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We report on the case of a 13-year-old female presenting with dizziness and nausea related to high blood pressure. A complete medical evaluation revealed that the patient had middle-aortic syndrome (MAS), in which there was a severe stenosis of the abdominal aorta that affected her renal and visceral arteries. If left untreated, this syndrome may lead to serious complications such as renal insufficiency or congestive heart failure. Therefore, the decision was made to perform a thoracoabdominal surgery with surgical patch augmentation. The patient had successful postoperative recovery. We have reported this case to draw attention to the coarctation of abdominal aorta, a condition which should be considered in pediatric patients with hypertension, and to emphasize a successful treatment approach for MAS.

Deep vein thrombosis (DVT) is a common disease associated with high rates of mortality and significant morbidity. The diagnostic approach of DVT has evolved over the years. Algorithmical use of pretest probability, D-Dimer testing and ultrasonography allow safe and accurate investigation of DVT. The anticoagulation therapy, used to treat DVT, includes vitamin K antagonists (VKAs) and low-molecular-weight heparin (LMWH) or unfractionated heparin (UF). The duration of anticoagulation therapy depends on the cause of DVT and patient's clinical profile. Although these conventional therapies are effective, narrow therapeutic index, need for frequent monitoring and various food-drug interactions cause difficulties for patients. In recent decades, new oral anticoagulants have been developed. These drugs focus directly on inhibiting either Factor Xa (rivaroxaban, apixaban, edoxaban) or thrombin (dabigatran). In contrast to warfarin, these new agents have shorter half-life, fewer drug or food interactions, no necessity for a close monitoring and ease of administration. This review summarizes current knowledge about deep vein thrombosis and new treatment aspects with novel oral anticoagulants.

Coronary artery aneurysms are rare entities with a prevalence of 0.15%-4.9%. Giant coronary artery aneurysms are known as more than 2 to 5 cm in size. We present a case of 74 year-old female who was admitted to our clinic with chest pain and dyspnea. Coronary angiography demonstrated a giant right coronary artery (RCA) aneurysm with a significant left-to-right shunt. The patient underwent an open heart surgery. During the exploration, an aneurysm of 40 mm in diameter of the RCA was seen. The aneurysmatic RCA was excluded and continuously closed with the support of intra-aortic balloon pump (IABP). The patient was discharged on the 13th postoperative day without any complication.

Although bullets penetrating into the heart are usually known as fatal, retained cardiac bullets can have a silent course without causing any cardiovascular complications. An 89 year-old patient with myocardial infarction was admitted to our department hemodynamically stable and widely awake. His electrocardiogram didn't show any evidence of ischemia. Cardiac catheterization revealed a three-vessel coronary artery disease. In addition, a circular structure projected on the area of the marginal branch and the V. magna cordis. Removal of the bullet showed that three quarters of myocardium were involved without any perforation. The history of the patient revealed an injury under the heavy fire during World War II. Thus, this case demonstrates that bullets can remain asymptomatic within in the heart for decades. The patient was dis-charged home at the 10th postoperative day after having an uneventful clinical course.

Current techniques to resolve heart valve defects involve the use of prosthetic and bioprosthetic materials. These materials lack the potential to grow and are not ideal, especially not for pediatric patients. Novel techniques like tissue engineering involve the use of biodegradable polymers coated with autologous myofibroblast and endothelial cells. We inspected morphological and marker gene expression differences between cells harvested from the saphenous vein, or from veins and arteries of the umbilical cord, and the cells they are designed to replace: the interstitial and endothelial cells of the pulmonary heart valve. We assessed the extent to which the endothelial cells from the inspected sources in vitro resemble endothelial cells of human pulmonary heart valves, and we found that myofibroblast cells, respective of their source, in vitro differ from the interstitial cells from human pulmonary heart valves regarding collagen and smooth muscle alpha-actin. Therefore we conclude that the cells isolated from the saphenous veins, or from veins and arteries of the umbilical cord might be feasible cell sources for tissue engineering of heart valve for the pulmonary position.

