[Perioperative use of levosimendan in cardiac surgery. Hungarian recommendation]. / A levoszimendán perioperatív alkalmazása a szívsebészetben. Magyar ajánlás.

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 (IK,ATP) opener agent. Due to these effects, it improves myocardium performance, does not influence adversely the balance between O2 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.

Decline of FOXN1 gene expression in human thymus correlates with age: possible epigenetic regulation.

BACKGROUND: Thymic involution is thought to be an important factor of age related immunodeficiency. Understanding the molecular mechanisms of human thymic senescence may lead to the discovery of novel therapeutic approaches aimed at the reestablishment of central and peripheral T cell repertoire. RESULTS: As an initial approach, here we report that the decline of human thymic FOXN1 transcription correlates with age, while other genes, DLL1, DLL4 and WNT4, essential for thymopoiesis, are constitutively transcribed. Using a human thymic epithelial cell line (hTEC), we show that FOXN1 expression is refractory to signals that induce FOXN1 transcription in primary 3D culture conditions and by stimulation of the canonical WNT signaling pathway. Blockage of FOXN1 induceability in the hTEC line may be mediated by an epigenetic mechanism, the CpG methylation of the FOXN1 gene. CONCLUSION: We showed a suppression of FOXN1 transcription both in cultured human thymic epithelial cells and in the aging thymus. We hypothesize that the underlying mechanism may be associated with changes of the DNA methylation state of the FOXN1 gene.

Striving for physiologic coaptation: An experimental and innovative approach towards designing and implanting aortic neo-leaflets.

Aortic valve repair has emerged as the treatment of choice for congenital aortic valvular disease, avoiding the need for a reoperation associated with stented prosthesis overgrowth. The introduction of a leaflet implant represents a recent advancement in a field that originated early techniques, such as simple commissurotomies. In our experimental approach, we assessed two established leaflet-sizing techniques by analysing their resultant coaptation areas. Although both techniques produced competent valves, the large coaptation areas differed significantly from the native aortic valve. This observation prompted us to revisit the functional anatomy of the aortic valve, our goal being to refine leaflet design and implantation for enhanced efficacy and longevity in neo-leaflet procedures. We designed a novel aortic valvar neo-leaflet, utilizing porcine pericardium as our primary source material, and we implanted four tri-leaflet valves in four porcine hearts. All tri-leaflet valves were competent and closely resembled the coaptation area of the native aortic valve. This study serves as a pilot for further experimental aortic valve repair surgery using neo-leaflet implants.

Aortic atresia combined with right aortic arch.

We present a case of an aortic atresia and a persistent right aortic arch with a retro-aortic brachiocephalic vein. A left neo-aortic arch was constructed using a reversed subclavian flap and a standard Norwood-type patch enlargement to provide systemic outflow. The intercarotid section of the aortic arch was hypoplastic. Therefore, we maintained the patency of the right arch, thus creating a vascular ring.

Pulmonary stenosis is a predictor of unfavorable outcome after surgery for supravalvular aortic stenosis.

We sought to evaluate whether the presence of pulmonary stenosis (PS), amongst other factors, influences the mortality and the rate of reoperations in the long-term follow-up of patients with supravalvular aortic stenosis (SVAS). We identified all patients with SVAS from our surgical database. The patients with multi-level aortic stenosis or concomitant cardiac procedures were excluded from this study. Follow-up (100 %) was conducted between 2008 and 2010. Twenty-six patients underwent surgery for SVAS between 1974 and 2006. Seventeen patients (65 %) were diagnosed with Williams-Beuren-Syndrome, six (17 %) had a diffuse form of SVAS and 10 (39 %) had PS. No patient had a surgical or interventional procedure for PS at the initial operation or during follow-up. There was no statistically significant association between PS and WBS (p = 0.30) or diffuse form of SVAS (p = 0.13). Patients with PS were operated at younger age (p = 0.028). Median follow-up time was 14.6 years. Overall mortality was 11.5 %. One patient with preoperatively severely decreased LV-function died 27 days postoperatively. Two late deaths occurred 7 and 10 years after the initial operation. Reoperations were required in 4 patients (15 %), 4-19 years after the original operation, due to aortic arch stenosis, supravalvular restenosis or poststenotic aortic dilatation. PS was found to be a risk factor for reoperation (p = 0.005) and for the combined reoperation/death end-point (p = 0.003). PS in patients with SVAS is a risk factor for reoperations in the aortic region and might be considered an indicator of the severity of the arterial disease and a predictor of an unfavourable outcome.

