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Coronary events are life-threatening long-term complications of the arterial switch operation for complete transposition of the great arteries. The aim of our study was to assess the dimensions of the reimplanted coronary arteries and their relationship with the various geometric characteristics to gain a better understanding of the involved mechanisms. Coronary computed tomography angiography (CCTA) scans of 78 asymptomatic pediatric patients were performed at the age of 10.7 (6.3-17.8) years. The position of the ostia, the branching angles, and the diameters of the coronary arteries were determined in a subgroup of 51 patients presenting the usual preoperative coronary anatomy. Mean Z-score of the left main coronary artery (LMCA), left anterior descending coronary artery (LAD), left circumflex coronary artery (LCX), and right coronary artery (RCA) diameters were 0.7 ± 1.2, - 0.4 ± 1.1, - 0.3 ± 1.1, and - 0.3 ± 1.1, respectively. The Z-score of the ostial LMCA diameter had a positive correlation with the remoteness from the main pulmonary artery (p < 0.001) and the branching angle (p = 0.001). The ostial diameter Z-score of the LMCA had a negative correlation with the interval between the arterial switch operation and the CCTA (p = 0.004). Even though most of the coronary diameters fell within the accepted range, which suggests normal overall development, acute branching angle and more anterior origin were associated with smaller ostial coronary artery diameter Z-scores. To prove the clinical relevance of the smaller ostial diameter of high-risk left coronary arteries and the decrease of ostial coronary artery Z-scores over time needs further follow-up studies., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

The classical histological features of the thymus are the cortex and medulla, the Hassall's bodies as well as the lobules. Anti-pan-cytokeratin immunocytochemistry shows that the keratin staining pattern of the cortical and medullary epithelial cells is different. The medulla is further compartmentalized: it consists of keratin-positive network and keratin-negative areas. Histology of the keratin-negative area is identical with the connective tissue of the septae. The basal lamina is continuous at the capsule and septae, but it becomes discontinuous at the border between the keratin-positive network and keratin-negative area. This immunohistochemical finding is the first histological sign, which may explain that the medulla has no blood-thymus barrier. The supporting tissue of the keratin-negative area is identical with that of the septae. The connective tissue of thymic capsule and septae develops from the cranial neural crest cells, therefore we hypothesize that the keratin-negative area has neural crest origin. Blood vessels of the thymic medulla localize in the keratin-negative area. Every emigrating or immigrating immunologically competent cells should enter the keratin-negative area, therefore this area is the transit zone of the thymus. The hematoxylin-eosin staining of the thymus shows that the thymic cortico-medullary border does not represent cellular background. However, the border between keratin-positive network and keratin-negative area is determined by cellular identity (epithelial and mesenchymal tissues). Therefore, it can be assumed that the real histological and functional border is the border between the keratin-positive network and the keratin-negative area. Orv Hetil. 2019; 160(5): 163-171.

