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Objective: The study objective was to analyze survival and incidence of Fontan completion of patients with single-ventricle and concomitant unbalanced atrioventricular septal defect., Methods: Data from 4 Dutch and 3 Belgian institutional databases were retrospectively collected. A total of 151 patients with single-ventricle atrioventricular septal defect were selected; 36 patients underwent an atrioventricular valve procedure (valve surgery group). End points were survival, incidence of Fontan completion, and freedom from atrioventricular valve reoperation., Results: Median follow-up was 13.4 years. Cumulative survival was 71.2%, 70%, and 68.5% at 10, 15, and 20 years, respectively. An atrioventricular valve procedure was not a risk factor for mortality. Patients with moderate-severe or severe atrioventricular valve regurgitation at echocardiographic follow-up had a significantly worse 15-year survival (58.3%) compared with patients with no or mild regurgitation (89.2%) and patients with moderate regurgitation (88.6%) (P = .033). Cumulative incidence of Fontan completion was 56.5%, 71%, and 77.6% at 5, 10, and 15 years, respectively. An atrioventricular valve procedure was not associated with the incidence of Fontan completion. In the valve surgery group, freedom from atrioventricular valve reoperation was 85.7% at 1 year and 52.6% at 5 years., Conclusions: The long-term survival and incidence of Fontan completion in our study were better than previously described for patients with single-ventricle atrioventricular septal defect. A concomitant atrioventricular valve procedure did not increase the mortality rate or decrease the incidence of Fontan completion, whereas patients with moderate-severe or severe valve regurgitation at follow-up had a worse survival. Therefore, in patients with single-ventricle atrioventricular septal defect when atrioventricular valve regurgitation exceeds a moderate degree, the atrioventricular valve should be repaired., (Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.)

Objectives: Variations in coronary anatomy are common and may relate to the position of the coronary ostium relative to the aortic sinus, the angle of coronary take-off, or the course of the coronary arterial branches. Several classification systems have been proposed. However, they all lack a simple rationale that is applicable irrespective of the relative position of the great arteries, as well as in bicuspid aortic valves. We present a modification of a relatively simple system introduced in the early 1980s, designated the "Leiden Convention.", Methods: The first step of the Leiden Convention is that the clinician takes position in the nonfacing sinus of the aorta looking toward the pulmonary orifice. The right-hand facing sinus is sinus 1, and the left-hand facing sinus is sinus 2. The coronary branches arising from sinus 1 are annotated proceeding in a counterclockwise fashion toward sinus 2. "Usual" (normal) coronary anatomy would be 1R-2LCx. Given their clinical relevance, single sinus coronary arteries are discussed separately., Results: This system was originally designed and highly applicable in hearts with an altered great artery relationship, such as in the variable and complicated patterns seen in transposition of the great arteries and double outlet right ventricle. The modified system also can be used in cases with normally related great arteries, cases with single sinus coronary arteries, and cases with bicuspid aortic valves., Conclusions: The modified Leiden Convention is not a strict classification but a simple coronary coding system that is broadly applicable., (Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: During normal left ventricular (LV) filling, a vortex ring structure is formed distal to the left atrioventricular valve (LAVV). Vortex structures contribute to efficient flow organization. We aimed to investigate whether LAVV abnormality in patients with a corrected atrioventricular septal defect (AVSD) has an impact on vortex ring formation., Methods: Whole-heart 4D flow MRI was performed in 32 patients (age: 26 ± 12 years), and 30 healthy subjects (age: 25 ± 14 years). Vortex ring cores were detected at peak early (E-peak) and peak late filling (A-peak). When present, the 3-dimensional position and orientation of the vortex ring was defined, and the circularity index was calculated. Through-plane flow over the LAVV, and the vortex formation time (VFT), were quantified to analyze the relationship of vortex flow with the inflow jet., Results: Absence of a vortex ring during E-peak (healthy subjects 0%, vs patients 19%; P = .015), and A-peak (healthy subjects 10% vs patients 44%; P = .008) was more frequent in patients. In 4 patients, this was accompanied by a high VFT (5.1-7.8 vs 2.4 ± 0.6 in healthy subjects), and in another 2 patients with abnormal valve anatomy. In patients compared with controls, the vortex cores had a more-anterior and apical position, closer to the ventricular wall, with a more-elliptical shape and oblique orientation. The shape of the vortex core closely resembled the valve shape, and its orientation was related to the LV inflow direction., Conclusions: This study quantitatively shows the influence of abnormal LAVV and LV inflow on 3D vortex ring formation during LV inflow in patients with corrected AVSD, compared with healthy subjects., (Copyright © 2015. Published by Elsevier Inc.)

