Infective endocarditis complicated with coronary artery septic embolization: is it worth to be mentioned? Case presentation and review of the literature.

Coronary artery septic embolization is a rare, but severe complication of infective endocarditis involving the leftside of the valves. The first case mentioned in the literature was a postmortem finding of a left anterior descending coronary artery occlusion by a vegetation fragment. Since this case, there have been several therapeutic strategies published with this clinical setting including medical treatment, percutaneous coronary angioplasty addressing coronary occlusion, surgical intervention for both the infected valve and coronary embolization, and hybrid procedures with transcatheter septic embolus aspiration followed by surgical valvular interventions. Out of the three interventions mentioned, the latter provided the best results and was in concordance with results observed in a case of mitral valve infected endocarditis complicated with acute occlusion of the left anterior descending coronary artery in patient whose comorbidities included hypertrophic obstructive cardiomyopathy. A transcatheter left anterior descending coronary artery embolus aspiration was performed , followed by a surgical mitral valve replacement and septal myectomy with an uneventful postoperative course. Although rare, this severe complication of infective endocarditis has a specific clinical course and therapeutic strategy, and in our opinion, it could be mentioned as a separate entity among embolic complications of infective endocarditis in future guidelines. Previously published cases suggest that the hybrid intervention might be the therapy of choice for this clinical setting; however, larger studies are necessary for confirmation.

Primary cardiac angiosarcoma: A case report.

Cardiac angiosarcomas are the most common primary malignant cardiac tumors in adults. The diagnosis is often delayed due to nonspecific clinical symptoms at presentation. The cornerstones of diagnosis are echocardiography and the histological evaluation of the cardiac biopsy. The knowledge on the treatment is limited; the outcomes of chemotherapy, radiotherapy, complete surgical removal, and heart transplantation are controversial. We report a 38-year-old woman with a primary heart tumor which infiltrated the right atrial wall and the pericardium and caused pericardial effusion. Angiosarcoma was verified histologically. The surgical excision could not be radical, and the patient died 3 months from diagnosis.

[Echocardiographic assessment of the left atrial appendage - transoesophageal, transthoracic and intracardiac approaches]. / A bal pitvari fülcse echokardiográfiás vizsgálata ­ transoesophagealis, transthoracalis és intracardialis lehetoségek.

Left atrial appendage is a cardiac chamber in variable shapes and sizes. Its condition is associated with atrial arrhytmias and the presence of a thrombus. Due to its difficult visualisation, the present review aimed to demonstrate the role of different echocardiographic methods in its assessment. Orv Hetil. 2018; 159(9): 335-345.

[The significance of N-terminal pro-B natriuretic peptide in the management of adult patients with congenital heart disease - Results from the CSONGRAD Registry]. / Az N-terminális pro-B natriureticus peptid jelentosége a felnott korú congenitalis szívbetegek gondozásában ­ Eredmények a CSONGRAD Regiszterbol.

INTRODUCTION: The prognostic role of B-type natriuretic peptide (BNP) level was confirmed in chronic heart failure and congenital heart diseases irrespective of the aetiology. AIM: The aim of this study was to compare NT­proBNP measured in the clinical practice and important clinical and echocardiographic parameters in patients with adult congenital heart diseases under our care. METHOD: Data of a total of 70 patients were analysed; 34 patients had corrected tetralogy of Fallot and 19 patients had corrected transposition of the great arteries. In 17 further cases, patients with other congenital vitiums have been involved in the study. In all cases, out-patient examination was performed with electrocardiography, echocardiography and NT-proBNP measurement. The New York Heart Association (NYHA) stages of the patients were determined and the patients filled in a questionnaire regarding their quality of life. RESULTS: Elevated NT-proBNP level resulted in a worse quality of life (visual analogue scale, VAS) and an increased right ventricular end diastolic diameter; the incidence of arrhythmia increased as well. Negative correlation was found between the NT-proBNP level and the VAS value (r = -0.45, p = 0.0001) and the left ventricular ejection fraction (LV-EF) (r = -0.67, p = <0.0001). ROC analysis showed that NT­proBNP≥668.1 pg/ml was the cut-off value that most accurately predicted NYHA class III-IV (sensitivity 93%, specificity 63%, area under the curve 80%, p = 0.001). Similarly, NT-proBNP≥184.7 pg/ml was found to be the cut-off value most accurately predicting LV-EF below 55% (sensitivity 66%, specificity 67%, area under the curve 77%, p = 0.02). CONCLUSIONS: NT-proBNP has a role in the evaluation of adult patients with congenital heart diseases and in determining the prognosis of these patients, and in addition to other examinations used in the follow-up of these patients, NT-proBNP may help in determining the time of reoperation or potential heart transplantation. Orv Hetil. 2018; 159(4): 141-148.