Background: Decision-making when offered a donor heart for transplantation is complex, and supportive data describing outcomes according to acceptance or non-acceptance choices are sparse. Our aim was to analyze donor heart acceptance decisions and associated outcomes at a single center, and after subsequent acceptance elsewhere., Methods: This investigation was a retrospective analysis of data obtained from the University of Vienna Medical Center and Eurotransplant centers for the period 2001 to 2015., Results: Our center accepted 31.8% (699 of 2,199) of donor hearts offered. Unlike other centers, the acceptance rate, with or without transplantation, did not increase over time. Of the donor hearts rejected by our center, 38.1% (572 of 1,500) were later accepted elsewhere. Acceptance rates were twice as high for donor hearts initially rejected for non-quality reasons (339 of 601, 56.4%) compared with initial rejection for quality reasons (233 of 899, 25.9%). Three-year patient survival rate was 79% at Vienna; for donor hearts initially rejected by Vienna for non-quality reasons or quality reasons, it was 73% and 63%, respectively (p < 0.001). Outcomes at other centers after transplantation of grafts rejected by Vienna varied according to the reason for rejection, with good 3-year survival rates for rejection due to positive virology (77%), high catecholamines (68%), long ischemic time (71%), or low ejection fraction (68%), but poor survival was observed for hearts rejected for hypernatremia (46%), cardiac arrest (21%), or valve pathology (50%)., Conclusions: A less restrictive policy for accepting donor hearts at our center, particularly regarding rejection for non-quality reasons or for positive virology, high catecholamine levels, longer ischemic time, or low ejection fraction, could expand our donor pool while maintaining good outcomes., (Copyright © 2018 International Society for the Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Low output syndrome significantly increases morbidity and mortality of cardiac surgery and lengthens the durations of intensive care unit and hospital stays. Its treatment by catecholamines can lead to undesirable systemic and cardiac complications. Levosimendan is a calcium sensitiser and adenosine triphosphate (ATP)-sensitive potassium channel (I K,ATP ) opener agent. Due to these effects, it improves myocardium performance, does not influence adversely the balance between O 2 supply and demand, and possesses cardioprotective and organ protective properties as well. Based on the scientific literature and experts' opinions, a European recommendation was published on the perioperative use of levosimendan in cardiac surgery in 2015. Along this line, and also taking into consideration cardiac surgeon, anaesthesiologist and cardiologist representatives of the seven Hungarian heart centres and the children heart centre, the Hungarian recommendation has been formulated that is based on two pillars: literature evidence and Hungarian expert opinions. The reviewed fields are: coronary and valvular surgery, assist device implantation, heart transplantation both in adult and pediatric cardiologic practice. Orv Hetil. 2018; 159(22): 870-877.

Background: The occurrence of postoperative chylothorax in children with congenital heart disease is a rare and serious complication in cardiac intensive care units (ICUs). The aim of our study was to identify the perioperative characteristics, treatment options, resource utilization and long term complications of patients having chylothorax after a pediatric cardiac surgery., Methods: Patients were retrospectively assessed for the presence of chylothorax between January 2002 and December 2012 in a tertiary national cardiac center. Occurrence, treatment options and long term outcomes were analyzed. Chylothorax patients less than 2 years of age were analyzed using propensity-matched statistical analysis in regard to postoperative complications after discharge., Results: During the 10-year period, 48 patients had chylothorax after pediatric cardiac surgery. The highest incidence was observed on the second postoperative day (7 patients, 14.6%). Seven patients (14.6% of the chylothorax population) died. During the follow up period, 5 patients had additional thromboembolic complications (2 had confirmed thrombophilia). Eleven patients had a genetic abnormality (3 had Down's syndrome, 3 had Di-Giorge's syndrome, 1 had an IgA deficiency and 4 had other disorders). During the reoperations (49 cases), no chylothorax occurred. After propensity matching, the occurrence of pulmonary failure (P=0.001) was significantly higher in the chylothorax group, and they required prolonged mechanical ventilation (P=0.002) and longer hospitalization times (P=0.01). After discharge, mortality and neurologic and thromboembolic events did not differ in the matched groups., Conclusions: Chylothorax is an uncommon complication after pediatric cardiac surgery and is associated with higher resource utilization. Chylothorax did not reoccur during reoperations and was not associated with higher mortality or long-term complications in a propensity matched analysis., Competing Interests: Conflicts of Interest: The authors have no conflicts of interest to declare.

