Bidirectional Cavopulmonary Connection Research Articles

Case example 2. Tricuspid valve regurgitation in a three-year-old patient with hypoplastic left heart syndrome who underwent bidirectional cavopulmonary connection.

Case example 2. Tricuspid valve regurgitation in a three-year-old patient with hypoplastic left heart syndrome who underwent bidirectional cavopulmonary connection.

Managing Bilateral Discontinuous Pulmonary Arteries of Ductal Origin in Single-Ventricle Anatomy

BackgroundWe describe outcomes and management strategies for single-ventricle and bilaterally discontinuous pulmonary arteries (PAs) originating from bilateral ductus arteriosus. MethodsWe reviewed 22 patients with aforementioned anatomy and PA centralization from 1995 to 2023, excluding those with biventricular repair. ResultsMedian age at centralization was 9 days (minimum-maximum, 0 days-2 years). Centralization was performed with systemic-to-pulmonary shunt (n =20 [91%]; 2 after bilateral ductal stents) or bidirectional cavopulmonary connection (n = 2 [9%]) using pericardial roll (n = 14 [64%]), patch-augmented direct anastomosis (n = 7 [32%]), and interposition graft (n = 1 [5%]) techniques. Concurrent total anomalous pulmonary venous connection (TAPVC, n = 11 [50%]) was associated with significantly inferior survival (P = .01). Five patients (23%) died at a median of 59 days (minimum-maximum, 6-257 days) after centralization, all with noncardiac TAPVC. At the latest follow-up for 17 survivors (median, 13.5 years; minimum-maximum, 0.5-25.1 years after centralization), 12 completed Fontan, 4 completed second-stage palliation, and 1 received a transplant before second-stage palliation. PA reintervention was required in 14 patients (64%), including 3 with reoperations independent of staged palliation. Echocardiography from baseline to before the second stage demonstrated branch PA growth with significantly increased diameters (left, P = .0006; right, P = .0002); z-scores significantly increased for right (P = .004) but not left (P = .11). ConclusionsSuccessful single-ventricle palliation is possible, although high risk, for patients with bilateral discontinuous ductal PAs. Early postcentralization mortality remains substantial, particularly with associated noncardiac TAPVC. Many require reintervention to maintain PA growth, typically concurrently with staged palliation.

Bilateral bidirectional cavopulmonary connection: a review of surgical techniques and clinical implications.

The presence of bilateral superior caval veins (bSCVs) could negatively influence the outcome of Fontan patients. In the setting of a bilateral bidirectional Glenn, the selective blood flow to the ipsilateral long with consequent flow stagnation in the connecting portion could lead to poor growth of the central portion of the pulmonary artery, potentially affecting the eligibility for Fontan completion and being associated with a higher incidence of thrombotic complications. Alternative surgical techniques have been described to perform a bidirectional cavopulmonary anastomosis in the presence of bSCVs aiming to achieve a balanced growth of the pulmonary bifurcation. The short-term results of these techniques such as the V- or Y-shape seem to be excellent; however, some anatomical settings could affect the feasibility of these techniques. The so-called "unifocalization" creates a configuration comparable to a "normal" bidirectional Glenn and could be a feasible alternative. However, the long-term results of this technique are not published yet. The positive effect of additional pulsatile pulmonary flow on pulmonary artery growth should be considered in case of bilateral bidirectional Glenn, despite the higher incidence of postoperative complications reported and the difficult calibration of the amount of additional flow. The role of computational fluid dynamic to simulate the surgical strategy in single ventricle patients is promising and could be worthwhile in the setting of bSCVs. In fact, the surgical techniques of bilateral bidirectional Glenn could be simulated testing their feasibility and allowing to identify the more favorable hemodynamic pattern, patient specific. This review article highlights the critical issues related to the presence of bSCVs in univentricular physiology, analyzing pros and cons of the different surgical techniques. Besides reviewing the literature, this manuscript focuses on the role of computational fluid simulation in identifying the most favorable surgical technique with an individualized approach, which could potentially improve the clinical outcome.