Objectives: In patients with univentricular heart disease, the total cavopulmonary connection (TCPC) is the preferred treatment. TCPC can be performed using the intra-atrial lateral tunnel (ILT) or extracardiac conduit (ECC) technique. The purpose of the present study was to evaluate exercise capacity in contemporary TCPC patients and compare the results between the 2 techniques., Methods: A total of 101 TCPC patients (ILT, n=42; ECC, n=59; age, 12.2±2.6 years; age at TCPC completion, 3.2±1.1 years) underwent cardiopulmonary exercise testing. The patients were recruited prospectively from 5 tertiary referral centers., Results: For the entire group, the mean peak oxygen uptake was 74%±14%, peak heart rate was 90%±8%, peak workload was 62%±13%, and slope of ventilation versus carbon dioxide elimination (VE/VCO2 slope) was 127%±30% of the predicted value. For the ILT and ECC groups, patient age, age at TCPC completion, body surface area, peak workload, and peak heart rate were comparable. The percentage of predicted peak oxygen uptake was lower in the ILT group (70%±12% vs 77%±15%; P=.040), and the percentage of predicted VE/VCO2 slope was greater in the ILT group (123%±36% vs 108%±14%; P=.015). In a subgroup analysis that excluded ILT patients with baffle leak, these differences were not statistically significant., Conclusions: Common exercise parameters were impaired in contemporary Fontan patients. Chronotropic incompetence was uncommon. The peak oxygen uptake and VE/VCO2 slope were less favorable in ILT patients, likely related to baffle leaks in some ILT patients. These results have shown that a reduced exercise capacity in Fontan patients remains an important issue in contemporary cohorts. The ECC had a more favorable exercise outcome at medium-term follow-up., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: Recent descriptions of decreased exercise capacity 10 to 15 years after arterial switch operation (ASO) suggest subclinical hemodynamic restrictions. Persistent impairment of ventricular performance following ASO may add to this. We aimed to characterize the time course of changes in biventricular performance within the first year following ASO., Methods: We prospectively included 26 patients with transposition of the great arteries undergoing ASO and 20 age-matched controls. Left and right ventricular systolic and diastolic performance was assessed using tissue Doppler imaging-derived peak systolic velocity, peak diastolic velocity, and peak early wave Doppler flow velocity/early diastolic tissue Doppler imaging velocity as well as mitral and tricuspid annular plane systolic excursion. Furthermore, left ventricular longitudinal, radial, and circumferential strain were assessed using speckle tracking strain imaging. Studies were performed preoperatively, 1 day postoperatively, at discharge, and at medium-term follow-up (9 months [interquartile range, 6-23 months] postoperatively)., Results: After an initial decrease in biventricular systolic and diastolic performance 1 day postoperatively versus preoperatively, recovery was observed in all parameters during medium-term follow-up. At medium-term follow-up left ventricular systolic and diastolic performance parameters were comparable in patients and controls. In contrast, right ventricular systolic and diastolic performance were still impaired in patients versus controls roughly 1 year postoperatively (tricuspid annular plane systolic excursion, 11.6 ± 2.2 vs 18.6 ± 3.1 mm; right ventricular peak systolic velocity, 8.1 ± 2.3 vs 12.6 ± 1.8 cm/second; right ventricular peak diastolic velocity, 12.4 ± 3.0 vs 18.2 ± 4.2 cm/second; and right ventricular peak early wave Doppler flow velocity/early diastolic tissue Doppler imaging velocity, 6.7 ± 2.1 vs 4.3 ± 1.3; all Ps < .001)., Conclusions: If early ASO is performed, left ventricular performance recovers to control values within the first postoperative year. In contrast, right ventricular systolic and diastolic performance remained impaired during follow-up, which stresses the importance of postoperative follow-up of right ventricular performance., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objectives: Borderline left ventricle is the left ventricular morphology at the favorable end of the hypoplastic left heart syndrome. In contrast to the severe end, it is suitable for biventricular repair. Wondering whether it is possible to identify cases suitable for biventricular repair from a developmental viewpoint, we investigated the myocardial histology of borderline and severely hypoplastic left ventricles., Methods: Postmortem specimens of neonatal, unoperated