Extracardiac Total Cavopulmonary Connection Research Articles

The effect of the conduit size on middle-term outcomes in patients with extracardiac total cavopulmonary connection.

The 18- and 16-mm conduits in extracardiac total cavopulmonary connection (eTCPC) were reported to be optimal based on energy loss and flow stagnation at the relatively early phase. However, because the artificial conduit lacks growth potential, we have recently encountered some cases in which the conduit needs to be changed several years after eTCPC. These cases prompted us to reconsider the surgical strategy for eTCPC. We reviewed our 20-year single-centre experience with eTCPC patients (n = 256) to compare the 18-mm conduit (n = 195) and 16-mm conduit (n = 61) in terms of mortality and morbidity. The 16-mm conduit was used significantly more frequently in patients whose main chamber was right ventricle (P < 0.001). There was also a significant difference in preoperative inferior vena cava pressure (P = 0.008). There was a significant difference in the actuarial rate of freedom from late-occurring complications, including mortality, between the 2 groups (P = 0.003). There was a significant difference in the actuarial rate of reoperation-free survival (P = 0.042); however, there was no significant difference in resurgical intervention for the conduit (P = 0.333). In multivariate analysis, preoperative inferior vena cava pressure was an independent predictor for late-occurring complications (hazard ratio 1.19; P = 0.026). Conduit size (18 or 16 mm) itself was not an independent predictive factor for late-occurring complications (P = 0.690). The mid-term clinical outcomes in patients who underwent eTCPC were excellent with low mortality. Preoperative inferior vena cava pressure was the only predictive risk factor for postoperative morbidity, and the 16 mm conduit was not predictive thereof.

Pleural and mediastinal effusions after the extracardiac total cavopulmonary connection: Risk factors and impact on outcome.

This study investigated the volume and duration of pleural and mediastinal effusions following extracardiac total cavopulmonary connection, as well as preoperative risk factors and their impact on outcome. A total of 210 patients who underwent extracardiac total cavopulmonary connection at our center between 2012 and 2020 were included in this study. Postoperative daily amount of pleural and mediastinal drainage were collected and factors influencing duration and amount of effusions were analyzed. The impact of effusions on adverse events was analyzed. Median age at extracardiac total cavopulmonary connection was 2.2 (interquartile range, 1.8-2.7) years with median weight of 11.6 (10.7-13.0) kg. Overall duration of drainage after extracardiac total cavopulmonary connection was 9 (6-17) days. The total volume of mediastinal, right pleural, and left pleural drainage was 18.8 (11.9-36.7), 64.4 (27.4-125.9), and 13.6 (0.0-53.5) mL/kg, respectively. Hypoplastic left heart syndrome (p = 0.004) and end-diastolic pressure (p = 0.044) were associated with high volume of drainages, and hypoplastic left heart syndrome (p = 0.007), presence of aortopulmonary collaterals (p = 0.002), and high end-diastolic pressure (p = 0.023) were associated with long duration of drainages. Dextrocardia was associated with higher volume (p < 0.001) and longer duration (p = 0.006) of left pleural drainage. Duration of drainage was associated with adverse events following extracardiac total cavopulmonary connection (p = 0.015). Volume and duration of pleural and mediastinal effusions following extracardiac total cavopulmonary connection were related with hypoplastic left heart syndrome, aortopulmonary collaterals, and end-diastolic pressure. The duration of drainage for effusions was a risk factor for adverse events after total cavopulmonary connection.

The long-term outcome of extra cardiac lateral tunnel total cavopulmonary connection

