Coagulation Profile of Neonates Undergoing Arterial Switch Surgery With Crystalloid Priming of the Cardiopulmonary Bypass Circuit.

OBJECTIVES: The aim was to evaluate changes in the coagulation profile of cyanotic neonates, to analyze the effects of cardiopulmonary bypass (CPB) with crystalloid priming on their coagulation status, and to determine factors predicting a requirement for hemostasis-derived transfusion. DESIGN: Retrospective cohort. SETTING: Single-center, tertiary academic hospital. PARTICIPANTS: In total, 100 consecutive neonates who underwent arterial switch surgery between December 2014 and June 2020. INTERVENTIONS: Rotational thromboelastometry (ROTEM) and coagulation parameters before surgery and before termination of CPB were evaluated. Transfusion of platelets, fresh frozen plasma, and fibrinogen, defined as hemostasis-derived transfusion (HD transfusion), were determined. Patients with and without HD transfusion were compared to identify predictors. MEASUREMENTS AND MAIN RESULTS: After CPB, fibrinogen was reduced by 24.5% (interquartile range [IQR] 8.9-32.1) to 201 mg/dL (IQR 172-249), resulting in a reduction of FIBTEM A10 by 20% (1.8-33.3) to 8 mm (6-11). The platelet count decreased by a median of 47.2% (25.6-61.3) to 162 × 103/µL (119-215). However, the median fibrinogen concentration and platelet count remained within normal range. Neonates with abnormal ROTEM results were more likely to receive HD transfusions. The HD transfusions were more likely with lower preoperative FIBTEM maximum clot firmness values (p = 0.031), lower hemoglobin concentrations at termination of CPB (p = 0.02), and longer CPB duration (p = 0.017). Perioperative hemostasis without any HD transfusion was achieved in 64 neonates. CONCLUSIONS: Guidance from ROTEM analyses facilitates hemostasis management after neonatal CPB. Circuit miniaturization with transfusion-free CPB is associated with acceptable changes in ROTEM in most patients, and allows sufficient hemostasis without any HD transfusions in most patients.

Cardiopulmonary Bypass Strategy to Facilitate Transfusion-Free Congenital Heart Surgery in Neonates and Infants.

Priming the cardiopulmonary bypass (CPB) circuit without the addition of homologous blood constitutes the basis of blood-saving strategies in open-heart surgery. For low-weight patients, in particular neonates and infants, this implies avoidance of excessive hemodilution during extracorporeal circulation. The circuit has to be miniaturized and tubing must be cut as short as possible to reduce the priming volume to prevent unacceptable hemodilution with initiating CPB. During perfusion, measures should be taken to prevent blood loss from the primary circuit to avoid replacement by additional volume. Favorable factors such as mild hypothermia/normothermia and high heparin concentrations during extracorporeal circulation promote earlier hemostasis after coming off bypass.Lower mortality score, first chest entry, higher hemoglobin concentration before going on bypass, and shorter CPB duration support transfusion-free CPB procedure. Reduced postoperative morbidity and mortality were observed when CPB was performed without blood transfusion. In our experience, this can be achieved in at least 70% of CPBs, even in low-weight patients.Bloodless CPB circuit priming should become a widespread reality, even in neonates and young infants, in any open-heart procedure.

Asanguineous Cardiopulmonary Bypass in Infants: Impact on Postoperative Mortality and Morbidity.

BACKGROUND: We routinely start cardiopulmonary bypass (CPB) for pediatric congenital heart surgery without homologous blood, due to circuit miniaturization, and blood-saving measures. Blood transfusion is applied if hemoglobin concentration falls under 8 g/dL, or it is postponed to after coming off bypass or after operation. How this strategy impacts on postoperative mortality and morbidity, in infants weighing ≤ 7 kg? METHODS: Six-hundred fifteen open-heart procedures performed from January 2014 to June 2018 were selected. One-hundred sixty-three patients (26.5%) were transfused on CPB (group 1), while 452 (73.5%) patients were not transfused on CPB (group 2). Operative risk and complexity were similar in both groups. Postoperative mortality and morbidity were compared. Multiple logistic regression was used to detect factors independently associated with outcome. RESULTS: Observed mortality in nontransfused group (0.7% = 3/452) was significantly lower than expected (4.2% = 19/452): p = 0.0007, and much lower than in transfused group (6.7% = 11/163): p < 0.0001. CPB transfusion (p = 0.001) was independently associated with mortality, either acting as the sole factor or in combination with the Society of Thoracic Surgeons morbidity score (p = 0.013). Patients not transfused during CPB required less frequently vasoactive inotropic drugs (p = 0.011) and duration of their mechanical ventilation was shorter (93 ± 134 hours) than for transfused patients (142 ± 170 hours): p = 0.0003. CPB transfusion was an independent determinant factor for morbidity (p = 0.05), together with body weight (p < 0.0001), vasoactive inotropic score (p < 0.0001), CPB duration (p = 0.001), and postoperative transfusion (p = 0.009). CONCLUSION: The strategy of transfusion-free CPB course, feasible in most patients ≤ 7kg, was associated with improved outcome. Asanguineous priming of CPB circuit should become standard, even in neonates and infants.