Aim: The procedure of coronary bypass grafting (CABG) with coronary endarterectomy (CE) is controversial. However, in the setting of severely calcified coronary arteries CE may enable complete revascularization. Complete revascularization, especially of the left anterior descending artery (LAD), is important for long-term outcome. In this study we assessed long-term LAD graft patency and anterior wall function after CABG with CE of the LAD., Methods: Between 1984 and 1992, 283 patients underwent CABG with CE of the LAD. In 50 patients (47 men), aged 59+/-7.6 (40-77), clinical reassessment and surveillance angiography were performed. In all patients complete revascularization had been achieved with 3.5+/-1 (1-5) grafts/patient with 1-3 CE/patient. The LAD was grafted either with a saphenous vein segment (N=38) or with left intern thoracic artery (N=12). A graft obstructed less than 50% in diameter was defined as patent., Results: At follow-up 39 patients (78%) were in CCS class I/II and had improved significantly (P<0.000). Control angiography after 7.6+/-2.5 (3.5-11.7) years after CABG revealed a patent LAD graft in 30/50 patients (60%). Actuarial graft patency was 100%, 96%, and 56% after 2, 5, and 10 years and was lower in patients with diabetes (P=0.001). Deterioration of anterior wall motion was observed in 17 patients (34%) and was more frequent if anterior wall motion was preoperatively normal (P=0.002), irrespective of LAD graft patency., Conclusion: Clinical status and long-term graft patency of grafts on endarterectomized LAD is considerable. However, patients with preoperatively normal anterior wall function are at increased risk for myocardial damage in the long-term.

We report a rare case of a successful cardiac transplantation in a patient suffering from cardiomyopathy and complex mitochondrial disease. The patient presented with severe heart failure and malignant ventricular arrhythmias requiring implantation of a defibrillator and advanced medical treatment. The patient was listed for urgent heart transplantation and received a donor heart after 36 days. One yr post-operatively, the patient has completely recovered.

Chronic rejection is still the major limitation of long-term outcome of heart transplant recipients. Several recent studies demonstrated that a not negligible proportion of chronic allograft rejection episodes are not only mediated by T-cell response but also triggered by pre-transplant and de novo post-transplant donor-specific alloantibodies. This points at a fundamental role of humoral immune response mechanisms that contribute to early and late graft failure. This type of rejection is an unsolved problem solid organ transplantation because current immunosuppressive regimens are generally intended to interfere in T-cell signalling pathways. Various options for the removal of circulating alloantibodies and the prevention of alloantibody formation by B-cell depletion have been described. Nevertheless, effective standardized schemes for the treatment of antibody-mediated graft rejection have to be defined. We present a heart transplant recipient with sustained antibody-mediated graft rejection who was successfully managed with a combination treatment consisting of 3 cycles of immunoadsorption and a single-dose administration of the anti-CD20 monoclonal antibody rituximab.

Background: The aim of this study was to evaluate early and late outcomes after mechanical systemic heart valve replacement in pediatric patients., Methods: Between October 1981 and December 2003, 32 children (mean age 7.2 +/- 5.4 years; 4 months - 15.9 years) underwent mechanical mitral (MVR, n = 17), aortic (AVR, n = 13) or double valve replacement (DVR, n = 2) with St. Jude Medical valves. Twenty-two patients (69 %) had undergone previous cardiac surgery. Anticoagulation self-management was used since 1995., Results: The operative mortality was 3.1 %. Perioperative complications were complete heart block (n = 5), ventricular fibrillation (n = 1) and myocardial infarction (n = 1) and were exclusively related to patients with MVR. Mean calculated valve size ratio (geometric prosthesis orifice area/normal valve size area) was 1.72 (1.07 - 2.85) for AVR and 1.4 (0.88 - 3.12) for MVR. Mean follow-up was 9.1 +/- 6.6 years (range 0.4 - 23.2 years, cumulative 283 patient-years). There were two late deaths in patients with MVR. Actuarial survival after 10 years was 93.8 %. Late complications were endocarditis (n = 2), minor hemorrhagic event (n = 1), and stroke (n = 1). Anticoagulation self-management is well accepted by all patients/parents. Overall 10-year freedom from any anticoagulation-related adverse event with phenprocoumon was 89.1 % (1.2 %/patient year). Nine patients required reoperations: redo-MVR (outgrowth of prostheses (n = 3), pannus overgrowth (n = 2), closure of paravalvular leak after AVR (n = 2), partial aortic valve thrombosis (n = 1) and redo-DVR (n = 1 for endocarditis). Freedom from reoperation after 10 years was 80.9 %., Conclusions: Mechanical valve prostheses are a valuable option for left-sided heart valve replacement in pediatric patients. Perioperative morbidity was exclusively related to patients with MVR. Oversizing was often possible to avoid early reoperation for outgrowth. The operative mortality and long-term morbidity are acceptable. Anticoagulation self-management is safe and well accepted.