Abstract Background Although early results of total cavopulmonary connection (TCPC) using an extracardiac conduit are favorable, the patients' growth leads to linearization of the Fontan route causing energy loss by collision of blood flow from the SVC and the IVC, and PV compression. Use of oversized conduit in young children also causes energy loss due to caliber change, and a risk of thrombosis by flow stagnation. We have performed TCPC with the Extra Cardiac Lateral Tunnel (ECLT) technique using e-PTFE patch to make an angled route design for laminar IVC and SVC blood flow confluence, and to avoid caliber change of the IVC-Fontan route. Purpose To clarify the long-term outcome of the Extra Cardiac Lateral Tunnel TCPC (ECLT-TCPC) using e-PTFE patch. Methods Medical records of the patients who underwent staged ECLT-TCPC from April 2003 to March 2020 were reviewed retrospectively. The surgical technique consists of creation of IVC and PA opening connected by atrial appendage and free wall as a floor and suturing a cut-open e-PTFE graft over the anterior external wall of the right atrium to make a Fontan route 14–16 mm in diameter entering the PA from the ventral aspect to merge the SVC flow with an angle. Sixty five patients were included in the study: 46 cases of functional single ventricle, 10 HLHS, 9 tricuspid atresia. Age at surgery were 33.0±22.2 month and the weight was 11.1±3.7 kg. Preoperative catheterization data were following; PA pressure: 10.3±4.0 mmHg, PVR: 1.9±0.65 woods. Results We had one in-hospital death (1.8%) and three long-term deaths (6.2%) during a follow-up period of 7.3±4.3 years. Long-term complications included 6 cases of arrhythmias requiring medical or surgical treatment. Other complication included 2 cases of thrombotic complications, and 1 case of hemorrhagic complication. Two patients underwent re-operation for TCPC: 1 cyanosis due to residual shunt and 1 cases for occlusion of the Fontan route. The anticoagulant therapy was usually terminated eight months after surgery. At the latest follow-up, 55 (84.6%) patients were anticoagulantion-free. At the one-year follow-up, the diameters of the Fontan route were 11.0±2.4mm at the PA anastomosis level, 10.8±2.5mm at the middle level, 10.0±2.5mm at the IVC anastomosis level. At the latest follow-up, the diameters of the Fontan route at the three levels were 14.9±3.4mm, 13.0±3.2mm, and 13.6±4.9mm, respectively. Using the normal IVC diameter as a reference, the Fontan route diameter at the three levels were 127±35%, 123±28%, and 143±44% of Normal, respectively, remaining above 100%Normal. The pressure gradient between IVC and PA was 1.5±1.0mmHg at the latest follow-up, and no significant changes in the route design were noted. Conclusion The long-term results of ECLT TCPC using e-PTFE patch seemed acceptable. This technique may provide advantages of a long-term maintenance of the Fontan route diameter and design, and an option of a long-term freedom form anticoagulation. Funding Acknowledgement Type of funding sources: None.

Risk factors and outcomes associated with acute kidney injury following extracardiac total cavopulmonary connection: a retrospective observational study.

BackgroundTotal cavopulmonary connection (TCPC) is an important operation for the treatment of complex congenital heart disease. Epidemiology and outcomes for pediatric patients with acute kidney injury (AKI) following extracardiac TCPC have not been well documented. This study investigates the prevalence, risk factors, and outcomes of AKI in children after extracardiac TCPC surgery.MethodsWe retrospectively evaluated patients (age at surgery <18 years) who underwent extracardiac TCPC surgery between January 2008 and January 2020 in the Pediatric Cardiac Surgical Center of Fuwai Hospital, Beijing, China. AKI was defined according to the pediatric-modified risk, injury, failure, loss of function, and end-stage renal disease criteria.ResultsA total of 377 pediatric patients were included in this study; 123 patients (32.6%) had some degree of AKI. Among the patients with AKI, 101 (82.1%) were diagnosed with AKI-risk (AKI-R), while 22 (17.9%) were diagnosed with acute kidney injury/failure (AKI/F) (16 with AKI, and 6 with AKF). Preoperative estimated creatinine clearance (OR: 1.039, 95% CI: 1.024–1.055, P<0.001), neutrophil-to-lymphocyte ratio (OR: 1.208, 95% CI: 1.128–1.294, P<0.001), and renal perfusion pressure (OR: 0.962, 95% CI: 0.938–0.986, P=0.002) on postoperative day (POD) 0 were significantly associated with AKI after TCPC. Having previously undergone a bidirectional Glenn was significantly associated with the severity of postoperative AKI (OR: 0.253, 95% CI: 0.088–0.731, P=0.011). Furthermore, AKI was associated with prolonged mechanical ventilation time, prolonged intensive care unit stay, and composite adverse outcome. Compared with non-AKI patients, the 10-year survival rate of patients with severe AKI was significantly lower (95.5% vs. 65.9%, P=0.009).ConclusionsAlthough the incidence of AKI was high in patients undergoing TCPC surgery, most cases were AKI-R. Severe AKI was significantly associated with early adverse outcomes and poor long-term survival.

Pulsatility Index in Different Modifications of Fontan Palliation: An Echocardiographic Assessment.