Introduction: The aim of this study was to investigate the role of the insulin and glucose content of the maintenance fluid in influencing the outcomes of pediatric patients undergoing heart surgery., Methods: A total of 2063 consecutive pediatric patients undergoing cardiac surgery were screened between 2003 and 2008. A dextrose and an insulin propensity-matched group were constructed. In the dextrose model, 5% and 10% dextrose maintenance infusions were compared below 20 kg of weight., Results: A total of 171 and 298 pairs of patients were matched in the insulin and glucose model, respectively. Mortality was lower in the insulin group (12.9% vs. 7%, p = 0.049). The insulin group had longer intensive care unit (ICU) stay [days, 10.9 (5.8-18.4) vs. 13.7 (8.2-21), p = 0.003], hospital stay [days, 19.8 (13.6-26.6) vs. 22.7 (17.6-29.7), p < 0.01], duration of mechanical ventilation [hours, 67 (19-140) vs. 107 (45-176), p = 0.006], and the incidence of severe infections (18.1% vs. 28.7%, p = 0.01) and dialysis (11.7% vs. 24%, p = 0.001) was higher. In the dextrose model, the incidence of pulmonary complications (13.09% vs. 22.5%, p < 0.01), low cardiac output (17.11% vs. 30.9%, p < 0.01), and severe infections (10.07% vs. 20.5%, p < 0.01) was higher, and the duration of the hospital stay [days, 16.4 (13.1-21.6) vs. 18.1 (13.8-24.6), p < 0.01] was longer in the 10% dextrose group., Conclusions: Insulin treatment appeared to decrease mortality, and lower glucose content was associated with lower occurrence of adverse events.

Objectives: The incidence of congenital heart disease is ~50%, mostly related to endocardial cushion defects. The aim of our study was to investigate the postoperative complications that occur after paediatric cardiac surgery., Methods: Our perioperative data were analysed in paediatric patients with Down syndrome undergoing cardiac surgery. We retrospectively analysed the data from 2063 consecutive paediatric patients between January 2003 and December 2008. After excluding the patients who died or had missing data, the analysed database (before propensity matching) contained 129 Down patients and 1667 non-Down patients. After propensity matching, the study population comprised 222 patients and 111 patients had Down syndrome., Results: Before propensity matching, the occurrences of low output syndrome (21.2 vs 32.6%, P = 0.003), pulmonary complication (14 vs 28.7%, P < 0.001) and severe infection (11.9 vs 22.5%, P = 0.001) were higher in the Down group. Down patients were more likely to have prolonged mechanical ventilation [median (interquartile range) 22 (9-72) h vs 49 (24-117) h, P = 0.007]. The total intensive care unit length of stay [6.9 (4.2-12.4) days vs 8.3 (5.3-13.2) days, P = 0.04] and the total hospital length of stay [17.3 (13.3-23.2) days vs 18.3 (15.1-23.6) days, P = 0.05] of the Down patients were also longer. Mortality was similar in the two groups before (3.58 vs 3.88%, P = 0.86) and after (5.4 vs 4.5%, P = 1.00) propensity matching. After propensity matching, there was no difference in the occurrence of adverse events., Conclusions: After propensity matching Down syndrome was not associated with increased mortality or complication rate following congenital cardiac surgery.

Unlabelled: The leading interventions due to congenital heart defects performed in adults are: (I) reconstructive operations, including: (a) newly diagnosed malformations, (b) previously adjudged to be inoperable defects, (c) so called "tardive" interventions due to pulmonary hypertension or right ventricle insufficiency. There is a growing number of (II) REDO operations, including: (a) correction of residual defects, (b) replacement of damaged or outgrown homografts, (c) recoarctation (aneurysm, dissection) of the aorta after surgical or catheter interventions, (d) Ross procedure, valve replacements due to previously performed aortic valvulotomy/valvuloplasty or corrections of different malformations (e.g. TGA)., Patients and Results: 166 of all 4496 operations were performed in adolescents and adults (age: 16-52 years, mean: 28 years) between years 2001-2008. The distribution of these interventions: Ia: 77, Ib: 15, Ic: 4, IIa: 11, IIb: 22, IIc: 9, IId: 28. Mortality rates were the following: 0 intraoperative, 4 early postoperative, 1 pulmonary hypertensive crisis, 1 malignant rhythm disturbances, 2 multi-organ failure., Conclusions: The main risk factors are pulmonary hypertension and right ventricle failure. Complex surgical solutions do not mean extreme problems for a congenital cardiac surgeon, but earlier diagnosis and the use of ECMO during therapy may improve the results.

