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OBJECTIVES Options for paediatric aortic valve replacement (AVR) are limited if valve repair is not feasible. Results of paediatric Ross procedures are inferior to adult Ross results, and mechanical AVR imposes constant anticoagulation with the inherent risks. METHODS The study design was a prospective, multicentre follow-up of all paediatric patients receiving decellularized aortic homografts (DAHs) for AVR in 8 European centres. RESULTS A total of 106 children (77 boys) were operated (mean age 10.1 ± 4.8 years, DAH diameter 20.5 ± 3.8 mm). A total of 60 (57%) had undergone previous surgical interventions: 34 with 1, 15 with 2 and 11 with ≥3. There was one early death in a 12-year-old girl, who underwent her fourth aortic valve operation, due to intracerebral haemorrhage on extracorporeal membrane oxygenation after coronary reimplantation problems following 3-sinus reconstruction 1 year earlier. One 2-year-old patient died due to sepsis 2 months postoperatively with no evidence for endocarditis. In addition, a single pacemaker implantation was necessary and a 2.5-year-old girl underwent successful HTx due to chronic myocardial failure despite an intact DAH. After a mean follow-up of 3.30 ± 2.45 years, primary efficacy end points mean peak gradient (18.1 ± 20.9 mmHg) and regurgitation (mean 0.61 ± 0.63, grade 0–3) were very good. Freedom from death/explantation/endocarditis/bleeding/stroke at 5 years was 97.8 ± 1.6/85.0 ± 7.4/100/100/100% respectively. Calculated expected adverse events were lower for DAH compared to cryopreserved homograft patients (mean age 8.9 years), lower than in Ross patients (9.4 years) and in the same range as mechanical AVR (12.8 years). CONCLUSIONS Even though the overall number of paediatric DAH patients and the follow-up time span are still limited, our data suggest that DAHs may present a promising additional option for paediatric AVR.

Fueled by the uncertainty and the time required to obtain a donor heart, mechanical circulatory support (MCS) forms an essential part of end-stage heart failure. Extracorporeal membrane oxygenation (ECMO) use is limited to a few days before serious complications like bleeding occur. Prolonged support in terms of ventricular assist device (VAD) as a bridge to transplantation (BTT) became mandatory to overcome death on the waiting list. Within the last decade, VADs in adults have evolved drastically with the introduction of continuous flow (cf) devices. Increased miniaturization of VADs and new support strategies have increased its use in the pediatric population even in small children and patients with congenital heart disease (CHD). Nevertheless, patient and device selection in this patient population remain challenging to achieve optimal outcome and decrease complication rates. This comes with the need for care providers specialized in this field. Size issues and anatomical diversity make decision making complex and unique when compared to general adult practice. Neonates with single ventricle physiology are the highest risk candidates for VADs. This chapter reviews the most relevant durable VADs used in children including the rapid evolution of using adult designed cf-VADs to support children with anatomical normal hearts and CHD.

Objectives:Mechanical circulatory support for pediatric heart failure patients with the Berlin Heart EXCOR ventricular assist system is the only approved and established bridging strategy for recovery or heart transplantation. In recent years, the burden of thromboembolic events has led to modifications of the recommended antithrombotic therapy. Therefore, we aimed to assess modifications of antithrombotic practice among the European EXCOR Pediatric Investigator Group members.Methods:We sent a questionnaire assessing seven aspects of antithrombotic therapy to 18 European hospitals using the EXCOR device for children. Returned questionnaires were analyzed and identified antithrombotic strategies were descriptively compared to “Edmonton protocol” recommendations developed for the US EXCOR pediatric approval study.Results:Analysis of 18 received surveys revealed substantial deviations from the Edmonton protocol, including earlier start of heparin therapy at 6–12 h postoperatively and in 50% of surveyed centers, monitoring of heparin effectiveness with aPTT assay, administering vitamin K antagonists before 12 months of age. About 39% of centers use higher international normalized ratio targets, and platelet inhibition is changed in 56% including the use of clopidogrel instead of dipyridamole. Significant inter-center variability with multiple deviations from the Edmonton protocol was discovered with only one center following the Edmonton protocol completely.Conclusion:Current antithrombotic practice among European EXCOR users representing the treatment of more than 600 pediatric patients has changed over time with a trend toward a more aggressive therapy. There is a need for systematic evidence-based evaluation and harmonization of developmentally adjusted antithrombotic management practices in prospective studies toward revised recommendations.