BACKGROUNDAdding pulsation to the Fontan circulation might change the fate of patients palliated by this procedure. Our aim was to compare the pulsatility index (PI) of the pulmonary artery (PA) between the various modifications of Fontan palliation.METHODSDoppler-derived PI was measured in PA branches of a cohort of 28 patients palliated by 6 modifications of Fontan procedure. A group of normal individuals was included for comparison.RESULTSAtriopulmonary connection (APC) group had the highest PA branches PI and statistically was close to the PI of the normal individuals (right pulmonary artery [RPA] PI of 1.58 vs. 1.63; p = 0.99 and left pulmonary artery [LPA] PI of 1.54 vs. 1.68; p = 0.46, respectively). The lowest PA branches PI was seen in the group of extracardiac total cavopulmonary connection (RPA PI of 0.62 and LPA PI of 0.65). Other 4 modifications including the extracardiac conduit with oversewn pulmonary valve, extracardiac conduit with preserved adjusted antegrade flow, extracardiac conduit from inferior vena cava onto the rudimentary right ventricle and lateral tunnel had a mean “RPA and LPA” PI of “1.19 and 1.17”, “1.16 and 1.11”, “1.13 and 1.11”, “0.82 and 0.84”, respectively. The modified Dunnett's post hoc test has shown a significant statistical decline in PI of all modifications compared to the normal individuals except for the APC group.CONCLUSIONSFontan palliated patients in different groups of surgical modification showed a spectrum of Doppler-derived PI with the highest amounts belong to the groups of pulsatile Fontan.

The Epidemiology of Persons Living with Fontan in 2020 and Projections for 2030: Development of an Epidemiology Model Providing Multinational Estimates.

IntroductionFontan surgery is a palliative procedure performed in children with a functionally univentricular heart. Improvements in surgical technique over the past 30 years have increased life expectancy in this rare population. However, the epidemiology of persons living with Fontan is poorly understood. This study aimed to estimate the 2020 and 2030 prevalence of persons living with a Fontan circulation in 11 countries across the US, Europe, Australia and New Zealand, by procedure type: [atriopulmonary connection (AP), lateral tunnel total cavopulmonary connection (LT-TCPC) or extracardiac total cavopulmonary connection (EC-TCPC)]; and age group: [children (< 12 years), adolescents (12–17 years), and adults (≥ 18 years old)] by building an epidemiologic model.MethodsThe annual number of Fontan surgeries by country in 2010–2020 were extracted from hospital or claims databases, via procedure codes. The epidemiology of persons living with Fontan was modelled by applying these surgery frequencies to mid-year populations from 1972 to 2020 and overlaying an uptake curve. A literature search identified: 30-day mortality rates, long-term survival, and median age at surgery. Averages of these estimates were inputted into the model to project prevalence in 2030.ResultsThe number of persons living with Fontan in 2020 across the 11 countries was estimated to be 47,881 [66 people per million (ppm)], rising to 59,777 (79 ppm) by 2030. In 2020, this population was 55% adults, 17% adolescents and 28% children shifting to 64%, 13% and 23%, respectively, in 2030. Among all persons living with Fontan, 74%/18%/9% are estimated to have EC-TCPC/LT-TCPC/AP, respectively, in 2020, and 83%/14%/4% in 2030.ConclusionsAccording to this epidemiology model, the Fontan population is growing, partly driven by increased survival rates with the more recent LT-TCPC and EC-TCPC procedures (compared with AP). The 2020/2030 prevalence of persons living with Fontan is 66/79 ppm.Supplementary InformationThe online version contains supplementary material available at 10.1007/s12325-021-02002-3.