Bilateral Pulmonary Artery Banding before Norwood Procedure: Survival of High-Risk Patients.

BACKGROUND: This study reports midterm results of high-risk patients with hypoplastic left ventricle treated with initial bilateral pulmonary artery banding (PAB) before secondary Norwood procedure (NP). METHODS: Retrospective study of 17 patients admitted between July 2012 and February 2017 who underwent this treatment strategy because diagnosis or clinical status was associated with high risk for NP. Survival was compared with that of patients who underwent primary NP. RESULTS: Mean Aristotle comprehensive complexity score for NP would have been 19.7 ± 2.6. Risk factors included obstructed pulmonary venous return (n = 9), body weight < 2.5 kg (n = 7), total anomalous pulmonary venous connection (n = 3), and necrotizing enterocolitis (n = 1). Ten patients had a score ≥ 19.5. Early survival after PAB was 82.4% (14/17). NP was performed in 14 patients after improvement of clinical condition at a median age of 56 days and a weight ≥2,500 g. There was no 30-day mortality, but one interstage death. One patient died later after Glenn operation. One-year survival after primary PAB followed by NP was 70.6 ± 11.1%. During the same period, 35 patients with overall lower risk factors underwent primary NP; early postoperative survival and 1-year survival were 88.6 ± 5.4% and 68.6 ± 7.8%, respectively. There was no significant difference in survival between the two groups (p = 0.83) despite higher risk in the secondary Norwood group (p <0.0001). CONCLUSIONS: PAB before NP in high-risk patients constituted salvage management. Primary PAB provided enough time for stabilization and control of most risk factors. It allowed midterm survival equivalent to the survival after primary NP in lower risk neonates.

Bloodless priming of the cardiopulmonary bypass circuit: determinants of successful transfusion-free operation in neonates and infants with a maximum body weight of 7 kg.

We currently perform open-heart procedures using bloodless priming of cardiopulmonary bypass circuits regardless of a patient's body weight. This study presents results of this blood-saving approach in neonates and infants with a body weight of up to 7 kg. It tests with multivariate analysis factors that affect perioperative transfusion. A total of 498 open-heart procedures were carried out in the period 2014-2016 and were analysed. Priming volume ranged from 73 ml for patients weighing up to 2.5 kg to 110 ml for those weighing over 5 kg. Transfusion threshold during cardiopulmonary bypass was 8 g/dl of haemoglobin concentration. Transfusion factors were first analysed individually. Variables with a p-value lower than 0.2 underwent logistic regression. Extracorporeal circulation was conducted without transfusion of blood in 335 procedures - that is, 67% of cases. Transfusion-free operation was achieved in 136 patients (27%) and was more frequently observed after arterial switch operation and ventricular septal defect repair (12/18=66.7%). It was never observed after Norwood procedure (0/33=0%). Lower mortality score (p=0.001), anaesthesia provided by a certain physician (p=0.006), first chest entry (p=0.013), and higher haemoglobin concentration before going on bypass (p=0.013) supported transfusion-free operation. Early postoperative mortality was 4.4% (22/498). It was lower than expected (6.4%: 32/498). In conclusion, by adjusting the circuit, cardiopulmonary bypass could be conducted without donor blood in majority of patients, regardless of body weight. Transfusion-free open-heart surgery in neonates and infants requires team cooperation. It was more often achieved in procedures with lower mortality score.

Routine Application of Bloodless Priming in Neonatal Cardiopulmonary Bypass: A 3-Year Experience.