Objective: We aimed to evaluate the adaptive growth and remodeling behavior of the transplanted heart in pediatric heart-transplant recipients by comparing donor body surface area (BSA) and cardiac dimensions during transplantation with the corresponding parameters of the recipient over a period of time. Methods: A retrospective review of medical and echocardiographic records of 167 children (8.65 5.98, median 9; range 0—17 years) who underwent orthotopic heart transplantation between 1987 and March 2010 was done. Results: In the first 30 days posttransplantation, right- and left-ventricular end-diastolic diameters, volumes, and myocardial mass were found to be significantly increased (z score 3.96, p < 0.000) in relation to the recipients’ BSA. Within the first year of post-transplantation, there was a significant reduction in the right-ventricular diameter (z score, 1.0 to +1.6, p = 0.000), left-ventricular diameter (z score 1.0 to +1.9, p = 0.000), right-ventricular enddiastolic volume (z score 1.3 to +1.9, p = 0.000) and left-ventricular end-diastolic volume (z score 1.3 to +1.8, p = 0.000), right-ventricular mass (z score, 1.4 to +1.7, p = 0.000) and left-ventricular mass (z score, 1.4 to +1.8, p = 0.000). During subsequent follow-up periods of 2—5 and 6—10 years, the aforementioned cardiac dimensions and volumes increased appropriately in accordance to the BSA ( p = 0.000). In all the cardiac dimensions and volumes measured, donor—recipient mismatch did not influence the continuous growth of the measured parameters, which was in accordance to the recipients’ BSA over time. Kaplan—Meier survival analysis showed a survival rate of 61.7% at 10 years. There is no statistically significant difference in survival rate among patients with varying donor—recipient weight ratios and donor—recipient BSA ratios ( p = 0.53). Conclusions: This study demonstrates that the transplanted heart undergoes remodeling processes and grows adaptively, in accordance to the BSA, over a period of time. # 2011 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Objective Owing to the mismatch between cardiopulomary bypass priming volume and infants' blood volume, pediatric cardiac surgery is often associated with transfusion of homologous blood, which may increase the risk of perioperative complications. Here we report the impact of a very low volume (95-110 mL) cardiopulmonary bypass circuit during arterial switch operations in neonates with transposition of the great arteries on blood requirements, tissue oxygenation, and patient outcome. Methods Twenty-three consecutively treated neonates aged 2 to 17 days were treated with the blood-sparing approach. Asanguineous priming was used in all cases and packed red blood cells were added when hemoglobin concentration decreased below 7 g/dL. Cerebral and lower body tissue oxygenation was monitored by near-infrared spectroscopy. Intraoperative and postoperative transfusion, duration of ventilation and intensive care unit stay, wound infection, and 30-day mortality were assessed for patient outcome. Results Intraoperative blood transfusion was necessary in 6 of 23 neonates. An additional 11 neonates received postoperative blood transfusions on the intensive care unit, leaving 6 infants who received no blood at all. Preoperative hemoglobin concentration was the only predictor for intraoperative transfusion requirement (11.6 ± 0.9 and 13.3 ± 0.4 g/dL in infants with and without intraoperative transfusion, respectively). Despite marked differences in hemoglobin concentrations between infants with and without transfusion, regional tissue oxygenation increased in both groups during cardiopulmonary bypass and returned to baseline at the end of surgery. In-hospital patient outcome was similar in both groups. Conclusions Transfusion-free complex cardiac surgery can be achieved even in neonates without jeopardizing tissue oxygenation or patient safety.

OBJECTIVES Extracorporeal membrane oxygenation (ECMO) is commonly used in children to allow recovery from ischemic injury or cardiac surgery, to support the circulation in case of end-stage cardiomyopathy, as bridge-to-bridge therapy and as bridge to transplantation as well. It has achieved success in providing cardiac support for these kind of patients with expected mortality due to severe myocardial dysfunction. In this modern era, ECMO support should be considered an important option for children with cardiopulmonary failure refractory to medical therapy or resuscitation. We report our experience in pediatric patients supported by ECMO for intraoperative cardiac failure between November 1991 and December 2006. METHODS AND RESULTS Sixty-six patients with a mean age of 5.2+/-4 years (range: 1 day-17 years) and mean weight of 14.3+/-11 kg (range: 2.8-69 kg) had intraoperative ECMO support for failure to wean off cardiopulmonary bypass (n=46, 69.7%), low cardiac output syndrome (n=8, 12.1%), isolated right ventricular failure (n=6, 9.1%), isolated left ventricular failure (n=3, 4.5%), malignant arrhythmia (n=1, 1.5%) and pulmonary hypertension (n=2, 3.1%). Mean duration of ECMO support was 5.1+/-3 days. Overall 30 (45.4%) patients were successfully weaned off ECMO and survived to decannulation. Overall 6 (9.1%) patients were successfully bridged to heart transplantation while on ECMO support. Thirty patients died (54.4%) (16 while on ECMO and 14 after decannulation) because of multi-factorial complications, i.e. cerebral hemorrhage, pulmonary failure, consumption coagulopathy and therapy-resistant myocardial insufficiency, leding to an overall hospital mortality rate of 45.4%. Mean survival time after decannulation was 28+/-16 h. Overall survival rate on ECMO as bridge to recovery and transplantation has been 54.5% with successful hospital discharge of patients. CONCLUSIONS Our experience shows that ECMO support can be offered intraoperatively to any children after palliative or corrective surgery for congenital heart disease with potentially reversible pulmonary, cardiac or cardiopulmonary failure. In the majority of patients who did not survive late after weaning from ECMO support, significant myocardial dysfunction persisted or pulmonary hypertensive events. Nevertheless, an acceptable proportion of patients who were successfully weaned from ECMO ultimately survived to leave the hospital.