Initial Clinical Trial of a Novel Pulmonary Valved Conduit.

Valved allografts and xenografts for reconstruction of the right ventricular outflow tract (RVOT) lack durability and do not grow. We report the first clinical use of a completely bioabsorbable valved conduit (Xeltis pulmonary valve - XPV) in children. Twelve children (six male), median age five (two to twelve) years and median weight 17 (10 to 43) kg, underwent RVOT reconstruction with the XPV. Diagnoses were: pulmonary atresia with ventricular septal defect (VSD) (n = 4), tetralogy of Fallot (n = 4), common arterial trunk (n = 3), and transposition of the great arteries with VSD and pulmonary stenosis (n = 1). All had had previous surgery, including prior RVOT conduit implantation in six. Two diameters of conduit 16mm (n = 5) and 18mm (n = 7) were used. At 24 months none of the patients has required surgical re-intervention, 9 of the 12 are in NYHA functional class I and three patients in NYHA class II. None of the conduits has shown evidence of progressive stenosis, dilation or aneurysm formation. Residual peak gradient of >40 mm Hg was observed in three patients, caused by kinking of the conduit at implantation in 1 and distal stenosis in the peripheral pulmonary arteries in 2 patients. Five patients developed severe pulmonary valve insufficiency (PI); the most common mechanism was prolapse of at least one of the valve leaflets. The XPV conduit is a promising innovation for RVOT reconstruction. Progressive PI requires however an improved design (geometry, thickness) of the valve leaflets.

A Novel Restorative Pulmonary Valve Conduit: Early Outcomes of Two Clinical Trials.

Objectives: We report the first use of a biorestorative valved conduit (Xeltis pulmonary valve-XPV) in children. Based on early follow-up data the valve design was modified; we report on the comparative performance of the two designs at 12 months post-implantation. Methods: Twelve children (six male) median age 5 (2 to 12) years and weight 17 (10 to 43) kg, had implantation of the first XPV valve design (XPV-1, group 1; 16 mm (n = 5), and 18 mm (n = 7). All had had previous surgery. Based on XPV performance at 12 months, the leaflet design was modified and an additional six children (five male) with complex malformations, median age 5 (3 to 9) years, and weight 21 (14 to 29) kg underwent implantation of the new XPV (XPV-2, group 2; 18 mm in all). For both subgroups, the 12 month clinical and echocardiographic outcomes were compared. Results: All patients in both groups have completed 12 months of follow-up. All are in NYHA functional class I. Seventeen of the 18 conduits have shown no evidence of progressive stenosis, dilation or aneurysm formation. Residual gradients of >40 mm Hg were observed in three patients in group 1 due to kinking of the conduit (n = 1), and peripheral stenosis of the branch pulmonary arteries (n = 2). In group 2, one patient developed rapidly progressive stenosis of the proximal conduit anastomosis, requiring conduit replacement. Five patients in group 1 developed severe pulmonary valve regurgitation (PI) due to prolapse of valve leaflet. In contrast, only one patient in group 2 developed more than mild PI at 12 months, which was not related to leaflet prolapse. Conclusions: The XPV, a biorestorative valved conduit, demonstrated promising early clinical outcomes in humans with 17 of 18 patients being free of reintervention at 1 year. Early onset PI seen in the XPV-1 version seems to have been corrected in the XPV-2, which has led to the approval of an FDA clinical trial. Clinical Trial Registration: www.ClinicalTrials.gov, identifier: NCT02700100 and NCT03022708.

[Surgical management of congenital heart defects in adolescent and adult patients, between years 2001-2008]. / Tapasztalataink a fiatal- és felnottkori veleszületett szívhibák sebészi kezelésében 2001-2008.

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.

[Structure of the thymus at the beginning of the 21th century]. / A thymus szerkezete a huszonegyedik század elején.

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.

Incidence and results of reoperations following the Senning operation: 27 years of follow-up in 314 patients at a single center.