A restrictive transfusion strategy led us to routinely try to conduct donor-blood free open-heart surgery even in neonates. The cardio-pulmonary bypass (CPB) circuit was minimized by priming volumina at 73 ml for the smallest patients with body weight up to 2.5 kg and 85-95 ml for those with body weight of more than 2.5 kg, and by positioning the console as close as possible to operation table. Measures were applied to save blood during the procedure. Transfusion threshold of 8 g/dl hemoglobin was retained. Effort was made to avoid transfusion while on CPB or to postpone transfusion towards CPB end. From 2013 to 2015, 149 consecutive neonates underwent 150 open-heart procedures without blood in priming volume. Weight was lower than 2.5 kg in five instances. The most frequent operations were arterial switch operation (n = 54) and Norwood procedure (n = 17). Transfusion-free operation was achieved in 44 procedures. The great majority (42/44 = 95%) involved biventricular repair and included 50% (27/54) of arterial switch operations. 106 patients were transfused: 63 mostly towards CPB end, and 43 after coming off bypass. Transfusion-free procedures were associated with postoperative lower lactate concentration (p = 0.0013) and shorter duration of mechanical ventilation (p = 0.0009). Seven patients were discharged from hospital without getting any transfusion of blood or blood products. In conclusion, routine application of bloodless priming in neonatal cardiopulmonary bypass is safe and beneficial. It results into a good number (29%= 44/150) of transfusion-free operations. Postponing transfusion towards CPB end favors an overall restrictive transfusion strategy for all patients.

Prolonged Activated Clotting Time after Protamine Administration Does Not Indicate Residual Heparinization after Cardiopulmonary Bypass in Pediatric Open Heart Surgery.

BACKGROUND: In open heart surgery, heparinization is commonly neutralized using an empirical heparin:protamine ratio ranging between 1:1 and 1:1.5. However, these ratios may result in protamine overdose that should be avoided for its negative side effects on the coagulation system. This study aimed to indicate the appropriate treatment for prolonged activated clotting time (ACT) after protamine administration following cardiopulmonary bypass (CPB) in pediatric open heart surgery by investigating the underlying reasons for it. METHODS: Twenty-seven children (<10 kg) undergoing open heart surgery were included. Heparin was administered only before CPB (400 IU/kg) and in the pump priming volume for CPB (2,000 IU) and was neutralized by 1:1 protamine after CPB. The blood heparin concentration was measured using anti-Xa assay. ACT and blood concentrations of heparin, coagulation factors, thrombin-antithrombin complex, and prothrombin fragment 1 + 2 were assessed. A rotational thromboelastometry (ROTEM; Tem International GmbH, München, Bayern, Germany) was used to confirm the coagulation status and residual heparin after protamine administration. RESULTS: Anti-Xa assay showed that there is no residual heparin in the blood after 1:1 protamine administration. Nevertheless, ACT (128.89 ± 3.09 seconds before heparin administration) remained prolonged (177.14 ± 5.43 seconds at 10 minutes after protamine, 182.00 ± 5.90 seconds at 30 minutes after protamine). The blood concentrations of coagulation factors were significantly lower than those before heparin administration (p < 0.01). The low FIBTEM MCF of ROTEM (4.43 ± 0.32 mm) at 10 minutes after protamine indicated low fibrinogen concentration. CONCLUSION: Prolonged ACT after heparin neutralization by 1:1 protamine administration does not necessarily indicate residual heparin, but low blood concentrations of coagulation factors should be considered as a reason as well. Accordingly, supply of coagulation factors instead of additional protamine should be considered.

Restrictive Atrial Septum Defect Becomes a Risk Factor for Norwood Palliation of Hypoplastic Left Heart Syndrome Only When It Is Combined with Mitral or Aortic Atresia.