Objective Detailed 3-dimensional anatomic information is essential when planning strategies of surgical treatment for patients with complex congenitally malformed hearts. Current imaging techniques, however, do not always provide all the necessary anatomic information in a user-friendly fashion. We sought to assess the practical clinical value of realistic 3-dimensional models of complex congenitally malformed hearts. Methods In 11 patients, aged from 0.8 to 27 years, all with complex congenitally malformed hearts, an unequivocal decision regarding the optimum surgical strategy had not been reached when using standard diagnostic tools. Therefore, we constructed 3-dimensional virtual computer and printed cast models of the heart on the basis of high-resolution whole-heart or cine magnetic resonance imaging or computed tomography. Anatomic descriptions were compared with intraoperative findings when surgery was performed. Results Independently of age-related factors, images acquired in all patients using magnetic resonance imaging and computed tomography proved to be of sufficient quality for producing the models without major differences in the postprocessing and revealing the anatomy in an unequivocal 3-dimensional context. Examination of the models provided invaluable additional information that supported the surgical decision-making. The anatomy as shown in the models was confirmed during surgery. Biventricular corrective surgery was achieved in 5 patients, palliative surgery was achieved in 3 patients, and lack of suitable surgical options was confirmed in the remaining 3 patients. Conclusion Realistic 3-dimensional modeling of the heart provides a new means for the assessment of complex intracardiac anatomy. We expect this method to change current diagnostic approaches and facilitate preoperative planning.

ObjectiveCardiac surgery with cardiopulmonary bypass for correction of congenital heart disease in neonates and small infants is associated with considerable neurologic sequelae. We assessed the extent to which mixed venous oxygen saturation as a measure for adequacy of perfusion, reflects the oxygenation status of upper and lower body compartments. Moreover, we evaluated potential benefits of near-infrared spectroscopic monitoring of regional tissue oxygenation.MethodsTwenty patients (body weight < 10 kg) undergoing open cardiac procedures with cardiopulmonary bypass were enrolled. Blood samples were obtained in parallel from inferior and superior caval vein cannulas and mixed venous line and assessed for venous oxygen saturation and lactate levels. Data were compared to simultaneously measured tissue oxygenation indices obtained by near-infrared spectroscopy from brain and lower limb.ResultsVenous oxygen saturation was lower and lactate concentration higher in blood from superior relative to inferior venous line. Mixed venous oxygen saturation correlated with venous oxygen saturation from inferior venous line and tissue oxygenation index of lower limb. No correlation was found between mixed venous oxygen saturation and venous oxygen saturation from superior venous line or cerebral tissue oxygenation index.ConclusionIn neonates and small infants undergoing cardiac surgery with cardiopulmonary bypass, considerable regional differences exist in venous oxygen saturation. Mixed venous oxygen saturation primarily represents lower-torso oxygen status but poorly reflects and systematically overestimates upper-body oxygenation. Near-infrared spectroscopy yields additional information on regional oxygenation and may be valuable in early and sensitive detection of regional malperfusion in critical organs such as the brain.

Le syndrome de Marfan est la maladie congenitale du tissu conjonctif la plus frequente. Il est cause par des mutations du gene FBN1 codant la fibrilline-1, un composant des microfibrilles qui forment les fibres elastiques du tissu conjonctif. Etant donne que les fibres elastiques sont omnipresentes dans l’organisme, le syndrome de Marfan peut affecter plusieurs organes.

Small infants undergoing cardiac surgery are at high risk for regional malperfusion during cardiopulmonary bypass. We report a 13-day-old neonate who underwent reconstruction of the aortic arch and closure of atrial and ventricular septum defects. Near-infrared spectroscopy probes were placed on the forehead and the calf to monitor tissue oxygenation and hemoglobin concentrations. During rewarming, after deep hypothermic circulatory arrest, the patient's calf hemoglobin concentration immediately increased but oxygenation remained low. Repositioning of the venous cannula resolved this suspected venous congestion. Simultaneous monitoring of tissue oxygenation and hemoglobin concentration allows differentiation of arterial obstruction from venous congestion.

