[Heart transplantation after corrective surgery of a congenital heart disease. Surgical technical problems]. / Sikeres szívátültetés veleszületett szívhiba korrekciós szívmutéte után. Sebésztechnikai problémák.

Heart transplantation was performed in a 17-year old boy with severe left ventricular failure. Previously a congenital heart disease, tetralogy of Fallot was reconstructed in one and half years old of age. Authors present the different surgical problems complicated this procedure. In Hungary this is the first heart transplantation after a corrective surgical procedure of a complex congenital heart disease.

[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.

[Surgical management with mitral valve replacement in young infants with congenital mitral valve diseases and complete atrioventricular septal defect]. / Veleszületett mitralisbillentyu-betegség és komplett atrioventricularis septumdefektus sebészi kezelése mubillentyupótlással csecsemokorban.

OBJECTIVES: The mechanical valve replacement may be the only option if the failing mitral valve cannot be repaired in complete AV septal defect (CAVSD), or congenital mitral valve stenosis and regurgitation in infants. In young infants the small mitral annulus-prosthesis mismatch is a big problem. AIM: To assess the possibility of the left AV orifice repair and the necessity of mechanical valve replacement in CAVSD in young infants. METHODS: Single center, retrospective study of 82 infants (13 pts under 3 months) who underwent complete repair of CAVSD between 2001 and 2007. Mechanical (bileaflet) valve replacement was required in 7 pts (5 weeks-7 months, 3.5-5 kg). The time interval between the two operations was 0-7 days, but the smallest baby spent 38 days in the intensive care unit waiting for increasing of his mitral annulus size from 11 to 15 mm. (Types of implanted valves: 2 Carbomedics 16 mm, 3 ATS 16 mm, 1 Sorin 17 mm, 1 Sorin 19 mm.) ATS 16 mm valves were implanted in 2 infants each with congenital mitral valve stenosis and regurgitation. RESULTS: Early mortality (30 days) was 0, but 2 pts died in sepsis on the 46th and 71st postoperative day, respectively. In the follow-up period of 1-6 (mean 3) years 1 child (18 months later) needed reoperation (pannus removal), now all pts are doing well. Anticoagulation therapy was difficult in some cases without complication. CONCLUSIONS: The surgical repair of congenital mitral valve diseases and CAVSD can be performed successfully in very young infants. If the anatomic characteristics of the mitral valve is not suitable for repair, only mechanical mitral valve replacement can be performed successfully even in sometimes hopeless situation (one of our pts of 3.5 kg weight, is the smallest baby mentioned in the literature). Our early and mid-time results are good, but the re-replacement will be an unavoidable problem in the future.

[Role of heart transplantation in pediatric heart surgery. The first successful pediatric heart transplantation in Hungary]. / A szívtranszplantáció helye a gyermekszívsebészetben. Az elsô sikeres gyermekszív-átültetés Magyarországon.

7-year-old boy, who underwent aortic valve replacement two years previously, suffered from idiopathic dilated cardiomyopathy. Because of poor condition (NYHA-IV), heart transplantation was performed on 18th October 2007. It was the first pediatric heart transplantation in Hungary. It was an uneventful early postoperative period, 6 months after the operation he is doing well, no biopsy-proven and tissue Doppler echocardiography (TDI-derived velocities measurement) rejection was detected. The immunosuppression was based on triple-drug therapy (tacrolimus+mycophenolate mofetil+corticosteroid) with use of induction therapy with interleukin-2 receptor blocker (basiliximab).

Aprotinin and renal dysfunction after pediatric cardiac surgery.

BACKGROUND: Aprotinin is a potent antifibrinolytic drug, which reduces postoperative bleeding and transfusion requirements. Recently, two observational studies reported increased incidence of renal dysfunction after aprotinin use in adults. Therefore, the aim of the study was to investigate the safety of aprotinin use in pediatric cardiac surgery patients. METHODS: Data were prospectively and consecutively collected from 657 pediatric patients undergoing cardiac surgery with cardiopulmonary bypass (CPB). The database was assessed with regard to a possible relationship between aprotinin administration and dialysis and between aprotinin and postoperative renal dysfunction [defined as 25% decrease in the creatinine clearance (Ccr) compared with the preoperative value] by propensity-score adjustment and multivariable methods. RESULTS: The incidence of dialysis (9.6% vs 4.1%; P = 0.005) and renal dysfunction (26.3% vs 16.1%; P = 0.019) was higher in patients who received aprotinin; however, propensity adjusted risk ratios were not significant [odds ratio (OR) of dialysis: 1.22; 95% confidence interval (CI) 0.46-3.22; OR of renal dysfunction 1.26; 95% CI: 0.66-1.92]. Aprotinin significantly reduced blood loss in the first postoperative 24 h. The main contributors of renal dysfunction were CPB duration, cumulative inotropic support, age, preoperative Ccr, amount of transfusion and pulmonary hypertension. CONCLUSIONS: Despite the higher incidences of renal dysfunction and failure in the aprotinin group, an independent role of the drug in the development of renal dysfunction or dialysis could not be demonstrated in pediatric cardiac patients undergoing CPB.

