[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.

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.

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.

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.

Provision of medical health care for adults with congenital heart disease associated with aortic involvement.

BACKGROUND: All patients with congenital heart disease (CHD) are chronically ill from their cardiac disease. Despite the increasing evidence that aortic alterations are becoming relevant, the importance of aortopathy in CHD has long been underestimated. This study was conducted to determine the health status of patients and/or the provision of health services of adults with CHD (ACHD) with manifest aortopathy or at risk thereof. METHODS: In a questionnaire-based cross-sectional survey, the "real life"-care of ACHD was analysed, comparing patients with risk of developing aortopathy and/or manifest aortopathy. RESULTS: Of the 563 enrolled ACHD (49.6% female, mean age 35.8±12.1, 18-86 years) 56.8% (n=320) had a risk of developing aortopathy and/or manifest aortopathy. Of the 320 patients at risk, 187 (33.2% of the total number) had a proven aortopathy. Within this subgroup, the basic medical care for CHD-independent medical problems was given by primary medical care providers [family doctors/general practitioners (GP) in 89.4% (n=286), internists in 13.4% (n=43), physicians of another specialty in 2.5% (n=8)]. Almost all primary medical care providers knew about the CHD of their patients. Even for CHD-specific health problems, the basic medical care of risk patients was provided by a family doctor or GP in 56.6% (n=181) and by an internist in 18.4% (n=59). 30.0% (n=96) primarily consulted another specialist, including cardiologists. Only 32.8% of ACHD at risk had ever been referred to a CHD specialist by a GP for cardiac problems related to their CHD. In contrast, the need for advice was high for ACHD with aortopathy and related mainly to physical activity, employment and education, pregnancy, rehabilitation or health and life insurance. Only 35.5% of patients at risk indicated that their information on specific care structures for ACHD was sufficient, and a further 38.1% of patients were aware of patient organizations. CONCLUSIONS: Even today, aortic involvement in ACHD is an often-overlooked condition, although considerable negative effects on morbidity and mortality exist. As aortopathy gains in importance with increasing age and complexity of CHD, almost all affected ACHD need lifelong medical advice and access to modern, scientifically based care concepts. According to the study-results, primary care providers and also patients are mostly insufficiently informed about the specialized ACHD facilities. The future goal is therefore to create a better awareness of CHD problems among both primary care physicians and the patients concerned.

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.

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.

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.

Pulmonary Hypertension in Adults with Congenital Heart Disease: Real-World Data from the International COMPERA-CHD Registry.

INTRODUCTION: Pulmonary hypertension (PH) is a common complication in patients with congenital heart disease (CHD), aggravating the natural, post-operative, or post-interventional course of the underlying anomaly. The various CHDs differ substantially in characteristics, functionality, and clinical outcomes among each other and compared with other diseases with pulmonary hypertension. OBJECTIVE: To describe current management strategies and outcomes for adults with PH in relation to different types of CHD based on real-world data. METHODS AND RESULTS: COMPERA (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension) is a prospective, international PH registry comprising, at the time of data analysis, >8200 patients with various forms of PH. Here, we analyzed a subgroup of 680 patients with PH due to CHD, who were included between 2007 and 2018 in 49 specialized centers for PH and/or CHD located in 11 European countries. At enrollment, the patients´ median age was 44 years (67% female), and patients had either pre-tricuspid shunts, post-tricuspid shunts, complex CHD, congenital left heart or aortic disease, or miscellaneous other types of CHD. Upon inclusion, targeted therapies for pulmonary arterial hypertension (PAH) included endothelin receptor antagonists, PDE-5 inhibitors, prostacyclin analogues, and soluble guanylate cyclase stimulators. Eighty patients with Eisenmenger syndrome were treatment-naïve. While at inclusion the primary PAH treatment for the cohort was monotherapy (70% of patients), with 30% of the patients on combination therapy, after a median observation time of 45.3 months, the number of patients on combination therapy had increased significantly, to 50%. The use of oral anticoagulants or antiplatelets was dependent on the underlying diagnosis or comorbidities. In the entire COMPERA-CHD cohort, after follow-up and receiving targeted PAH therapy (n = 511), 91 patients died over the course of a 5-year follow up. The 5-year Kaplan-Meier survival estimate for CHD associated PH was significantly better than that for idiopathic PAH (76% vs. 54%; p < 0.001). Within the CHD associated PH group, survival estimates differed particularly depending on the underlying diagnosis and treatment status. CONCLUSIONS: In COMPERA-CHD, the overall survival of patients with CHD associated PH was dependent on the underlying diagnosis and treatment status, but was significantly better as than that for idiopathic PAH. Nevertheless, overall survival of patients with PAH due to CHD was still markedly reduced compared with survival of patients with other types of CHD, despite an increasing number of patients on PAH-targeted combination therapy.

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.

Mitral annulus is enlarged and functionally impaired in adult patients with repaired tetralogy of Fallot as assessed by three-dimensional speckle-tracking echocardiography-results from the CSONGRAD Registry and MAGYAR-Path Study.

BACKGROUND: Fibrous mitral annulus (MA) is an important part of the mitral valve having a role in forwarding blood from the left atrium (LA) to the left ventricle (LV). MA can be assessed by three-dimensional speckle-tracking echocardiography (3DSTE) respecting the cardiac cycle. The present study was designed to test whether repaired Tetralogy of Fallot (TOF) is associated with morphological and functional alterations of the MA. The role of the type of treatment (early total reconstruction vs. early palliation, late correction) was also assessed. METHODS: The study population consisted of 29 consecutive adults repaired TOF patients (mean age: 35.4±15.5 years, 18 men), from which 13 patients underwent early total reconstruction (etrTOF), while 16 patients were firstly palliated and later corrected (pcTOF). Their data were compared to that of 76 age- and gender-matched healthy controls (mean age: 35.9±7.6 years, 33 men). All repaired TOF patients and controls were assessed by two-dimensional (2D) Doppler echocardiography and 3DSTE. RESULTS: Dilated end-systolic and end-diastolic MA diameter, area and perimeter and reduced MA fractional area change and MA fractional shortening could be demonstrated in repaired TOF patients as compared to controls. Increased body surface area-indexed end-diastolic and end-systolic MA diameter and perimeter could be demonstrated in pcTOF patients as compared to that of etrTOF cases. CONCLUSIONS: MA enlargement and functional impairment could be detected in adult patients with repaired TOF regardless of the type of correction. However, pcTOF patients have worse results.

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.

[Echocardiographic evaluation of the right atrial appendage and its clinical consequences]. / A jobb pitvari fülcse echokardiográfiás vizsgálata és annak klinikai jelentosége.

In case of atrial fibrillation, there is a higher risk of thrombus formation, which could affect the right heart as well. Visualization of the right atrial appendage is difficult; the aim of the present review was to demonstrate the role of routine echocardiographic techniques and to show related clinical data. Orv Hetil. 2019; 160(12): 443-447.

[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.

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.

[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.

The role of echocardiography in the management of adult patients with congenital heart disease following operative treatment.

Treatment of congenital heart diseases has significantly advanced over the last few decades. Due to the continuously increasing survival rate, there are more and more adult patients with congenital heart diseases and these patients present at the adult cardiologist from the paediatric cardiology care. The aim of the present review is to demonstrate the role of echocardiography in some significant congenital heart diseases.