Impact of Atrial Fibrillation on Fontan Circulation: Fontan Computational Model

BackgroundPatients with Fontan circulation may have heart failure resulting in atrial fibrillation during the late phase. Inotropic effects to ameliorate hemodynamics on the Fontan circulation are not well understood, especially when in atrial fibrillation. This study was performed to determine whether dobutamine therapy in patients with Fontan circulation has limited effects on improving hemodynamics. MethodsLumped computational models (sinus and atrial fibrillation) were used, including biventricular, atriopulmonary connection, and extracardiac total cavopulmonary connection Fontan models. The condition of atrial fibrillation including lack of atrial beat, irregular ventricular contraction, and time-varying elastance for the ventricle was introduced. A different dose of dobutamine was given by varying the elastance of the ventricle, heart rate, and peripheral resistance. ResultsIn all models, the cardiac output decreased by 22.5% to 25.8% in atrial fibrillation. At 10 μg · kg−1 · min−1 dobutamine in sinus rhythm, the cardiac output increased by 32.3% in the biventricular model but by only 9.2% (P < .001) and 9.1% (P < .001) in the atriopulmonary connection and total cavopulmonary connection Fontan models, respectively. At 10 μg · kg−1 · min−1 dobutamine in atrial fibrillation, the percent increase in the cardiac output in the Fontan circulation (11.8% increase in atriopulmonary connection, P < .001; and 11.9% increase in total cavopulmonary connection, P < .001) was significantly less than that in the biventricular circulation (32.3% increase). ConclusionsIn the Fontan circulation, atrial fibrillation itself reduced the cardiac output by approximately 25%, and dobutamine had a limited effect on increasing the cardiac output, especially when in atrial fibrillation. Maintaining sinus rhythm in patients with Fontan circulation is very important.

Risk factors for the development of arrhythmias after the fontan surgery

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Fontan surgery or total cavopulmonary connection is a palliative procedure for complex congenital heart disease with univentricular physiology, without the possibility of biventricular surgical correction. This surgery has a high mortality rate (70%), with heart failure and arrhythmias being the main causes of death. Objective Identify the pre, intra and post-surgical factors that may predispose to arrhythmias in patients undergoing to Fontan surgery. Methods We conducted a retrospective review of the patients who underwent a total cavopulmonary connection from January 2003 to December 2018. We performed an analysis employing the Chi-square test to determine which variables were associated with the development of arrhythmias. Results During the mentioned period, 115 patients underwent to Fontan surgery, of which 51.3% were women, the average age at the time of surgery was 8 years. The most frequent diagnosis was tricuspid atresia in 44 patients (38.2%), followed by pulmonary atresia in 18 patients (15.7%) and double outlet right ventricle in 14 patients (12.2%). Regarding the type of the procedures performed, 93.9% (n = 108) were total extracardiac cavopulmonary connection and 6.1% (n = 7) were intracardiac. Also, 8.6% (n = 10) were taken to fenestration, the mean size of the fenestration was 7 mm (7 ± 3 mm) After Fontan surgery, there was a high incidence of arrhythmias which was 38% (n = 44). 22 patients (50%) presented them in the late postoperative period, being this, the most frequent time for the appearance of arrhythmias. We found that the intracardiac surgery presented an OR of 4.423 (95% CI 0.819 - 23.879, p&amp;gt; 0.05), being not statistically significant. Instead, the bidirectional cavopulmonary shunt previous to the Fontan surgery presented an OR of 0.255 (95% CI 0.081 - 0.806, p &amp;lt;0.05) and on the other hand the QRS duration &amp;gt; 120 ms was significantly associated with the appearance of arrythmias with an OR 2.99 (95% CI 1.2-4.2, p &amp;lt;0.05). Conclusions In patients undergoing to Fontan surgery, the previous bidirectional cavopulmonary bypass is a protective factor for the development of arrhythmias. And an QRS duration &amp;gt; 120 ms is a parameter that predicts the apparence of arrhythmias. Abstract Figure. QRS INTERVAL POST FONTAN SURGERY

Long-term Outcomes of Extracardiac Total Cavopulmonary Connection for Apicocaval Juxtaposition

The long-term outcomes of a total cavopulmonary connection (TCPC) with an extracardiac conduit (ECC) for patients with apicocaval juxtaposition (ACJ) remain unclear. A total of 38 patients with ACJ who underwent TCPC with ECC between 1998 and 2014 were enrolled in this study. For 19 patients with a superior vena cava - inferior vena cava contralateral position, a long-curved route rounding the opposite side of the apex was selected (CC group). For 11 patients with a superior vena cava-inferior vena cava ipsilateral position, a long-curved route was principally selected (IC group); however, a short, straight route was selected for 8 patients because there was sufficient space behind the ventricular apex (IS group). Follow-up was completed in all patients, with a mean follow-up duration of 13.2 ± 4.9 years. The angles of the caudal conduit anastomosis site measured from the frontal view of cineangiography had significantly straightened in the CC group from 1 year to 15 years (P < .05) and in the IC group from 1 year to 10 years (P < .05). There were 2 late mortalities and 6 reoperations during follow-up. Overall survival and freedom from reoperation rates at 15 years were 95% and 82%, respectively. There were no conduit-related or route-related complications such as death, reoperations, pulmonary venous obstructions, conduit obstructions, or pulmonary arteriovenous malformations in any of the groups. Even though chronologic geometric changes of curved ECCs were observed, TCPC with ECC for patients with ACJ can be safely applied without conduit- or route-related complications in long-term follow-up.