The number of grown-up patients with congenital heart disease (GUCH) is constantly increasing and will equal the number of children requiring surgery for congenital heart disease (CHD). Specialized centers dealing with the medical and paramedical problems of these patients are required. GUCH patients can be divided into the following groups: (1) patients with minor cardiac malformations presenting at adult age for first treatment; (2) patients presenting for correction as adults because they are either naturally balanced or were surgically palliated; (3) patients presenting for expected reoperations after correction in childhood; (4) patients requiring repair of residual defects after correction; (5) patients developing heart failure after correction or palliation of CHD requiring thoracic transplantation; and (6) patients developing acquired heart disease in addition to CHD. Special aspects of malformations frequently occurring in GUCH patients are discussed in detail. Acquired heart disease in this patient population is expected to increase in the coming decades as survivors of CHD grow old.

Heart transplantation represents a valuable therapeutic option for patients with congenital heart disease and end-stage heart failure. We report the case of a 15-year-old patient with situs inversus and additional complex congenital malformations of the heart who underwent several previous palliative operations. Orthotopic cardiac transplantation with several technical modifications was performed successfully. At 18 months post-operatively the patient is free from rejection and doing well in New York Heart Association (NYHA) Class I status.

Objective: Donor organ shortage in pediatric heart transplantation (HTx) is causing mortality rates of 30-50% on the waiting list. Due to immaturity of the immune system of newborns and infants, ABO-incompatible HTx may be an option to increase donor availability. We present our experience with ABO-incompatible HTx., Methods: Three infants were transplanted ABO-incompatible since 12/2004: (1) hypoplastic left heart complex, (2) restrictive hypertrophic cardiomyopathy, (3) dilative cardiomyopathy. Age at HTx was 7, 5, and 3.5 months. All recipients had blood type O, donors were A, A, and B. Informed consent was given by parents, the ethics committee, and Eurotransplant., Results: Preoperative isohemagglutinin titers were low (Patient 1: 1:4 for anti-A1, A2, B, Patient 2: 1:4, 1:1, 1:4 for anti-A1, A2, B, respectively, and Patient 3: 0 for all, but quick spin 1+ for all). Intraoperatively, plasma was separated from red blood cells and discarded up to six times until antibodies were eliminated. Immunosuppressive induction with ATG was started for 5 days. Basic immunosuppression consisted of tacrolimus, mycophenolate mofetil, and prednisone. Extubation was performed on days 15, 2, and 1, respectively. After a follow-up of 17, 16, and 12 months all patients are well, ventricular function is excellent without any acute rejection periods; Patient 1 is still on dialysis. Isohemagglutinin titers against donor blood type have disappeared in follow-up., Conclusions: ABO-incompatible cardiac transplantation shows good short-term results in young infants and seems to be a safe procedure to lower the mortality on the waiting list.

We report on 2 children, aged 3 and 4 years, with single ventricle physiology who underwent Fontan operation in the presence of a single right lung successfully with good midterm outcome. Therefore, the absence of one lung is not a contraindication for a Fontan palliation in selected patients with optimal hemodynamics.

Truncus arteriosus communis is a congenital heart malformation, which is usually repaired in the neonatal period or early infancy. Although results of repair are good, there is long-term morbidity caused by reoperations mainly owing to right ventricle to pulmonary artery conduit exchange or stenosis at the pulmonary artery bifurcation as recently reported for Contegra conduits. We present a new technique for complete autologous reconstruction of the aorta and the pulmonary bifurcation in truncus arteriosus type I and II.

In children, systemic heart valve replacement with bioprostheses is associated with accelerated valve degeneration, and mechanical prostheses require permanent anticoagulation. Novel "biomechanical" polymeric valve prostheses ("bio" = flexible, "mechanical" = synthetic), solely made of polycarbonate urethane (PCU), were tested in vitro and in a growing animal (calf) model with the aim of improved durability without permanent anticoagulation. The trileaflet aortic prosthesis has diminished pressure loss and reduced stress and strain peaks. The asymmetric bileaflet mitral valve mimics natural nonaxial inflow. The valves underwent long-term in vitro testing and in vivo testing in growing calves for 20 weeks [mitral (7), aortic (7)] with comparison to different commercial bioprostheses [mitral (7), aortic (2)]. In vitro durability of PCU valves was proved up to 20 years. Survival of PCU valves versus bioprostheses was 7 versus 2 mitral and 5 versus 0 aortic valves, respectively. Two animals with PCU aortic valves died of pannus overgrowth causing left ventricular outflow tract obstruction. Degeneration and calcification were mild (mitral) and moderate (aortic) in PCU valves but were severe in biological valves. There was no increased thrombogenicity of the PCU valves compared to bioprostheses. The novel polymeric valve prostheses revealed superior durability compared to current bioprostheses in growing animal model without permanent anticoagulation and thus, may be a future option for pediatric patients.