human hearts with severe hypoplastic left heart syndrome and borderline left ventricle were compared with normal specimens and hearts from patients with transposition of the great arteries. After tissue sampling of the lateral walls of both ventricles, immunohistochemical and immunofluorescence stainings against cardiac troponin I, N-cadherin, and connexin 43, important for proper cardiac differentiation, were done., Results: All severely hypoplastic left hearts (7/7) and most borderline left ventricle hearts (4/6) showed reduced sarcomeric expressions of troponin I in left and right ventricles. N-cadherin and connexin 43 expressions were reduced in intercalated disks. The remaining borderline left ventricle hearts (2/6) were histologically closer to control hearts., Conclusions: Four of 6 borderline left ventricle hearts showed myocardial histopathology similar to the severely hypoplastic left hearts. The remainder were similar to normal hearts. Our results and knowledge regarding the role of epicardial-derived cells in myocardial differentiation lead us to postulate that an abnormal epicardial-myocardial interaction could explain the observed histopathology. Defining the histopathologic severity with preoperative myocardial biopsy samples of hearts with borderline left ventricle might provide a diagnostic tool for preoperative decision making., (Copyright © 2012 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: Management of pulmonary atresia with intact ventricular septum is challenging and depends on the severity of right ventricular hypoplasia. Clinical outcomes of biventricular repair seem favorable to univentricular palliation, but data on superiority of biventricular repair regarding exercise capacity are conflicting. We investigated the response to physical and pharmacologic stress in patients with surgically corrected pulmonary atresia with intact ventricular septum., Methods: Sixteen patients (7 patients after univentricular palliation, age 11.8 ± 2.6 years; 7 patients after biventricular repair, age 12.9 ± 2.7 years; and 2 patients after 1.5 ventricular repair, age 12 and 19 years) underwent cardiopulmonary exercise test, dobutamine stress magnetic resonance imaging, and delayed contrast-enhanced magnetic resonance imaging., Results: The univentricular group showed impaired exercise capacity in contrast with normal exercise capacity in the biventricular group. Left ventricular ejection fraction increased in both groups. With dobutamine, left ventricular stroke volume increased only in the biventricular group (+11.3 ± 5.0 mL, P < .001) and not in the univentricular group (-0.04 ± 3.6 mL, P = .9). Heart rate increase was inadequate in the univentricular group. Maximum oxygen consumption and oxygen pulse were strongly correlated with left ventricular stroke volume during stress but not at rest. The results of the 2 patients after 1.5 ventricular repair were comparable to those of the univentricular group. No myocardial fibrosis was detected., Conclusions: Impaired exercise capacity in children and adolescents with pulmonary atresia with intact ventricular septum after univentricular palliation is related to decreased cardiac reserve and inadequate chronotropic response. Young patients with pulmonary atresia with intact ventricular septum after biventricular repair show normal exercise capacity and cardiac reserve., (Copyright © 2012 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: The outcome of surgical correction of atrioventricular septal defect with double-orifice left atrioventricular valve has improved in recent years but is still reported to be associated with high mortality and reoperation rates. Controversy exists about the management of the accessory orifice. We evaluated our results with correction of atrioventricular septal defect with double-orifice left atrioventricular valve., Methods: Between 1975 and 2006, 21 patients underwent correction of atrioventricular septal defect with double-orifice left atrioventricular valve. Clinical data were obtained by means of retrospectively reviewing inpatient and outpatient medical records. To evaluate the influence of double-orifice left atrioventricular valve on mortality and the need for reoperation, a comparison was made with 291 consecutive patients who, during the same period, underwent correction of atrioventricular septal defect without double-orifice left atrioventricular valve., Results: None of the 21 patients with double-orifice left atrioventricular valve had undergone a previous operation. The accessory orifice was managed with different techniques depending on the severity of the regurgitation. There