Clinical profile, prenatal detection and predictors of outcome of heterotaxy syndromes in Western Australia

BackgroundHeterotaxy syndromes encompass left and right atrial isomerism (LAI and RAI respectively) and are associated with variable cardiac and non-cardiac anomalies which greatly influence outcomes. RAI is usually associated with complex congenital heart disease (CHD), early surgical intervention and increased mortality. LAI is less commonly associated with complex CHD but can be associated with heart block. The objective of this study was to review the clinical features and outcomes of patients with heterotaxy syndromes in Western Australia (WA). MethodsA retrospective review was performed of live born patients diagnosed with heterotaxy from 2003 to 2022 in a statewide tertiary cardiac service, representing all cases in WA with a view to compare the outcomes between LAI and RAI at our centre. Results30 patients (53% male) were diagnosed with heterotaxy; 16 (53%) with LAI and 14 (47%) with RAI. Overall incidence was 0.48 per 10,000 live births over the defined period. RAI patients were significantly more likely to have an antenatal diagnosis (81.8% versus LAI 28.6%, p = 0.03). Overall, 5-year survival was 56% for RAI and 87% for LAI. No deaths occurred after the first 12 months of life with a median follow-up of 65 months (IQR 114.8). RAI was associated with asplenia (91%), atrioventricular septal defect (91%) and a functionally univentricular circulation (71%). LAI was associated with polysplenia (100%) and complete heart block in 3 patients (19%). Surgical pathways included repair of anomalous pulmonary venous return (45%), Blalock Taussig shunt (60%), bidirectional cavopulmonary connection (50%) and Fontan completion (30%). ConclusionsPatients with RAI suffer high mortality and early surgical intervention, with few making it to Fontan completion. By comparison patients with LAI have less morbidity and mortality. The management of heterotaxy continues to be challenging due to widely associated cardiac and extracardiac manifestations.

Long-term outcomes of hypoplastic left heart syndrome with analysis of the Norwood procedure in infants following bilateral pulmonary artery banding

ObjectiveTo investigate the early and long-term outcomes of the deferred Norwood procedure by bilateral pulmonary artery banding (BPAB) versus the neonatal Norwood procedure. MethodsThis retrospective study examined 46 patients with hypoplastic left heart syndrome and its variants undergoing the Norwood procedure for single ventricle physiology between 2004 and 2022 at 3 institutions. The patients were divided into 2 groups: neonatal Norwood procedure (group N; n = 23) and staged Norwood procedure in infants following BPAB (group I; n = 23). Preoperative risk factors, surgical results, survival rates, Fontan candidacy, and long-term complications were compared. ResultsEarly survival rates after the Norwood procedure were 91.3% (21 of 23) in both groups. Late survival rates after the Norwood procedure were similar at the 10-year follow-up (group N, 76.3%; group I, 68.7%; P = .63). Fontan completion rates also were comparable in the 2 groups (group N, 77.8%; group I, 85.7%; P = .67). Group N showed a higher median pulmonary artery (PA) index before bidirectional cavopulmonary connection (group N, 177 [interquartile range (IQR), 147-243] mm2/m2; group I, 152 [IQR, 146-163] mm2/m2; P = .03); this trend continued until 5 years after Fontan completion (P = .01). Group N also had a lower rate of freedom from protein-losing enteropathy (PLE) at 9.0 years after the Fontan operation (90.0% vs 52.5% for group I; P = .04), although the incidences of other Fontan-associated events were not significantly different. ConclusionsFontan candidacy and survival rates were similar regardless of the timing of the Norwood procedure. Early performance of the Norwood procedure may lead to lower rates of late Fontan-associated events, such as PLE.

Risk Factors and Outcome of Pulmonary Artery Stenting After Bidirectional Cavopulmonary Connection (BDCPC) in Single Ventricle Circulation.