Unlabelled: We report the early and late outcome following left-sided mechanical heart valve replacement in children. Between 10/1981 and 02/2001, 27 children (13 male, mean age 7.2 +/- 5.2 years, range 0.53-15.7 years) underwent mechanical mitral (MVR 16), aortic (AVR 9) or double valve replacement (DVR 2) with St. Jude Medical valves. Eighteen children (66.7%) had undergone previous cardiac surgery. Valve disease was congenital in 23, due to endocarditis in 2 and rheumatic in 2 patients. Concomitant cardiac surgery was performed in 12 patients (44.4%). Operative mortality was 3.7% (1/27). Perioperative complications were complete heart block (5) and myocardial infarction (1). Mean follow-up was 6.5+/-5.9 years (range 0.4-19 years, total 169.9 patient-years). There was one valve-related late death due to mitral valve thrombosis without phenprocoumon. Actuarial survival after 1, 5 and 10 years was 93, 93 and 93%. Late complications included endocarditis (2), minor hemorrhagic event (1) and stroke (1). Overall 10-year freedom from any anticoagulation-related adverse event under phenprocoumon was 91% (1.3%/patient year). Eight patients required reoperations: re-MVR (5; outgrowth of the prostheses (3), pannus overgrowth (2)), closure of paravalvular leak after AVR (2), and re- DVR (1; endocarditis). Actuarial freedom from reoperation after 1, 5 and 10 years was 96, 88 and 76%., Conclusion: Mechanical valve prostheses are a valuable option for left-sided heart valve replacement in pediatric patients with good results. Operative mortality and the incidence of any valve-related events as endocarditis, reoperation, thromboembolism or anticoagulation related bleeding is acceptable.

Background: The development of hypertensive pulmonary vascular disease (HPVD) is considered a risk factor in the long-term course of patients with secundum atrial septal defects (ASD). The aim of this study was to assess the prevalence and histologic degree of HPVD and pulmonary hypertension in relation to preoperative clinical and hemodynamic data, intraoperative findings, and operative outcome in adults., Methods: Lung biopsies of 75 patients, mean age 44 +/- 14 years (18-71 years), with secundum ASD or sinus venosus defect including ten patients with partial anomalous pulmonary venous return were analyzed in accordance with preoperative and intraoperative findings as well as operative outcome. Lung biopsy was performed at the time of defect closure and was classified according to Heath and Edwards., Results: Structural changes of the pulmonary vasculature were found in 59% of patients; grade 3 and higher changes were present in 19%. There were no statistically significant relations between histologic findings and preoperative clinical and hemodynamic data, intraoperative findings, and operative outcome. The prevalence of moderate (32-50 mm Hg) and severe (> 50 mm Hg) systolic pulmonary hypertension was 27% and 17%, respectively. Increased systolic pulmonary arterial pressure was associated with increased pulmonary vascular resistance (p < 0.000) and patients' age (p = 0.001). Patients with a lower functional capacity had a higher prevalence of pulmonary hypertension (p = 0.011)., Conclusions: The prevalence of HPVD and pulmonary hypertension in adult patients with secundum ASD or sinus venosus defect is considerable. Preoperative hemodynamic data do not predict the degree of HPVD in lung biopsy. Closure is generally advised to prevent increasing pulmonary arterial pressure and decreasing functional capacity over time.

Objective: Current prosthetic heart valves necessitate permanent anticoagulation or have limited durability and impaired hemodynamic performance compared to natural valves. Recently a polymeric valve prostheses with special design for mitral position demonstrated excellent in vitro and in vivo results with improved durability and no need for permanent anticoagulation. In this study, a respective flexible polymeric aortic valve is presented and in vitro and in vivo results are reported., Methods: The aortic prosthesis (ADIAM lifescience AG, Erkelenz, Germany) is entirely made of polycarbonaturethane. The tri-leaflet flexible prosthesis mimicks the natural aortic valve and has a diminished pressure loss and reduced stress and strain peaks at the commissures. The valve underwent long-term in vitro testing and in vivo-testing in a growing calve animal model (20 weeks, 7 aortic valves) and was compared to two different commercial bioprostheses., Results: The polymeric aortic heart valve substitute demonstrated excellent in vitro and in vivo hemodynamics. Five/seven animals with aortic PCU-prostheses had an excellent clinical long-term course. The explanted valves showed a variable degree of calcification. Two of the seven animals died at 27 and 77 days due to pannus overgrowth causing severe LVOTO without degeneration of the valve itself. Both animals with commercial bioprostheses had to be sacrificed because of congestive heart failure related to structural degeneration of the bioprosthesis after 10 and 30 days of implantation. There was no increased thrombogenity of the PCU valves compared to bioprostheses., Conclusion: The new flexible polymeric aortic valve prosthesis is superior to current bioprostheses in animal testing.

