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Objective: We sought to identify the impact of pulmonary artery size on outcomes after nonfenestrated total cavopulmonary connection. In a subgroup of patients with right-sided bidirectional cavopulmonary shunt, the impact of each branch pulmonary artery size was individually determined., Methods: The medical records of all patients who underwent nonfenestrated total cavopulmonary connection between 2009 and 2021 were reviewed. The pulmonary artery index was calculated using angiography before the operation., Results: A total of 247 patients were included in this study. A right-sided bidirectional cavopulmonary shunt was performed in 217 patients (88%). Median pulmonary artery index was 162 (133-207) mm 2 /m 2 before total cavopulmonary connection. Chylothorax occurred in 55 patients (22%). Pulmonary artery index was an independent factor for chylothorax (odds ratio, 0.98, 95% confidence interval, 0.97-0.99, P < .001) with a cutoff value of 170 mm 2 /m 2 . In a subgroup of patients with right-sided bidirectional cavopulmonary shunt, the left pulmonary artery index was identified as an independent risk factor for longer stay in the intensive care unit (coefficient B -0.02, 95% confidence interval, -0.04 to -0.002, P = .034) and for adverse events (hazard ratio, 0.98, 95% confidence interval, 0.96-0.99, P = .011) with a cutoff value of 56 mm 2 /m 2 ., Conclusions: The pulmonary artery index is significantly associated with the occurrence of chylothorax after nonfenestrated total cavopulmonary connection with a cutoff value of 170 mm 2 /m 2 . In patients with right-sided bidirectional cavopulmonary shunt, left pulmonary artery index has a significant predictive value for longer stay in the intensive care unit and adverse events with a cutoff value of 56 mm 2 /m 2 ., (Copyright © 2022 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: We sought to investigate the impact of early postoperative low arterial oxygen saturation on mortality and morbidity after bidirectional cavopulmonary shunt (BCPS)., Methods: The medical records of all patients who underwent BCPS between 2013 and 2018 were reviewed., Results: A total of 164 patients were included in this study. Forty-seven patients underwent reintervention during hospital stay at median 7 days after BCPS. Before reintervention, 30 patients were intubated or had SpO2 of <75%. All re-interventions for Glenn pathway obstruction and 4 out of 5 venovenous coil embolization resulted in hospital discharge, while high mortality was observed after other re-interventions (atrioventricular valve surgery, thrombolysis, systemic ventricular outflow obstruction relief, extracorporeal membrane oxygenation implantation and diaphragmatic plication). Additional aortopulmonary shunt with pulmonary artery discontinuation was performed in 8 patients who showed severe cyanosis with median SpO2 of 59% under maximal ventilation support. In the univariable Cox regression analysis, the associated factors for mortality before total cavopulmonary connection were reduced ventricular function [hazard ratio (HR) 6.89, 95% confidence interval (CI) 1.76-26.9, P-value 0.006], greater than moderate atrioventricular valve regurgitation (HR 5.89, 95% CI 1.70-20.4, P-value 0.005), SpO2 1 h after extubation (HR 0.87, 95% CI 0.80-0.96, P-value 0.004) and mean pulmonary artery pressure 1 h after extubation (HR 1.14, 95% CI 1.02-1.26, P-value 0.016)., Conclusions: After BCPS, unacceptable cyanosis persisted with various aetiologies. Low arterial oxygen saturation within 1 h after extubation is significantly associated with high mortality after BCPS., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: This study sought to determine long-term outcomes of a primary Damus-Kaye-Stansel (DKS) procedure in patients with a functional single ventricle and to compare the results with those of our historical control subjects who underwent pulmonary artery banding before the DKS procedure., Methods: The study reviewed the medical records of all patients who underwent the DKS procedure at the German Heart Center of Munich, Germany between December 1994 and December 2019., Results: The DKS procedure was performed as initial palliation in 52 patients (primary DKS group) and as staged palliation after pulmonary artery banding in 24 patients (staged DKS group). The median follow-up period after the DKS procedure was 8.9 years in the primary DKS group and 8.0 years in the staged DKS group. The survival rates at 10 years after the DKS procedure were 89% in the primary DKS group and 68% in the staged DKS group (log-rank P = 0.04). Before total cavopulmonary connection, the pressure gradient through the systemic ventricular outflow tract was significantly lower in the primary DKS group than in the staged DKS group (P < .001). At last follow-up echocardiography, reduced ventricular function was observed in 1 patient in the primary DKS group and in 7 patients in the staged DKS group (P < .001). The degree of neoaortic regurgitation was significantly higher in the staged DKS group than in the primary DKS group (P < .001)., Conclusions: A primary DKS procedure in patients with a functional single ventricle and potential systemic ventricular outflow tract obstruction is recommended to obtain favorable long-term survival with preserved ventricular function and competent semilunar valve function., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: Heterotaxy is a known risk factor for morbidity and mortality in single ventricle palliation. In this study, we examined our experience with this challenging group of patients., Methods: Records of patients born between 2001 and 2019 with heterotaxy, who needed staged single ventricle palliation were retrospectively analysed., Results: A total of 53 patients were included in this study. Thirty-five (66%) patients had a right ventricular dominance, common