Árpád Kormányos

Left atrial dysfunction in light-chain cardiac amyloidosis and hypertrophic cardiomyopathy – A comparative three-dimensional speckle-tracking echocardiographic analysis from the MAGYAR-Path Study

INTRODUCTIONnWhile cardiac amyloidosis (CA) is a rare systemic disease characterized by extracellular deposition of protein-derived fibrils, hypertrophic cardiomyopathy (HCM) is histopathologically characterized by myocyte hypertrophy and disarray, interstitial fibrosis, and small intramural coronary arteriole dysplasia. The aim of the present study was to compare left atrial (LA) volumetric and functional characteristics between light-chain (AL) CA and HCM by three-dimensional (3D) speckle-tracking echocardiography (STE).nnnMETHODSnThe AL-CA group initially consisted of 17 patients with AL-CA, but one patient was excluded due to inadequate image quality, and so the study population consisted of 16 patients (mean age: 64.0±9.6 years, five men). Their results were compared with data on 20 age-matched HCM patients (mean age: 59.8±5.2 years, 10 men) and on 16 age-matched healthy controls (mean age: 58.2±7.2 years, six men). Complete two-dimensional Doppler echocardiography and 3D-STE were performed in all cases.nnnRESULTSnSignificantly increased LA volumes were observed in both AL-CA and HCM compared with the control group. Only active atrial emptying fraction was found to be significantly reduced in AL-CA patients compared to controls. Peak global and mean segmental circumferential, longitudinal and area strains showed significant reductions in AL-CA patients compared with controls, but only peak mean segmental longitudinal strain differed significantly between HCM patients and controls. While no differences were demonstrated in global and mean segmental strain at atrial contraction between HCM patients and controls, AL-CA patients showed reductions in certain strain parameters compared to controls and HCM patients.nnnCONCLUSIONSnDifferent patterns of LA functional characteristics were demonstrated in AL-CA and HCM patients by 3D-STE.

Three-dimensional speckle-tracking echocardiography detects different patterns of right atrial dysfunction in selected disorders: a short summary from the MAGYAR-Path Study

The right atrium (RA) is the most under-evaluated heart chamber in the clinical practice. Three-dimensional (3D) speckle-tracking echocardiography (STE) merges the benefits of 3D echocardiography and STE: volumetric and strain assessment of the RA can be performed at the same time from the same digitally acquired 3D echocardiographic dataset with a special equipment using a virtual 3D model of the RA. A diagnostic focused study is under way at the Second Department of Medicine and Cardiology Center, University of Szeged, Hungary to assess 3D-STE-derived RA variables in different patient populations, which serves as a substudy of Motion Analysis of the heart and Great vessels bY three-dimensionAl speckle-tRacking echocardiography in Pathological cases (MAGYAR-Path) Study. The aim of the present review is to summarize these early results.

Rigid body rotation of the left ventricle in hidradenitis suppurativa (a case from the three-dimensional speckle-tracking echocardiographic MAGYAR-Path Study)

In healthy subject, the left ventricular (LV) apex rotates in counterclockwise direction, while the LV base shows a clockwise rotation resulting in a towel-wringing-like motion called LV twist (1). However, this is a very sensitive motion, which could be altered early by several pathological states.

Cardiac amyloidosis is associated with increased aortic stiffness

Cardiac amyloidosis (CA) is as an infiltrative disorder primarily caused by extracellular tissue deposition of amyloid fibrils in the myocardial interstitium. The current study was designed to test whether alterations in ascending aortic elastic properties could be detected by echocardiography in CA patients, and to compare their results to controls.

Left ventricular twist is impaired in acromegaly: insights from the three- dimensional speckle tracking echocardiographic MAGYAR-Path Study

Acromegaly is a rare, chronic, disfiguring, and debilitating disease caused, in 90% of cases, by a benign monoclonal growth hormone–secreting pituitary adenoma. The present study aimed to assess left ventricular (LV) rotational and twist mechanics in acromegalic patients and to compare their results to age‐ and gender‐matched healthy controls.

Change of left ventricular “rigid body rotation” during dipyridamole-induced vasodilation: A case from the three-dimensional speckle tracking echocardiographic MAGYAR-Stress Study

A patient with previous coronary stenting presented with stable angina and positive exercise treadmill test. Echocardiography with three‐dimensional speckle‐tracking demonstrated left ventricular rigid body rotation (near absence of left ventricular twist), whose characteristics changed dramatically during dipyridamole‐induced vasodilatation.

Three-dimensional speckle tracking echocardiography-derived left atrial deformation analysis in acromegaly (Results from the MAGYAR-Path Study)

Acromegaly is a chronic and disfiguring hormonal disease caused by a benign growth hormone‐secreting pituitary adenoma in most of the cases. The objective of this study was to investigate three‐dimensional (3D) speckle tracking echocardiography (3DSTE)‐derived left atrial (LA) volumetric and functional properties in patients with acromegaly. It was also examined whether the activity of acromegaly is associated with further alterations in these LA parameters.