A novel procedure of puncture to access the pulmonary venous atrium in complex congenital heart disease with surgical correction

BackgroundIn complex congenital heart disease, supraventricular arrhythmia develops long term after surgical repair. The arrhythmia could have pharmacological tolerance and sometimes be fatal. We report our experiences with puncturing tough or calcified surgical repair material to access the pulmonary venous atrium for the purpose of the management of arrhythmia in complex congenital heart disease with surgical correction. MethodsFrom June 2016, subsequent 9 patients underwent the procedure. Their age at the procedure ranged from 11 to 43 years old (median 26.4 years old). Surgical procedures were Mustard procedure (XenomedicaⓇ) in 6, lateral tunnel total cavo-pulmonary connection with autologous pericardium in 2, and extra-cardiac total cavo-pulmonary connection with expanded polytetrafluoroethylene (ePTFE) tube in 1. ResultsMulti-planar reconstruction imaging by cardiac computed tomography was done for making plans before the procedures. Under intracardiac echocardiography guidance, an 8 French steerable sheath was perpendicularly adjusted to target objects. The sharpened edge of 0.014 inch guide wire penetrated with gentle forward force. Subsequently, the puncture site was dilated from a small balloon to a large one step by step. In all patients, penetration was successfully completed. Catheter ablation was achieved in 8 patients and a pacemaker was implanted through the re-canalized superior caval vein in 1 patient. No complications were recorded. ConclusionThe trans-catheter puncture of the synthetic or calcified material is safe and feasible, although careful planning is required with imaging. A steerable sheath could easily be controlled to the make appropriate angle to the target object for preventing slippage and conveying effective force.

Perioperative and long-term management of Fontan patients.

A Fontan circulation requires a series of three-staged operations aimed to palliate patients with single-ventricle CHD. Currently, the most frequent technique is the extracardiac total cavopulmonary connection, an external conduit connecting the IVC and right pulmonary artery, bypassing the right side of the heart. Fontan candidates must meet strict criteria; they are assessed utilising both cardiac catheterisation and cardiac magnetic resonance. Postoperatively, treatment protocols prioritise antibiotic prophylaxis, diuretics, angiotensin-converting enzyme inhibitors, anticoagulation, and oxygen therapy with fluid restriction and a low-fat diet. These measures aim to reduce length of stay in the ICU and hospital by preventing acute complications such as infection, venous thromboembolism, low cardiac output, pleural effusion, and acute kidney injury. Late complications of a Fontan procedure include circulation failure, protein-losing enteropathy, plastic bronchitis, and Fontan-associated liver disease. The definitive management is cardiac transplantation, with promising innovations in selective embolisation of lymphatic vessels and Fontan-specific ventricular assist devices. Further research assessing current protocols in the perioperative management of Fontan patients would be beneficial for standardising current practice and improving outcomes.

An Anterior Anastomosis for the Modified Fontan Connection: A Hemodynamic Analysis

This hemodynamic feasibility study examined total cavopulmonary connection (TCPC) designs connecting the extracardiac conduit to the anterior surface of pulmonary arteries (PAs) or superior vena cava (SVC) rather than to the inferior PA surface (traditional TCPC). The study involved twenty-five consecutive Fontan patients meeting inclusion criteria from a single institution. A virtual surgical platform mimicked the completed traditional TCPC and generated three anterior anastomosis designs: Anterior-PA, Middle-SVC, and SVC-Inn (Inn: innominate vein). Hemodynamic performance of anterior anastomosis designs was compared with the traditional TCPC regarding indexed power loss (iPL) and hepatic flow distribution (HFD). Compared to the traditional TCPC, the Anterior-PA design produces a similar iPL. The Middle-SVC design is also similar, though the iPL difference is positively correlated with the anastomosing height. The SVC-Inn design had significantly more iPL. The three anterior anastomosis designs did not have a significant difference in HFD (from traditional TCPC). Pulmonary flow distribution (PFD) has a stronger correlation with HFD from the anterior anastomosis designs than the traditional TCPC. This hemodynamic feasibility study examined anterior anastomosis, extracardiac TCPC designs that may offer surgeons clinical dexterity. The Anterior-PA design may be equivalent to the traditional TCPC. Fontan extracardiac conduit anastomosis just superior to the PAs (Middle-SVC) also preserves hemodynamic performance and avoids direct PA anastomosis. These designs could simplify surgical Fontan completion, and may particularly benefit patients requiring surgical dissection, having atypical PA orientation, or after PA stent angioplasty.

