[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.

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.

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.

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.

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.

Left Atrial Deformation Analysis in Patients with Corrected Tetralogy of Fallot by 3D Speckle-Tracking Echocardiography (from the MAGYAR-Path Study) / Análise por Speckle-Tracking com Ecocardiografia 3D da Deformação de Átrio Esquerdo em Pacientes com Tetralogia de Fallot Corrigida (do MAGYAR-Path Study)

Abstract Background: Three-dimensional (3D) echocardiography coupled with speckle-tracking echocardiographic (STE) capability is a novel methodology which has been demontrated to be useful for the assessment of left atrial (LA) volumes and functional properties. There is increased scientific interest on myocardial deformation analysis in adult patients with corrected tetralogy of Fallot (cTOF). Objectives: To compare LA volumes, volume-based functional properties and strain parameters between cTOF patients and age- and gender-matched healthy controls. Methods: The study population consisted of 19 consecutive adult patients with cTOF in sinus rhythm nursing at the University of Szeged, Hungary (mean age: 37.9 ± 11.3 years, 8 men, who had repair at the age of 4.1 ± 2.5 years). They all had undergone standard transthoracic two-dimensional Doppler echocardiographic study extended with 3DSTE. Their results were compared to 23 age- and gender-matched healthy controls (mean age: 39.2 ± 10.6 years, 14 men). Results: Increased LA volumes and reduced LA emptying fractions respecting cardiac cycle could be demonstrated in cTOF patients compared to controls. LA stroke volumes featuring all LA functions showed no differences between the 2 groups examined. LA global and mean segmental uni- and multidirectional peak strains featuring LA reservoir function were found to be diminished in adult patients with cTOF as compared to controls. Similarly to peak strains reduced global and mean segmental LA strains at atrial contraction characterizing atrial booster pump function could be demonstrated in cTOF patients as compared to controls. Conclusions: Significant deterioration of all LA functions could be demonstrated in adult patients with cTOF late after repair.

Resumo Fundamento: Ecocardiografia tridimensional (3D) acoplada à técnica de speckle-tracking (3DSTE) é uma nova metodologia útil para a avaliação de volumes e propriedades funcionais do átrio esquerdo (AE). Há crescente interesse científico na análise da deformação miocárdica em adultos com tetralogia de Fallot corrigida (cTOF). Objetivos: Comparar os volumes de AE, propriedades funcionais baseadas no volume e parâmetros de strain entre pacientes com cTOF e controles saudáveis pareados por idade e sexo. Métodos: A população do estudo consistiu em 19 adultos com cTOF consecutivos, em ritmo sinusal, acompanhados na Universidade Szeged, Hungria (idade média: 37,9 ± 11,3 anos; 8 homens; com correção cirúrgica aos 4,1 ± 2,5 anos de idade). Todos foram submetidos a ecocardiografia transtorácica bidimensional com Doppler padrão e 3DSTE. Os resultados foram comparados aos de 23 controles saudáveis pareados por idade e sexo (idade média: 39,2 ± 10,6 anos; 14 homens). Resultados: Aumento dos volumes de AE e redução das frações de esvaziamento de AE em relação ao ciclo cardíaco foram demonstrados em pacientes com cTOF em comparação aos dos controles. Os volumes de ejeção de AE caracterizando todas as funções do AE não diferiram entre os dois grupos. Strains de AE global e segmentar médio uni- e multidimensional, caracterizando função de reservatório de AE, estavam diminuídos em adultos com cTOF em comparação aos de controles. À semelhança dos strains de pico, reduzidos strains de AE global e segmentar médio na contração atrial, caracterizando função de bomba atrial, foram demonstrados em pacientes com cTOF em comparação aos de controles. Conclusões: Demonstrou-se significativa deterioração das funções de AE em adultos com cTOF em fase tardia após correção.

[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.

[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.

[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.