was no in-hospital mortality, and there were 3 late deaths. Seven patients required 12 reoperations, 7 for left atrioventricular valve insufficiency. Double-orifice left atrioventricular valve had no influence on mortality but was a significant predictor for reoperation compared with repair of atrioventricular septal defect without double-orifice left atrioventricular valve. At the latest follow-up, all 18 survivors were in New York Heart Association functional class capital I, Ukrainian without medication. Only 1 patient showed residual mild left atrioventricular valve insufficiency., Conclusion: Atrioventricular septal defect with double-orifice left atrioventricular valve can be repaired with low mortality. However, double-orifice left atrioventricular valve is a predictor for reoperation. The accessory orifice is often competent and should then be left untouched. If regurgitation of the accessory orifice is present, this is best managed with suture or patch closure.

Objective: We sought to determine the histologic features of pulmonary autografts explanted after the Ross operation., Methods: Histologic sections of 30 explanted autografts and 8 normal heart valves were compared and semiquantitatively scored by a blinded cardiovascular pathologist., Results: Pulmonary autografts (n = 30) were explanted on average 6.1 +/- 0.6 years (median, 6.6 years; range, 0.1-11.7 years) after the Ross operation (n = 28) or removed at autopsy (n = 2). Twelve (43%) of the patients undergoing reoperation had no or negligible autograft insufficiency on early transthoracic echocardiography, 12 (43%) had grade 1 autograft insufficiency, and 4 (14%) had grade 1-2 autograft insufficiency. Valve regurgitation with root dilatation was the most common indication for reoperation after root replacement (n = 26 [93%]) and regurgitation after subcoronary implanted autografts (n = 2 [7%]). Microscopy of the autograft explants revealed normal laminar architecture and cellularity. Wall specimens were characterized by reduced and fragmented elastin and increased collagen levels (fibrosis). Medial elastin changes were associated with the presence of hypertrophic smooth muscle cells. Fibrosis was most severe in the adventitia. Intimal thickening was a common finding. Valve explants showed significant thickening caused by fibrocellular tissue on the ventricular surface and marked thickening of the free margin. An autopsy explant with normal function before death showed similar features., Conclusions: Pulmonary autograft explants showed severe aneurysmal degeneration of the wall, which was characterized by intimal thickening, medial elastin fragmentation, and adventitial fibrosis. Valve leaflets were thickened. The presence of these features in a nonfailing explant suggests these changes represent a common mode of remodeling.

Objectives: Little attention has been paid to the effect of the systemic inflammatory response syndrome on intestinal dysfunction in the postoperative period. Several proinflammatory cytokines have been reported to increase the permeability of intestinal mucosa in vitro. We investigated the effect of dexamethasone on gut permeability in pediatric patients undergoing cardiac surgery by using the dual sugar permeability test and absorption of 2 other saccharides., Methods: Thirty-four patients scheduled for cardiac surgery with cardiopulmonary bypass were prospectively randomized to either act as control subjects or to receive dexamethasone (1 mg . kg -1) during induction of anesthesia. Intestinal permeability was measured with 3-O-methyl-D-glucose, D-xylose, L-rhamnose, and lactulose administered orally after induction of anesthesia and 12 and 24 hours later., Results: Lactulose/rhamnose ratios were increased from the outset in both groups (mean 0.57 [95% confidence interval, 0.24-0.91] for the control group and 0.76 [95% confidence interval, 0.35-1.17] for patients receiving dexamethasone). Although the ratios decreased 12 hours (0.29 [95% confidence interval, 0.17-0.42]) and 24 hours later (0.17 [95% confidence interval, 0.08-0.15]) in the dexamethasone group, in the control group there was a rise at 12 hours (0.77 [95% confidence interval, 0-1.64]), with a slight reduction 24 hours later (0.46 [95% confidence interval, 0.06-0.85])., Conclusions: Infants and children undergoing cardiac surgery with cardiopulmonary bypass show a significant reduction in gut permeability when dexamethasone is used during induction of anesthesia. Dexamethasone does not affect the intestinal barrier at the functional level, as assessed on the basis of 3-O-methyl-D-glucose and D-xylose absorption.