Risk Factors for Mid-Term Liver Disease After the Fontan Procedure.

The Fontan procedure is a palliative surgery performed for patients with complex congenital heart disease who exhibit functional single ventricular physiology. Although clinical outcomes of the Fontan procedure have improved in recent years and most patients who undergo the procedure reach adulthood, Fontan-associated liver disease (FALD) is a noncardiovascular complication that has become increasingly common; its risk factors remain unknown.A total of 95 patients who underwent the Fontan procedure and who were followed up for at least three years at Gunma Children's Medical Center and Kitasato University Hospital between 1996 and 2015 were retrospectively enrolled in this study.The mean age of the patients at the time of Fontan procedure was 2.3 ± 1.4 years. Overall, 21 patients (23.1%) experienced FALD. All Fontan procedures were performed with extracardiac total cavopulmonary connection using 16-mm expanded polytetrafluoroethylene grafts. The presence of systemic right ventricle, requirement of pulmonary vasodilator, application of a non-fenestrated Fontan procedure, and absence of fenestration flow at the time of follow-up catheter examination were identified as predictors of FALD using univariate analysis. All these factors, except the requirement of pulmonary vasodilator, remained significant predictors of FALD in multivariate logistic regression analysis.Patients with a systemic right ventricle who undergo the Fontan procedure are at a high risk of FALD in the mid-term. Creating fenestration at the time of Fontan and maintaining the fenestration flow may reduce the mid-term risk of FALD.

Total cavopulmonary connection with a new restorative vascular graft: results at 2 years

BackgroundTo present a 2-year follow-up regarding safety and hemodynamic performance of a new restorative vascular graft used as extracardiac cavo-pulmonary connection in patients with univentricular congenital heart malformations.MethodsThe graft was implanted in five patients (aged 4–12 years) as extracardiac connection between the inferior vena cava and the pulmonary artery. The conduit consists of a bioabsorbable polymer-based implant able to generate endogenous tissue restoration leading to a fully functional neo-vessel while the polymer progressively absorbs. All patients have reached more than 24 months following surgery and underwent echocardiography and magnetic resonance imaging.ResultsAll patients are doing well at 24 months follow-up, with no graft-related serious adverse events. Transthoracic echocardiography demonstrated adequate function of the conduit in all patients while magnetic resonance imaging showed anatomical and functional stability of the restorative grafts.ConclusionsThe new restorative conduit has been successfully used for the second step of the Fontan procedure as extracardiac total cavopulmonary connection. The results are promising because they suggest that complete transformation of a bioabsorbable polymer and replacement through endogenous tissue may represent a major advantage in the treatment of congenital heart disease patients. Further monitoring will allow to evaluate the long-term behavior of this new graft, in terms of clinical and hemodynamic performance, thrombogenicity and ability to grow.

Meropenem pharmacokinetics during extracorporeal membrane oxygenation and continuous haemodialysis: a case report

ObjectivesPharmacokinetic (PK) parameters can change significantly during extracorporeal membrane oxygenation (ECMO) and continuous haemodialysis. This case report describes the pharmacokinetics of a 3-h meropenem infusion in an infantile anuric patient on ECMO with continuous haemodialysis. CaseA 19-month-old female patient with asplenia syndrome was admitted to the paediatric intensive care unit for postoperative management of an extracardiac total cavopulmonary connection procedure. Veno-arterial ECMO and continuous haemodialysis were initiated on postoperative Day 2 for circulatory insufficiency due to septic shock and thrombosis of the inferior vena cava extending to the pulmonary artery. Blood and ascites cultures were positive for extended-spectrum β-lactamase-producing Escherichia coli, and 3-h meropenem infusions [120–300 mg/kg/day divided every 8 h (q8h)] were commenced. Following dose escalation to 300 mg/kg/day q8h, sustained negative blood cultures were confirmed. The estimated meropenem clearance and volume of distribution (Vd) were 2.21 mL/kg/min and 0.59 L/kg, respectively. These patient-specific PK parameters were used to predict the PK profile of various dosing regimens. Both 1-h and 3-h infusions of meropenem at 60, 120 and 200 mg/kg/day q8h predicted that the free drug concentration would remain above the minimum inhibitory concentration (fT>MIC) at an MIC of 1 μg/mL for >40% of the dosing interval. However, when the target was set at 100% fT>MIC, only a 3-h infusion of 200 mg/kg/day q8h could achieve the target in this patient despite the presence of anuria. ConclusionTo optimise meropenem dosing in paediatric patients on ECMO and continuous haemodialysis, further study and PK monitoring are warranted.

