The Introduction of an MR-Conditional Prototype for Cardiopulmonary Bypass Support: Technical Aspects and System Requirements.

INTRODUCTION: The use of cardiopulmonary bypass (CBP; also known as a heart-lung machine) in newborns with complex congenital heart defects may result in brain damage. Magnetic resonance imaging (MRI) assessments cannot be performed safely because the metal components used to construct CBP devices may elicit adverse effects on patients when they are placed in a magnetic field. Thus, this project aimed to develop a prototype MR-conditional circulatory support system that could be used to perform cerebral perfusion studies in animal models. METHODS: The circulatory support device includes a roller pump with two rollers. The ferromagnetic and most of the metal components of the roller pump were modified or replaced, and the drive was exchanged by an air-pressure motor. All materials used to develop the prototype device were tested in the magnetic field according to the American Society for Testing and Materials (ASTM) Standard F2503-13. The technical performance parameters, including runtime/durability as well as achievable speed and pulsation behavior, were evaluated and compared to standard requirements. The behavior of the prototype device was compared with a commercially available pump. RESULTS: The MRI-conditional pump system produced no image artifacts and could be safely operated in the presence of the magnetic field. The system exhibited minor performance-related differences when compared to a standard CPB pump; feature testing revealed that the prototype meets the requirements (i.e., operability, controllability, and flow range) needed to proceed with the planned animal studies. CONCLUSION: This MR-conditional prototype is suitable to perform an open-heart surgery in an animal model to assess brain perfusion in an MR environment.

Biventricular circulatory support with two miniaturized implantable assist devices.

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.

Major adverse cardiac and cerebrovascular events after the Ross procedure: a report from the German-Dutch Ross Registry.

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.

[Technical possibilities and limitations of mechanical circulatory support]. / Herzinsuffizienz - Technische Möglichkeiten und Grenzen der Mechanischen Kreislaufunterstützung.

Ventricular assist devices (VAD) to support the left (LVAD), the right (RVAD) or both ventricles (BVAD) have emerged as one standard of care for advanced heart failure patients. Initially used to bridge patients to transplantation (BTT) they are now more frequently implanted as permanent support (destination therapy, DT). Bridge to recovery (BTR) is a valid option for only a small number of patients. Although there are different devices available, patient selection, preoperative and intraoperative management, and the timing of VAD implantation are the elements critical to successful circulatory support.

Autograft reinforcement to preserve autograft function after the ross procedure: a report from the german-dutch ross registry.

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 6x 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<0.001). SC and Root groups had similar rate of aortic regurgitation (AR) development over time. Root+R patients had no progression of AR, whereas Root-R had 6 times higher AR development compared to Root+R. In SC, R had no remarkable effect on the annual AR progression. The SC technique was associated with lower rates of autograft dilatation at all levels of the aortic root compared to the Root techniques. R did not influence autograft dilatation rates in the Root group. 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.

Fate of the aortic valve following the arterial switch operation.

OBJECTIVE: We evaluated the long-term outcome of aortic valve after arterial switch operation (ASO). METHODS: A retrospective review of 324 hospital survivors after ASO was performed. Echocardiographic findings and incidence and progression of aortic regurgitation (AR) were investigated. Endpoints of the study were the first documented occurrence of moderate or severe AR or need for aortic valve replacement (AVR). RESULTS: Upon discharge from hospital 5.2% of the patients showed mild AR, progressing to 11.4% after 1 year. At a mean follow-up time of 14.4 ± 0.54 years, AR was absent in 307 (94.7%), trivial in six (1.8%), mild in nine (2.7%), and moderate-to-severe in two (0.6%) patients. There is a progression of AR with time after ASO (p < 0.001). A total of two patients reached the combined endpoint, both of whom underwent AVR at a mean time of 10.82 years after ASO. Freedom from aortic valve reoperation was 98.7 ± 0.9% at 5 and 10 years and 94.7 ± 4% at 15 years. Univariate analysis identified the following risk factors: ventricular septal defect (VSD) (p = 0.005), prior pulmonary artery banding (p < 0.003), and postoperative incidence of mild AR (p < 0.0001). CONCLUSION: AR is not rare after ASO, but it is stable without progressive intensity; new AR developed in 5.2% after 10 years, and is present only in 2.7% after a median follow-up 14.4 ± 0.54 years. If trivial AR is excluded, it is present only in 0.9%. New AR can develop even up to 15 years. However, severe AR and need for AVR are rare.

Paediatric aortic valve replacement using decellularized allografts.

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.

Partial anomalous pulmonary venous drainage in young pediatric patients: the role of magnetic resonance imaging.