Unlabelled: Nowadays, the complex congenital heart diseases with a functional single ventricular haemodynamics can be treated with good results with bi-directional Glenn (BDG) and total cavopulmonary connection (TCPC) procedure. The late results are determined by the ventricular function., Aims: To assess the surgical results of "high risk" BDG and TCPC procedures, where the functional single ventricle were complicated by left sided SVS and IVC, total anomalous pulmonary venous connection (TAPVC) and severe atrioventricular valve insufficiency. These patients' were refused for surgery earlier., Patients: Between 01. 01. 1994. and 30. 06. 2004. 112 BDG and 93 TCPC (42 pts with extracardiac conduit--ECC) procedures were performed. AGE: 3 months-29 yrs mean: 4,7 yrs, weight: 4-46 kg, mean: 15,5 kg. Among the "high risk" pts.: 1. Bilateral BDG in 19, left BDG in 9, left sided TCPC with ECC in 3 pts. were performed, 2. The associated TAPVC were operated on successfully in 3 pts. (bilateral BDG in 1, left BDG in 1, left sided TCPC with ECC in 1), 3. Mitral valvuloplasty in 2, artificial (mitral) valve implantation in 1, tricuspid valve closure or valvuloplasty in 3, (tricuspid) valve replacement in 1, common AV valve replacement in 1 pts were performed, 4. In 4 infants BDG was performed after Norwood I. procedure., Results: Five pts. were lost after BDG procedure (Damous-Kaye-Stansen operation were performed as well in 3 pts.), and another 5 patients' systemic pulmonary shunts were re-done. Four patients after TCPC were lost and reoperation to BDG (n = 1) or shunt procedure (n = 2) was performed in 3 pts. Pre-discharge echocardiography confirmed mitral valvuloplasty with good results. No intervention was necessary in the follow up (1-3 yrs period) due to AV insufficiency and ventricular functions improved., Conclusion: On the basis of our series the "high risk" BDG and TCPC operations can be performed with an encouraging early result. The authors previously contraindicated associated defects can be surgically managed. Our short and mid-term results are feasible in this aspect. The long-term control of the "uni-ventricular" heart's ventricular function needs further investigation.

Objective: Low-risk cardiac surgery approaches zero morbidity/mortality, therefore, cosmetic issues append. Right thoracotomy has been advocated as cosmetically attractive alternative to median sternotomy. Posterolateral thoracotomy involves chest wall musculature division that contributes to postoperative pain/morbidity; access to the heart may be difficult. Longitudinal subaxillary incision and muscle-sparing thoracotomy can overcome these disadvantages and provide better visibility., Patients and Methods: Between April 2000 and April 2004 the authors performed open-heart repair from right thoracotomy in 161 patients. Diagnoses comprised perioval and sinus venosus ASDs (137), VSD (11), incomplete/intermediate AVD (8), miscellaneae (5). According to both patients' and surgeon's preference a longitudinal midaxillary incision and muscle-sparing lateral thoracotomy was utilised in 98 cases. Intracardiac repair was performed by extracorporal circulation, usual cannnulation, cardioplegia. Data of 79 ASD closures via midline sternotomy were used as controls., Results: Sternotomy and thoracotomy groups did not differ in demographic characteristics albeit thoracotomies exhibited wider age and weight range. The skin incision could be easily stretched up by 60% providing excellent visibility/access. No mortality occurred. Phrenic nerve palsies (2), transient tetraparesis (1) fully recovered. A mitral valve replacement was necessary for valve pathology unrelated to access. No breast and/or upper limb vascular/neurologic problems were encountered., Conclusions: Right subaxillary muscle-sparing thoracotomy offers safe option for open-heart repair of selected anomalies. Despite the shorter skin incision subaxillary approach provides better visibility than conventional thoracotomy as mediastinal organs are closer to the operator. Intermediate cosmetic result are appealing. With no muscles divided less postoperative pain is anticipated. The authors recommend to expand the spectrum of anomalies repaired via this approach.

