Introduction: The anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital defect. Without surgical correction, the mortality rate within the first year of life remains exceptionally high (90%). Prompt surgical reestablishment of a dual coronary perfusion system is the treatment of choice as it significantly improves left ventricular (LV) function and long-term survival (86% survival after 20 years). A subset of patients experiences refractory LV failure due to extensive myocardial ischemia and might require prolonged mechanical circulatory support.

Training in congenital cardiac surgery is potentially lengthier and more demanding than training in any other surgical field. The duration of training is proportional to the complexity of the specialization. The expertise of a wide range of procedures is required. There is no doubt that some individuals may acquire the requisite abilities with greater ease than others, but fundamentally, these are capabilities that can be taught and learnt. Moreover, congenital cardiac surgeons are required to have a detailed understanding of pathophysiology and morphology, in addition to the stamina and empathy required to manage these complex patients. A fellowship is just the start of such training and is followed by a long road eventually leading to a lifelong journey to become a qualified congenital cardiac surgeon. Effective mentorship is a prerequisite throughout training to guide surgeons on this journey.

The Berlin Heart EXCOR Pediatric ventricular assist device is the only approved device for long-term mechanical circulatory support of pediatric patients to recovery or heart transplantation. Over the past years, the burden of thromboembolic events has led to many center-specific modifications of the original anticoagulation protocol. The aim of this survey was to systematically identify and evaluate changes of current antithrombotic practice among the European EXCOR Pediatric Investigator Group (EEPIG) members compared to earlier recommendations.

Background and aims: Acute kidney injury (AKI) is a frequent complication after cardiac surgery with cardiopulmonary bypass in infants. Renal near-infrared spectroscopy (NIRS) evaluates regional oximetry in a non-invasive continuous fashion and reflects tissue perfusion. Aims: The aim of this study was to evaluate the relation between renal oximetry and development of AKI in infants undergoing cardiopulmonary surgery. Methods: We prospectively enrolled 59 infants undergoing cardiopulmonary bypass surgery for uni- (n = 26) or biventricular (n = 33) repair. IRB approval was given and parents signed written informed consent. Renal NIRS was continuously measured intraoperatively and at least 24 hours postoperatively. Renal oximetry values were correlated with the pediatric Renal-Injury-Failure-Loss-End (pRIFLE) classification for AKI, renal biomarkers and the postoperative course. Results: 28 (48 %) infants developed AKI. Already in the intraoperative renal oximetry and further in the first 12, 24 and 48 hours postoperatively significantly lower renal oximetry values in AKI patients as compared to patients with normal renal function could be shown (p < 0.05). 11 % (3/28) of infants with AKI needed renal replacement therapy and 2/28 (7 %) died. In the non AKI group no fatal course occurred. Infants with decreased renal oximetry values developed significantly higher lactate levels 24 hours after surgery. NGAL values were not correlated with AKI. Conclusions: Our results suggest that prolonged lower renal oximetry values during cardiac surgery correlate with the development of AKI and are superior to conventional biomarkers. Renal NIRS is a promising tool to develop kidney-protective strategies during cardiac surgery with cardiopulmonary bypass in infants.

Background: Remote ischemic preconditioning (remote IPC) induced by short non-deleterious ischemic episodes prior to an index ischemic event is known to protect the heart from lethal myocardial ischemia-reperfusion (I/R) injury.Although demonstrated to be effective across almost all species, the underlying signaling pathways and specifically a role for nitric oxide (NO) remain poorly understood.We and others recently showed that brief episodes of limb I/R via blood pressure cuff in-/deflations increases endothelial NO synthase (eNOS) activity with a subsequent formation of NO, nitroso species (RNO) and nitrite.Nitrite, in turn, has been shown to protect the myocardium from lethal ischemia-reperfusion when activated by the heme globin myoglobin (Mb).We therefore hypothesized that remote IPC initiates the release of NO species in an eNOS-dependent manner, and that Mb is required to reduce this endogenously formed species to NO to protect the myocardium.Methods and Results: This study was conducted using a mouse model of remote IPC followed by open-chest I/R via reversible ligation of the left coronary artery in vivo.Four cycles of remote IPC consisting of 5 minutes of hindlimb ischemia followed by 5 minutes of reperfusion, checked by laser Doppler perfusion imaging, caused a release of NO, RNO and nitrite into the circulation.Remote IPC furthermore stimulated a post-translational modification of mitochondrial complex I by S-nitrosation and a subsequent decrease in reactive oxygen species in the reperfused myocardium.This finally caused a reduction in myocardial infarct size per area at risk from 36±2% to 17±1% in wild-types (n=5,p<0.0001).Targeted disruption of endothelial nitric oxide synthase in eNOS-/-mice impaired the release of NO species during remote IPC and completely abolished the beneficial effects on myocardial necrosis (control vs. remote IPC: 45±3% vs. 41±3%, p=n.s.).Finally, in Mb-deficient mice (Mb-/-) remote IPC caused an increase in NO species, but was without effects on infarct size (control vs. remote IPC: 31±1% vs. 35±2% p=n.s.).All values are means±SEM. Conclusion:A concerted action between vascular and myocardial signaling pathways is involved in the NO triggered protection from remote ischemic preconditioning.While eNOS is essentially required for the formation of circulation NO species as triggering mechanisms, Mb in the heart is required for the activation of nitrite to nitric oxide leading to protection of the myocardium at risk.

