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Background: Patients requiring extracorporeal life support (ECLS) support post-Norwood operation constitute an extremely high-risk group. Materials and methods: We retrospectively aimed to evaluate the relationship of hyperoxia with mortality and other clinical outcomes in patients who required ECLS following Norwood operation between January/2010 and December/2020 in a large volume center. Results: During the study period 65 patients required ECLS post-Norwood. Using receiver operating characteristic (ROC) curve analysis, mean PaO2 of 182 mmHg in the first 48-hour on ECLS was determined to have the optimal discriminatory ability for mortality (sensitivity 68%, specificity 70%). Of the 65 patients, 52% had PaO2 > 182 mmHg and were designated as hyperoxia group. Patients in the hyperoxia-group had longer cardiopulmonary bypass time (187 vs. 165 min, p = 0.023), shorter duration from CICU arrival to ECLS-cannulation (13.28 vs. 132.58 h, p = 0.003), higher serum lactate within 2-hours from ECLS-canulation (14.55 vs. 5.80, p = 0.01), higher ECLS flows in the first 4-hours (152.68 vs. 124.14, p = 0.006), and higher mortality (77% vs. 39%, p = 0.005). In the unadjusted-analysis, using a derived cut-point, patients in the hyperoxia-group had 5.15 higher odds of mortality (p = 0.003). However, this association was insignificant when adjusting for confounding variables (p = 0.104). Using a functional status scale, new morbidity (38% vs. 21%), and unfavorable outcomes (13% vs. 5%) were higher in the hyperoxia group. Despite being higher in the hyperoxia group, this did not reach statistical significance. Conclusion: Neonates with hyperoxia (PaO2 > 182 Torr) during the first 48-hour of ECLS post-Norwood operation had 5 times higher odds of mortality in the unadjusted analysis, however, this was insignificant when adjusting for confounding variables. Patients in the hyperoxia group had shorter duration from CICU arrival to ECLS-cannulation, higher serum lactate prior to ECLS-canulation, and higher ECLS flows in the first 4-hours, (p

Leadless pacemakers have provided new treatment modalities that can be especially useful in patients with complex cardiac anatomy and contraindications toward other pacemaker approaches. The Aveir™ single-chamber (VR) leadless pacemaker (LP) (Abbott Laboratories, Chicago, IL) is a recently approved device that can be placed in the right ventricle for patients with bradycardia. In this case, we present a novel use for the device through placement in the atrium to control atrial flutter in a patient with a hypoplastic right ventricle.

Leadless pacemakers have provided new treatment modalities that can be especially useful in patients with complex cardiac anatomy and contraindications toward other pacemaker approaches. The Aveir™ single-chamber (VR) leadless pacemaker (LP) (Abbott Laboratories, Chicago, IL) is a recently approved device that can be placed in the right ventricle for patients with bradycardia. In this case, we present a novel use for the device through placement in the atrium to control atrial flutter in a patient with a hypoplastic right ventricle.

BackgroundUneven hepatic venous blood flow distribution (HFD) to the pulmonary arteries is hypothesized to be responsible for the development of intrapulmonary arteriovenous malformations (PAVM) in patients with univentricular physiology. Thus, achieving uniform distribution of hepatic blood flow is considered favorable. However, no established method for the prediction of the post-interventional hemodynamics currently exists. Computational fluid dynamics (CFD) offers the possibility to quantify HFD in patient-specific anatomies before and after virtual treatment. In this study, we evaluated the potential benefit of CFD-assisted treatment planning.Materials and methodsThree patients with total cavopulmonary connection (TCPC) and PAVM underwent cardiovascular magnetic resonance imaging (CMR) and computed tomography imaging (CT). Based on this imaging data, the patient-specific anatomy was reconstructed. These patients were considered for surgery or catheter-based intervention aiming at hepatic blood flow re-routing. CFD simulations were then performed for the untreated state as well as for different surgical and interventional treatment options. These treatment options were applied as suggested by treating cardiologists and congenital heart surgeons with longstanding experience in interventional and surgical treatment of patients with univentricular physiology. HFD was quantified for all simulations to identify the most viable treatment decision regarding redistribution of hepatic blood flow.ResultsFor all three patients, the complex TCPC anatomy could be reconstructed. However, due to the presence of metallic stent implants, hybrid models generated from CT as well as CMR data were required. Numerical simulation of pre-interventional HFD agreed well with angiographic assessment and physiologic considerations. One treatment option resulting in improvement of HFD was identified for each patient. In one patient follow-up data after treatment was available. Here, the virtual treatment simulation and the CMR flow measurements differed by 15%.ConclusionThe combination of modern computational methods as well as imaging methods for assessment of patient-specific anatomy and flow might allow to optimize patient-specific therapy planning in patients with pronounced hepatic flow mismatch and PAVM. In this study, we demonstrate that these methods can also be applied in patients with complex univentricular physiology and extensive prior interventions. However, in those cases, hybrid approaches utilizing information of different image modalities may be required.