[Hybrid solution in aortic arch surgery]. / Hybrid megoldások az aortaív sebészetben.

Since the introduction of endografts, treatment of vascular diseases has remarkably changed. Due to less surgical trauma patients--those were not amenable to open surgery--now have the chance to be treated with remarkably lower risk. At certain segments of the aorta with life important side branches combination of open surgery is needed to get free segment for deployment of endografts. These "hybrid" interventions have opened new horizon at aortic arch surgery without use of cardiopulmonary bypass and deep hypothermia. In selected types of diseases by debranching of the aortic arch and transposition of the supra-aortic trunks we can achieve suitable landing zones to fix the endografts properly. In this paper we provide an overview of the possible solutions.

Evolution of left ventricular function in paediatric patients with permanent right ventricular pacing for isolated congenital heart block: a medium term follow-up.

AIMS: We aimed to assess the evolution of left ventricular (LV) systolic function in children with right ventricular apical (RVA) pacing for isolated congenital heart block (ICHB) and to identify possible predictors of LV function deterioration. Right ventricular apical pacing can be detrimental to LV function in a significant number of adults. Effects in children are still controversial. METHODS AND RESULTS: Left ventricular shortening fraction (LV SF) and QRS duration were retrospectively assessed in 45 children with RVA pacing for ICHB: before pacemaker (PM) implantation, immediately after and then regularly during a follow-up of 58.69 +/- 45.23 months. Patients were categorized as stable or deteriorators according to an arbitrarily chosen cut-off point of > or =7% decrease in LV SF. Lupus status was unknown. Overall LV SF did not change significantly (41.42% +/- 8.21 before pacing, 39.77% +/- 7.03 immediately after PM implant, 37.43% +/- 9.91 with chronic pacing, P = NS). Deteriorators (n = 13) had significantly higher baseline heart rate (57.5 +/- 8.7 vs. 46.9 +/- 10.5 bpm, P < 0.05) and baseline LV SF (46.17 +/- 8.13 vs. 38.4 +/- 6.4%; P < 0.05), a significantly higher proportion of them being implanted before 2 years of age: 8 of 13 (61.5%) vs. 5 of 25 (20%) in the stable group (P < 0.05). Deteriorators had a higher incidence of an initial epicardial lead and narrower native QRS. CONCLUSION: Permanent RVA pacing for ICHB does not necessarily affect LV function in children. The risk of deterioration of LV function seems to be higher in children with higher baseline heart rate and better baseline LV SF, especially with pacing at a younger age, a narrower native QRS and RVA epicardial pacing site.

[Anatomic correction of the congenitally corrected transposition of the great vessels by the first successful "double switch" operation in Hungary]. / Korrigált nagyér-transzpozíció anatómiai korrekciója ("double switch"). Elso sikeres hazai mutéti megoldás.

Congenitally corrected transposition of the great arteries is a rare defect characterized by discordant atrioventricular and ventriculoarterial connections. Symptoms result from one or a combination of associated cardiovascular malformations, including ventricular septal defect, pulmonary stenosis or atresia, tricuspid valve dysfunction, dextrocardia, hypoplastic left or right ventricle. Correcting exclusively the associated defects, leaving the morphologic right ventricle in systemic position, will determine the patient's life-long prognosis. Anatomic repair by double switch technique may improve survival of patients with congenitally corrected transposition of the great arteries by establishing the morphologic left ventricle in the systemic circulation. A 3-year-old girl with congenitally corrected transposition of the great arteries, ventricular septal defect, hypoplastic right ventricle, and previous palliative procedure was corrected by double switch technique and patch closure of ventricular septal defect. The authors prefer the double switch procedure inspite of its many surgical challenges because it has very good long-term outcome. The more simple surgery namely the operation of only associated cardiac defects will involve the possibility of deterioration of right ventricular function.

[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.

[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.