Background: Since introduction in 1999, pulmonary valve replacement in pediatric patients with the Contegra conduit (Medtronic Inc, Minneapolis, MN) has gained widespread application with increasing enthusiasm. However, unexpected graft related adverse effects may occur., Methods: Between April 2001 and December 2002, 29 patients (20 male; mean age, 3.39 +/- 3.66 years; range, 0.01 to 13.0 years; mean weight, 11.62 +/- 8.73 kg) underwent right ventricular outflow tract reconstruction with the Contegra conduit. Seventeen patients underwent primary repair, 8 had prior homografts, and 4 had other previous operations., Results: There were no deaths. Three early graft related complications were observed. In two infants (age, 1.8 and 3.5 months; weight, 3.6 and 3.8 kg, respectively) thrombus formation at the conduit valve was detected 2 weeks postoperatively. Under anticoagulation with low-molecular-weight heparin, thrombi resolved completely in both patients. One patient (4.5 months, 4.43 kg) developed severe regurgitation due to a fibrous layer covering the inner conduit wall and required conduit exchange 3 weeks postoperatively. After a mean follow-up time of 10.2 +/- 6.4 months all patients are in good clinical condition. However, one patient with systemic right ventricular pressure developed pseudoaneurysm at both graft insertion sites and is scheduled for reoperation. Two other patients underwent balloon dilation. Freedom from reoperation and intervention at 1 year is 89.4%. With regard to regurgitation and conduit stenosis all other conduits perform well., Conclusions: Contegra conduits are an alternative to homografts for right ventricular outflow tract reconstruction. However, there is a risk of thrombus formation in small infants so that prophylactic anticoagulation may be necessary. Patients with systemic right ventricular pressure require close observation as pseudoaneurysm formation has been observed.

Objective: Current prosthetic heart valves necessitate permanent anticoagulation or have limited durability and impaired hemodynamic performance compared with natural valves. We report in vivo and in vitro results with new polymeric valve prostheses that have a special design for the mitral and aortic positions. The aims are improved durability and elimination of the need for permanent anticoagulation., Methods: The mitral and aortic prostheses (Adiam Life Science, Erkelenz, Germany) are made entirely of polycarbonate urethane (PCU). The bileaflet asymmetric mitral valve mimics natural, nonaxial inflow, which creates a left ventricular vortex, saving energy for systolic ejection of blood. The trileaflet aortic prosthesis has diminished pressure loss and reduced stress and strain peaks at the commissures. The valves were subjected to long-term in vitro testing and in vivo testing in a growing calf model (20 weeks; 7 mitral and 7 aortic valves) with comparison with 2 commercial bioprostheses (7 mitral, 2 aortic). Two-dimensional echocardiography was performed after implantation and prior to sacrifice with autopsy and valve examination., Results: In vitro durability of the PCU valves was proved up to 20 years. In vivo durability and hemodynamics were superior to those of all bioprostheses. Survival of PCU valves versus bioprostheses was 7 versus 2 mitral valves and 5 versus 0 aortic valves, respectively. Two animals with PCU aortic valves died of pannus overgrowth that caused severe left ventricular outflow tract obstruction without changes in the valves. Degeneration and calcification were mild (mitral) and moderate (aortic) in PCU valves but were severe in biological valves. There was no increased thrombogenicity of the PCU valves compared with bioprostheses., Conclusion: The new flexible polymeric aortic and mitral valve prostheses were superior to current bioprostheses in animal testing.

Background: Current heart valve prostheses are constructed mimicking the native aortic valve. Special hemodynamic characteristics of the mitral valve such as a nonaxial central inflow with creation of a left ventricular vortex have so far not been taken into account. A new polycarbonaturethane (PCU) bileaflet heart valve prosthesis with special design for the mitral position is introduced, and results of animal testing are presented., Methods and Results: After in vitro testing, 7 PCU-prostheses and 7 commercial bioprostheses (Perimount, n=4; Mosaic, n=3) were implanted in mitral position into growing Jersey calves (age 3-5 months, weight 60-97 kg) for 20 weeks. 2-Dimensional echocardiography was performed after implantation and before sacrification. Autopsy included histologic, radiographic, and electron microscopic examination of the valves. In vitro durability was proven for >15 years. After implantation 2-dimensional-echocardiography showed no relevant gradient or regurgitation of any prosthesis. Clinical course of the animals with PCU valves was excellent. In contrast, 5 of 7 calves with bioprostheses were sacrificed after 1-9 weeks because of congestive heart failure. 2-Dimensional echocardiography of the PCU valves after 20 weeks showed mild leaflet thickening with trivial regurgitation; mean gradient was 8.1+/-5.0 mm Hg (weight: 160-170 kg). The explanted PCU prostheses revealed mild calcification and no structural degeneration. All of the Perimount bioprostheses were severely calcified and degenerated after 11+/-7 weeks. One Mosaic bioprosthesis was thrombosed after 1 week, and 2 showed severe and mild-to-moderate degeneration after 4 and 22 weeks, respectively., Conclusions: Polycarbonaturethane valve prostheses with special design for mitral position show excellent hemodynamic performance and durability in vivo. Calcification and structural changes are mild compared with bioprostheses. Controlled clinical studies are planned.

