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This study aims to clarify the relation of development of aortopulmonary collateral arteries (APCs) with anatomical sub-types and the shunt types at Norwood procedure in patients with hypoplastic left heart syndrome (HLHS). A total of 140 patients with HLHS who completed 3 staged palliation between 2003 and 2019 were included. Incidence of APCs and corresponding interventions were examined using angiogram by cardiac catheterization, with respect to the anatomical sub-types and shunt types. Totally, APCs were observed in 87 (62%) of the patients; pre-stage II in 32 (23%), pre-stage III in 64 (46%), and after stage III in 40 (29%). The incidence of APCs before stage II was significantly higher in patients with aortic atresia/mitral atresia (AA/MA) compared with other sub-types (P = 0.022). Patients with right ventricle to pulmonary artery conduit (RVPAC) had a higher incidence of APCs originating from the descending aorta, compared with those with modified Blalock-Taussig shunt (20% vs 2%, P = 0.002). Interventions for APCs were performed in 58 (41%) patients; before stage II in 10 (7%), after stage II in 7 (5%), before stage III in 22 (16%), and after stage III in 32 (23%). Patients with AA/MA had more interventions before stage II (P = 0.019), and patients with aortic stenosis/mitral stenosis (AS/MS) had a lower incidence of interventions after stage III (P = 0.047). More than half of the patients with HLHS developed APCs. Before stage II, patients with AA/MA sub-type had a higher incidence of APCs, and those with RVPAC had significantly more APCs from the descending aorta. [Display omitted] [ABSTRACT FROM AUTHOR]

Apicocaval juxtaposition (ACJ) may complicate the selection of conduit route in patients with single ventricles when total cavopulmonary connection (TCPC) is performed. We reviewed our experience of pathway selection and evaluated the clinical results. Of 128 patients who underwent TCPC at our hospital between January 2009 and April 2016, 31 with ACJ were included in this study. In 24 patients, the conduit was placed between the inferior vena cava (IVC) and the ipsilateral pulmonary artery. To avoid compression of the conduit and pulmonary veins in 5 patients, the conduit was placed between the IVC and the contralateral pulmonary artery. In 2 patients, the tube graft was anastomosed with the IVC orifice within the atrium, then guided through the atrial free wall and anastomosed with the contralateral pulmonary artery outside the heart (intra/extracardiac Fontan). Patient demographics were compared with those of patients without ACJ. The mean age and body weight at surgery were 58.5 ± 32.4 months and 16.2 ± 6.0 kg, respectively. The mean postoperative pulmonary artery pressure was 15 ± 3 mm Hg. The postoperative data did not differ significantly from that of patients without ACJ who underwent extracardiac TCPC. One patient died of overwhelming infection. The mean follow-up was 17.5 ± 15.4 months (range, 1-65 months). There were no conduit-related early or late complications. TCPC in patients with ACJ can be performed with excellent early and midterm results. The route between the IVC and the ipsilateral pulmonary artery is our preference. [ABSTRACT FROM AUTHOR]

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. 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. 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) mm2/m2 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 mm2/m2. 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 mm2/m2. The pulmonary artery index is significantly associated with the occurrence of chylothorax after nonfenestrated total cavopulmonary connection with a cutoff value of 170 mm2/m2. 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 mm2/m2. [Display omitted] [ABSTRACT FROM AUTHOR]

Background/purpose: Fontan operation has evolved from atriopulmonary connection to total cavopulmonary connection (TCPC) due to its advantages in terms of hemodynamics and reduction of atrium-related complications. We analyzed the early and intermediate-term results of extracardiac conduit TCPC (EC-TCPC) procedure in patients with functional single ventricle to investigate the risk factors of surgical mortality and intermediate failure.Methods: Retrospective review of the medical records of 88 consecutive patients with functional single ventricle who underwent EC-TCPC from 2000 to 2013 was conducted.Results: The follow-up was 100% complete, ranging from 3 months to 13 years (mean 7.0 ± 3.8 years). There were two (2.3%) hospital and 18 (20.4%) late deaths. The estimated event-free survival rates at 1 year, 5 years, and 10 years were 90.6%, 89.3%, and 77.2%, respectively. On univariate analysis, fenestration was the only risk factor for surgical mortality (p = 0.027). On multivariate analysis, the significant atrioventricular valve regurgitation was the only risk factor for intermediate failure (p = 0.017).Conclusion: The clinical results of EC-TCPC in patients with functional single ventricle were satisfactory. The patients who needed fenestration during operation had higher risk of surgical mortality. Significant atrioventricular valve regurgitation had negative impact on intermediate survival. [ABSTRACT FROM AUTHOR]

The article explores the effectiveness of combined remote-controlled magnetic navigation and ablation three-dimensional (3D) image integration in the treatment of supraventricular tachycardia in patients with previous intra-atrial baffle procedures. The 13 patients in the study were divided into Group A who had a medical history of a Mustard or Senning operation and Group B who underwent total cavopulmonary connection. Higher inducible SVTs were noted in Group A than B.