Neonates and small infants with congenital heart disease and complex cardiac and vascular anatomy are particularly prone to episodes of complete or incomplete regional ischemia during cardiopulmonary bypass. These episodes may result either from inhomogeneous distribution of arterial blood flow via the aortic cannula or from impaired drainage of blood via the venous cannulae. However, techniques for continuous routine monitoring of regional perfusion in neonates or small infants undergoing cardiopulmonary bypass are extremely limited. Over recent years, transcranial near-infrared spectroscopy has become established as a useful technique for the non-invasive monitoring of cerebral oxygenation. Here we present a case in which simultaneous near-infrared spectroscopic monitoring of the oxygenation status in the brain and the right upper thigh revealed lower torso ischemia due to accidental cross-clamping of a hypoplastic descending aorta which would otherwise have been unnoticed. This shows that parallel near-infrared spectroscopy of the brain and the lower extremities may represent a novel non-invasive monitoring technique to ensure adequate cerebral and extracerebral perfusion during cardiopulmonary bypass.

Due to impressive improvements in surgical repair options, even patients with complex congenital heart disease (CHD) may survive into adulthood and have a high risk of end-stage heart failure. Thus, the number of patients with CHD needing heart transplantation (HTx) has been increasing in the last decades. This paper summarizes the changing etiology of causes of death in heart failure in CHD. The main reasons, contraindications, and risks of heart transplantation in CHD are discussed and underlined with three case vignettes. Compared to HTx in acquired heart disease, HTx in CHD has an increased risk of perioperative death and rejection. However, outcome of HTx for complex CHD has improved over the past 20 years. Additionally, mechanical support options might decrease the waiting list mortality in the future. The number of patients needing heart-lung transplantation (especially for Eisenmenger’s syndrome) has decreased in the last years. Lung transplantation with intracardiac repair of a cardiac defect is another possibility especially for patients with interatrial shunts. Overall, HTx will remain an important treatment option for CHD in the near future.

Objectives Postimplantation right ventricular dysfunction is associated with increased morbidity and mortality in ventricular assist device (VAD) recipients. This study aimed to determine the preoperative risk factors for severe right heart failure needing biventricular mechanical circulatory support in children with end-stage heart failure. Methods We reviewed data from 84 children supported with long-term VADs at the German Heart Institute Berlin between January 1999 and October 2010. Right ventricular assist device (RVAD) support was needed for 24 (29%) patients, and the other 60 (71%) were implanted with left ventricular assist devices (LVADs). Results The median age at implantation was 7 years (12 days-18 years), and the median support time was 41 days (1-432 days). Of the 84 patients, the overall survival to transplantation or recovery of ventricular function was 69%. Compared with children implanted with LVAD, patients receiving biventricular support had significantly higher postoperative mortality (P = 0.04). The multivariate logistic regression indicated that decreased milrinone use was the only preoperative factor independently associated with increased requirement for biventricular support (odds ratio: 0.1, 95% confidence interval: 0.04-0.64, P = 0.01). Children treated with milrinone preoperatively showed improved survival after implantation (P = 0.04). Conclusions Paediatric patients needing biventricular support had significantly higher postoperative mortality. Preoperative milrinone use might decrease the risk of severe right ventricular failure requiring additional RVAD insertion and improve postimplantation survival in children with advanced heart failure.

Since August 2009, 22 patients with aortic root aneurysm have been successfully operated on with our new aortic remodeling technique as follows: after placement of the Gelweave (Vascutek, Ltd., Inchinnan, UK) Valsalva vascular graft in the reverse manner to the Florida sleeve procedure, the aortic annulus was fixed with the collar of this prosthesis at the level of the basal ring and the aortic root was wrapped with the prosthesis. Furthermore, the aortic valve commissures were resuspended. The distal end of the graft and the transected aortic wall were sutured together with running sutures when they were anastomosed to the stump of the distal ascending aorta.

Cardiopulmonary bypass (CPB) has been known to induce an inflammatory response that is influenced by various factors. Hypothermia is supposed to reduce inflammation after CPB. We developed an in vitro coculture model for CPB and compared the effects of hypothermia on the inflammatory response in the coculture model with results from a clinical prospective randomized trial. The coculture model consisted of endothelial cells and monocytes. Cells were stimulated with tumor necrosis factor (TNF)-α and exposed to deep hypothermia (20°C) or normothermia (37°C). In the clinical trial, 20 patients undergoing CPB for ventricular septum defect receive either normothermic (37°C) or mild hypothermic (32°C) CPB. We observed a significant interleukin (IL)-6 and IL-8 release in the coculture model 2 and 24 h after the experimental start. In the clinical trial, cytokines were significantly increased directly after weaning from CPB and remained elevated until 24 h. IL-8 and IL-6 secretions were similar in the hypothermic and normothermic group of the coculture model and the patients after 24 h. These results demonstrate that the inflammatory reaction observed in our coculture model is comparable with the cytokine increase in the blood of children undergoing CPB. Our coculture model could be useful for studies on the mechanisms of CPB-induced inflammation.