BACKGROUND: Restrictive atrial septal defect (ASD) is described as risk factor for Norwood procedure because of elevated pulmonary resistance. We hypothesized that it invariably could not cause pulmonary hypertension, unless it was combined with mitral valve or aortic valve atresia. We investigated how restrictive ASD influenced survival of patients with hypoplastic left heart syndrome (HLHS) who underwent Norwood operation. PATIENTS AND METHODS: A total of 118 HLHS patients who underwent surgery between January 2005 and December 2012 were grouped into three groups. Group 1 included 31 patients with restrictive ASD combined with mitral or aortic atresia; Group 2 composed of 12 patients with restrictive ASD and mitral and aortic stenosis; Group 3 (n = 75) had no ASD restriction. Survival was determined for each group. Multivariate analysis was conducted to test risk factors for mortality. RESULTS: Mean follow-up was 26.3 ± 24.1 months. Survival was 78.7% ± 4.2% at 30-month interval and onward after Norwood procedure for the whole cohort; it was 43.8% ± 10.0%, 91.7% ± 8.0%, and 77.3% ± 5.0% for Group 1, 2, and 3, respectively. The difference was significant between Group 1 and Group 2 and 3: p < 0.001. Survival was similar for Group 2 and Group 3: p = 0.45. Combination of restrictive ASD and mitral or aortic atresia was found to be the sole risk factor for early and late mortality (odds ratio: 3.5, 95% confidence interval: 1.8-7.1, p < 0.001). CONCLUSION: Restrictive ASD only affects survival of HLHS patients following Norwood procedure if it is associated with mitral or aortic atresia.

Reimbursement by current German diagnosis-related groups system penalises complex congenital heart surgery.

A total of 458 hospital stays during the year 2011 were analysed to determine whether reimbursement by the current German Diagnosis-Related Groups system covers the costs incurred during hospital stay for congenital heart surgery. The costs of every hospital stay were estimated according to the guidelines of the Institute for the Hospital Remuneration System, an institute responsible for encoding hospital reimbursement in Germany. Cost-weight values of the year 2012 were applied for reimbursement. Related additional compensations were also included. Hospital costs ranged from 8896.26 to 193,671.94 euros per case, with a mean of 30,597 and standard deviation of 25,032 euros. Reimbursement varied from 8630.35 to 173,710.65 euros, with a mean of 25,514 and standard deviation of 18,497 euros: an underfunding of 17%. Fifty-nine per cent (271/458) of cases were classified, according to Aristotle complexity score, in higher comprehensive complexity: Levels 4-6. Costs highly correlated with complexity levels (Spearman's r coefficient = 0.89) and the regression was linear. Underfunding increased, linearly, from 6% for procedures with Level 1, lowest comprehensive complexity, to 23% for those with Level 6, highest complexity. In conclusion, this study demonstrates that reimbursement by the current German Diagnosis-Related Groups system increasingly penalises complex congenital heart surgery. Aristotle complexity score could help to correct this prejudicial situation.

Quantification of morbidity associated with congenital heart surgery.

OBJECTIVE: The objective of this study was to estimate the morbidity according to observed complications after congenital heart surgery over 1-year period. METHODS: The previously established list of conditions prone to affect patients' well-being or increase cost of in-hospital stays was used systematically to score the severity of postoperative complications from 1 to 4 points. The morbidity score was calculated by adding the scores of observed complications. When the sum amounted to more than 5 points, a morbidity score of only 5 points was attributed. If no complication was detected, a score of 0.5 points was assigned. The resulting morbidity scores were correlated with the length of stay in the intensive care unit (ICU) and in the hospital, the duration of mechanical ventilation, and Aristotle complexity scores. RESULTS: A total of 542 primary procedures performed in the year 2011 were studied. Aristotle basic and comprehensive scores amounted to 7.78 ± 2.65 and 10.15 ± 3.83, respectively. Mortality was 1.85% (10/542). The standardized ratio of surgical performance reached 103.10%. Total cavopulmonary connection with extracardiac fenestrated conduit constituted the most frequent operation (n = 34). No complication was observed following 183 (33.8%) procedures. More than two complications were observed in 114 cases (21%). The three most frequent unfavorable conditions were "mechanical ventilation 25 to 95 hours" (n = 150), low cardiac output syndrome (n = 56), and cardiac arrhythmia requiring medication (n = 50). The estimated mean morbidity score amounted to 2.26 ± 1.80 points. Scores ranged from 0.68 ± 0.50 for primary closure of atrial septal defect to 4.50 ± 0.79 for the Norwood procedure. They were perfectly related to the length of ICU stay and to the duration of mechanical ventilation (Spearman coefficient r = 1). Correlation was high with the length of hospital stay (r = 0.83), Aristotle basic score (r = 0.89) (r = 0.96), and comprehensive score (r = 0.94) (C-index = 0.97). The observed mean morbidity score was statistically not different from the expected mean morbidity score according to the basic Aristotle complexity: p = 0.73. CONCLUSION: Quantification of morbidity indicates the length of ICU stay and the duration of mechanical ventilation as the best surrogates for morbidity. Such benchmarking and scoring of observed postoperative complications paves the way for an accurate assessment and improvement of quality care in congenital heart surgery.