Right Atrial Deformation Analysis in Cardiac Amyloidosis - Results from the Three-Dimensional Speckle-Tracking Echocardiographic MAGYAR-Path Study

Background Light-chain (AL) cardiac amyloidosis (CA) is characterized by fibril deposits, which are composed of monoclonal immunoglobulin light chains. The right ventricle is mostly involved in AL-CA and impairment of its function is a predictor of worse prognosis. Objectives To characterize the volumetric and functional properties of the right atrium (RA) in AL-CA by three-dimensional speckle-tracking echocardiography (3DSTE). Methods A total of 16 patients (mean age: 64.5 ± 10.1 years, 11 males) with AL-CA were examined. Their results were compared to that of 15 age- and gender-matched healthy controls (mean age: 58.9 ± 6.9 years, 8 males). All cases have undergone complete two-dimensional Doppler and 3DSTE. A two-tailed p value of less than 0.05 was considered statistically significant. Results Significant differences could be demonstrated in RA volumes respecting cardiac cycle. Total (19.2 ± 9.3% vs. 27.9 ± 10.7%, p = 0.02) and active atrial emptying fractions (12.1 ± 8.1 vs. 18.6 ± 9.8%, p = 0.05) were significantly decreased in AL-CA patients. Peak global (16.7 ± 10.3% vs. 31.2 ± 19.4%, p = 0.01) and mean segmental (24.3 ± 11.1% vs. 38.6 ± 17.6%, p =0.01) RA area strains, together with some circumferential, longitudinal and segmental area strain parameters, proved to be reduced in patients with AL-CA. Global longitudinal (4.0 ± 5.2% vs. 8.2 ± 5.5%, p = 0.02) and area (7.8 ± 8.1% vs. 15.9 ± 10.3%, p = 0.03) strains at atrial contraction and some circumferential and area strain parameters at atrial contraction were reduced in AL-CA patients. Conclusion Significantly increased RA volumes and deteriorated RA functions could be demonstrated in AL-CA.

Left ventricular ‘rigid body rotation’ in a patient with acromegaly (from the MAGYAR-Path Study)

Cardiovascular complications are known features in acromegaly due to growth hormone (GH) and insulin-like growth factor 1 (IGF-1) excess (1,2). In healthy subjects, left ventricular (LV) apex and base rotate in opposite directions resulting in a towel-wringing-like motion called as LV twist, which could be easily and non-invasively quantified by three-dimensional (3D) speckle-tracking echocardiography (3DSTE) (3). However, little is known about LV myocardial rotational mechanics in acromegaly.

Mit kell tudnunk a cardialis amyloidosisról? A tünettantól a kezelésig

Absztrakt: A szisztemas amyloidosis ritka betegseg, amelyben a sziverintettseg viszonylag gyakran fordul elő es a tulelest jelentősen befolyasolja. Az alapbetegseg es a szervi erintettseg szempontjabol uj diagnosztikus eljarasok segitenek a korai diagnozis felallitasaban es a mihamarabbi kezeles megkezdeseben. Sziverintettseg inkabb a monoklonalis immunglobulin-konnyűlanc (AL-amyloidosis) es a transthyretin formaban fordul elő. AL-amyloidosis eseten a sziverintettseg sulyos kovetkezmenyekhez vezet. A kezdeti sulyossag megiteleseben es a kezelesre adott valasz mertekenek mereseben a szivfunkcioval kapcsolatos biomarker-vizsgalatok segitenek. Amyloidosis eseten az eletkorral nő a sziverintettseg, a prevalencia nem ismert pontosan, de feltetelezhető, hogy tobb eset van valojaban, mint amennyi felismeresre kerul. A szerzők a klinikai tunetek, diagnosztikus eljarasok, kiemelten a kardiologiai vizsgalatok jelentőseget ismertetik. Orv Hetil. 2017; 158(46): 1811–1818.Systemic amyloidosis is a rare disease, in which the heart involvement is rather frequent and determines survival remarkably. Regarding the disease and organ involvement, new diagnostic procedures help to establish the diagnosis and to start the adequate treatment as soon as possible. Cardiac involvement is more likely to be characterised by monoclonal immunglobulin free light chain (AL amyloidosis) type and transthyretin type. In case of AL amyloidosis, heart involvement can lead to serious consequences. Biomarker assessments for cardiac function are important to determine disease severity at the beginning and to measure response to the treatment. In case of amyloidosis, the incidence of the heart involvement grows with age. The prevalence is not known exactly, but probably there are more cases than recognised. The authors present the clinical signs and diagnostic methods, emphasizing the importance of the cardiac examination methods. Orv Hetil. 2017; 158(46): 1811-1818.