We developed models of pulmonary artery (PA) size in Fontan patients as a function of age and body surface area (BSA) using linear regression and breakpoint analyses based on data from 43 Fontan patients divided into two groups: the extracardiac conduit (ECC) group (n = 24) and the non-ECC group (n = 19). Model predictions were compared against those of a non-Fontan control group (n = 18) and published literature. We observed strong positive correlations of the mean PA diameter with BSA (r = 0.9, p < 0.05) and age (r = 0.88, p < 0.05) in the ECC group. The absolute percentage differences between our BSA and age model predictions against published literature were less than 16% and 20%, respectively. Predicted PA size for Fontan patients was consistently smaller than the control group. These models may serve as useful references for clinicians and be utilized to construct 3D anatomic models that correspond to patient body size or age. [ABSTRACT FROM AUTHOR]

Morbidity and mortality in Down syndrome (DS) are mainly related to congenital heart defects (CHDs). While CHDs with high prevalence in DS (typical CHDs), such as endocardial cushion defects, have been extensively described, little is known about the impact of less common CHDs (atypical CHDs), such as aortic coarctation and univentricular hearts. In our single‐center study, we analyzed, in observational, retrospective manner, data regarding cardiac features, surgical management, and outcomes of a cohort of DS patients. Literature review was performed to investigate previously reported studies on atypical CHDs in DS. Patients with CHDs were subclassified as having typical or atypical CHDs. Statistical analysis was performed for comparison between the groups. The study population encompassed 859 DS patients, 72.2% with CHDs, of which 4.7% were atypical. Statistical analysis showed a significant excess in multiple surgeries, all‐cause mortality and cardiac mortality in patients with atypical CHDs (p =.0067, p =.0038, p =.0001, respectively). According to the Kaplan–Meier method, survival at 10 and 40 years was significantly higher in typical CHDs (99 and 98% vs. 91 and 84%, log rank <0.05). Among atypical CHDs, it seems that particularly multiple complex defects in univentricular physiology associate with a worse outcome. This may be due to the surgical difficulty in managing univentricular hearts with multiple defects concurring to the clinical picture or to the severity of associated defects themselves. Further studies need to address this specific issue, also considering the higher pulmonary pressures, infective complications, and potential comorbidities in DS patients. [ABSTRACT FROM AUTHOR]

Management of rhythm related issues might be particularly challenging in patients with congenital heart disease due to complex anatomy and restricted vascular access. The leadless technology appears a suitable and attractive alternative for this population. We describe a patient with single ventricle physiology who successfully underwent implantation of a leadless pacemaker.

Tissue engineering holds great promise for the advancement of cardiovascular surgery as well as other medical fields. Tissue-engineered vascular grafts have the ability to grow and remodel and could therefore make great advances for pediatric cardiovascular surgery. In 2001, we began a human clinical trial evaluating these grafts in patients with a univentricular physiology. Herein, we report the long-term results of patients who underwent implantation of tissue-engineered vascular grafts as extracardiac total cavopulmonary conduits. Tissue-engineered vascular grafts seeded with autologous bone marrow mononuclear cells were implanted in 25 patients with univentricular physiology. The graft is composed of a woven fabric of poly-l-lactide acid or polyglycolic acid and a 50:50 poly (l-lactic-co-ε-caprolactone) copolymer. Patients were followed up with postoperatively in a multidisciplinary clinic. Median patient age at operation was 5.5 years and the mean follow-up period was 11.1 years. There was no graft-related mortality during the follow-up period. There was also no evidence of aneurysmal formation, graft rupture, graft infection, or calcification. Seven (28%) patients had asymptomatic graft stenosis and underwent successful balloon angioplasty. Stenosis is the primary complication of the tissue-engineered vascular graft. Avoidance of anticoagulation therapy would improve patients' quality of life. Tissue-engineered vascular grafts have feasibility in pediatric cardiovascular surgery. [ABSTRACT FROM AUTHOR]