Interventions after Norwood procedure: comparison of Sano and modified Blalock-Taussig shunt.

Improved results have evolved from the modified Norwood procedure (NP). This study compares the incidence of interventions after NP with the Sano (n = 37) and modified Blalock-Taussig (BT n = 70) shunt. Incidence, location, interval of interventions, and weight were retrospectively analysed for 107 neonates undergoing NP during the period from October 2002 to December 2009. Forty-six (43.0 %) patients underwent interventions, mostly for dilatation of the aortic arch ([DAA] n = 26 [24.3 %]; Sano n = 10, BT n = 16, p = 0.6), dilatation of the shunt ([DS] n = 15 [14.0 %]; Sano n = 11, BT n = 4; p = 0.002), or closure of aortopulmonary collaterals ([APC] n = 15 [14.0 %]; Sano n = 3, BT n = 12; p = 0.08). Mean interval after NP and body weight at DAA, DS, and APC were 72.4 ± 18.9, 108.5 ± 15.8, and 110.7 ± 17.8 days and 4.5 ± 1.3, 4.9 ± 1.9, 5.3 ± 1.2 kg, respectively. The interventions were not associated with mortality but with a greater rate of complications (9 of 46 [21.4 %]) compared with the rate after diagnostic catheterization (0 of 45, p = 0.03). Complications included closure of the femoral or subclavian artery (n = 5), cerebral embolic or bleeding events (n = 4), cardiopulmonary resuscitation (n = 3), and temporary heart block (n = 2). Actuarial survival was similar from the postoperative month 8 onward at 78.6 ± 4.9 % (95 % confidence interval [CI] 67.0-86.5 %) for Sano and 78.4 ± 6.8 % (95 % CI 61.4-88.6 %) for BT (p = 0.95). Interventions after NP were common irrespective of shunt type. However, a significantly greater rate of shunt interventions was noted in the Sano group. In particular, interventions addressing the aortic arch and the shunt were related with a significant rate of complications.

Does bilateral pulmonary banding in comparison to Norwood procedure improve outcome in neonates with hypoplastic left heart syndrome beyond second-stage palliation? A review of the current literature.

Best evidence protocol was applied and the question addressed, whether there is an outcome advantage of a hybrid approach including bilateral pulmonary banding (BPB) and ductal stenting over Norwood procedure (NP) to maintain systemic circulation and to restrict pulmonary blood flow in neonates with hypoplastic left heart syndrome (HLHS). Out of 80 articles published since the original description of BPB in 2002, eight were selected to answer the clinical question. All studies were retrospective case-series after BLB, only three compared results with NP controls, but not in a randomized fashion. Only three chose high-risk patients. Mean follow-up interval was mentioned only in one study. Reported mean hospital, interstage, and stage 2 mortalities after BPB was 17.3, 12.4, and 25.3%, respectively. Estimated transplant-free survival beyond stage 2 palliation ranged from 7 to 80%, with no significant differences to NP controls in three studies. Reintervention rate was high, ranging from 20 to 42% at different sites. Due to study design or inhomogeneity of patient groups, current literature does not show evidence that bilateral pulmonary banding improves outcome with respect to survival beyond second-stage HLHS palliation.

Aortopulmonary window associated with interrupted aortic arch: report of surgical repair of eight cases and review of literature.