Fontan completion during winter season is not associated with higher mortality or morbidity in the early post-operative period.

The aim of our study was to compare post-operative outcome after total cavopulmonary connection between patients operated during winter and summer season. We retrospectively studied 211 patients who underwent extracardiac total cavopulmonary connection completion at our institution between 1995 and 2015 (median age 4 (1-42) years). Seventy (33%) patients were operated during winter (November to March) and 141 (67%) patients during summer season (April to October). Patients operated during winter and summer season showed no difference in early mortality (7% versus 5%, p = 0.52) and severe morbidity like need for early Fontan takedown (1% versus 1%, p = 0.99) and need for mechanical circulatory support (9% versus 4%, p = 0.12). The post-operative course and haemodynamic outcome were comparable between both groups of patients (ICU (4 versus 3 days, p = 0.44) and hospital stay (15 versus 14 days, p = 0.28), prolonged pleural effusions (36% versus 31%, p = 0.51), need for dialysis (16% versus 11%, p = 0.37), ascites (37% versus 33%, p = 0.52), supraventricular tachyarrhythmia (16% versus 13%, p = 0.56) and chylothorax (26% versus 16%, p = 0.12), change of antibiotic treatment (47% versus 36%, p = 0.06), prolonged inotropic support (24% versus 14%, p = 0.05), intubation time (15 versus 12 hours, p = 0.33), and incidence of fast-track extubation (11% versus 22%, p = 0.06). Outcomes after total cavopulmonary connection completion during winter and summer season were comparably related to mortality, severe morbidity, or longer hospital stay in the early post-operative period. These results suggest that total cavopulmonary connection completion during winter season is as safe as during summer season.

Pulmonary venous obstruction after extracardiac total cavopulmonary connection in right atrial isomerism.

Patients with functional single ventricle and right atrial isomerism (RAI) often have multiform cardiac pulmonary venous (PV) connection, which could be a risk factor for pulmonary venous obstruction (PVO) after extracardiac total cavopulmonary connection (EC-TCPC) owing to compression of the conduit. To investigate the anatomical risk factors for PVO after EC-TCPC in RAI. Twenty-nine patients with RAI without extracardiac total anomalous pulmonary venous connection were enrolled. No patients had PVO before EC-TCPC. A total of 14 and 15 patients had PV orifices ipsilateral and contralateral to the extracardiac conduit, respectively. The former 14 patients were assigned to two groups based on development of PVO after EC-TCPC (groups O and N). The pre- and post-operative cardiac morphologies and their relationship with the conduit were compared. After the EC-TCPC, the pressure gradients between the atrium and the PV were 5.0 ± 2.5 and 0.44 ± 0.2mmHg in groups O and N, respectively (p < 0.01); however, the pressure gradients in the left and right PVs were not significantly different, suggesting stenosis of the common PV orifice. The ratio of the horizontal distance from the vertebrae to the PV orifice and to the lateral edge of the atrium was significantly higher (0.38 ± 0.2 vs. 0.17 ± 0.1; p = 0.04) and the orifice was smaller (8.9 ± 2.0 vs. 15 ± 4.7mm; p < 0.01) in group O than in group N. In cases with ipsilateral locations of the conduit and PV orifice, small size and more lateral location of the PV orifice may be preoperative risk factors for development of PVO.

