 14 women with lipedema after the use of compression garment however six probands have special LV rotational abnormalities at baseline and/or after compression.

Active acromegaly is associated with enhanced left ventricular contractility: Results from the three-dimensional speckle-tracking echocardiographic MAGYAR-Path Study.

INTRODUCTION: Acromegaly is a relatively rare chronic hormonal disease resulting in disfigurement. In 90% of cases, acromegaly is caused by a benign pituitary monoclonal human growth hormone-secreting tumor. The aim of the present study was to determine the presence of left ventricular (LV) deformation abnormalities using three-dimensional speckle-tracking echocardiography in a group of acromegalic patients. METHODS: Thirty-eight acromegalic patients were involved in the study. Thirteen patients were excluded due to inadequate image quality. The mean age of the remaining patients was 57.2±13.6 years and seven were male. Their data were compared to an age- and gender-matched control population, which consisted of 34 healthy volunteers (mean age: 52.7±4.9 years, 15 male). RESULTS: Global and mean segmental LV radial strain (RS) (33.2±13.4% vs. 25.2±10.8%, p=0.01 and 36.0±12.1% vs. 28.2±10.0%, p=0.009, respectively) proved to be significantly higher in acromegaly compared to controls. Active acromegalic patients had significantly higher global and mean segmental LV-RS (35.5±14.4% vs. 25.2±10.8%, p=0.03 and 37.9±13.3% vs. 28.2±10.0%, p=0.03, respectively) compared to controls. Between the active and inactive acromegaly groups, only basal LV circumferential strain (-30.2±4.8% vs. -26.7±4.1%, p=0.02) was found to be significantly different. CONCLUSION: The presented clinical, demographic, therapeutic and echocardiographic features demonstrate that active acromegaly is associated with enhanced LV RS as compared to healthy controls and those with inactive acromegaly.

Are increased left ventricular strains compensatory effects in lipedema? Detailed analysis from the three-dimensional speckle-tracking echocardiographic MAGYAR-Path Study.

PURPOSE: Lipedema is a chronic, common but underdiagnosed disease masquerading obesity, with female predominance, characterized by disproportional abnormal adipose tissue distribution of the lower and also upper extremities. The present study was designed to determine whether lipedema is associated with three-dimensional (3D) speckle-tracking echocardiography (3DSTE)-derived left ventricular (LV) deformation abnormalities, and to assess the effects of 1-hour use of medical compression stockings (MCS). METHODS: The present study comprised 19 female patients with lipedema (mean age: 42.2 ± 12.4 years), compared to 28 age-matched healthy female controls (mean age: 42.0 ± 9.8 years). RESULTS: Lipedema patients showed larger left atrial and LV dimensions and greater LV ejection fraction than controls, without significant difference in other echocardiography variables. Lipedema patients had greater 3DSTE-derived global and mean segmental LV circumferential and area strains than controls. Following 1-hour use of wearing MCS, neither global and nor mean segmental LV strains showed significant impairment or improvement. CONCLUSIONS: Increased LV strains could be compensatory effects maintaining LV pumping function in lipedema. Short-term wearing of MCS has no global effect on LV strains.

The right atrium in acromegaly-a three-dimensional speckle-tracking echocardiographic analysis from the MAGYAR-Path Study.