Studies of larger patient groups for systematic assessment of the anatomical accuracy of magnetic resonance imaging (MRI) for partial anomalous pulmonary venous drainage (PAPVD) have been performed so far only in adults. This study was undertaken to evaluate whether MRI can precisely depict pulmonary venous anatomy in infants and young children. Data on 26 children under 10 years old that underwent MRI over the past 2 years for suspected PAPVD were assessed. The MRI protocol included shunt quantification by velocity-encoded cine as well as morphological and functional assessment by multislice multiphase and contrast-enhanced MR techniques. MRI was performed in the compliant patient in breath-hold (n = 8; age range, 4.6-9.5 years) and in the noncompliant patient in conscious-sedation free breathing (n = 18; age range, 0.4 to 7.5 years). In 22 patients, PAPVD was diagnosed with MRI and confirmed during surgery. In four patients with large atrial septal defects not accessible to percutaneous closure, normal pulmonary venous return was demonstrated by MRI and confirmed during surgery. MRI under conscious sedation accurately specifies the anatomy of pulmonary veins in infants and small children. Therefore, we suggest performing MRI in patients with inconclusive transthoracic echocardiographic results in the preoperative assessment of PAPVD.

Right ventricular failure following heart transplantation--recovery after extended mechanical support.

Early graft failure, particularly right ventricular dysfunction, remains a significant determinant of early morbidity and mortality in heart transplant recipients. If conservative medical management fails to help recover cardiac allograft function, mechanical circulatory support either bridging to recovery or retransplantation remains the last option. We report on a 16-year-old patient with hypertropic nonobstructive cardiomyopathy who was weaned successfully from a right ventricular assist device (RVAD) after extended right ventricular mechanical support for three months following heart transplantation.

Antithrombotic therapy in pediatric ventricular assist devices: Multicenter survey of the European EXCOR Pediatric Investigator Group.

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.

Transfusion-free cardiac reoperation in an 11-kg Jehovah's Witness child by use of a minimized cardiopulmonary bypass circuit.

Herein, we describe the design of a perfusion system for a complex cardiovascular reoperation in an 11-kg Jehovah's Witness patient. The goal of safe, transfusion-free surgery was achieved chiefly by minimizing the priming volume of the cardiopulmonary bypass circuit to 200 mL while providing adequate flow and standard safety features.

Transfusion-free cardiopulmonary bypass in Jehovah's Witness patients weighing less than 5 kg.

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.

Release patterns of astrocytic and neuronal biochemical markers in serum during and after experimental settings of cardiac surgery.

OBJECTIVE: Brain injury and altered psychomotor development in infants, children and adults after cardiac surgery using cardiopulmonary bypass (CPB) and deep hypothermic circulatory arrest (DHCA) is still a matter of concern. Early diagnosis and identification of brain injury that has occurred or is ongoing by measurement of biochemical markers in serum may have diagnostic and prognostic value. The aim of the experimental studies in an animal model was therefore to investigate the release patterns of astroglial and neuronal markers in serum and to determine the morphological and immunohistochemical changes in the brain of animals undergoing similar perfusion conditions of CPB and a period of DHCA. METHODS: Fourteen New Zealand rabbits, (weight, 3.1 +/- 0.25 kg) were anesthetized, intubated and mechanically ventilated. Four animals were sham operated and served as controls. After median sternotomy the animals were connected to CPB by cannulation of the aorta and right atrium. Full flow CPB (200-250 ml/kg/min) was initiated to achieve homogeneous systemic cooling. Circulatory arrest of 60 minutes was induced when rectal and nasopharyngeal temperature of 14 degrees C was achieved. After rewarmed reperfusion and establishment of stable cardiac ejection the animals were weaned from CPB and monitored for 6 hours. Then the animals were killed, the brain was immediately removed and cut in standardized sections. These were fixated, embedded in paraffin and stained for further quantitative histological studies. In the brain astrocyte reactivity for S-100B was assessed immunocytochemically (DPC Immustain Los Angeles, USA). Monoclonal mouse anti-human neurospecific enolase (NSE) antibody was used for the localization of NSE in the fixed and paraffin embedded brain (NSE-DAKO, H14). The concentrations of S-100B protein and neurospecific enolase (NSE) in the serum were analyzed using a commercially available immunoluminometric assay (LIA-mat, Sangtec 100, Byk-Sangtec). Immunospecific monoclonal anti-parvalbumin antibody was used for the detection of parvalbumin in the brain. Serum concentrations of parvalbumin were analyzed using a newly developed ELISA method. RESULTS: In all experimental animals a significant increase of the serum concentration of the astroglial protein S-100B was found immediately after reperfusion and the termination of CPB. In contrast the serum levels of the neuronal proteins parvalbumin and NSE were not increased, but rather decreased. Light microscopy and electron microscopy revealed perivascular astrocytic swelling and minor neuronal cell injury. In comparison to the sham operated animals, increased immunohistochemical staining of S-100B was found. This increased reactivity of S100B antibody was found in the astrocytic processes with immediate connection to the perivascular space and around the perivascular oedema. The immunocytochemical stainings for NSE and parvalbumin in the neuronal cells was not different from that of sham-operated animals and indicated well preserved neurons.