[Surgical management of congenital heart defects beyond the age of 60]. / Veleszületett szívhibák sebészi kezelése 60 éves kor felett.

INTRODUCTION: The population with congenital heart disease is increasing and ageing. AIM: The aim of the authors was to examine the outcome of surgical management of congenital heart diseases beyond the age of 60 years. METHOD: Between 2013 and 2015, 77 adults were operated (36 younger, and 41 older than forty years, including 12 patients aged over 60 years. The numbers of procedures were as follows (in brackets the number of operations in the three age groups): Ross surgery 5 (3, 2, 0); aortic valve replacement 19 (12, 1, 6); subaortic membrane resection 1 (0, 0, 1); Bentall/ascending aortic plasty 8 (4, 3, 1); myectomy with or without mitral valve replacement in left ventricular outflow obstruction 5 (0, 3, 2); aortic coarctation 1 (1, 0, 0); ligation of ductus arteriosus 2 (1, 1, 0); reconstruction of right ventricular outflow tract with biological valve 4 (0, 3, 1); homograft 5 (5, 0, 0); BioValsalva graft 1 (0, 1, 0); primary reconstruction of complete atrioventricular septum defect 3 (1, 2, 0); valve replacement 2 (1, 1, 0); ventricular septum defect 10 (4, 6, 0); atrial septum defect closure 5 (2, 3, 0); total cavopulmonal anastomosis 1 (1, 0, 0); valve replacement in congenital transposition of great arteries 1 (0, 1, 0), Ebstein operation with valve plasty 2 (0, 1, 1); valve replacement 2 (1, 0, 1). RESULTS: There was no operative mortality, while early mortality occurred in one patient with total cavopulmonal anastomosis due to multiorgan failure. CONCLUSIONS: Congenital heart defects can be operated beyond the age of 60 years with good results in a tertiary heart centre having great experience in the management of congenital and acquired heart disease.

[Long-term follow-up of patients with transposition of the great arteries following Senning or Mustard operations. Results from the CSONGRAD Registry]. / A teljes nagyér-transzpozíción átesett betegek Senning- és Mustard-féle mutéti megoldásának hosszú távú utánkövetése. Eredmények a CSONGRAD Regiszterbol.

INTRODUCTION: Transposition of the great arteries is one of the most common cyanotic congenital heart diseases. AIM: The present study aimed to examine and compare long-term survival, functional grading, arrhytmologic and quality of life control in patients with transposition of the great arteries following Senning- and Mustard-operations. METHODS: The present study comprised 85 patients with transposition of the great arteries, of whom Senning-operation was performed in 37 cases and Mustard-operation in 48 subjects. Follow-up study was performed in all cases. RESULTS: The success rate of long-term follow-up proved to be 74%. Twelve out of the 31 Senning-operated and 16 out of 32 Mustard-operated patients died during the follow-up (39% vs. 50%, p = 0.45). Neither features of heart failure, nor those of arrhythmias showed differences between the groups, but parameters of quality of life and functional capacity proved to be favourable in Senning-operated patients. CONCLUSIONS: There is no significant difference in mortality and morbidity of patients with transposition of the great arteries following Mustard- and Senning-operations. Regarding to long-term follow-up quality of life and functional capacity of Senning-operated patients were more favourable.

Evaluation of right atrial dysfunction in patients with corrected tetralogy of Fallot using 3D speckle-tracking echocardiography. Insights from the CSONGRAD Registry and MAGYAR-Path Study.