ObjectiveRecently we suggested a comprehensive blood-sparing approach in pediatric cardiac surgery that resulted in no transfusion in 71 infants (25%), postoperative transfusion only in 68 (24%), and intraoperative transfusion in 149 (52%). We analyzed the effects of transfusion on postoperative morbidity and mortality in the same cohort of patients.MethodsThe effect of transfusion on the length of mechanical ventilation and intensive care unit stay was assessed using Kaplan-Meier curves. To assess whether transfusion independently determined the length of mechanical ventilation and length of intensive care unit stay, a multivariate model was applied. Additionally, in the subgroup of transfused infants, the effect of the applied volume of packed red blood cells was assessed.ResultsThe median length of mechanical ventilation was 11 hours (interquartile range, 9-18 hours), 33 hours (interquartile range, 18-80 hours), and 93 hours (interquartile range, 34-161 hours) in the no transfusion, postoperative transfusion only, and intraoperative transfusion groups, respectively (P

ObjectivesReinterventions after the Ross procedure are a concern for patients and treating physicians. The scope of the present report was to provide an update on the reinterventions observed in the large patient population of the German-Dutch Ross Registry.Patients and MethodsFrom 1988 to 2011, 2023 patients (age, 39.05 ± 16.5 years; male patients, 1502; adults, 1642) underwent a Ross procedure in 13 centers. The mean follow-up was 7.1 ± 4.6 years (range, 0-22 years; 13,168 patient-years).ResultsIn the adult population, 120 autograft reinterventions in 113 patients (1.03%/patient-year) and 76 homograft reinterventions in 67 patients (0.65%/patient-year) and, in the pediatric population, 14 autograft reinterventions in 13 patients (0.91%/patient-year) and 42 homograft reinterventions in 31 patients (2.72%/patient-year) were observed. Of the autograft and homograft reinterventions, 17.9% and 21.2% were performed because of endocarditis, respectively. The subcoronary technique in the adult population resulted in significantly superior autograft durability (freedom from autograft reintervention: 97% at 10 years and 91% at 12 years; P

Background— Up to 30% of patients with end-stage heart failure experience biventricular failure that requires biventricular mechanical support. For these patients, only bulky extracorporeal or implantable displacement pumps or the total artificial heart have been available to date, which enables only limited quality of life for the patients. It was our goal to evaluate a method that would allow the use of 2 implantable centrifugal left ventricular assist devices as a biventricular assist system. Methods and Results— Seventeen patients have been implanted with 2 HeartWare HVAD pumps, 1 as a left ventricular assist device and 1 as a right ventricular assist device. Seventy-seven percent of the patients had idiopathic dilated or ischemic cardiomyopathy. Their age ranged from 29 to 73 years (mean 51.8±14.5 years), and 11 (64.7%) received intravenous catecholamine support preoperatively. The right ventricular assist device pump was implanted into the right ventricular free wall. The afterload of this pump was artificially increased by local reduction of the outflow graft diameter, and the effective length of its inflow cannula was reduced by the addition of two 5-mm silicon suture rings to the original HVAD implantation ring. All right ventricular assist device devices could be operated in appropriate speed ranges and delivered a flow of between 3.0 and 5.5 L/min. Thirty-day survival was 82%, and 59% of the patients could be discharged home after recovering from the operation. There was no clinically relevant hemolysis in any of the patients. Conclusions— Two HeartWare HVAD pumps can be used as a biventricular assist system. This implantable biventricular support gives the patients more comfort and mobility than usual biventricular ventricular assist devices with large and noisy displacement pumps.

Objective We aimed to evaluate the influence of size disparity of the transplanted heart on cardiac growth in infant and child recipients by comparing donor body surface area (BSA) and cardiac dimensions during transplantation to the corresponding parameters of the recipient over a period of time. Methods A retrospective review of medical and echocardiographic records of 147 children (5.3 ± 4.0; median, 4.1; range, 1 month–15 years) who underwent orthotopic heart transplantation was done. The patients were divided into age groups as follows: less than 1 year (n = 23), 1 to 2 years (n = 26), more than 2 to 5 years (n = 18), more than 5 to 10 years (n = 27), and more than 10 to 15 years (n = 53). Donor/recipient BSA ratio was determined during transplantation. Cardiac dimensions were measured 30 days after transplantation and compared at 1 year, 2 to 5 years, and 5 to 10 years after transplantation. Results There were no significant differences in the ventricular end-diastolic diameter, volumes, and mass among those with a donor/recipient BSA ratio of less than 0.80, 0.8 to 1.2, and more than 1.2 ( P = .80, .44, and .48, respectively). In all the cardiac dimensions and volumes measured, donor–recipient mismatch did not influence the continuous growth of the heart, as indicated by the measured parameters, in accordance with the recipients' increase in BSA over time. All calculated Z-scores at 1 year, 2 to 5 years, and 6 to 10 years after transplantation were normal when indexed to BSA. Conclusions This study demonstrates that despite size disparity of a transplanted heart, it undergoes normal growth in diastolic dimensions, volumes, and myocardial mass over time as appropriate for body growth after cardiac transplantation in infants and children.

