Significantly elevated hepatic extracellular volume in adult patients with a Fontan circulation and its correlation with impaired functional capacity.

Fontan-associated liver disease is a major concern in patients who have undergone the procedure. Regular imaging of the liver is currently recommended for Fontan patients, but not other congenital heart diseases. The extracellular volume (ECV) of the liver obtained during CMR scanning and studies can show the high liver ECV in Fontan patients. However, the correlation between the liver ECV and the functional capacity of Fontan patients has not yet been reported. This study aimed to compare the liver ECV between Fontan patients and other congenital heart diseases with significant pulmonic regurgitation (PR) or tricuspid regurgitation (TR), and to evaluate the correlation between the liver ECV in adult Fontan patients and their functional capacity as well as clinical characteristics. Retrospective analysis of cardiovascular magnetic resonance imaging from patients with history of Fontan surgery between 2017 and 2021 were conducted. The clinical characteristics and liver ECV were evaluated and compared between patients and control group. Functional capacity was evaluated using a 6-min walk distance (6MWD). The correlation between the liver ECV and functional capacity was analyzed. 35 patients were enrolled in the study, including 13 Fontan patients, 12 Ebstein's anomaly or repaired tetralogy of Fallot (rTOF) patients with significant PR or TR, and 10 patients for the control group. The liver ECV were significantly higher in Fontan patients compared with Ebstein's anomaly/rTOF and the control group (41.% in Fontan group, 33.9% in Ebstein's anomaly/rTOF, and 31.7% in control group with p = 0.01 and 0.0008 in Fontan vs. Ebstein's anomaly/rTOF and Fontan vs. control group, respectively). In Fontan patients, there was a significant correlation between the liver ECV and the liver blood biochemistry with r = 0.879, p = 0.01 for AST/ALT ratio and r = 0.65, p = 0.005 for AST. The liver ECV was inversely correlated with the six-minute walk distance (r = -0.55, p = 0.02). The liver ECV in patients who had undergone Fontan operation showed a significantly elevated and has significantly inversed correlation with their functional capacity. These findings indicated that the liver ECV may be a potentialmarker for adverse clinical outcomes. However, due to small size population, further prospective study with larger number of patients may validate this findings.

A simple-to-use nomogram for predicting prolonged mechanical ventilation for children after Ebstein anomaly corrective surgery: a retrospective cohort study.

BACKGROUND: Prolonged mechanical ventilation (PMV) after pediatric cardiac surgery imposes a great burden on patients in terms of morbidity, mortality as well as financial costs. Ebstein anomaly (EA) is a rare congenital heart disease, and few studies have been conducted about PMV in this condition. This study aimed to establish a simple-to-use nomogram to predict the risk of PMV for EA children. METHODS: The retrospective study included patients under 18 years who underwent corrective surgeries for EA from January 2009 to November 2021. PMV was defined as postoperative mechanical ventilation time longer than 24 hours. Through multivariable logistic regression, we identified and integrated the risk factors to develop a simple-to-use nomogram of PMV for EA children and internally validated it by bootstrapping. The calibration and discriminative ability of the nomogram were determined by calibration curve, Hosmer-Lemeshow goodness-of-fit test and receiver operating characteristic (ROC) curve. RESULTS: Two hundred seventeen children were included in our study of which 44 (20.3%) were in the PMV group. After multivariable regression, we obtained five risk factors of PMV. The odds ratios and 95% confidence intervals (CI) were as follows: preoperative blood oxygen saturation, 0.876(0.805,0.953); cardiothoracic ratio, 3.007(1.107,8.169); Carpentier type, 4.644(2.065,10.445); cardiopulmonary bypass time, 1.014(1.005,1.023) and postoperative central venous pressure, 1.166(1.016,1.339). We integrated the five risk factors into a nomogram to predict the risk of PMV. The area under ROC curve of nomogram was 0.805 (95% CI, 0.725,0.885) and it also provided a good discriminative information with the corresponding Hosmer-Lemeshow p values > 0.05. CONCLUSIONS: We developed a nomogram by integrating five independent risk factors. The nomogram is a practical tool to early identify children at high-risk for PMV after EA corrective surgery.

Cardiac Implantable Electronic Devices in Ebstein Anomaly: Management and Outcomes.