BACKGROUND: Patients with combined aortopulmonary window (APW) and interrupted aortic arch (IAA) malformations are rarely seen. We reviewed cases with such association with emphasis on surgical management and long-term outcome. METHODS: 109 patients with IAA and 25 patients with APW were operated upon between 1981 and 2011. The clinical records, operation and follow-up data were analyzed. Long-term outcome was completed with the help of either outpatient data or inquiring. Related literature was investigated. RESULTS: Combined APW/IAA was found in 8 cases: 7.3% (8/109) of those with IAA diagnosis and 32% (8/25) with APW. All APWs were proximal. 7 patients had interruption type A. A ventricular septal defect (VSD) was associated in one case only. Median age at surgery was 10 days. The two first patients were operated upon without cardio-pulmonary bypass (CPB) and one died during operation. The other six underwent single stage approach under CPB with no death: overall early mortality of 12.5% (⅛). APW was closed with one or two patches; aortic arch was reconstructed either directly (extended end-to-side anastomosis) or by patch augmentation. There was no late death and no reoperation during mean follow-up of 118 months (range 1-360 months). Six patients were in functional NYHA class I, the remainder in class II. The cumulative APW/IAA incidence from literature varies between 4.9% (56/1105) and 22.2% (42/189). Early mortality reaches 15.1% (8/53) (95% CI: 6.8% - 27.6%). CONCLUSION: Neonatal repair with thorough mobilization of the aortic arch and extended end-to-side anastomosis or use of patch augmentation carries potential for the best early and late outcome for combined APW/IAA malformation.

Surgical management of congenital heart disease: contribution of the Aristotle complexity score to planning and budgeting in the German diagnosis-related groups system.

Planning and budgeting for congenital heart surgery depend primarily on how closely reimbursement matches costs and on the number and complexity of the surgical procedures. Aristotle complexity scores for the year 2010 were correlated with hospital costs and with reimbursement according to the German diagnosis-related groups (DRG) system. Unit surgical performance was estimated as surgical performance (complexity score × hospital survival) times the number of primary procedures. This study investigated how this performance evolved during years 2006 to 2010. Hospital costs and reimbursements correlated highly with Aristotle comprehensive complexity levels (Spearman r = 1). Mean costs and reimbursement reached 35,050 ± 32,665 and 31,283 ± 34,732, respectively, for an underfunding of 10.7%. Basic and comprehensive unit surgical performances were respectively 3036 ± 1009 and 3891 ± 1591 points in 2006. Both performances increased in sigmoid fashion to reach 3883 ± 1344 and 5335 ± 1314 points, respectively, in 2010. Top performances would be achieved in year 2011, and extrapolated costs would comprise about 19,434,094.92 (95% confidence interval, 11,961,491.22-22,495,764.42). The current underfunding of congenital heart surgery needs correction. The Aristotle score can help to adjust reimbursement according to complexity of procedures. Unit surgical performance allows accurate budgeting in the current German DRG system.

Mid-term performance of decellularized equine pericardium in congenital heart surgery.

OBJECTIVE: The aim was to report mid-term performance of decellularized equine pericardium used for repair of various congenital heart defects in the pediatric population. METHODS: A retrospective review of all patients undergoing patch implantation between 2016 - 2020 was performed. Patch quality, surgical handling, hemostasis and early patch-related complications were studied on all patients. Mid-term performance was observed in patients with ≥12 months follow-up and intact patch at discharge (without reoperation/stent implantation). RESULTS: A total of 201 patients with median age of 2.5 years [interquartile range (IQR): 0.6-6.5] underwent 207 procedures at 314 implant locations. The patch was used in following numbers/locations: 171 for pulmonary artery (PA) augmentation, 36 for aortic repair, 35 for septal defect closure, 22 for valvular repair and 50 at other locations. Early/30-day mortality was 6.5%. Early patch-related reoperations/stent implantations occurred in 28 locations (8.9%). No patch-related complications were noted except for bleeding from implant site in three locations (1%). Follow-up ≥ 12 months was available for 132 patients/200 locations. During a median follow-up of 29.7 months [IQR: 20.7-38.3], 53 patch-related reoperations/catheter reinterventions occurred (26.5%) with the majority in PA position (88.7%, 47/53). Overall 12- and 24-months freedom from patch-related reoperation/catheter reintervention per location was 91.5% (95% CI: 86.7%-94.6%) and 85.2% (95% CI: 78.9%-89.6%) respectively. CONCLUSION: Decellularized equine pericardium used for repair of various congenital heart defects showed acceptable mid-term performance. Reoperation/reintervention rates were in a range as observed with other xenogeneic materials previously reported articles, occurring most frequently after PA augmentation.

Surgical management of Ebstein anomaly: impact of the adult congenital heart disease anatomical and physiological classifications.