Objective: To analyze the aerobic fitness and evolution of exercise tolerance in patients with single-ventricle physiology after total cavopulmonary connection (TCPC) with an extracardiac conduit (ECC).Study Design: This retrospective cohort study included patients with previous ECC-TCPC who underwent cardiopulmonary exercise testing (CPET) between September 2010 and September 2019. Patients who completed at least 2 tests (≥6 months apart) with adequate levels of effort were recruited for the serial CPET evaluation.Results: We identified 70 patients (50% male) with a mean age of 6.45 ± 5.14 years at ECC-TCPC and 15.67 ± 5.03 years at the initial CPET. The peak oxygen consumption (peak VO2) to predicted value (peak PD) was 55.90 ± 16.81%. Twenty of the 70 identified patients (50% male) were recruited for serial analysis. The average number of CPETs was 2.6 per patient. The average duration from the first CPET to the last CPET was 3.64 years. The peak VO2 and PD increased slowly, with mean rates of 38.77 ± 129.01 mL/min and 1.66 ± 6.40%, respectively, during the study period.Conclusions: Although the patients had lower exercise tolerance after ECC-TCPC compared with their normal peers, exercise tolerance appears to have been preserved over the adolescent period in those who underwent serial testing after ECC-TCPC. [ABSTRACT FROM AUTHOR]

A longer length of stay (LOS) in the intensive care unit (ICU) after the total cavopulmonary connection (TCPC) is thought to be a predictive sign of late Fontan failure. This study was performed to determine the clinical risk factors for ICU LOS. In total, 483 patients who underwent a TCPC between May 1994 and December 2016 were included the study. Patients' main diagnosis, morphologic characteristics, palliative procedures, hemodynamic parameters, and perioperative variables, were analyzed to identify risk factors influencing ICU stay based on Cox regression. Causes of longer ICU LOS and the impact of ICU LOS on late outcomes were evaluated. Age at TCPC, type of TCPC, and fenestration at TCPC did not affect the ICU LOS. With multivariable model, hypoplastic left heart syndrome (P =.001) and anomalous systemic venous drainage (P <.001) were identified as independent morphologic risk factors for prolonged ICU LOS. Of hemodynamic variables, preoperative high transpulmonary gradient (P =.037), and low aortic oxygen saturation (P =.031) were risks for longer ICU LOS. Of postoperative variables, pleural effusion (P <.001), chylothorax (P =.001), ascites (P <.001), and infection (P =.028) were risks for longer ICU LOS. The ICU LOS was found to be significantly associated with late mortality (P <.001) and late cardiac reoperation (P =.007). Patients with hypoplastic left heart syndrome and anomalous systemic venous drainage had longer ICU LOS. Extended cyanosis and elevated pulmonary artery pressure affect the ICU LOS. Special care should be provided during the initial postoperative phase in patients with such risk factors. Patients with anomalous systemic venous drainage (SVD), dextrocardia, hypoplastic left heart syndrome (HLHS), dominant right ventricle (RV), and single tricuspid valve (TV) had longer intensive care unit (ICU) length of stay (LOS). Preoperative extended cyanosis, demonstrated by greater hemoglobin level and lower arterial oxygen saturation (SO 2), elevated pulmonary artery (PA) pressure, increased transpulmonary gradient, and presence of aortopulmonary collaterals (APCs), affected the ICU LOS. Special care should be provided during the initial postoperative phase in patients with such risk factors. TCPC , Total cavopulmonary connection. [ABSTRACT FROM AUTHOR]

Objectives Patients with a single ventricle infrequently undergo total cavopulmonary connection as preadolescents, adolescents, or adults. The purpose of this study was to clarify the characteristics of this cohort and to analyze the factors influencing outcomes. Methods Between 1994 and 2015, 50 of 460 patients underwent total cavopulmonary connection as preadolescents, adolescents, or adults (group A). The patients' characteristics and operative results were compared with those of the remaining 410 patients aged less than 9 years who underwent total cavopulmonary connection (group B). Post–total cavopulmonary connection echocardiogram reports (n = 4862) were used to evaluate longitudinal ventricular function, and ejection fraction was characterized using nonlinear mixed-effects models and compared between groups. Results The median follow-up time was 10.3 (2.8-15.5) years. The differences between groups in 30-day mortality ( P = .20), intensive care unit stay ( P = .20), and incidence of prolonged effusion ( P = .08) were not significant. The estimated survival at 15 years was lower in group A (86.5%) than in group B (94.0%, P = .04) patients. Long-term systemic ventricular ejection fraction, analyzed with linear mixed-effect models, was significantly reduced in group A than in group B patients ( P < .001). At a median postoperative period of 8.4 (7.1-10.5) years, the peak oxygen uptake as measured by exercise capacity testing was lower in group A than in group B patients, respectively (22.3 ± 6.5 [n = 25] vs 30.6 ± 8.1 [n = 100] mL/kg/min, P < .001). Conclusions The total cavopulmonary connection procedure was performed in preadolescent, adolescent, and adult patients with no significant difference in 30-day or hospital mortality compared with those in young children. However, long-term survival and ventricular performance were reduced in this older cohort. [ABSTRACT FROM AUTHOR]

Abstract: Many groups, including ours, have played a more or less significant role in the development and evolution of the Fontan operation that has taken place over the past four decades. This is a brief review of the contributions developed by our institution. Contributions to clinical and technical areas include: development of the ventricular exclusion concept; promoting the staged approach to the Fontan operation by bidirectional cavopulmonary anastomosis with or without a modified Damus–Kaye–Stansel anastomosis; endorsement of the total extracardiac cavopulmonary connection; a rational approach to the management of systemic venous aberrances in single-ventricle patients; and criteria for transition to heart transplantation. Contributions to computational fluid dynamics include: investigation of the best spatial arrangement for the total extracardiac cavopulmonary anastomosis and identification of the so-called ‘beneficial vortex’; studies on the effect of unbalanced pulmonary resistances on the hydrodynamic performance of the total extracardiac cavopulmonary connection; and a rationale for the management of systemic venous anomalies in single-ventricle patients. [Copyright &y& Elsevier]