Background: In selected cases, minimally-invasive approaches are favoured for the correction of congenital heart defects with regard to better cosmetic results., Methods: Between July 1999 and April 2001, 25 children (9 male; mean age 5.8 +/- 4.1 years; mean weight 19.6 +/- 12.6 kg) were operated on using minimally invasive approaches. Diagnoses were: ASD (n = 19), VSD (n = 2), ostium primum defect (n = 3) and Tetralogy of Fallot (n = 1). Female patients with ASD underwent a limited right anterolateral thoracotomy. A ministernotomy was chosen in male patients, in patients under 6 months of age, and in patients with malformations other than ASD. Cannulation was always performed via the chest incision., Results: There were no perioperative complications. Mean operation time was 3.23 +/- 0.89 h. Twelve patients were extubated immediately after surgery, mean ventilation time in the others was 12.1 +/- 11.7 h. Mean ICU stay and hospital stay were 1.5 +/- 0.75 days and 8.3 +/- 2.2 days, respectively. Follow-up (mean 4.8 +/- 4.6 months) was uneventful., Conclusions: Intracardiac repair of some congenital heart defects can be performed safely and effectively via minimally-invasive approaches. Indications are expanding towards more complex defects. Exposure for precise repair is good, additional incisions can be avoided, and cosmetic results have been excellent.

Background: Patients with hypoplastic left heart syndrome (HLHS) and associated malformations undergo Norwood palliation or potentially a two-ventricle repair., Methods: Since 8/99, 8 patients with typical HLHS and two with DILV underwent Norwood/Fontan palliation (group I). Three other patients (group II) had two-ventricle repair. Anatomy was: aortic atresia, coarctation, unrestrictive VSD (patient 1), hypoplastic mitral and aortic valve, arch and LV, coarctation (patients 2 and 3). Surgical procedures were Norwood arch reconstruction with either Rastelli operation (patient 1) or ASD-closure (patients 2 and 3)., Results: Operative mortality in group I was 1/8 (day 22; RV-failure). Two patients died before Glenn (sepsis, RV-failure). Six patients underwent Glenn procedure successfully. No patient died in group II. Echocardiography after 13 +/- 7.4 months showed mild homograft dysfunction (patient 1) and an LVOT-gradient of 20 mmHg (patient 3). Clinical condition of all survivors in both groups is good., Conclusion: Some anatomical subsets of HLHS with borderline mitral valves and small left ventricles may undergo two-ventricle repair despite severe LVOTO. Mortality and morbidity seem to be lower, but selection criteria are so far not defined.

Background: Patients with absent pulmonary valve syndrome (APVS) with respiratory distress (RD) have previously had a high mortality. In 1990 we adopted a strategy of primary repair including total replacement of the aneurysmal central pulmonary arteries (PAs) for patients with RD., Methods: Retrospective review was made of 54 consecutive patients with APVS between 1960 and 1998. Median age and weight were 4 months and 4.8 kg. RD was present in 23 patients (10 neonates, 16 required ventilation). Fifteen patients had repair with homograft replacement of the PAs and VSD closure (group 1). Twenty-seven patients had transannular patch with VSD closure with PA-plasty (group 2, n = 21) or without PA plasty (group 3, n = 6). Twelve had miscellaneous procedures (group 4); in 6 the VSD was left open., Results: Operative, 1-, 5-, and 10-year survivals were 83%, 80%, 78%, and 78%, respectively. Risk factors for operative mortality in multivariate analysis were RD (p = 0.04), neonates (p = 0.02), weight less than 3 kg (p = 0.02), open VSD (p = 0.02) and surgery before 1990 (p = 0.04). Since 1990 operative mortality has decreased to 11% (p = 0.04). RD was the only time-related predictor of survival in multivariate analysis (p = 0.004). In patients with RD, survival with homograft was 73% versus 41% with other techniques (p = 0.2). Mean follow-up was 72 +/- 50 months. There were no significant differences in freedom from reintervention rates among the surgical groups (p = 0.08)., Conclusions: Aggressive homograft replacement of the central pulmonary arteries has been associated with improved survival in patients with APVS especially in neonates with severe RD.