Impact of Phrenic Nerve Palsy on Late Fontan Circulation

Although adverse effects of phrenic nerve palsy (PNP) on early Fontan circulation have been reported, detailed late impact remains unclear. Of 218 patients undergoing extracardiac total cavopulmonary connection between 1995 and 2008, 160 who all underwent cardiac catheter examination, spirometry, and exercise capacity testing 10 years after the operation were enrolled. The cohort was divided into 2 groups: with (N= 21) or without PNP (control group, N= 139). The patients with PNP were further divided into the recovered PNP group (n= 10) and the persistent PNP group (n= 11). All but 2 patients who developed PNP (90.9%) underwent diaphragmatic plication. There was no difference in hemodynamic indices at pre-Fontan evaluation among the three groups. Ten years after the Fontan procedure, the averaged forced vital capacity was 81% ± 18% of predicted in the control group, 86% ± 17% in the recovered PNP group, and 56% ± 12% in the persistent PNP group (P < .001). Peak oxygen consumption was linearly correlated to the forced vital capacity (r= 0.222, P= .009). There was no significant difference in the peak oxygen consumption between groups. Significant veno-venous collaterals into the diaphragm from lower body to pulmonary vein(s) or atria more frequently developed in patients who underwent diaphragmatic plication compared with those who did not (P < .001). Persistent PNP resulted in reduced forced vital capacity; however, its influence on exercise intolerance could not be identified. Diaphragmatic plication should be reserved for patients who experience clinically significant respiratory or hemodynamic sequelae of PNP.

Early extubation improves outcome following extracardiac total cavopulmonary connection.

The aim of this study was to investigate the impact of an early extubation strategy on the outcome following extracardiac total cavopulmonary connection. From 1999 through 2017, 458 patients underwent extracardiac total cavopulmonary connection; 257 (56%) patients were managed with an early extubation strategy adopted in 2009 (group A). Their outcome was compared with those of 201 (44%) patients treated before 2009 (group B). In group A, the outcome of unstable patients, defined as >75th percentile for volume administered and inotrope scores, was compared with those of stable patients. Ventilation time (median: 4 h vs 16 h, P < 0.001), fluid volume administered during the first 24 h (mean: 110 ml/kg vs 164 ml/kg, P = 0.003), chest tube duration (median: 3 days vs 4 days, P = 0.028) and length of intensive care unit stay (median: 6 days vs 7 days, P = 0.001) were less in group A than in group B. The reintubation rate (7% vs 6%, P = 0.547) and early mortality (0.8% vs 1.5%, P = 0.465) were similar between groups. The 80 unstable group A patients received more inotropic support (P < 0.001) and fluid volume (P < 0.001) than stable patients, but the ventilation time (6 h vs 5 h, P = 0.220), the reintubation rate (10% vs 6%, P = 0.283) and the length of intensive care unit stay (7 days vs 6 days, P = 0.590) were similar. In unstable patients, mean arterial pressure before extubation was significantly lower than stable patients (P = 0.001). However, mean arterial pressure in unstable patients increased significantly (P < 0.001) soon after extubation, and became similar to the value in stable patients. Early extubation following extracardiac total cavopulmonary connection improves postoperative haemodynamics and recovery regardless of the initial haemodynamic status.

Approaches to Establish Extracardiac Total Cavopulmonary Connections in Animal Models-A Review.

Long-term survival of patients with a single ventricle palliated with a Fontan procedure is still limited. No curative treatment options are available. To investigate the pathophysiology and potential treatment options, such as mechanical circulatory support (MCS), appropriate large animal models are required. The aim of this review was to analyze all full-text manuscripts presenting approaches for an extracardiac total cavopulmonary connection (TCPC) animal model to identify the feasibility and limitations in the acute and chronic setting. A literature search was performed for full-text publications presenting large animal models with extracardiac TCPCs on Pubmed and Embase. Out of 454 reviewed papers, 23 manuscripts fulfilled the inclusion criteria. Surgical procedures were categorized and hemodynamic changes at the transition from the biventricular to the univentricular condition analyzed. Surgical procedures varied especially regarding coronary venous flow handling and anatomic shape of the TCPC. In most studies (n = 14), the main pulmonary artery was clamped and the coronary venous flow redirected by additional surgical interventions. Only in five reports, the caval veins were connected to the right pulmonary artery to create a true TCPC shape, whereas in all others (n = 18), the veins were connected to the main pulmonary artery. An elevated pulmonary vascular resistance was identified as a limiting hemodynamic factor for TCPC completion in healthy animals. A variety of acute TCPC animal models were successfully established with and without MCS, reflecting the most important hemodynamic features of a Fontan circulation; however, chronic animal models were not reported.