Introduction: Autograft and homograft endocarditis remain a concern after the Ross procedure. Aim of the present study was to identify the incidence and characteristics of autograft and homograft endocarditis in the adult population of the German-Dutch Ross Registry.

Introduction: Reinterventions after the Ross procedure remain a concern for patients as well as treating physicians. Aim of the present study was to provide an update on reinterventions after the Ross procedure in the large patient population of the German-Dutch Ross Registry

Background: epsilon-Aminocaproic acid (EACA) and tranexamic acid (TXA) are used for antifibrinolytic therapy in neonates undergoing cardiac surgery, although data directly comparing their blood-sparing efficacy are not yet available. We compared two consecutive cohorts of neonates for the effect of these two medications on perioperative blood loss and allogeneic transfusions.Material and Methods: Data from the EACA group (n = 77) were collected over a 12-month period; data from the tranexamic acid group (n = 28) were collected over a 5-month period. Blood loss, rate of reoperation due to bleeding, and transfusion requirements were measured.Results: There was no significant difference in blood loss at 6 hours (EACA 24 [17-30] mL/kg [median (interquartile range)] vs. TXA 20 [11-34] mL/kg, p = 0.491), at 12 hours (EACA 31 [22-38] mL/kg vs. TXA 27 [19-43] ml/kg, p = 0.496) or at 24 hours postoperatively (EACA 41 [31-47] mL/kg vs. TXA 39 [27-60] mL/kg; p = 0.625) or transfusion of blood products.Conclusions: epsilon-Aminocaproic acid and tranexamic acid are equally effective with respect to perioperative blood loss and transfusion requirements in newborns undergoing cardiac surgery.

A previously non-operated 37-year-old patient presented with esophageal dysphagia for solid food and stridorous breathing. Computed tomography and magnetic resonance showed a 33-mm wide Kommerell's diverticulum. The diverticulum and the obliterated ductus arteriosus arising from it towards the pulmonary artery were resected. The left subclavian artery, which originated from the diverticulum, was reimplanted into the descending aorta. In addition to dissecting any tissue which is making vascular ring-like structures around the trachea and the esophagus, it is important to resect the diverticulum itself and reimplant the left subclavian artery arising from it, in this way leaving the aortic arch free from any additional dislocating forces.

Until today, no perfect valve substitute has been developed. Some essential requirements for a perfect valve substitute are proposed in Table 1. Regarding the aortic valve, the Ross procedure (pulmonary autograft operation) is closer to this ideal than any other substitute in many ways. For this reason, it was enthusiastically adopted by many surgeons after it became widely known in the late 1980s and was technically simplified by Stelzer and Elkins [27] (total root replacment, Fig. 1). In recent years, however, several groups report high reoperation rates and a worrysome tendency for the development of neoaortic regurgitation and/or ascending aortic aneurysms [7, 10, 16, 18, 20, 29]. A recent systematic review concluded that "durability limitations become apparent by the end of the first postoperative decade, in particular in younger patients" [28], and it was asked whether the ross procedure is a "Trojan horse" [15]. As a result of these newer data, many centers appear to have stopped performing the Ross procedure.

BACKGROUND:Pulmonary atresia with intact ventricular septum (PA-IVS) is a complex congenital heart defect with a large variety of right heart-sided morphologies.METHODS:We undertook a retrospective review of 86 patients with PA-IVS with a special emphasis on the angiographic findings. The aim of the study was to determine predictors for biventricular repair. Initial surgical procedures depended on the right ventricular morphology, the tricuspid valve size and coronary anomalies.RESULTS:Fifty-five patients (64%) underwent decompression of the right ventricle (RV) as an initial procedure; 16 of them required an additional systemic-to-pulmonary artery shunt. Twenty-six patients (30%) had only a systemic-to-pulmonary artery shunt as their initial procedure. Five patients underwent interventional procedures performed by pediatric cardiologists. Biventricular repair was possible in 56 patients (65%). Univentricular palliation was achieved in 16 patients. Fourteen patients had only palliation with a systemic-to-pulmonary artery shunt. Mean tricuspid valve size was significantly bigger in patients with biventricular repair (z-score -3.6 +/- 2.6) than in patients who did not undergo biventricular repair (-5.2 +/- 1.7, P = 0.003). Predictors for biventricular repair were right ventricular decompression with or without systemic-to-pulmonary artery shunt ( P < 0.001), tripartite right ventricle ( P < 0.001) and the absence of coronary fistulae ( P < 0.001). Long-term survival was 80% +/- 13% at 25 years for patients undergoing biventricular repair.CONCLUSIONS:Decompression of the RV as an initial surgical procedure improves the possibility of achieving biventricular repair with good long-term results. However, morphological factors such as right ventricular size and the absence of coronary fistulae are significant predictors for biventricular repair.

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<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.