Objective: Transposition of the great arteries is currently treated by performing the arterial switch operation. Dilatation of the neoaortic root is a late complication with unknown cause. Samples of patients with untreated transposition of the great arteries and patients with normally related great arteries were compared to investigate a possible role for vascular remodeling in the dilatation process., Methods: Aortic and pulmonary artery vessel wall and sinus samples were taken from 20 untreated human heart specimens with transposition of the great arteries and 9 age-matched, normal, postmortem human heart specimens, divided into 2 groups according to age. Routine histology and immunohistochemical staining for smooth muscle cell differentiation markers alpha-smooth muscle actin, SM22, and calponin were performed., Results: This study revealed structural differences between the normal aorta and pulmonary artery in the early group, which became more pronounced in the late group. In the early stage in transposition of the great arteries, no marked differences were seen between the aorta and pulmonary artery. With increasing age, however, there was a pronounced down-regulation of all smooth muscle cell markers in the pulmonary artery., Conclusions: There is a structural difference between the normal neonatal aorta and pulmonary artery. The great arteries in transposition of the great arteries differ from each other and from normal vessels, indicating a structural vascular difference in transposition of the great arteries. In the pulmonary artery and sinus of untreated transposition of the great arteries, there is a dedifferentiation of smooth muscle cells with increasing age that we could not correlate to altered flow. This structural abnormality might provide an explanation for the neoaortic root dilatation that has been reported as a late complication of the arterial switch operation.

Background: Lev's contribution to the understanding of the morphology of hearts with double-outlet right ventricle and the surgical feasibility for correction is important and remains in current use. However, the term noncommitted ventricular septal defect remains enigmatic. The aim of this study was to elucidate the morphologic nature of the noncommitted ventricular septal defect in view of its surgical implications., Methods: We examined 67 specimens with double-outlet right ventricle, focusing on the relationship of the ventricular septal defect to the semilunar orifices., Results: The defect was subaortic, subpulmonary, or doubly committed in 55 specimens. In a further 8 specimens, the defect opened into the outlet portion of the right ventricle, but the distance between the ventricular septal defect and the semilunar orifice was extensive, either because of extreme dextroposition of the aorta or a broad ventriculoinfundibular fold, which, in some cases, was associated with a long-outlet septum. A truly noncommitted ventricular septal defect was found in the inlet in the remaining 4 specimens. An atrioventricular septal defect without extension to the outlet was present in 3 cases, and a ventricular septal defect limited to the inlet was found in another case. The ventriculoinfundibular fold, part of the outlet septum and septal band or septomarginal trabeculation, had fused to form a crestlike structure. The septomarginal trabeculation is a useful landmark in the right ventricle to differentiate the inlet from the outlet in different forms of double-outlet right ventricle., Conclusion: We do not suggest to discard the Lev terminology but rather to differentiate the noncommitted ventricular septal defect into 2 types: the truly noncommitted defect of the inlet type and the not-directly-committed defect, which does open into the outlet portion of the right ventricle. The implication for the surgeon is 2-fold. The tricuspid valve or right part of the atrioventricular valve is interposed between the noncommitted ventricular septal defect and the semilunar orifice. The not-directly-committed defect opens into the outlet portion of the right ventricle but is not directly subaortic or subpulmonary.