We describe our experience with extracorporeal cardiopulmonary resuscitation (CPR) using extracorporeal membrane oxygenation (ECMO) in children with refractory cardiac arrest, and determine predictors for mortality. ECMO support was instituted on 42 children, median age 0.7 years (1 day-17.8 years), median weight 7.05 (range 2.7-80) kg who suffered refractory cardiac arrest (1992-2008). Patients were postcardiotomy (n=27), or had uncorrected congenital heart diseases (n=3), cardiomyopathy (n=3), myocarditis (n=2), respiratory failure (n=3), or had trauma (n=4). Cannulation site was the chest in all except for three neonates who were cannulated through the neck vessels and two children who had femoral cannulation. ECMO was successfully discontinued in 17 patients. Primary cause of mortality was neurological injury. Pre-ECMO CPR duration for survivors against those who died was a mean of 35±1.3 min vs. a mean of 46±4.2 min. Age, weight, sex, anatomic diagnosis, etiology (surgical vs. medical) were not significant predictors of poor outcome. Prolonged CPR and high-dose inotropes are significant predictors of mortality. Rescue ECMO support in children with refractory cardiac arrest can achieve acceptable survival and neurological outcomes.

Background— The purpose of the study is to report major cardiac and cerebrovascular events after the Ross procedure in the large adult and pediatric population of the German-Dutch Ross registry. These data could provide an additional basis for discussions among physicians and a source of information for patients. Methods and Results— One thousand six hundred twenty patients (1420 adults; 1211 male; mean age, 39.2±16.2 years) underwent a Ross procedure between 1988 and 2008. Follow-up was performed on an annual basis (median, 6.2 years; 10 747 patient-years). Early and late mortality were 1.2% (n=19) and 3.6% (n=58; 0.54%/patient-year), respectively. Ninety-three patients underwent 99 reinterventions on the autograft (0.92%/patient-year); 78 reinterventions in 63 patients on the pulmonary conduit were performed (0.73%/patient-year). Freedom from autograft or pulmonary conduit reoperation was 98.2%, 95.1%, and 89% at 1, 5, and 10 years, respectively. Preoperative aortic regurgitation and the root replacement technique without surgical autograft reinforcement were associated with a greater hazard for autograft reoperation. Major internal or external bleeding occurred in 17 (0.15%/patient-year), and a total of 38 patients had composite end point of thrombosis, embolism, or bleeding (0.35%/patient-year). Late endocarditis with medical (n=16) or surgical treatment (n=29) was observed in 38 patients (0.38%/patient-year). Freedom from any valve-related event was 94.9% at 1 year, 90.7% at 5 years, and 82.5% at 10 years. Conclusions— Although longer follow-up of patients who undergo Ross operation is needed, the present series confirms that the autograft procedure is a valid option to treat aortic valve disease in selected patients. The nonreinforced full root technique and preoperative aortic regurgitation are predictors for autograft failure and warrant further consideration. Clinical Trial Registration— URL: http://www.clinicaltrials.gov. Unique identifier: NCT00708409.

Background Weaning from left ventricular assist devices (LVADs) after myocardial recovery in patients with idiopathic dilated cardiomyopathy is a clinical option. With the broad application of continuous-flow pumps, we observed a decrease in the numbers of possible LVAD explantations due to myocardial recovery in these particular patients. We investigated this phenomenon and its causes. Methods Between July 1992 and December 2009, 387 patients (age, 0.1 to 82 years) with idiopathic dilated cardiomyopathy underwent LVAD implantation at our institution. Patients were divided into two groups depending on whether they were weaned from the LVAD (group A) or not (group B). Univariate and multivariate analyses were performed on 24 different factors with a possible influence on myocardial recovery. Results In 34 patients, LVAD removal due to myocardial recovery was performed with long-term stable cardiac function (weaning rate, 8.8%). Patients with a pulsatile-flow LVAD had an almost threefold chance for myocardial recovery (odds ratio, 2.719; 95% confidence interval, 1.182 to 6.254) than patients who received continuous-flow devices. Younger patients had significantly higher recovery rates than older patients (odds ratio, 1.036; 95% confidence interval, 1.016 to 1.057). Conclusions Pulsatile-flow LVADs and young age were important factors for myocardial recovery in idiopathic dilated cardiomyopathy patients in our analysis. Further studies should investigate whether pulsatility in itself or the different degrees of left ventricular unloading by the two types of systems play a role in myocardial recovery.