OBJECTIVES: Our goal was to evaluate the impact of the adult congenital heart disease anatomical and physiological (ACHD AP) classification system on the surgical management of Ebstein anomaly (EA) in adult patients. METHODS: From February 2000 through August 2017, data of patients aged at least 16 years, who underwent primary EA surgery, were retrospectively evaluated. The cohort was divided in 2 groups according to their ACHD AP classification: the moderate EA group (IIB, IIC) and the severe EA group (IID). Survival, freedom from reoperation and freedom from occurrence of major adverse advents were estimated. RESULTS: There were 33 patients (21 women, 12 men). Eighteen belonged to the moderate group, 15 to the severe group. There were 12 female patients (80%) in the severe group. Patients in the moderate group were younger than those in the severe group (P = 0.02): 32 ± 12 vs 44 ± 15 years old. Thirty tricuspid valve repairs and 3 replacements were performed. Repair was mainly performed in the moderate group (P = 0.02). Overall survival was 90.1 ± 5.4% at 9 months after the operation and did not change in the later follow-up period. It was 100% for patients in the moderate group and 80.0 ± 10.3% in the severe group (P = 0.07), and 75.0 ± 12.5% for female patients of in the severe group compared to 100% for the remaining patients (P = 0.025). Survival free from major adverse events, including reoperation, at 10 years was 60.0 ± 12.6% in the moderate and 38.1% ± 12.9% in the severe group (P = 0.03). No patient in the moderate group evolved to be in the severe group at late follow-up. CONCLUSION: Adult EA patients should undergo surgery earlier when they are still in the moderate ACHD AP classification.

Acute Kidney Injury After Neonatal Aortic Arch Surgery: Deep Hypothermic Circulatory Arrest Versus Moderate Hypothermia With Distal Aortic Perfusion.

BACKGROUND: Acute kidney injury (AKI) is a common complication observed after neonatal aortic arch repair. We studied its incidence after procedures carried out using deep hypothermic circulatory arrest (DHCA) versus moderate hypothermia with distal aortic perfusion (MHDP), usually through the common femoral artery. In both groups, continuous regional cerebral perfusion (RCP) was used during the time required for aortic arch repair. METHODS: A total of 125 neonates underwent aortic arch repair. Between 2007 and 2012, DHCA with RCP was used in 51 neonates. From 2013 to 2019, MHDP with RCP was performed on 74 newborns. Operative complexity was similar in both periods. Acute kidney injury was defined as a significant elevation of serum creatinine and was classified according to the neonatal modified n-KDIGO (neonatal Kidney Disease: Improving Global Outcomes) stages 1 to 3 (Kidney Disease Improving: Global Outcomes). RESULTS: Acute kidney injury was observed in a total of 68 patients (68/125: 54.4%). In the majority (44/68: 64.7%), n-KDIGO stage 1 occurred. Stage 2 (n = 14) and stage 3 (n = 10) were observed more frequently after DHCA versus MHDP: 29.4% (15/51) versus 12.2% (9/74), P = .02. At cardiopulmonary bypass end, lactate levels were significantly higher (P = .001) after DHCA: 3.4 (2.9-4.3) mmol/L compared to 2.7 (2.3-3.7) mmol/L after MHDP. Early mortality was 12% (15/125) in the entire cohort. It was 17.6% (9/51) after DHCA versus 8.1% (6/74) after MHDP, however not statistically significant (P = .16). CONCLUSION: Mild (stage 1) AKI occurred frequently after neonatal aortic arch repair. The use of MHDP was associated with a significantly lower incidence of moderate (stage 2) and severe (stage 3) AKI forms.

Fast-track extubation after cardiac surgery in infants: Tug-of-war between performance and reimbursement?