Aims: Cardiac magnetic resonance (CMR) imaging is a main diagnostic tool in the follow-up of Fontan patients. However, the value of serial CMR for the evaluation of Fontan attrition is unknown. The aim of this prospective study of serial CMR is to describe the analysis of time-dependent evolution of blood flow distribution, ventricular volumes, and function in patients after Fontan completion., Methods and Results: In this prospective single-centre study, between 2012 and 2022, 281 CMR examinations were performed in 88 Fontan patients with distribution of blood flows, measurements of ventricular volumes, and ejection fraction. Linear mixed model regression for repeated measurements was used to analyse changes of measurements across serial CMR examinations. During a time interval of 10 years, the median number of CMR per patient was 3 (range 1-5). Indexed flow of ascending aorta, caval veins, and pulmonary arteries decreased significantly across serial CMR examinations. Although a decrease of mean indexed aortic flow (3.03 ± 0.10 L/min/m 2 at first CMR vs. 2.36 ± 0.14 L/min/m 2 at fourth CMR, P < 0.001) was observed, ejection fraction did not decline (50 ± 1% at first CMR vs. 54 ± 2% at fourth CMR, P = 0.070). Indexed ventricular volumes did not differ significantly across serial CMR examinations., Conclusion: The decrease of indexed aortic and cavopulmonary flows reflects the attrition of univentricular circulation and can be detected by means of serial CMR. Ventricular systolic dysfunction does not contribute significantly to this attrition. In order to detect significant change of indexed aortic flow, we recommend performing serial CMR as routine practice in the Fontan population., Competing Interests: Conflict of interest: None declared., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Purpose: Systemic-to-pulmonary collateral flow is a well-recognised phenomenon in patients with single ventricle physiology, but remains difficult to quantify. The aim was to compare the reported formula's that have been used for calculation of systemic-to-pulmonary-collateral flow to assess their consistency and to quantify systemic-to-pulmonary collateral flow in patients with a Glenn and/or Fontan circulation using four-dimensional flow MRI (4D flow MR)., Methods: Retrospective case-control study of Glenn and Fontan patients who had a 4D flow MR study. Flows were measured at the ascending aorta, left and right pulmonary arteries, left and right pulmonary veins, and both caval veins. Systemic-to-pulmonary collateral flow was calculated using two formulas: 1) pulmonary veins - pulmonary arteries and 2) ascending aorta - caval veins. Anatomical identification of collaterals was performed using the 4D MR image set., Results: Fourteen patients (n = 11 Fontan, n = 3 Glenn) were included (age 26 [22-30] years). Systemic-to-pulmonary collateral flow was significantly higher in the patients than the controls (n = 10, age 31.2 [15.1-38.4] years) with both formulas: 0.28 [0.09-0.5] versus 0.04 [-0.66-0.21] l/min/m 2 (p = 0.036, formula 1) and 0.67 [0.24-0.88] versus -0.07 [-0.16-0.08] l/min/m 2 (p < 0.001, formula 2). In patients, systemic-to-pulmonary collateral flow differed significantly between formulas 1 and 2 (13% versus 26% of aortic flow, p = 0.038). In seven patients, veno-venous collaterals were detected and no aortopulmonary collaterals were visualised., Conclusion: 4D flow MR is able to detect increased systemic-to-pulmonary collateral flow and visualise collaterals vessels in Glenn and Fontan patients. However, the amount of systemic-to-pulmonary collateral flow varies with the formula employed. Therefore, further research is necessary before it could be applied in clinical care.