BACKGROUND: In recent studies, alterations in ventricular deformations were demonstrated in adult patients with corrected tetralogy of Fallot by three-dimensional speckle-tracking echocardiography. The present study was designed to assess three-dimensional speckle-tracking echocardiography-derived right atrial volumetric and strain parameters in corrected tetralogy of Fallot. METHODS: A total of 17 patients with corrected tetralogy of Fallot were included in the study. Their results were compared with 18 age- and gender-matched healthy controls. Complete two-dimensional echocardiography and three-dimensional speckle-tracking echocardiography were performed in all cases. RESULTS: Significantly increased right atrial volumes respecting heart cycle were detected in patients with corrected tetralogy of Fallot. Total and passive atrial emptying fractions proved to be significantly decreased in patients with corrected tetralogy of Fallot (26.4 ± 12.4 % vs. 39.1 ± 8.8 %, p = 0.001 and 11.2 ± 6.8 % vs. 19.8 ± 9.0 %, p = 0.003, respectively). Global and mean segmental peak longitudinal (17.3 ± 9.2 % vs. 30.8 ± 11.2 %, p = 0.0007 and 20.6 ± 10.7 % vs. 34.4 ± 10.5 %, p = 0.0005) and area strains (20.1 ± 17.6 % vs. 41.0 ± 19.8 %, p = 0.004 and 28.1 ± 19.8 % vs. 49.1 ± 19.7 %, p = 0.004) as well as global radial peak strain (-9.1 ± 5.1 % vs. -15.0 ± 10.0 %, p = 0.05) were reduced in patients with corrected tetralogy of Fallot compared with controls. CONCLUSIONS: The complexity of right atrial dysfunction can be demonstrated by three-dimensional speckle-tracking echocardiography in patients with corrected tetralogy of Fallot.

"Rigid body rotation" of the left ventricle in hypoplastic right-heart syndrome: a case from the three-dimensional speckle-tracking echocardiographic MAGYAR-Path Study.

Left ventricular twist results from the movement of two orthogonally oriented muscular bands of the helical myocardial structure, with a consequent clockwise rotation of the left ventricular base and counterclockwise rotation of the left ventricular apex. To the best of the authors' knowledge, this is the first time that left ventricular "rigid body rotation", the near absence of left ventricular twist in hypoplastic right-heart syndrome, has been demonstrated.

[Perspectives in the management of congenital heart defects in adult patients]. / Perspektívák a veleszületett szívhibák felnottkori sebészi kezelésében.

Due to improving results in congenital heart surgery, the number of adult patients with congenital heart defect is increasing. The question is: what kind of problems can be managed in this patient-group? The authors review the different problems of management of congenital heart defects in adults based on national and international literature data. Simple defects recognised in adults, postoperative residual problems, changing of small grafts and valves, correction of primary or operated coarctation aortae can be usually managed without problems. A very close follow-up is necessary to establish the correct period for heart transplantation in patients with transposition of great arteries with Senning/Mustard operation, and univentricular heart corrected with "Fontan-circulation" type surgical procedure. The authors conclude that although the number of patients increases, only a few congenital heart diseases may cause problems. It seems important (1) to monitor asymptomatic patient who underwent operation (Fallot-IV, Ross procedure, etc.), (2) follow up regularly patients who underwent Senning/Mustard procedure (magnetic resonance imaging, echocardiography, brain natriuretic peptide measurement), (3) define the proper period of preparation for heart transplantation of patients with a univentricular heart, with special attention to the possibility of multiorgan (lung, liver, etc.) failure. Due to the improvement of foetal diagnosis of congenital heart defects, the number of patients with complex congenital heart defects is decreasing. The standard management of these patients could be primary heart transplantation in infancy.

[More than 50 years' experience in the treatment of patients with congenital heart disease at a Hungarian university hospital]. / Több mint 50 év tapasztalat a congenitalis szívbetegek ellátásában egy magyar egyetemi központban. A CSONGRÁD Regiszter alapadatai.

Improvements in surgical techniques and technical advancements have made possible for several patients with congenital heart disease to grow up to adulthood. It has been decided to create a registry for their more precise treatment. This registry now includes 2770 patients with data on 3043 operations, with almost 30 different diagnoses. The purpose of this paper is to review the facts and the basics leading to the establishment of this registry.

[Efficacy and safety of apixaban compared with warfarin regarding time within the therapeutic range]. / Az apixaban hatékonysága és biztonságossága a warfarinhoz képest a terápiás tartományban töltött ido függvényében.