OBJECTIVES: To compare the safety and resource-efficacy of the fast-track (FT) concept (extubation ≤8 hours after surgery) versus the conventional approach (non-FT, >8 hours postoperatively) in infants undergoing open-heart surgery. METHODS: Infants <7 kg operated on cardiopulmonary bypass between 2014 and 2018 were analyzed. Propensity score matching (1:1) was performed for group comparison (FT vs non-FT). Intensive care unit (ICU) personnel use and unit performance were evaluated. Postoperative outcome and reimbursement based on German diagnosis-related groups were compared. RESULTS: Of 717 infants (median age: 4 months, Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality score: 0.1-4), FT extubation was achieved in 182 infants (25%). After matching, 123 pairs (FT vs non-FT) were formed without significant differences in baseline characteristics. FT versus non-FT showed a significantly shorter ICU stay (in days): 1.8 (0.9-2.8) versus 4.2 (1.9-6.4), P < .01, and postoperative length of stay (in days): 7 (6-10) versus 10 (7-15.5), P < .01; significantly lower postoperative transfusion rates: 61.3% versus 77%, P < .01; and tendency toward lower early mortality: 0% versus 2.8%, P = .08. Reintubation rate did not differ between the groups (P = .7). Despite a decrease in personnel capacity (2014 vs 2018), the unit performance was maintained. The mean case-mix-index of FT versus non-FT was 8.56 ± 6.08 versus 11.77 ± 12.10 (P < .01), resulting in 27% less reimbursement in the FT group. CONCLUSIONS: FT concept can be performed safely and resource-effectively in infants undergoing open-heart surgery. Since German diagnosis-related group systems reimburse costs, not performance, there is little incentive to avoid prolonged mechanical ventilation. Greater ICU turnover rates and excellent postoperative outcomes are not rewarded adequately.

Subcoronary Ross/Ross-Konno operation in children and young adults: initial single-centre experience.

OBJECTIVES: We sought to evaluate the outcome after modified subcoronary Ross/Ross-Konno operation in children and young adults. METHODS: Between January 2013 and January 2019, a total of 50 patients with median age of 6.3 years (range 0.02-36.5 years, 58% males), including 10 infants (20%), received modified subcoronary Ross/Ross-Konno operation at our institution. Survival, morbidity, reinterventions, aortic valve function and aortic root dimensions were analysed. RESULTS: At a median follow-up of 31.2 months (range 14.4-51 months), there were 1 early death and 1 late death, both in the infant group. The overall survival at 5 years after the operation was 95%. Two patients needed aortic valve replacement, 11 and 15 months after their Ross operation. At 5 years, freedoms from reoperation on the autograft and on the right ventricle to pulmonary artery conduit were 94% and 97%, respectively. Freedom from aortic valve regurgitation greater than mild was 97% at 5 years. Median dimensions of the aortic root at all levels remained in normal range at last visit. Forty-four patients (95%) were in New York Heart Association class I with normal left ventricular function. CONCLUSIONS: The initial experience with the subcoronary Ross/Ross-Konno operation in children and young adults showed excellent outcome. The mortality and morbidity among infants remain significant. The described technique is reproducible and might be advantageous in situations when prosthetic supporting techniques interfere with somatic growth.

Reimbursement After Congenital Heart Surgery in Germany: Impact of Early Postoperative Extubation.

BACKGROUND: Duration of mechanical ventilation is an important variable used by German Diagnosis-Related Groups (G-DRG) system to establish cost weight values for reimbursement after congenital heart surgery. Infants are commonly ventilated after open heart surgery. As of year 2015, we strived to achieve early postoperative extubation. This work studies how this approach impacted reimbursement after infant open heart surgery. METHODS: Data of infants who underwent surgery on cardiopulmonary bypass (CPB) from 2014 to 2018 were reviewed. Successful early extubation was defined as end of mechanical ventilation within 24 hours postoperatively, without reintubation at a later point. Mean cost weight values (case mix index [CMI]) of achieved DRGs were used for estimation of reimbursement. Evolutions over years of early extubation and of reimbursement were compared. RESULTS: A total of 521 infants underwent operations on CPB. Of these, 161 (31%) procedures were of higher risk Society of Thoracic Surgery and the European Association for Cardio-Thoracic Surgery (STAT) categories 3 and 4. Early extubation was achieved in 205 (39%) patients. The rate increased from 14% (year 2014) to 57% (year 2018). Case mix index amounted to 8.87 ± 7.00 after early extubation, and 12.37 ± 7.85 after late extubation: P value <.0001. It was 8.77 ± 6.09 after early extubation in patients undergoing lower risk STAT categories 1 and 2 operations, and 8.09 ± 2.95 when categories 3 and 4 procedures were performed (P = .18). An overall 14.4% decrease in hospital reimbursement per patient was observed. CONCLUSION: Early extubation could be progressively obtained in the majority of infants. This resulted in lower reimbursement. Surgical complexity was disregarded. The current G-DRG system appears to favor longer mechanical ventilation durations after infant open heart surgery.