After bidirectional cavopulmonary connection (BDCPC) central pulmonary arteries (PAs) of single ventricle (SV) patients can be affected by stenosis or even closure. Aim of this study is to compare SV patients with and without PA-stent implantation post-BDCPC regarding risk factors for stent implantation and outcome. Single center, retrospective (2006-2021) study of 136 SV consecutive patients with and without PA-stent implantation post-BDCPC. Patient characteristics, risk factors for PA-stent implantation and PA growth were assessed comparing angiographic data pre-BDCPC and pre-TCPC. A total of 40/136 (29%) patients underwent PA-stent implantation at median (IQR) 14 (1.1-39.0) days post-BDCPC. 37/40 (92.5%) underwent LPA-stenting. Multiple regression analysis showed single LV patients to receive less likely PA-stents than single RV patients (OR 0.41; p = 0.05). Reduced LPA/BSA (mm/m2) and larger diameter of neo-ascending aorta pre-BDCPC were associated with an increased likelihood of PA-stent implantation post-BDCPC (OR 0.89, p = 0.03; OR 1.05, p = 0.001). Stent re-dilatation was performed in 36/40 (89%) after 1 (0.8-1.5) year. Pulmonary artery diameters pre-BDCPC were lower in the PA-stent group: McGoon (p < 0.001), Nakata (p < 0.001). Indexed pulmonary artery diameters increased equally in both groups but remained lower pre-TCPC in the PA-stent group: McGoon (p < 0.001), Nakata (p = 0.009), and Lower Lobe Index (p = 0.003). LPA and RPA grew symmetrically in both groups. Single RV, larger neo-ascending aorta, and small LPA pre- BDCPC are independent risk factors for PA-stent implantation post-BDCPC. Pulmonary artery diameters after PA-stent implantation and stent re-dilatation showed significant growth together with the contralateral side, but the PA-system remained symmetrically smaller in the stent group.

How Does Cross-Sectional Imaging Impact the Management of Patients With Single Ventricle After Bidirectional Cavopulmonary Connection?

There is currently no consensus regarding the use of surveillance cross-sectional imaging in pediatric patients after bidirectional cavopulmonary connection (BDCPC). We sought to determine how computed tomography with angiography (CTA) and cardiac magnetic resonance (CMR) imaging impacted the clinical management of pediatric patients after BDCPC. A single-center retrospective study including patients with single ventricle who had BDCPC between 2010 and 2019, and CTA/CMR studies obtained in these patients, at ≤5 years of age, and with Glenn physiology. Repeat studies on the same patient were included if the clinical situation had changed. The impact of CTA/CMR studies was categorized as major, minor, or none. Twenty-four patients (63% male) and 30 imaging studies (22 CTAs) were included. 60% were obtained in patients with hypoplastic left heart syndrome (HLHS); most common indication was Follow-up after an intervention (23%). 6 CMRs were performed on stable HLHS patients as part of a research protocol, with no clinical concerns. The overall impact of CTA/CMR studies was major in 13 cases (43.3%). CTA/CMR studies performed ≥1 year of age (62.5% vs 21.4%, P = .02) and in non-HLHS patients (66.7% vs 27.8%, P = .035) were associated with major impact. Also, 2/6 Research studies were associated with a major impact. CTA/CMR imaging in pediatric patients with SV after BDCPC was associated with significant clinical impact in over 40% of cases, with a higher impact if obtained in patients ≥1 year of age and in non-HLHS patients. We cannot disregard the possibility of CMR as a surveillance imaging modality in this population.

Pulmonary arteries growth after bidirectional cavopulmonary connection in functionally univentricular heart program