BACKGROUND: Acromegaly is a chronic, rare hormonal disease associated with major cardiovascular co-morbidities. The disease, in the majority of the cases, is caused by a benign human growth hormone (hGH) secreting adenoma. Cardiovascular involvement is especially common in acromegalic patients from the most common hypertension to cardiomyopathy. Left ventricular hypertrophy and myocardial fibrosis are considered common findings in acromegalic cardiomyopathy, which might result in severe heart failure at end-stages. It was set out to quantify right atrial (RA) morphology and function in a group of acromegalic patients using three-dimensional (3D) speckle-tracking echocardiography (3DSTE). METHODS: The study comprised 30 patients from which 8 patients were excluded due to inadequate image quality. Mean age of the remaining acromegaly patients were 53.7±14.5 years (7 males). In the control group 44 healthy adults were enrolled (mean age: 50.7±12.6 years, 15 males). In each case, complete two-dimensional Doppler echocardiography was performed followed by 3DSTE. RESULTS: All three RA volumetric parameters (Vmax, Vmin, VpreA) were significantly higher in case of acromegaly compared to the healthy controls. Strain analysis revealed that RA function may be enhanced in acromegalic patients, which is more notable in case of active acromegaly. Numerous independent strain parameters had significant correlations with different hormonal variables in the active acromegaly group. These correlations were not present in the inactive acromegaly subgroup. CONCLUSIONS: Acromegaly has a profound effect on RA function and with proper treatment these changes partly seem to be reversible.

Left Atrial Volumetric and Deformation Analysis in Adult Patients with Dextro-Transposition of the Great Arteries (Insights from the CSONGRAD Registry and MAGYAR-Path Study).

BACKGROUND: In complete or dextro-transposition of the great arteries (dTGA), the aorta and the pulmonary artery are transposed. The present study was designed to examine dTGAassociated left atrial (LA) volumetric and functional abnormalities in adult patients late after repair and to compare their results to those of healthy controls. METHODS: The present study consisted of 15 dTGA patients (30.3 ± 8.1 years, 9 males), the patients had Mustard (n = 8) or Senning (n = 7) procedure performed. Their results were compared to those of 36 age- and gender-matched healthy subjects (28.7 ± 1.5 years, 24 males). RESULTS: Increased maximum LA volume and reduced LA emptying fractions respecting the cardiac cycle could be demonstrated in our dTGA patients. LA stroke volumes representing all LA functions were significantly reduced. Peak LA circumferential, longitudinal, and area strains and LA circumferential, longitudinal, and area strains measured at atrial contraction were reduced in our dTGA patients. Most LA strains were reduced in patients having Mustard surgery compared to controls and patients undergoing Senning operation. CONCLUSIONS: Significant LA volumetric and functional abnormalities could be demonstrated in adult patients with dTGA late after repair. Senning procedure seems to have more beneficial long-term effects on LA volumetric and functional features as compared to the Mustard procedure.

[Left ventricular deformation in cardiac light-chain amyloidosis and hypereosinophilic syndrome. Results from the MAGYAR-Path Study]. / Bal kamrai deformáció cardialis könnyulánc-amyloidosisban és hypereosinophilia-szindrómában. Eredmények a MAGYAR-Path Tanulmányból.

Introduction: Hypereosinophilic syndrome (HES) and immunoglobulin light-chain amyloidosis (ALA) are two, rare haematological disorders associated with cardiac alterations. Aim: The goal of the present study was a comparative assessment of left ventricular (LV) deformational parameters in HES and ALA patients using three-dimensional speckle-tracking echocardiography (3DSTE). Method: In the present study, results of 10 HES patients (mean age: 60.9 ± 14.7 years) and 19 ALA patients (mean age: 63.4 ± 7.8 years, 13 males) were analysed. The control group contained 13 age- and gender-matched healthy adults (mean age: 59.2 ± 4.3 years, 5 males). All patients underwent a complete two-dimensional Doppler echocardiography followed by 3DSTE. Results: All basal segmental LV strains were significantly reduced in ALA patients as compared to the control group. Global and mean segmental LV longitudinal strain (LS) values of ALA patients proved to be significantly decreased as compared to those of the healthy control group. During comparison of HES patients and healthy controls, significant difference could be detected in global LV-LS, while segmental basal LV-LS was also significantly reduced in HES patients. Basal LV radial and 3D strains showed significant differences when parameters of HES and ALA patient groups were compared. Conclusion: 3DSTE is a feasible tool for the detailed assessment of LV deformation in HES and ALA patients. Significant LV deformational abnormalities could be detected in both groups. In the case of ALA, these abnormalities are more prominent. Orv Hetil. 2020; 161(5): 169-176.