Objective: Early and late mortality have significantly improved during recent decades in pediatric patients after heart transplantation (HTx). Nevertheless early and late morbidity and mortality are influenced by acute rejection, cardiac allograft vasculopathy (CAV), malignancy, renal failure, and graft failure. Methods: We evaluated our results after HTx in children under the age of 18 years with 23 years of follow-up. Perioperative characteristics, probability of survival, and time-related morbidity were retrospectively analyzed. Results: We included 169 pediatric HTx recipients, transplanted between 05/1986 and 05/2010. One hundred and one were males with a median age of 8.7 (0.02—23.2) years at the time of HTx. Main preoperative diagnoses were cardiomyopathy (CMP) (n = 139) with a median survival of 7.0 (0—23.2) years and congenital heart disease (CHD) (n = 30), median survival 11.3 (0—19.9) years. Overall survival at 1, 5, 10, and 15 years was 87%, 76%, 68%, and 50%, respectively. Patient survival was signifi cantly reduced in patients with 0—1 year at the time of HTx versus 1— 10 and 11—18 years: 2.3 (0—13.2) years versus 1—10 years = 8.6 (0—23.2) years; 11—18 years = 5.9 (0.003—18.5) years. Fifty-one patients were on mechanical circulatory support as a bridge-to-HTx with increased early but not late mortality. Ten patients underwent retransplant due to acute or chronic graft failure after a median posttransplant time of 12.25 (0.3—17.45) years. Late mortality was influenced by rejection, infection, posttransplant lymphoproliferative disease (PTLD) (11.8%), or CAV with an incidence of 25% at 5 years, 50% at 10 years, and approximately 75% at 15 years. Conclusions: Pediatric HTx is a safe and effective treatment for terminal heart failure. In our experience, there is no adverse effect of previous cardiac assist device implantation in long-term follow-up. Virtually all anatomic malformations are amenable to orthotopic HTx. Significant progress has been achieved in controlling rejection through improved immunosuppression and noninvasive rejection monitoring. # 2011 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Purpose We have introduced a new surgical technique for aortic root remodeling in aortic root aneurysm, comprised of aortic annuloplasty, reduction of sinus of Valsalva, and wrapping of the aortic root. Description At the level of the basal ring, the aortic annulus is fixed with the collar of a Gelweave Valsalva vascular graft (Vascutek, Ltd, Inchinnan, UK). After plication of the dilated sinuses of Valsalva from outside the aorta, the aortic root is wrapped with this prosthesis. The distal end of the graft and the transected aortic wall are sutured together with running sutures. Evaluation Since September 2006, 10 patients suffering from aortic root aneurysm were successfully operated on using this technique. After a median follow-up of 20.7 months, there were no early deaths and no reoperations due to failure of remodeling surgery. Median diameter of the sinus of Valsalva was reduced from 49.5 mm to 34.0 mm. All patients showed aortic regurgitation of grade 0 or grade 1 at follow-up. Conclusions Long-term follow-up is necessary to prove the durability of this operation.

Background— Autograft reinforcement interventions (R) during the Ross procedure are intended to preserve autograft function and improve durability. The aim of this study is to evaluate this hypothesis. Methods and Results— 1335 adult patients (mean age:43.5±12.0 years) underwent a Ross procedure (subcoronary, SC, n=637; root replacement, Root, n=698). 592 patients received R of the annulus, sinotubular junction, or both. Regular clinical and echocardiographic follow-up was performed (mean:6.09±3.97, range:0.01 to 19.2 years). Longitudinal assessment of autograft function with time was performed using multilevel modeling techniques. The Root without R (Root−R) group was associated with a 6× increased reoperation rate compared to Root with R (Root+R), SC with R (SC+R), and without R (SC-R; 12.9% versus 2.3% versus 2.5%.versus 2.6%, respectively; P Conclusions— For the time period of the study surgical autograft stabilization techniques preserve autograft function and result in significantly lower reoperation rates. The nonreinforced Root was associated with significant adverse outcome. Therefore, surgical stabilization of the autograft is advisable to preserve long-term autograft function, especially in the Root Ross procedure.