Introduction: Owing to excellent survival following primary repair over 80% of congenital cardiac patients reach adulthood, half of them requiring continuous specialist care and one-third needing further reoperation. The ample variety and complexity of lesions warrant individualised treatment strategy., Objective: This study focuses on grown-up congential heart (GUCH) programme in the settings of a tertiary pediatric cardiac centre., Methods: Patients underwent corrective surgical procedures in pediatric facilities (theatre, ICU, wards) with a close involvement of adult cardiology/anaesthetic team. Patients were divided into simple/complex groups., Results: Simple group of comprised patients (n = 20) having ASD-II (18/20) sinus venosus ASD (2/20) repair without morbidity/mortality from right subaxillary thoracotomy in 17/20. Complex group (n = 20): corrective surgery for tetralogy of Fallot (6), LVOT-aortic valve repairs (4), allograft conduit exchange (3), TCPC (3), miscellaneous procedures (4) were performed as reoperations in 16/20 at 16.1 +/- 8.1 years following previous operations (median: 1.96, range 1-4). Postoperative right ventricle- (1), acute renal (1) failure and ARDS (1) fully recovered. One patient was lost for multi-organ-failure due to low cardiac output syndrome caused by chronic RV failure. Preoperative cyanosis was a risk factor for postoperative complications (p = 0.01). All survivors are symptom-free. No significant difference in ITU stay/LOS was observed between simple and complex groups., Conclusions: This study represents the initial experience of authors with GUCH. The number of GUCH patients is expected to rise with an upgrade shift in surgical complexity and severity requiring a multidisciplinary approach. It is advocated that complex GUCH cases should be performed by teams experienced in congenital reconstructive surgery. Patients following complex GUCH procedures have a comparable hospital course to patients undergoing simple cardiac operations.

Unlabelled: Nowadays, due to the development of cardiac surgery, pediatric cardiology and anesthesia, almost every congenital heart disease can be corrected totally or partially. The increasing number of surgical corrections will lead to better life quality. The surgical mortality has decreased significantly, even in the most complex cases. Only few cases can not be treated surgically. The aim of this study was to examine these positive changes, what they mean in the treatment of premature babies with congenital heart disease. These patients are endangered because of their age as well, they require special treatment and the surgical treatment has always meant high risk, urgent interventions., Patients: between 01. 01. 1975. and 31. 12. 2003. 447 premature babies were operated on. The patients were divided into 3 subgroups by their weight: I: 470-1500 gr. - 19 patients, II: 1500-2000 gr. - 93 pts., IIl: > 2000 gr. - 335 pts. There were 69 corrective surgical procedures performed mainly after 1998., Results: Overall early mortality: 81 patients (18.1%). Detailed mortality: group I.: 0%, group 11: 23 (24.7%), group III.: 58 (17.3%). The early mortality reduced from 21.6% to 6.3% (closed procedure), and 27.7% to 10.8% (ECC operation). The successful operations in the smallest weight groups were as follows: On ECC: Total Anomalous Pulmonary Venous Return (1600 g), Transposition of the Great Arteries--Arterial Switch (1800 g), Ventricular Septal Defect (1800 g), Aortopulmonary fenestration (2000 g), Hypoplastic Left Heart Syndrome-Norwood procedure (2200), AV septal defect (2300 g), Interrupted aortic arch + Ventricular Septal Defect (2300 g), Truncus arteriosus (2500 g). Without ECC: Coarctation of the aorta (930 g), Patent Ductus Arteriosus (470 g)., Conclusion: Nowadays the possibilities and the chances of the corrective procedures of congenital heart diseases in those patients with bodyweight of over 2000 g and in those that are mature babies are the same. In those patients with bodyweight 1500-2000 g procedures without ECC had good results, on-pump procedures had higher mortality rate, but the long-term results were acceptable. At the moment only procedures without ECC are performed on babies with weight under 1500 g--but with higher risk. Our effort is to perform ECC operations in patients with bodyweight less than 1500 g.