The optimization of the pulmonary arteries (PA) size is an important concern after the bidirectional cavopulmonary connection (BDCPC) in patients with functionally univentricular heart as it precedes the completion of the Fontan program. We analyzed the PA growth between the time of the BDCPC and the time of the total cavopulmonary connection (TCPC) in a large monocentric cohort of children, and we explored the factors associated with differential growth profiles. In total, 118 patients (median age at the BDCPC 8 months (IQR 5–18)) were included. Diameters of the right and the left PA were measured proximally to the first lobar bifurcation on the pulmonary artery angiography obtained before BDCPC and compared to angiographies obtained before TCPC. Nakata index was also calculated using these two measurements. Associations of pertinent covariables with the PA growth were explored. Both pulmonary artery branches grew between BDCPC and TCPC (9.1 mm of diameter (IQR 7.2–12.2) to 12.2 mm (IQR 9.9–14.4) for right PA; P < 0.0001, 8.5 mm of diameter [db1] (IQR 6.5–7.3) to 9.6 mm (IQR 8.0–12.4) for left PA; P = 0.0001). However, PA branches growth was not proportional to child's growth as we noted a decrease of the Nakata index over this period. The main factor associated with a better PA branch growth was the presence of an additional pulmonary blood flow through a pulsatile antegrade flow (+28.2 mm 2 of section area for right PA; P = 0.020, +16.8 mm 2 for left PA; P = 0.043). No other factor preceding BDCPC or associated procedure performed simultaneously had an impact on PA branch growth. PA branches growth is reduced after BDCPC and this may hamper the functioning of the future TCPC. Maintaining a pulsatile antegrade blood flow whenever possible could optimize PA growth and prevent deleterious early remodeling of the pulmonary vascular bed.

Fontan-associated liver disease.

Fontan-associated liver disease.

Outcomes of single ventricle palliation in infants with heterotaxy syndrome.

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. Records of patients born between 2001 and 2019 with heterotaxy, who needed staged single ventricle palliation were retrospectively analysed. 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). 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.

Augmentation of Pulmonary Arterial Growth in Single Ventricle Patients by Interim Selective Shunts

Stenosis or diffuse hypoplasia of central pulmonary arteries (PA) is common in patients with single ventricle physiology, often requiring surgical patching. Such repairs are prone to failure, particularly with low pressure venous flow (bidirectional cavopulmonary connection or Fontan). We describe our experience of disconnection of central PA and selective systemic-PA shunt to the hypoplastic vessel. Single ventricle patients (n = 12) with diffuse left pulmonary artery (LPA) hypoplasia (LPA:right pulmonary artery diameter <0.7) underwent PA disconnection (ligation clip) and selective arterial shunt to the LPA. Patients with ≤mild atrioventricular valve regurgitation, and no more than mild systolic dysfunction on echocardiogram were considered. Following systemic-LPA shunt, patients were reassessed by cardiac catheterization prior to further surgery, with follow-up catheterization later performed and description of changes observed. Increased volume loading was well tolerated with no greater than mild atrioventricular valve regurgitation and preserved systolic function (normal or mildly reduced). Selective arterial shunting increased the caliber of the LPA from 4.1 mm (1.2-5.6) to 6.5 mm (1.7-11.9) and this increase was preserved post-Fontan (6.7 mm [1.3-8.0]) (median [range]). Ventricular end diastolic pressure increased with arterial shunting but resolved after shunt takedown and Fontan completion (median +3 and -4 mm Hg respectively). Post-Fontan hospital length of stay was not prolonged (median 11 days, range 7-14). No deaths occurred. In univentricular hearts and PA hypoplasia, selective systemic-PA shunting physiologically increases the caliber of the distal vessels. In selected patients this can be done safely with maintenance of PA growth and resolution of the elevated end diastolic pressure with Fontan completion.

Pulmonary arteriovenous malformations in children after the Kawashima procedure: Risk factors and midterm outcome.

Background:Pulmonary arteriovenous malformations (PAVMs) are the major cause of progressive cyanosis in patients palliated with bidirectional cavopulmonary connection (BCPC). The aim of our study is to analyze the occurrence of PAVMs in patients after Kawashima procedure, to study the effect of total cavopulmonary connection (TCPC) on PAVMs, to evaluate the effect of axillary arteriovenous fistula (AAVF) creation on PAVMs, and to study the risk factors for PAVMs.Methods:In this retrospective cohort study, all patients with left isomerism and azygous continuation of an interrupted inferior vena cava who underwent Kawashima procedure from July 2001 to December 2017 were included.Results:Twenty.six patients after Kawashima procedure were included in our study. PAVMs were diagnosed in 12 patients (46%). Five of these 12 patients underwent TCPC with complete resolution of hypoxemia. Three patients underwent AAVF creation, 2 had complete resolution, while 1 had partial resolution of hypoxemia. Fourteen patients (54%) did not develop PAVMs. Nakata index below 267 mm2/m2and McGoon ratio below 1.9 predicted the development of PAVMs with high sensitivity and specificity.Conclusions:PAVMs represent a serious complication in patients who undergo Kawashima procedure. Small size of pulmonary arteries is an important risk factor for the development of PAVMs. Resolution of hypoxemia after TCPC completion supports the hepatic factor hypothesis. Early TCPC completion in these patients may help to avoid the development of PAVMs by restoring the hepatic factor. Resolution of hypoxemia after AAVF creation may support the lack of pulsatile flow hypothesis.