When mitral annuloplasty is performed in small children, room for annular growth should be allowed. However, it has not been reported how the valve develops after mitral annuloplasty of the entire posterior annulus. We report a case showing traces of annular growth at redo surgery. A female patient suffering from mitral valve insufficiency due to annular dilatation underwent modified Paneth plasty with Kay-Wooler commissural plication annuloplasty at the age of two years one month. In redo surgery 8.4 years after initial repair, enlargement of the commissural portion of the posterior annulus in addition to enlargement of the anterior leaflet and anterior annulus was observed. Modified Paneth plasty reinforced with a pericardial strip and Kay-Wooler annuloplasty of the posteromedial commissure were performed. Mitral orifice size measured with the Hegar dilator was 18 mm after the re-repair, increasing from 16 mm after the initial repair. Taking into account the normal mitral annulus diameter related to body surface area (BSA) of 16 mm at initial operation and 20 mm at redo surgery, the increase in mitral orifice size from 16 mm to 18 mm in this patient may be regarded as the annular growth in 8.4 years.

Fig. 2. Completion of modified Paneth plasty with 3-0 Prolene (polypropylene monofilament) sutures. The patient underwent modified Paneth suture plasty, in which two double-armed 3-0 Prolene sutures were anchored at each fibrous trigone, and each second running suture was placed around the first simple running suture already placed on both halves of the posterior mitral annulus. A 23-mm sizer was able to pass through the mitral valve. Intra-operative transoesophageal echocardiography revealed no mitral regurgitation, no mitral stenosis and no left ventricular outflow tract obstruction after valve repair.

Performing cardiac surgery on pediatric Jehovah’s Witness patients is a great challenge for the surgical team and especially for the perfusionist. Jehovah’s Witnesses reject blood transfusions on the grounds of their literal interpretation of passages of the Bible. In accordance with this belief, Jehovah’s Witnesses feel that it is also forbidden to retransfuse autologous blood that has been separated from their own circulatory system. We report the use of cardiopulmonary bypass (CPB) during open-heart surgery in three infants with a body weight of 4.5 kg, 3.5 kg, and 3.1 kg, respectively, without transfusion of blood components. A small-volume CPB circuit with a priming volume of 200 mL, including the arterial line filter, was designed to decrease the degree of hemodilution. A dedicated pediatric heart lung machine console with remote pump heads and intensive blood conservation efforts allowed the operation without the use of donor blood. The CPB circuits were primed with crystalloid solution only. The procedures were performed in normothermia or in moderate hypothermia. Pre-CPB hemoglobin levels were 10.8 g/dL, 10.6 g/dL, and 8.5 g/dL. The hemoglobin concentrations measured during CPB ranged from 5.9 to 6.5 g/dL, 6.4 to 6.8 g/dL, and 5.5 to 5.9 g/dL, respectively. The patients did not receive any blood or blood products during their entire hospital stay.

Conflicting results have been reported with regard to the neuroprotective effects of steroid treatment with cardiopulmonary bypass (CPB) and deep hypothermic circulatory arrest (DHCA). We evaluated the mode and severity of neuronal cell injury in neonatal piglets after prolonged DHCA and the possible neuroprotective effect of systemic pretreatment (>6 h before surgery) with large-dose methylprednisolone (MP). Nineteen neonatal piglets (age

Complex cardiac surgery using cardiopulmonary bypass normally requires the transfusion of autologous blood components, particularly in neonates. This is predominately caused by the relatively high priming volume of the circuit with subsequent extreme hemodilution and the often extended and complex perfusions leading to progressive consumption of platelets and coagulation factors. We report on a strategy to minimize the cardiopulmonary bypass circuit and adjust the perfusion technique that resulted in transfusion-free correction of tetralogy of Fallot with an absent pulmonary valve and an aneurysm of the left pulmonary artery in a 3.55 kg Jehovah's Witness neonate boy.

Background: Sildenafil (Viagra®) has been shown to be a specific and effective pulmonary vasodilator and is increasingly used in patients with pulmonary hypertension. We investigated the effect of sildenafil medication on cerebral oxygenation using near-infrared spectroscopy (NIRS) in children with elevated pulmonary vascular resistance after cardiac surgery.

a calcified tear in the noncoronary leaflet with perforation was observed. It is difficult to determine whether the tear was primary or calcium related. Probably, the presence of these calcium depositions encouraged tearing. We speculate that the postoperative finding of elevated peak transvalvular gradient was associated with a dynamic obstruction in the left ventricular outlet, and this situation led to mechanical stress during the systolic phase. The right coronary leaflet had intrinsic and vegetating calcifications with stiffness but without tearing. In addition, the commissural areas were severely calcified. These areas are designed to absorb and distribute the mechanical stress during the cardiac cycle. Thus when the leaflets are opened, they are completely free of stresses. Although a stent distortion is extremely rare and unusual, we can not reject this possibility completely.