Unlabelled: A 36 year old male with tetralogy of Fallot and pulmonary atresia was corrected successfully. Previous palliative operation (Cooley shunt) was performed 35 years before. He was in NYHA functional class III. because of chronic hypoxia, polyglobulia, cerebral accident, pulmonary complications, myocardial (ventricular) dysfunction and syncopes. After an uneventful surgical reconstruction, the postoperative period was complicated with haemostasis complication and pulmonary distress syndrome. He left the hospital in NYHA functional class I on the 20th postoperative day in very good condition., Conclusion: Complete cardiac recovery will be possible after successful surgically reconstruction of tetralogy of Fallot and pulmonary atresia even in adulthood. The reconsideration of operability of such cases is recommended. As the patient may have different extracardiac complications in every case new complete reconsideration is necessary for the indication of surgical reconstruction.

Aim: This article presents the early and late surgical results of 401 newborns and infants among the 569 children with coarctation of aorta who were operated on between 1975-2001., Results: The early results were dependent on the anatomy of the aortic arch, the age and weight of babies and the types of the associated heart defects. The mortality rate was reduced from 15% (isolated coarctation 7.3%, complex coarctation 34%) to 3.0% (isolated 1.9%, complex 4.9%). 77.3% of 320 infants (follow-up 1 month-26 years, mean: 17 years) were free from re-operation or intervention. The (extended) end-to-end anastomosis and the subclavian flap method produced the best surgical results., Conclusions: They suggest the extended end to end anastomosis technique for repair of the aortic arch together with the reconstruction of the associated heart defects in the youngest age if it is possible. The balloon angioplasty of the recoarctation of the aorta produces a good result.

Aim: To analyze the causes and role of right ventricle failure in the morbidity and mortality after arterial switch operation for transposition of the great arteries in neonates., Method: Between January 1999 and December 2001, 62 neonates underwent arterial switch operation. The simple transposition group was comprised of 39 patients with transposition of the great arteries and intact ventricular septum. The complex transposition group included 23 patients with large ventricular septal defects, accompanied with left ventricle outflow tract obstruction in 6 cases and dextrocardia in 1 case. Arterial switch operation was performed on elective basis in all but 3 patients who underwent emergency operation., Results: Patients with complex heart defects had significantly lower body weight (p = 0.008) than patients with simple trasposition of great arteries. The usual coronary artery pattern (ie, the left anterior descending artery and circumflex artery arising from the right aortic sinus; the right coronary artery arising from the left aortic sinus) was found in 74% of the neonates in the simple transposition group and 65% of the neonates in the complex transposition group. Age, weight, coronary artery anatomy, cardiopulmonary bypass, duration of aortic cross-clamp, bleeding, and the need for delayed chest closure did not influence the outcome of surgery. Low cardiac output after surgery was more common in the complex transposition group (p = 0.0001), although it was not a predictor of fatal outcome. Preoperative hypoxia coupled with acidosis (odds ratio (OR), 5.70; 95% confidence intervals (CI), 4.45-7.44), and emergency operations (OR, 3.62; 95% CI, 2.22-5.59) were strong predictors of unfavourable outcome. We lost 4 patients out of 62 (6.5%) because of right ventricle failure caused by persistent pulmonary hypertension. Right ventricle failure on the second postoperative day, e.g., sustained increased central venous pressure > 15 mm Hg (p < 0.001) and high velocity tricuspid regurgitation > 4 m/s (p = 0.002), indicated bad prognosis., Conclusion: Difficult coronary anatomy was not a risk factor for morbidity and mortality after arterial switch operation. Poor preoperative health condition, hypoxia (despite effective balloon atrioseptostomy), and acidosis contributed to persistent pulmonary hypertension. Operation on the emergency basis and tricuspid valve insufficiency with right ventricle failure were strong predictors of unfavorable outcome.