Relation of cerebral tissue oxygenation index to central venous oxygen saturation in children.

OBJECTIVE: To evaluate the relationship between the cerebral tissue oxygenation index measured by near-infrared spectroscopy and central venous oxygen saturation (SvO2) after corrective surgery of congenital heart defects in children. DESIGN: Prospective observational clinical study. SETTING: A tertiary neonatal and paediatric intensive care unit for paediatric cardiology. PATIENTS: Neonates and children consecutively admitted to the paediatric cardiology intensive care unit after corrective surgery of non-cyanotic congenital heart defects. MEASUREMENTS AND RESULTS: Forty-three children were studied. Cerebral tissue oxygenation index, measured non-invasively by near-infrared spectroscopy, was compared to SvO2, measured by a catheter placed in the right atrium, and to haemodynamic and respiratory parameters. Pearson's correlation coefficients and p values were calculated. Simultaneously measured values for SvO2 (62.2+/-9.8%, 39.8-80.4%) and cerebral tissue oxygenation index (56.7+/-8.8%, 35.8-71.2%) showed a significant correlation ( r=0.52, p<0.001). CONCLUSION: Cerebral tissue oxygenation index and SvO2 are not interchangeable parameters, but cerebral tissue oxygenation index reflects the haemodynamic influence on cerebral oxygenation after cardiovascular surgery. Further work is necessary to confirm the clinical role of continuous non-invasive measurement of cerebral tissue oxygenation index with regard to the variations of global systemic oxygen consumption after cardiac surgery in children.

Mitral annulus after mitral repair: geometry and dynamics.

The study of systolic changes in both the inflow and outflow of the left ventricle after mitral repair elucidates the geometric characteristics after surgery. The study included eight normal subjects and six patients after mitral repair without a prosthesis. The left ventricular (LV) base, consisting of both mitral valve annulus (MVA) and left ventricular outflow tract (LVOT) orifice, was reconstructed from magnetic resonance images. The angle between the planes of the MVA and LV base (MB angle), and the proportionate share of the LVOT at the LV base were calculated. After mitral repair, both the MVA and LV base became almost normal in size, showing flexible change (i.e., contraction and dorsiflexion) in the MVA and contraction of the LV base in systole. Compared with the normal heart at 100 ms delay from the electrocardiogram R wave, the hearts of the patients at the same phase had a mean 1.05 cm2 larger LVOT orifice, resulting in a mean 8.0 degree larger MB angle. Furthermore, the LVOT orifice occupied a mean of 49.5% of the LV base (41.9% in normal subjects). We hypothesize that the higher MB angle at early systole may weaken the tension of the chordae of the anterior mitral leaflet.

Blood transfusion determines postoperative morbidity in pediatric cardiac surgery applying a comprehensive blood-sparing approach.

OBJECTIVE: Recently 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. METHODS: The 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. RESULTS: The 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 < .00001). The corresponding median lengths of intensive care unit stay were 1 day (interquartile range, 1-2 days), 3.5 days (interquartile range, 2-5 days), and 8 days (interquartile range, 3-9 days; P < .00001). The multivariate hazard ratio for early extubation was 0.24 (95% confidence interval, 0.16-0.35) and 0.37 (95% confidence interval, 0.25-0.55) for the intraoperative transfusion and postoperative transfusion only groups, respectively (P < .00001). In addition, the cardiopulmonary time, body weight, need for reoperation, and hemoglobin during cardiopulmonary bypass affected the length of mechanical ventilation. Similar results were obtained for the length of intensive care unit stay. In the subgroup of transfused infants, the volume of packed red blood cells also independently affected both the length of mechanical ventilation and the length of intensive care unit stay. CONCLUSIONS: The incidence and volume of blood transfusion markedly affects postoperative morbidity in pediatric cardiac surgery. These results, although obtained by retrospective analysis, might stimulate attending physicians to establish stringent blood-sparing approaches in their institutions.

Reverse graft placement in the Florida sleeve procedure for aortic root aneurysm.

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.

Factors associated with the need of biventricular mechanical circulatory support in children with advanced heart failure.

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.

Heart transplantation in congenital heart disease: in whom to consider and when?

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.