Background: To evaluate the impact of intramural coronary arteries for the peri-, postoperative and long-term course after arterial switch operation (ASO)., Methods: ASO was performed in 351 patients between 1981 and 2000 with dextrotransposition of the great arteries (d-TGA). Five patients (1.4 %) had an intramural coronary artery. Coronary transfer was performed with a collar under dissection of the commissure without longitudinal splitting of the intramural section., Results: None of these patients died; the intraoperative course was uneventful, and no myocardial ischemic changes were observed. In three patients, follow-up cardiac catheterization after 5, 16 and 53 months revealed an occlusion of the intramural coronary ostium. Exercise electrocardiography and myocardial scintiscan showed myocardial ischemia. Two of these patients underwent a successful internal mammary artery bypass., Conclusions: The intramural course of coronary arteries in patients with d-TGA is rare and does not cause increased mortality or myocardial infarction rates. However, the risk of coronary occlusion over time seems to be high. Therefore, patients with this condition require selective coronary angiography and frequent exercise investigations. Revascularization with an internal mammary artery bypass may be indicated.

Background: Failure of the systemic right ventricle after atrial switch operation can be treated by conversion into an arterial switch operation., Methods: Four patients, age 38 to 59 months, presented with right ventricular failure after Senning operation and ventricular septal defect closure. One patient had elevated left ventricular pressure; in the other three patients the left ventricle was retrained to a left ventricular/right ventricular pressure ratio of 0.8 or greater by pulmonary artery banding in 12 to 24 months., Results: Postoperative course after arterial switch operation was prolonged, but clinical condition was good at discharge. Fractional shortening ranged from 20% to 28%. Trace-to-moderate aortic regurgitation was present; only 1 patient had preserved sinus rhythm. After a mean follow-up of 43.5 months 1 patient had died due to left ventricular dysfunction. The survivors are in New York Heart Association functional class I to II. Fractional shortening has improved (29% to 37%); aortic regurgitation has not increased. No patient has undisturbed sinus rhythm., Conclusions: Conversion of an atrial into an arterial switch is an alternative to cardiac transplantation in childhood. However, the procedure is demanding. Long-term morbidity is caused by rhythm disturbances. Aortic valve performance and left ventricular function require close observation.

Background: Tissue engineering is a new approach, whereby techniques are being developed to transplant autologous cells onto biodegradable scaffolds to ultimately form new functional tissue in vitro and in vivo. Our laboratory has focused on the tissue engineering of heart valves, and we have fabricated a trileaflet heart valve scaffold from a biodegradable polymer, a polyhydroxyalkanoate. In this experiment we evaluated the suitability of this scaffold material as well as in vitro conditioning to create viable tissue for tissue engineering of a trileaflet heart valve., Methods: We constructed a biodegradable and biocompatible trileaflet heart valve scaffold from a porous polyhydroxyalkanoate (Meatabolix Inc, Cambridge, MA). The scaffold consisted of a cylindrical stent (1 x 15 x 20 mm inner diameter) and leaflets (0.3 mm thick), which were attached to the stent by thermal processing techniques. The porous heart valve scaffold (pore size 100 to 240 microm) was seeded with vascular cells grown and expanded from an ovine carotid artery and placed into a pulsatile flow bioreactor for 1, 4, and 8 days. Analysis of the engineered tissue included biochemical examination, enviromental scanning electron microscopy, and histology., Results: It was possible to create a trileaflet heart valve scaffold from polyhydroxyalkanoate, which opened and closed synchronously in a pulsatile flow bioreactor. The cells grew into the pores and formed a confluent layer after incubation and pulsatile flow exposure. The cells were mostly viable and formed connective tissue between the inside and the outside of the porous heart valve scaffold. Additionally, we demonstrated cell proliferation (DNA assay) and the capacity to generate collagen as measured by hydroxyproline assay and movat-stained glycosaminoglycans under in vitro pulsatile flow conditions., Conclusions: Polyhydroxyalkanoates can be used to fabricate a porous, biodegradable heart valve scaffold. The cells appear to be viable and extracellular matrix formation was induced after pulsatile flow exposure.