Prevention of thromboembolism by lifelong anticoagulation is an important therapeutic goal in patients with atrial fibrillation according to recent guidelines. Major drawback of vitamin K antagonists are their narrow therapeutic range and interactions with other drugs and food. These have significant impact on the pharmacokinetics and pharmacodynamics requiring regular measurements of the international normalized ratio. Efficiency of the anticoagulant therapy depends considerably on time within the therapeutic range of prothrombin international normalized ratio. Time within the therapeutic range represents the percentage of time within the required range of prothrombin international normalized ratio. Prothrombin international normalized ratio outside the therapeutic range increases the risk of thromboembolism or bleeding according to whether it falls below or above the range. New oral anticoagulants do not require routine monitoring of anticoagulation. Their efficacy and safety are shown to be at least as good as or better than those of warfarin. In patients with nonvalvular atrial fibrillation ARISTOTLE study revealed that antithrombotic effect of apixaban compared with warfarin is better and with lower bleeding risk irrespective of the quality of prothrombin international normalized ratio control. Orv. Hetil., 2014, 155(5), 177-181.

[Congenital heart diseases in patients above the age of 60 years. Results from the CSONGRAD Registry] / Veleszületett szívbetegségek 60 éves kor felett. Eredmények a CSONGRAD regiszterbol

INTRODUCTION: Development in paediatric and interventional cardiology and cardiac surgery resulted in an increase in the number and average age of adult congenital heart disease patients. Comorbidities may appear with increased age leading to new challenges in the diagnosis and treatment of this complex group of patients. OBJECTIVE: The aim of this study is to compare clinical and echocardiographic parameters in adult congenital heart disease patients under our care below the age of 40 years, between 40 and 59 years and above the age of 60 years. METHOD: Data of a total of 346 patients were analyzed; 154 patients were under 40 years of age, 133 patients were between 40 and 59 years of age, and 59 patients were 60 years old or older. All adult congenital heart disease patients who underwent an outpatient examination were included in the study. As part of the physical examination, the New York Heart Association (NYHA) clinical classification of heart failure was determined, electrocardiography, echocardiography and 6-minute walk test were performed. RESULTS: Above the age of 40, the ratio of comorbidities increased, significantly more patients were classified into NYHA functional classes III-IV and the ratio of patients having a left ventricular ejection fraction below 55% significantly increased. The prevalence of arrhythmia was similar in all age groups, but an increasing tendency could be seen with age. CONCLUSION: There is a growing number of elderly adult congenital heart disease patients with comorbidities that play an important role in the management and in the outcome of congenital heart disease. New protocols and recommendations are required in the follow-up of these patients to help determining the optimal time for reoperation, intervention or heart transplantation. Orv Hetil. 2023; 164(6): 219-226.

[Multimodality imaging of adult patients with surgically corrected tetralogy of Fallot. Results from the CSONGRAD Registry] / A mutétileg korrigált Fallot-tetralógiával élo felnott betegek multimodális képalkotó vizsgálata. Eredmények a CSONGRAD Regiszterbol

INTRODUCTION: There is an increasing ratio of surviving patients with congenital heart disease including tetralogy of Fallot. OBJECTIVE: The aim of the present study was a comparative assessment of echocardiographic and cardiac magnetic resonance imaging-derived findings in patients with tetralogy of Fallot following early total reconstruction versus early palliation/late correction. METHODS: The study comprised 17 patients with tetralogy of Fallot (mean age: 28.6 ± 10.4 years; 10 males). Early total reconstruction was performed in 10 cases (mean age: 25.0 ± 8.0 years; 8 males), while early palliation/late construction was done in 7 subjects (mean age: 33.7 ± 11.8 years; 2 males). Complete two-dimensional Doppler echocardiography and cardiac resonance imaging were performed in all patients. RESULTS: During long-term follow-up, lower left ventricular end-systolic (40.50 ± 10.55 ml/m2 vs. 58.14 ± 19.07 ml/m2, p = 0.013) and end-diastolic volume indices (86.60 ± 12.62 ml/m2 vs. 116.70 ± 23.70 ml/m2, p = 0.002) and stroke volume index (46.00 ± 6.77 ml/m2 vs. 58.43 ± 7.11 ml/m2, p = 0.001) could be detected, which were associated with higher echocardiographic left ventricular ejection fraction (69.75 ± 6.80% vs. 61.67 ± 8.80%, p = 0.038) in patients with tetralogy of Fallot following early total reconstruction as compared to that of subjects late after early palliation/late correction. In regard with the right heart, larger right ventricular muscle mass (72.33 ± 21.03 g/m2 vs. 51.33 ± 22.33 g/m2, p = 0.044) could be seen in patients with tetralogy of Fallot following early total reconstruction. CONCLUSION: Beneficial left ventricular morphological and functional parameters, but more pronounced right ventricular hypertrophy could be detected in patients with tetralogy of Fallot following early total reconstruction as compared to that of subjects late after early palliation/late correction. Orv Hetil. 2023; 164(5): 186-194.