The impact of compliance on Stage 2 uni-ventricular heart circulation: An experimental assessment of the Bidirectional Glenn

The Bidirectional Glenn (BDG) or cavopulmonary connection is typically undertaken to volume unload the single ventricle in an effort to preserve ventricular and atrioventricular valve function. The geometry of this surgical palliation has been shown to influence the fluid energy loss as well as the distribution of flow that enters through the superior vena cava. In-vitro and in-silico studies to date have been performed on rigid wall models, while this investigation looks at the impact of flexible thin walled models versus rigid walls. Rigid and compliant models of two patient-specific Glenn geometries were fabricated and tested under various flow conditions, within a biosimulator capable of replicating patient specific flow conditions. It was found that the compliant models exhibit greater levels of energy loss compared to the rigid models. Along with these findings greater levels of turbulence was found in both compliant models compared to their rigid counterparts under ultrasound examinations. This shows that vessel compliance has a significant impact on the hemodynamics within hypoplastic left heart syndrome.

Loss of Ventricular Function After Bidirectional Cavopulmonary Connection: Who Is at Risk?

Decline of single ventricle systolic function after bidirectional cavopulmonary connection (BDCPC) is thought to be a transient phenomenon. We analyzed ventricular function after BDCPC according to ventricular morphology and correlated this evolution to long-term prognosis. A review from Mayo Clinic databases was performed. Visually estimated ejection fraction (EF) was reported from pre-BDCPC to pre-Fontan procedure. The last cardiovascular update was collected to assess long-term prognosis. A freedom from major cardiac event survival curve and a risk factor analysis were performed. 92 patients were included; 52 had left ventricle (LV) morphology and 40 had right ventricle (RV) morphology (28/40 had hypoplastic left heart syndrome (HLHS)). There were no significant differences in groups regarding BDCPC procedure or immediate post-operative outcome. EF showed a significant and relevant decrease from baseline to discharge in the HLHS group: 59 ± 4% to 49 ± 7% or − 9% (p < 0.01) vs. 58 ± 3% to 54 ± 6% or − 4% in the non-HLHS RV group (p = 0.04) and 61 ± 4% to 60 ± 4% or − 1% in the LV group (p = 0.14). Long-term recovery was the least in the HLHS group: EF prior to Fontan 54 ± 2% vs. 56 ± 6% and 60 ± 4%, respectively (p < 0.01). With a median follow-up of 8 years post-BDCPC, six patients had Fontan circulation failure, four died, and three had heart transplantation. EF less than 50% at hospital discharge after BDCPC was strongly correlated to these major cardiac events (HR 3.89; 95% Cl 1.04–14.52). Patients with HLHS are at great risk of ventricular dysfunction after BDCPC. This is not a transient phenomenon and contributes to worse prognosis.

OUTCOMES OF BIDIRECTIONAL GLENN PROCEDURE IN CARDIAC MALFORMATIONS OTHER THAN HYPOPLASTIC LEFT HEART SYNDROME VERSUS HYPOPLASTIC LEFT HEART SYNDROME

Various cardiac malformations would default to a single ventricle physiology. The Glenn bidirectional cavopulmonary connection (BCPC) is the second stage in a multistage surgical palliation pathway. Objective of this study is to compare BCPC outcomes in patients with hypoplastic left heart syndrome

Conexão cavopulmonar parcial como primeiro estágio no tratamento do coração univentricular em um centro na Amazônia: resultados imediatos