Aim: To present our experience with modified cannulation with continuous, moderately hypothermic systemic perfusion in extensive aortic arch repair. The technique has fewer complications and preserves cerebral blood flow autoregulation., Method: Nine neonates, 6 with the hypoplastic left heart syndrome and 3 with the interrupted aortic arch with ventricular septal defect, were surgically treated with this technique between June and December 2001. Before extracorporeal circulation, 3.5-mm polytetrafluoroethylene tube was sutured onto the innominate artery and the arterial perfusion cannula inserted into the tube. Aortic arch repair was then performed with extracorporeal circulation. Right radial artery and femoral artery pressures were continuously monitored. Perfusion flows were built up gradually, with strict attention to the upper body (right radial artery) pressures not to exceed normal values. Procedures were carried out at moderate hypothermia (>28 degrees C), preferably with the beating heart., Results: No morbidity or mortality attributable to continuous perfusion occurred. Mean+/-SD extracorporeal circulation duration was 114+/-26 min. Maximum perfusion rate (actual/required flow for body surface area) was 1.65 at normal perfusion pressures. Right radial artery pressure at full flow (2.2 L/m2/min) was 56.1+/-6.7 mm Hg, whereas femoral artery pressure was 34.2+/-8.2 mm Hg. Decrease in right radial-to-femoral artery pressure was 21.9+/-5.6 mm Hg. The lowest nasopharyngeal temperature was 28.5 degrees C. There were no neurologic complications., Conclusion: Continuous, moderately hypothermic systemic perfusion via collaterals seems to be a method of choice in aortic arch repair in neonates. As there is no need for deep hypothermic total circulatory arrest, its numerous sequelae, such as increased postoperative bleeding and permanent neurologic deficit, can be avoided.

Introduction: For infants and children with congenital aortic valve disease root replacement with pulmonary allograft (Ross procedure) is the preferred method of choice., Patients/results: The authors have successfully applied this operation in 12 children (age range from 2.5 to 17 years--mean 9 years, body weight from 12 to 58 kg--mean 46 kg), one of whom has also required a Konno extension for long segment left ventricular outflow tract obstruction. The operation was complicated by early postoperative endocarditis in one case, and the child required redo homograft root replacement on the ninth postoperative day. All patients, including this one survived, and are doing well at present., Conclusions: In the Hungarian literature this is the first report on the Ross and Konno procedure in children. On the basis of our excellent early results, Ross procedure is the method of choice in aortic valve disease in children.

Truncus arteriosus communis (TAC) is a complex heart disease, it presents 1% of the congenital heart defects. A 7 weeks old infant with cardiac failure was operated on with TAC. The bicuspidized (14 mm diameter) pulmonary homograft valved conduit was used to reconstruct the right ventricular outflow tract (RVOT). The sternum closure was delayed. The important points of the indications for the surgical management of TAC: 1. Management or prevention of the pulmonary hypertensive crisis. 2. Patient's age. 3. Reconstruction of the RVOT (choices: homograft, dacron valve conduit, autologous pericardial valve conduit, porcine aortic root, direct anastomosis of the pulmonary trunc to the right ventricle). The surgical management is based on the optimal combination of these three important points. This is the first successfully performed primary surgical repair of an infant with TAC using modified pulmonary homograft in Hungary.

At 28th week of gestation a conotruncal malformation with ventricular septal defect was diagnosed by fetal echocardiography. Postnatal echocardiographic and angiocardiographic examinations confirmed the diagnosis of conotruncal malformation (pulmonary atresia, ventricular septal defect, patent ductus arteriosus, aortopulmonary collateral arteries). The unifocalization (age: 11 months) and total correction with aortic homograft (age: 7 years) were performed. To our knowledge our case is the first whose intrauterine diagnosis of complex congenital heart disease was confirmed after delivery and had successful two-stage surgical management.