atrioventricular septal defect was present in 37 (70%) patients. Anomalous pulmonary venous drainage was present in 29 (55%) patients. Forty-six (86%) patients underwent first-stage palliation. Forty-one (77.3%) patients received a bidirectional cavopulmonary connection. Thirty-one (58%) patients received total cavopulmonary connection (TCPC). Overall survival rate was 92% at 1 month, 74% at 1 year and 68% at 10 years. Twelve (22.6%) patients died before second palliation stage. Four (10%) patients died before TCPC. No patient died after TCPC. Independent risk factors for mortality in the multivariate COX regression were a presence of restrictive pulmonary blood flow (HR 3.23; 95% CI 1.02-10.2; P = 0.05) and greater than mild atrioventricular valve regurgitation (HR 3.57; 95% CI 1.27-10.0; P = 0.02)., Conclusions: Mortality and morbidity in patients with single ventricle and heterotaxy are high. Restrictive pulmonary blood flow needing early modulation and greater than mild atrioventricular valve regurgitation at presentation are independent risk factors for mortality. Total anomalous pulmonary venous connection was not identified as a risk factor in the current era., (© The Author(s) 2021. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background  Understanding the anatomy and physiology of congenital heart defects is crucial for planning interventions in these patients. Congenital heart procedures often involve complex three-dimensional (3D) reconstructions. Excellent imaging techniques are required to depict all anatomical details. We have used and evaluated fast 3D prototyping technology for reconstruction and planning of corrections of complex congenital heart defects. Materials and Methods  3D physical models were constructed from contrast-enhanced computed tomography (CT) datasets of patients with complex congenital heart defect. Two different commercially available printing technologies were used and their clinical application compared. Results  Physical models of three different patients were used for preoperative surgical planning. All models showed good correspondence to patient anatomy. Both printing technologies gave excellent results. Conclusion  Physical models could be easily constructed with the use of CT datasets. The printing process could be done efficiently, quite rapidly, and cost effectively. Surgical corrections could be planned based on these models., (Georg Thieme Verlag KG Stuttgart · New York.)

Purpose: Implanting the largest valved conduit possible - oversizing - to reconstruct an absent connection from the right ventricle to the pulmonary artery in certain types of congenital heart defects has been suggested as a compensating measure for somatic outgrowth of the patient. However, one effect that has not been investigated yet is the hemodynamic consequence. For this purpose, virtual implantation and flow simulations were conducted in this study., Methods: To isolate the effects of conduit oversizing on the hemodynamics observed after conduit implantation and outgrowth, calculated wall shear stresses (WSS) of image-based computational fluid dynamic (CFD) simulations were used as indicators. Three different sizes of valved conduits (20 mm, 22 mm, and 24 mm), including the largest possible conduit size, virtually implanted in a child-sized healthy pulmonary artery and the corresponding adult-sized model were investigated., Results: The child and adult models show a decrease of the mean WSS (approx. 26%) in the whole domain with an increase of the conduit size. When looking at the mean WSS at the anastomosis, for the child model the WSS is significantly increased (approx. 40%) when oversizing (Z-score +3.21). In contrast, the stresses are decreased for the adult model (34%) when using the largest conduit (Z-score +0.25)., Conclusions: Based on the results of this study, it must be considered that choosing a prosthesis size that will lead to high WSS and an associated intimal reaction, possibly leading to stenosis, can defeat the benefit of having a nominally larger orifice area directly after implantation.

Background: Bicarotid trunk is the most common variation of aortic arch branching patterns. Bicarotid trunk can be a decisive factor in the surgical management of congenital heart defects. Our objective was to determine the prevalence of the bicarotid trunk among pediatric patients with congenital heart defects and to identify whether there is an association between bicarotid trunk and aortic coarctation., Methods: From a total of 4,371 pediatric cardiac catheterizations between the years 1979 and 2010, a group of 2,033 patients were selected. Prevalence of the aortic coarctation in patients with bicarotid trunk and with a normal branching pattern was determined by evaluating the catheterization reports. In addition, associated congenital defects, vascular anomalies, and genetic syndromes were also assessed., Results: Of 2,033 patients in our database, 84.8% of patients had a normal branching pattern of the aortic arch and 15.2% had a bicarotid trunk. The most frequent congenital heart defects in patients with a bicarotid trunk were ventricular septal defects, valvular pulmonary stenosis, and atrial septal defect. The most common associated vascular anomaly was the aberrant right subclavian artery. Most frequent genetic syndromes were trisomy 21, Williams syndrome, and CHARGE syndrome., Conclusions: The prevalence of bicarotid trunk of 17.6% among patients with aortic coarctation and 15.2% among the entire population studied made it not at all a rare phenomenon. Because bicarotid trunk can be a risk factor for surgery, the anatomy of the aortic arch needs to be clearly depicted and described. If ultrasonography examination cannot exclude bicarotid trunk, enhanced magnetic resonance imaging or aortic arch angiography may be necessary., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)