Objetivo: Analisar a eficácia da conexão cavopulmonar parcial como paliação primária no tratamento do coração univentricular em pacientes com mais de três meses. Métodos: Foram avaliados retrospectivamente os prontuários de pacientes portadores de cardiopatias congênitas com fisiologia univentricular submetidos à conexão cavopulmonar como primeiro estágio. Foram avaliados os seguintes parâmetros: idade, sexo, diagnóstico anatômico, cardiopatias associadas, tempo de circulação extracorpórea, tempo de intubação, tempo de internação hospitalar, complicações pós-operatórias, saturação periférica de oxigênio e da pressão sistólica da artéria pulmonar pré e pós-operatória. Resultados: Foram identificados 11 casos de crianças submetidas a conexão cavopulmonar parcial como paliação primária. A média de idade foi 10,54 meses, com leve prevalência do sexo feminino (54,54%). As principais características anatômicas das cardiopatias foram Atresia tricúspide e Ventrículo Único tipo esquerdo. Houve aumento na saturação de oxigênio variando de uma média de 74,46% para 88,27% (p&lt;0,01), com adequação da pressão sistólica de artéria pulmonar de 20,80mmHg para 17,80mmHg (p&lt;0,05). Ocorreram dois casos de paresia frênica e um caso de quilotórax os quais foram corrigidos cirurgicamente. Não houve óbitos na amostra. Conclusão: A conexão cavopulmonar parcial como paliação primária no tratamento do coração univentricular teve resultados imediatos satisfatórios quando realizada após os três meses de idade.

Perioperative Assessment of Cerebral Oxygen Metabolism in Infants With Functionally Univentricular Hearts Undergoing the Bidirectional Cavopulmonary Connection.

The transition from single-ventricle lesions with surgically placed systemic-to-pulmonary artery shunt to the circulation following a bidirectional cavopulmonary connection results in higher pressure in the superior vena cava when compared with the preceding circulation. The aim of this study was to evaluate the impact of this transition on the perioperative cerebral oxygen metabolism. Prospective observational cohort study. Pediatric critical care unit of a tertiary referral center. Sixteen infants after bidirectional cavopulmonary connection. Cardiac surgery (bidirectional cavopulmonary connection). We measured regional cerebral oxygen saturation, amount of hemoglobin, blood flow velocity, and microperfusion immediately before, 12-24 hours, and 36-48 hours following bidirectional cavopulmonary connection. Based on these measurements, we calculated cerebral fractional tissue oxygen extraction and approximated cerebral metabolic rate of oxygen. Mean pressure in the superior vena cava increased significantly (8 vs 17 mm Hg; p < 0.001) following bidirectional cavopulmonary connection. Mean cerebral oxygen saturation increased from 49.0% (27.4-61.0) to 56.9% (39.5-64.0) (p = 0.008), whereas mean cerebral blood flow velocity decreased from 80.0 arbitrary units (61.9-93.0) to 67.3 arbitrary units (59.0-83.3) (p < 0.001). No change was found in the cerebral amount of hemoglobin and in the cerebral microperfusion. Mean cerebral fractional tissue oxygen extraction (0.48 [0.17-0.63] vs 0.30 [0.19-0.56]; p = 0.006) and approximated cerebral metabolic rate of oxygen (5.82 arbitrary units [2.70-8.78] vs 2.27 arbitrary units [1.19-7.35]; p < 0.001) decreased significantly. Establishment of bidirectional cavopulmonary connection is associated with postoperative improvement in cerebral oxygen metabolism. Cerebral amount of hemoglobin did not increase, although creation of the bidirectional cavopulmonary connection results in significant elevation in superior vena cava pressure. Improvement in cerebral oxygen metabolism was due to lower cerebral blood flow velocity and stable microperfusion, which may indicate intact cerebral autoregulation.