Features of the right atrium in repaired dextro-transposition of the great arteries following atrial switch operations (Insights from the CSONGRAD Registry and MAGYAR-Path Study).

BACKGROUND: In dextro-transposition of the great arteries (dTGA), the aorta and the pulmonary artery are transposed. The present study aimed a three-dimensional speckle-tracking echocardiography-derived determination of volumetric and functional features of the atrium being on the morphologic right side ['right atrium (RA)] in adult patients with dTGA. METHODS: The present study comprised 18 adult dTGA patients, four of whom were excluded due to inferior image quality. From the remaining 14 patients (mean age: 29.7 ±â€¯8.1 years, 9 males), 7 cases underwent Mustard-procedure, while another 7 subjects underwent Senning-procedure. Their results were compared to that of 28 age- and gender-matched healthy subjects (28.8 ±â€¯1.4 years, 20 males). RESULTS: Increased RA volumes respecting the cardiac cycle could be demonstrated in dTGA patients compared to controls. RA stroke volumes (SVs) for reservoir and conduit function were reduced together with impaired RA emptying fractions (EFs) featuring all phases of RA function. Mustard-operated patients showed tendentiously lower RA volumes and increased SVs and EFs respecting the cardiac cycle compared to those of Senning-operated patients suggesting beneficial results for Mustard-procedure. Reduced RA global and mean segmental peak RA strains and RA strains at atrial contraction could be detected in dTGA patients compared to those of controls with tendentiously lower values in Mustard-operated patients compared to those of Senning-operated subjects. CONCLUSIONS: Significant RA volumetric and functional abnormalities could be detected in adult dTGA patients following atrial switch repair. While RA volumetric data proved to be better in Mustard-operated patients, RA strains were enhanced in Senning-operated subjects.

Left ventricular rotational abnormalities in patients with transposition of the great arteries late after atrial switch operation: detailed analysis from the CSONGRAD Registry and MAGYAR-Path Study.

Background: Dextro-transposition of the great arteries (dTGA) is one of the most common cyanotic congenital heart defects, when the origins of the main arteries are switched in position. The present retrospective cohort study aimed three-dimensional speckle-tracking echocardiography-derived determination of apical and basal morphologic left ventricular (mLV) rotations and twist in adults with dTGA late after atrial switch. It was also purposed to compare whether differences in mLV rotational parameters were present in Senning- and Mustard-operated subjects. Methods: Sixteen dTGA patients were willing to participate late after atrial switch in this study, however, 6 subjects were excluded due to inferior image quality. The remaining group of 10 dTGA patients had a mean age of 29.4±8.8 years (5 males). Their clinical data were from the CSONGRAD Registry. Their results were compared to 24 age- and gender-matched healthy controls with a mean age of 34.4±12.6 years (14 males). Results: From the dTGA patient population, only 5 out of 10 subjects had normally directed mLV rotational mechanics, 5 dTGA cases had significant mLV rotational abnormality with counterclockwise mLV basal rotation in 4 patients (mLV rigid body rotation, mLV-RBR). One patient had complete reversal of apical and basal mLV rotations. Compared to the matched healthy controls, dTGA patients showed mLV-RBR significantly more frequently (50% vs. 0%, P=0.0009) regardless of the fact whether Senning- or Mustard-procedure was performed. dTGA patients with normally directed mLV rotational mechanics proved to have increased mLV basal rotation (-7.9±4.1 vs. -3.7±1.9 degree, P=0.001) with preserved mLV twist (16.4±3.3 vs. 14.0±4.1 degree, P=ns) as compared to matched controls. Conclusions: Significant mLV rotational abnormalities are present in dTGA late after atrial switch procedures including mLV-RBR and reversed mLV twist. In dTGA patients with normally directed mLV rotational mechanics, mLV basal rotation is increased with preserved mLV twist. Some differences in mLV rotational abnormalities are present between Senning- and Mustard-procedures.