BACKGROUND: Optimal management of cardiac implantable electronic devices (CIEDs) in patients with Ebstein anomaly during tricuspid valve (TV) surgery is unknown. Thus, we aimed to characterize CIED management/outcomes in patients with Ebstein anomaly undergoing TV surgery. METHODS: Patients at the Mayo Clinic from 1987 to 2020 with Ebstein anomaly and CIED procedure were reviewed for procedural details, complications, echocardiogram, and lead parameters. Five-year cumulative incidence of CIED complications were estimated using the Kaplan-Meier method. RESULTS: Ninety-three patients were included; 51 were female, and mean age was 40.7±17.5 years. A new CIED was implanted in 45 patients at the time of TV surgery with the majority receiving an epicardial (n=37) CIED. Among 34 patients who had preexisting CIED (11 epicardial, 23 transvenous) at time of TV surgery, 20 had a transvenous right ventricular lead managed by externalizing the lead to the TV (n=15) or extracting the transvenous lead with epicardial lead implantation (n=5). Fourteen patients underwent CIED implantation (4 epicardial, 10 transvenous) without concurrent surgery. Placement of lead across the TV was avoided in 85% of patients. The 5-year cumulative incidence of CIED complications was 24% with no significant difference between epicardial and transvenous CIEDs (26% versus 23%, P=0.96). Performance of lead parameters was similar in epicardial and transvenous leads during median (interquartile range) follow-up of 44.5 (61.1) months. CONCLUSIONS: In patients with Ebstein anomaly undergoing TV surgery, the use of epicardial leads and externalization of transvenous leads to the TV can avoid lead placement across the valve leaflets. Lead performance and CIED complications was similar between epicardial and transvenous CIEDs.

Potential risk factors for Ebstein anomaly, National Birth Defects Prevention Study, 1997-2011.

BACKGROUND: Ebstein anomaly is a rare congenital heart defect (CHD) that, when severe, requires corrective surgery or other catheter-based intervention in the first year of life. Due to its rarity, risk factors for Ebstein anomaly remain largely unknown. Using national data, we examined 18 potential risk factors for Ebstein anomaly. METHODS: Using 1997-2011 data from the National Birth Defects Prevention Study, a population-based case-control study, we calculated crude and adjusted odds ratios and 95% confidence intervals for paternal age, maternal socio-demographics, reproductive history, and modifiable risk factors, and infant characteristics reported by mothers of 135 Ebstein anomaly cases and 11,829 controls. RESULTS: Mothers of Ebstein anomaly cases had 4.1 (95% confidence interval: 1.8, 9.5) times the odds of reporting a family history of CHD compared with mothers of controls. Ebstein anomaly was associated with maternal second-hand cigarette smoke exposure at home (odds ratio = 2.2 [95% confidence interval: 1.1, 4.4]), but not maternal cigarette smoking (odds ratio = 1.3 [95% confidence interval: 0.8, 2.1]). Odds were elevated, but the 95% confidence interval included 1.0, for maternal marijuana use (odds ratio = 1.8 [95% confidence interval: 0.9, 3.8]) and paternal age ≥40 years at delivery (odds ratio = 1.9 [95% confidence interval: 1.0, 3.5]). CONCLUSIONS: Maternal exposure to second-hand cigarette smoke at home and a family history of CHD were associated with elevated odds of Ebstein anomaly. Genetic analyses could clarify the potential heritability of Ebstein anomaly.

[A morphological study of 23 hearts with Ebstein's anomaly]. / Estudio morfológico de 23 corazones con anomalía de Ebstein.

The literature published of the Ebstein anomaly of the tricuspid valve have been given little attention to the study of the trabecular portion of the right ventricle. This motivate us to study the morphology of twenty-three hearts by the segmentary sequential method determining with special interest the extension and grade of valvular attachment, dysplasia, characteristics of the trabecular portion of the right ventricle and associated anomalies. The three valves had some grade of attachment but this was less frequently in the anterior valve. However in our material this occurred in 43% which is consider high comparing with the rest of the literature; the trabecular portion had several bands at this zone and it was covered by the valve attachment. The downward displacement of the functional ring was directly proportional to the valve attachment. The structural anomalies of the right ventricle related to the valve malformation lead us to consider that the primary damage that causes this cardiopathy occurred at the right ventricle during the embryonic development altering the morphogenesis of the tricuspid valve.