Determinants of acute events leading to mortality after shunt procedure in univentricular palliation

ObjectiveThe study objective was to identify determinants of adverse outcomes in patients undergoing univentricular palliation after the shunt procedure. MethodsWe performed a retrospective study of patients with univentricular lesions undergoing modified Blalock–Taussig shunt and central shunt placement, with or without concomitant Norwood/Damus–Kaye–Stansel procedures, between 2004 and 2014. ResultsOverall, 246 patients were included, with 150 patients undergoing concomitant Norwood/Damus–Kaye–Stansel procedure. The 30-day and in-hospital mortality were 7.3% and 14.6%, respectively. Progression to bidirectional cavopulmonary connection was achieved in 76% of patients. Acute events occurred in 66 patients (27%), with 42 having an acute event in the first 72 hours postoperatively. Of all in-hospital deaths, 75% had preceding acute events. On multivariable analysis, the risk factor for 30-day or in-hospital mortality was the incidence of an acute event (odds ratio [OR], 13.5; 95% confidence interval [CI], 5.51-36.4; P < .001). Postoperative pH was associated with fewer acute events (OR, 0.61 per 0.1 unit increase; 95% CI, 0.45-0.82; P = .002). Risk factors for shunt thrombosis associated with an acute event were increased postoperative hematocrit (OR, 2.12 per 0.1 unit increase; 95% CI, 1.01-4.58; P = .049) and 3.0-mm shunt size (OR, 3.78; 95% CI, 1.47-10.6; P = .007). Damus–Kaye–Stansel/Norwood procedure, shunt type, morphology, and extracardiac or genetic anomaly were not risk factors for mortality or acute events. ConclusionsMore than one-fifth of patients shunted during univentricular palliation die before the second stage. The majority of these deaths are associated with acute events occurring early after surgery. Strategies to improve survival should focus on prevention and management of acute events.

Increased extracellular volume in the liver of pediatric Fontan patients

BackgroundPatients with single ventricle physiology are at increased risk for developing liver fibrosis. Its extent and prevalence in children with bidirectional cavopulmonary connection (BCPC) and Fontan circulation are unclear. Extracellular volume fraction (ECV), derived from cardiovascular magnetic resonance (CMR) and T1 relaxometry, reflect fibrotic remodeling and/or congestion in the liver. The aim of this study was to investigate whether pediatric patients with single ventricle physiology experience increased native T1 and ECV as markers of liver fibrosis/congestion.MethodsHepatic native T1 times and ECV, using a cardiac short axis modified Look-Locker inversion recovery sequence displaying the liver, were measured retrospectively in children with BCPC- and Fontan circulations and compared to pediatric controls.ResultsHepatic native T1 time were increased in Fontan patients (n = 62, 11.4 ± 4.4 years, T1 762 ± 64 ms) versus BCPC patients (n = 20, 2.8 ± 0.9 years, T1 645 ± 43 ms, p = 0.04). Both cohorts had higher T1 than controls (n = 44, 13.7 ± 2.9 years, T1 604 ± 54 ms, p < 0.001 for both). ECV was 41.4 ± 4.8% in Fontan and 36.4 ± 4.8% in BCPC patients, respectively (p = 0.02). In Fontan patients, T1 values correlated with exposure to cardiopulmonary bypass time (R = 0.3, p = 0.02), systolic and end diastolic volumes (R = 0.3, p = 0.04 for both) and inversely with oxygen saturations and body surface area (R = -0.3, p = 0.04 for both). There were no demonstrable associations of T1 or ECV with central venous pressure or age after Fontan.ConclusionFontan and BCPC patients have elevated CMR markers suggestive of hepatic fibrosis and/or congestion, even at a young age. The tissue changes do not appear to be related to central venous pressures.Trial registrationRetrospectively registered data.

Corrected Transposition: Anatomic Repair Using the Hemi-Mustard Atrial Baffle and Bidirectional Superior Cavopulmonary Connection.

Anatomic repair for congenitally corrected transposition requires thoughtful surgical planning at both initial (neonatal or late) presentation, and during definitive repair. An algorithmic approach to the overall management of this lesion, with its many associated intracardiac anomalies, is presented. Modified atrial switch with bidirectional superior cavopulmonary connection is commonly utilized and demonstrates favorable results through a 20-year experience. Herein, technical considerations learned during the implementation of this strategy are described and emphasize the concept, in selected cases, of native pulmonary root preservation by translocation as an adjunct that is uniquely suited by adopting this approach.