Tricuspid annular abnormalities following different surgical strategies in adults with corrected tetralogy of Fallot (Results from the CSONGRAD Registry and MAGYAR-Path Study).

BACKGROUND: Although there is an increasing number of patients with corrected tetralogy of Fallot (cTOF), who reach adulthood due to recent successful surgical procedures, some of them suffers from late complications, including functional tricuspid regurgitation (FTR). The purpose of the present study was to examine tricuspid annular (TA) abnormalities in adult cTOF patients and their relationship right atrial (RA) volumes respecting the cardiac cycle assessed by three-dimensional speckle-tracking echocardiography (3DSTE). Moreover, the effect of different surgical procedures on TA morphologic and functional parameters was examined as well. METHODS: The study comprised 24 cTOF patients (32.8±13.5 years, 9 males) in which early palliative surgery and late total correction were performed in 12 cases (pcTOF), while early total reconstruction was performed in 12 subjects (etrTOF). Their results were compared to those of 33 matched healthy adults (36.7±7.2 years, 15 males). RESULTS: Dilated end-systolic and end-diastolic TA morphologic parameters and their body surface area-indexed counterpart could be detected in cTOF patients as compared to that of controls. TA fractional area change and TA fractional shortening proved to be reduced in cTOF patients as well as in etrTOF and pcTOF patients compared to controls. None of the TA morphologic and functional parameters showed any differences between etrTOF and pcTOF patients. Increased maximum, preatrial contraction and minimum RA volumes could be detected in cTOF patients compared to controls, which correlated with TA dimensions. CONCLUSIONS: TA is dilated with reduced function in adult patients with cTOF. TA dilation is related to RA volumes. etrTOF and pcTOF patients have similar TA dimensions and TA functional properties. KEYWORDS: Fallot; tricuspid annulus; three-dimensional (3D); speckle-tracking; echocardiography.

Tricuspid annular abnormalities in repaired dextro-transposition of the great arteries following Senning and Mustard procedures (Insights from the CSONGRAD Registry and MAGYAR-Path Study).

BACKGROUND: In dextro-transposition of the great arteries (dTGA), the aorta and the pulmonary artery are transposed in position, when aorta arises from the right ventricle and pulmonary artery arises from the left ventricle. The present study was designed to assess three-dimensional speckle-tracking echocardiography-derived tricuspid annular (TA) abnormalities in adult patients with dTGA late after atrial switch operations. It was also examined whether differences in TA morphology and function exist between Senning- and Mustard-procedures. METHODS: The study consisted of 14 adult dTGA patients (mean age: 29.9±8.3 years, 6 males), who underwent Mustard-procedure (n=7) at the age of 1.57±0.53 years or Senning-procedure (n=7) at the age of 1.42±0.53 years. Their results were compared to 28 age- and gender-matched healthy subjects (30.3±4.9 years, 14 males). RESULTS: Dilated end-systolic and end-diastolic TA diameters, areas and perimeters could be detected in dTGA patients as compared to those of controls. TA functional properties calculated from TA diameter (TAFS) and area (TAFAC) data proved to be deteriorated in dTGA patients. No differences could be detected either in TA dimensions, or in TA functional properties between Senning- and Mustard-operated dTGA patients. TA plane systolic excursion (TAPSE) was reduced, which did not correlate with TAFAC and TAFS in dTGA patients. CONCLUSIONS: In dTGA, dilated end-systolic and end-diastolic TA is accompanied with deteriorated TA functional properties regardless of which atrial switch procedure was performed. Correlations between TAPSE representing longitudinal movement of the TA and TAFAC and TAFS representing sphincter-like movement of the TA disappeared, which could partially explain accompanying tricuspid functional regurgitations. KEYWORDS: Tricuspid annulus; function; three-dimensional echocardiography; speckle-tracking; transposition of the great arteries.

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.