Background: The arterial switch operation (ASO) is the treatment of choice for transposition of the great arteries., Methods: Anatomical risk factors on mortality and morbidity were analyzed retrospectively in 312 patients who underwent ASO between 1982 and 1997., Results: Survival was 95%, 92%, and 92% after 30 days, 5, and 10 years, respectively. Operative survival improved after 1990 to 97% (p < 0.001). Risk factors for operative mortality were complex anatomy (p = 0.018), coronary anomalies (p = 0.008), and prolonged bypass time (p < 0.001). Determinants of late mortality were coronary distribution (p = 0.03), position of the great arteries (p = 0.0095), bypass time (p = 0.047), and aortic coarctation (p = 0.046). After a follow-up of 3.6 +/- 2.7 years (0.1 to 14.9 years), 98% had good left ventricle function, 94% were in sinus rhythm, 2.4% had moderate to severe pulmonary stenosis, 0.3% had significant aortic regurgitation, and 1% had coronary stenosis. Freedom from reoperation was 100%, 96%, and 94% after 1, 5, and 10 years, respectively. No preoperative anatomic parameter correlated with long-term morbidity., Conclusions: ASO can be performed with low operative mortality (< 5%) and long-term morbidity. Malformations associated with complex transposition of the great arteries influence early and late mortality.

Objective: Permanent cardiac pacing in children and adolescents is rare and often occurs by means of epicardial pacing. Based on two decades of experience, operative and postoperative data of patients with epicardial and transvenous pacing were analyzed retrospectively., Methods: Between October 1979 and December 1998, 71 patients (mean age, 5.3+/-4.2, range, 1 day-16.2 years; mean body weight, 18+/-12; range, 8-56 kg) underwent permanent pacemaker implantation. Indications were sinus node dysfunction and atrio-ventricular block following surgery for congenital heart disease (69%), or congenital atrioventricular block (31%). Pacing was purely atrial (1.4%), purely ventricular (73%), ventricular with atrial synchronization (5. 6%), or atrioventricular synchronized (20%). Epicardial pacing was established in 49 (69%), transvenous in 22 (31%) patients. Follow-up was 3.4+/-3.8 years (epicardial) and 3.0+/-4.0 years (transvenous)., Results: Epicardial leads were implanted in younger patients (mean age: 4.5 vs. 7.0 years, P<0.05) and preferably after surgery induced atrioventricular block (78 vs. 46%, P<0.05). The youngest patient with transvenous pacing was 1.3 years old (weight, 8.5 kg). At implantation epicardial ventricular stimulation threshold at 1.0 ms was 1.07+/-0.46 vs. 0.53+/-0.31 V (transvenous) (P<0.05). The age-adjusted rate of lead-related reoperations was significantly higher in patients with epicardial leads (P<0.05), mainly due to increasing chronic stimulation thresholds resulting in early battery depletion. In three patients who received steroid-eluting epicardial leads initial low thresholds persisted after five month to one years. In two patients with recurrent epicardial threshold increase, steroid-eluting epicardial leads led to good acute and chronic thresholds after nine to 15 month. Two post-operative death (2.8%) were probably due to a dysfunction of the (epicardial) pacing system., Conclusions: Transvenous pacing in the pediatric population is associated with a lower acute stimulation threshold and a lower rate of lead-related complications. If epicardial pacing is necessary (e. g. small body weight, special intracardiac anatomy (e.g. Fontan), impossible access to superior caval vein), steroid-eluting leads may be considered.

Objective: We compared the Norwood stage I operation for hypoplastic left heart syndrome and other complex malformations with ductus-dependent systemic circulation., Methods: A retrospective study of 194 patients who underwent a Norwood stage I palliation between 1990 and 1998 was conducted. Malformations in 131 patients were classified as hypoplastic left heart syndrome, defined as aortic and mitral atresia or severe stenosis, normal segmental anatomy, intact ventricular septum, and hypoplasia of the left ventricle. Sixty three patients had other lesions: hypoplastic left ventricle with ventricular septal defect (n = 18), unbalanced complete atrioventricular canal (n = 9), complex double-outlet right ventricle (n = 14), double-inlet left ventricle (n = 11), tricuspid atresia with transposition of the great arteries (n = 6), and others (n = 5), including heterotaxia., Results: Operative (>30 days) and 1-year survivals were lower for patients with hypoplastic left heart syndrome than for those with other lesions (63.4% vs 81%, P =.008, and 51.2% vs 71.4%, P =.02, respectively). The presence of a nonhypoplastic left ventricle (n = 27) was associated with higher operative and 1-year survivals (96.3% vs 64.7%, P =.002; 88.9% vs 52. 7%, P <.001). A restrictive atrial septal defect and prematurity tended to increase mortality across both groups. Cox proportional hazards regression indicated that a single right ventricle was the most important independent predictor of death (P <.001). Operative mortality for all patients undergoing the stage I procedure decreased from 38.5% (1990-1994) to 21.4% after 1994 (P =.02)., Conclusions: The survival of patients with malformations other than hypoplastic left heart syndrome after the Norwood procedure is greater than for those with hypoplastic left heart syndrome. Staged palliation is valid surgical therapy in these patients, with good results in intermediate follow-up.