OBJECTIVES: Atrial switch procedures for transposition of the great arteries are associated with reoperations mainly for systemic ventricular dysfunction and baffle complications. This study aims at identifying the results of reoperations following the Senning operation. METHODS: Records of 314 hospital survivors who had undergone the Senning operation were reviewed for details concerning cardiac reoperations. RESULTS: Reoperations were required in 32 patients (systemic ventricular failure n=12, baffle complications n=11, left ventricular outflow tract obstruction n=7, aortic coarctation n=2) during a mean follow-up time of 18.2+/-5.7 years. Freedom from reoperation and survival at 25 years was 88.0+/-2.1% and 90.9+/-2.3%, respectively. Among patients who underwent reoperation for systemic ventricular failure (arterial switch and Senning take-down without prior pulmonary artery banding n=2, with prior banding n=3, banding without conversion n=4, tricuspid valve repair n=3), two patients died at the time of arterial switch and Senning take-down, and two patients died 4 and 16 months after tricuspid valve repair, respectively. Mean follow-up time after the first reoperation was 7.7+/-5.9 years. Survival after reoperation for systemic ventricular failure at 30 days, 1 year, and 10 years, was 91.7+/-8.0%, 83.3+/-10.8%, and 64.8+/-14.3%, respectively. Survival after reoperation for baffle complications and left ventricular outflow tract obstruction at 10 years was 85.7+/-13.2% and 83.3+/-15.2%, respectively. CONCLUSIONS: Reoperations following the Senning operation are rare. Reoperations for baffle complications or left ventricular outflow tract obstruction can be performed with good results in the mid-term. However, reoperations for systemic ventricular failure are demanding, and are associated with a high operative and mid-term mortality.

Donor heart selection and outcomes: An analysis of over 2,000 cases.

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.

Chylothorax after pediatric cardiac surgery complicates short-term but not long-term outcomes-a propensity matched analysis.

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.

Surgical therapy of an aberrant origin of the left anterior descending coronary artery from the right sinus of valsalva: a case report.

Anomalous aortic origin of a coronary artery from the incorrect coronary sinus of Valsalva is a rare congenital cardiac defect that is associated with an increased risk of sudden death. In most of these defects, the anomalous coronary artery takes an intramural course between the great arteries and leaves the aortic wall from the appropriate coronary sinus of Valsalva. Many times this anomalous artery shares a common orifice with the other coronary artery. We report on a previously healthy 15-year-old boy who presented with signs of an anterolateral myocardial infarction after physical activity (soccer game). Transthoracic echocardiography revealed an aberrant origin of the left anterior descending coronary artery from the right sinus of Valsalva. Repair was accomplished by unroofing the intramural segment. Because the intramural segment was below the commissure, detachment of the intercoronary commissure was not necessary. To conclude, transthoracic echocardiography can accurately depict this rare anomaly, and in symptomatic patients surgical therapy is indicated.

[Challenges in the surgical management of hearts with a functional single ventricle]. / Kihívások az egykamrás komplex veleszületett szívhibák sebészi kezelésében.

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.

[Right subaxillary and posterolateral thoracotomy for open repair of congenital heart defects]. / Jobb oldali subaxillaris és posterolateralis thoracotomia alkalmazása veleszületett szívhibák nyitott szívmutétei során.

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.

[Surgical management of preterm infants and low birth weight neonates with congenital heart disease]. / Veleszületett szívhibák sebészete koraszülötteken es kis súlyú ujszülötteken.

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.

[Adult congenital heart program in a tertiary care facility of pediatric cardiology]. / Felnottkori congenitalis szívsebészet gyermekkardiológiai és szívsebészeti központban.

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.

[Successful surgical correction of tetralogy of Fallot and pulmonary atresia in adulthood after reconsidering inoperability]. / A felnottkori Fallot-tetralogia és pulmonalis atresia sikeres sebészi rekonstrukciója korábbi inoperábilitás úijraértékelése után.

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.

[New strategies in surgical management of coarctation of the aorta 1975-2001]. / Szemléletváltozás a coarctatio aortae sebészi kezelésében 1975-2001.

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.

[Aortic root replacement with pulmonary allograft (Ross procedure) in children. Early results]. / Veleszületett aortabillentyú-betegség kezelése aortagyökpótlással pulmonalis allografttal (Ross-mútét) gyermekkorban. Korai eredmények.

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.