Background: With improved therapies, an increasing number of patients with Fontan circulation reach reproductive age. Pregnant patients with Fontan circulation are at high risk of obstetrical complications. Most data for pregnancies complicated by Fontan circulation and associated complications stem from single-center studies, with limited national epidemiologic data available., Objective: This study aimed to evaluate temporal trends in deliveries to pregnant individuals with Fontan palliation using nationwide data and to estimate associated obstetrical complications among these deliveries., Study Design: Delivery hospitalizations were abstracted from the 2000 to 2018 Nationwide Inpatient Sample. Deliveries complicated by Fontan circulation were identified using diagnosis codes, and trends in the rates of these deliveries were assessed using joinpoint regression. Baseline demographics and obstetrical outcomes (including severe maternal morbidity, a composite of serious obstetrical and cardiac complications) were assessed. Univariable log-linear regression models were fit comparing risks of outcomes among deliveries of patients with and without Fontan circulation., Results: A total of 509 pregnancies complicated by Fontan circulation were identified at a rate of 7 per 1 million delivery hospitalizations, with a temporal increase from 2.4 to 30.3 cases per 1 million from 2000 to 2018 (P<.01). Deliveries complicated by Fontan circulation were at higher risk of hypertensive disorders (relative risk, 1.79; 95% confidence interval, 1.42-2.27), preterm delivery (relative risk, 2.37; 95% confidence interval, 1.90-2.96), postpartum hemorrhage (relative risk, 4.28; 95% confidence interval, 3.35-5.45), and severe maternal morbidity (relative risk, 6.09; 95% confidence interval, 4.54-8.17) than deliveries not complicated by Fontan circulation., Conclusion: The rates of deliveries of patients with Fontan palliation are increasing on a national level. These deliveries have higher risks of obstetrical complications and severe maternal morbidity. Additional national clinical data are necessary to better understand the complications in pregnancies complicated by Fontan circulation, to improve patient counseling, and to reduce maternal morbidity., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Objective: Left ventricular non-compaction is an architectural abnormality of the myocardium, associated with heart failure, systemic thromboembolism, and arrhythmia. We sought to assess the prevalence of left ventricular non-compaction in patients with single ventricle heart disease and its effects on ventricular function., Methods: Cardiac MRI of 93 patients with single ventricle heart disease (mean age 24 ± 8 years; 55% male) from three tertiary congenital centres was retrospectively reviewed; 65 of these had left ventricular morphology and are the subject of this report. The presence of left ventricular non-compaction was defined as having a non-compacted:compacted (NC:C) myocardial thickness ratio >2.3:1. The distribution of left ventricular non-compaction, ventricular volumes, and function was correlated with clinical data., Results: The prevalence of left ventricular non-compaction was 37% (24 of 65 patients) with a mean of 4 ± 2 affected segments. The distribution was apical in 100%, mid-ventricular in 29%, and basal in 17% of patients. Patients with left ventricular non-compaction had significantly higher end-diastolic (128 ± 44 versus 104 ± 46 mL/m2, p = 0.047) and end-systolic left ventricular volumes (74 ± 35 versus 56 ± 35 mL/m2, p = 0.039) with lower left ventricular ejection fraction (44 ± 11 versus 50 ± 9%, p = 0.039) compared to those with normal compaction. The number of segments involved did not correlate with ventricular function (p = 0.71)., Conclusions: Left ventricular non-compaction is frequently observed in patients with left ventricle-type univentricular hearts, with predominantly apical and mid-ventricular involvement. The presence of non-compaction is associated with increased indexed end-diastolic volumes and impaired systolic function.

Total anomalous pulmonary venous drainage (TAPVD) is an uncommon cardiac defect in children. The mixed subset accounts for 5%-10% of the TAPVD and is variable in its anatomy. The outcomes associated with this subset of patients are rarely reported. A retrospective review of all patients with mixed TAPVD undergoing repair at a single institution (1984-2014) was conducted. A descriptive analysis was performed. Twenty-four patients underwent repair for mixed TAPVD (6 univentricular physiology, 18 biventricular physiology). The mixed TAPVD anatomy included 8 patients in group I (2 + 2 veins), 11 patients in group II (3 + 1 veins), and 5 patients in group III (atypical). Preoperative pulmonary venous obstruction occurred in 8 patients (33%). The median age at repair was 2.2 months (range 2 days to 3 years) and median weight was 4.2 kg (range 1.9 to 12.5 kg). Operative mortality was 13% (3 of 24), 33.3% (2 of 6) for patients with univentricular physiology, and 5.6% (1 of 18) for patients with biventricular physiology. There have been no operative deaths in the biventricular group since 1997 (n = 11). Survival at 30-days was 83% ± 15% (95% confidence interval: 27%-97%) and 94% ± 5% (95% confidence interval: 67%-99%) for the univentricular and biventricular groups, respectively. Reoperation for recurrent pulmonary venous obstruction was required in 2 patients (8.3%) where the sutureless technique was used. The average follow-up after surgery was 9.3 ± 6.4 years (5 months to 21 years), and all surviving patients were asymptomatic. Mixed TAPVD can be repaired with good results in children, particularly in those undergoing biventricular repair., (Crown Copyright © 2017. Published by Elsevier Inc. All rights reserved.)

Certain congenital heart anomalies make patients more susceptible to arrhythmia development throughout their lives. This poses the question whether prophylactic arrhythmia surgery should be incorporated into reparative open heart procedures for congenital heart disease. There is currently no consensus on what constitutes a standard prophylactic procedure, owing to the questions that remain regarding lesions to be performed; energy sources to use; proximity of energy source or incisions to coronary arteries, sinoatrial node, atrioventricular node; circumstances for right atrial, left atrial, or biatrial appendectomy; and whether to perform a right, left, or biatrial maze procedure. These considerations are important because prophylactic arrhythmia procedures are performed without knowing if the patient will actually develop an arrhythmia in his or her lifetime. By reviewing and summarizing the literature, congenital heart disease patients who are at risk for developing atrial arrhythmias can be identified and lesion sets can be suggested in an effort to standardize experimental protocols for prophylactic arrhythmia surgery., Competing Interests: The authors have no conflicts of interest to declare.