Extracardiac Fontan Research Articles

The long-term effects of the fenestration in patients with extracardiac Fontan circulation-a multicenter Korean cohort study based on national Fontan registry.

The long-term effects of fenestration in patients with Fontan circulation remain unclear. We aim to evaluate the fenestration impact on early and late outcomes in patients with extracardiac Fontan (ECF) using a propensity score matching analysis. We performed an extensive retrospective multicenter clinical data review of the Korean Fontan registry and included 1,233 patients with surgical ECF (779 fenestrated, 454 non-fenestrated). Demographics, baseline, and follow-up data were collected and comprehensively analyzed. Patients were divided into two groups according to the baseline presence or absence of surgical fenestration. Subsequently, patients were sub-divided according to the fenestration status at the last follow-up. Propensity-score matching was performed to account for collected data between the 2 groups using a multistep approach. The primary outcomes were survival and freedom from Fontan failure (FFF). We also looked at postoperative hemodynamics, cardiopulmonary exercise test results, oxygen saturations, and functional status. After propensity-score matching (454 matched pairs), there was no difference in survival or FFF between the 2 groups. However, ECF patients with baseline fenestration had significantly lower oxygen saturation (p = 0.001) and lower functional status (p < 0.001). Patients with fenestration had significantly longer bypass times, higher postoperative central venous pressure, higher postoperative left atrial pressure, and less prolonged pleural effusion in the early postoperative period. The propensity score matching according to the fenestration status at the last follow-up (148 matched pairs) showed that patients with a persistent fenestration had significantly lower oxygen saturation levels (p < 0.001). However there were no intergroup differences in the functional status, survival and FFF. Our results showed no long-term benefits of the Fenestration in terms of survival and FFF. Patients with persistent fenestration showed oxygen desaturation but no difference in exercise intolerance was shown between the 2 groups.

Novel approaches for leadless pacemaker implantation in the extra-cardiac Fontan cohort: Options to avoid leaded systems or epicardial pacing.

Fontan surgery, a palliative procedure for single ventricle patients, often leads to the need for permanent pacing. Epicardial pacing has limitations, while transvenous pacing carries risks in the Fontan circulation. This case series introduces a novel approach of leadless pacemaker implantation in the extra-cardiac Fontan (ECF) cohort to overcome these limitations. The study includes four cases of leadless pacemaker (Micra™) implantation in patients with ECF. Procedures were performed under general anesthesia with guidance from trans-esophageal echocardiography. Various access routes were used, including trans-carotid and trans-fenestration approaches. Procedural details, parameters, and follow-up data were collected. All leadless pacemaker implantations were successful, with satisfactory electrical parameters and stable pacing postprocedural outcomes during short-term follow-up. One patient required closure of their fenestration for symptomatic desaturation post procedurally. Leadless pacemaker implantation via trans-carotid and trans-fenestration approaches appears to be a feasible back up option for pacing in Fontan patients where other options have been exhausted or there is a patient choice to avoid surgery. These techniques provide an option to avoid leaded systems or epicardial pacing, reducing the need for multiple thoracotomies and addressing challenges associated with surgical pacing leads. Further studies are needed to evaluate long-term outcomes and assess the broader application of leadless pacemakers in the Fontan population.

The Rate of Hepatic Fibrosis Progression in Patients Post-Fontan.

This investigation analyzed the rate of hepatic fibrosis progression in post-Fontan patients that underwent hepatic biopsy. The study cohort comprised post-Fontan patients that underwent cardiac catheterization and transvenous liver biopsy between March 2012 and September 2019. We identified 126 patients that met inclusion criteria. Of the 126, 27 (21%) had a lateral tunnel Fontan, and 99 (79%) had an extracardiac Fontan. For the 27 lateral tunnel Fontan patients, age at Fontan was 4 ± 2years, and for the 99 extracardiac Fontan patients age at Fontan was 4 ± 2years (p = 0.98). For the 27 lateral tunnel Fontan patients, the average total fibrosis score was 3.0 ± 1.5; and for the 99 extracardiac Fontan patients, the average total fibrosis was 2.7 ± 1.7 (p = 0.48). For the lateral tunnel Fontan patients, the average Fontan duration was 20 ± 6years; and for the 99 extracardiac Fontan patients, the average Fontan duration was 11 ± 5years (p < 0.001). For the 27 lateral tunnel Fontan patients, the average rate of fibrosis progression was 0.16 ± 0.10 total fibrosis score/year; and for the 99 extracardiac Fontan patients, the average rate of fibrosis progression was 0.30 ± 0.23 total fibrosis score/year (p < 0.001). In conclusion, our findings suggest that those with extracardiac Fontans have a faster rate of hepatic fibrosis progression than those with lateral tunnel Fontans. More extensive or multi-institutional studies will be needed to confirm these findings and define the clinical significance of discrepant rates of hepatic fibrosis in post-Fontan patients.

Variation in care for children undergoing the Fontan operation for hypoplastic left heart syndrome.

The Single Ventricle Reconstruction Trial randomised neonates with hypoplastic left heart syndrome to a shunt strategy but otherwise retained standard of care. We aimed to describe centre-level practice variation at Fontan completion. Centre-level data are reported as median or median frequency across all centres and range of medians or frequencies across centres. Classification and regression tree analysis assessed the association of centre-level factors with length of stay and percentage of patients with prolonged pleural effusion (>7days). The median Fontan age (14 centres, 320 patients) was 3.1years (range from 1.7 to 3.9), and the weight-for-age z-score was -0.56 (-1.35 + 0.44). Extra-cardiac Fontans were performed in 79% (4-100%) of patients at the 13 centres performing this procedure; lateral tunnels were performed in 32% (3-100%) at the 11 centres performing it. Deep hypothermic circulatory arrest (nine centres) ranged from 6 to 100%. Major complications occurred in 17% (7-33%). The length of stay was 9.5 days (9-12); 15% (6-33%) had prolonged pleural effusion. Centres with fewer patients (<6%) with prolonged pleural effusion and fewer (<41%) complications had a shorter length of stay (<10 days; sensitivity 1.0; specificity 0.71; area under the curve 0.96). Avoiding deep hypothermic circulatory arrest and higher weight-for-age z-score were associated with a lower percentage of patients with prolonged effusions (<9.5%; sensitivity 1.0; specificity = 0.86; area under the curve 0.98). Fontan perioperative practices varied widely among study centres. Strategies to decrease the duration of pleural effusion and minimise complications may decrease the length of stay. Further research regarding deep hypothermic circulatory arrest is needed to understand its association with prolonged pleural effusion.

Immediate and early post-operative sequelae of off-pump total cavopulmonary connection.

Extracardiac Fontan (ECF) is currently the final operation of choice for patients with a univentricular heart. Performing this procedure without cardiopulmonary bypass (CPB) carries potential benefits. In this study, we report the early results of ECF without CPB. Between 2012 and 2015, 72 consecutive patients underwent Fontan without CPB. Their medical records were examined in detail. Mean age was 11.8 ± 5.2 (range 5 to 23, median 10) years. Intraoperative mean superior vena cava clamp time was 15.19 ± 3.8min, and the inferior vena cava clamp time was 16.93 ± 3.31min. There were three early deaths. No patient required conversion from off-CPB to CPB. Mean inotropic score was 4.73 ± 5.9 (range 0 to 25, median 2.5). Mean time to extubation was 9.5 ± 5.82 (range 3 to 29, median 8) hours. Pleural drainage in intensive care unit (ICU) was 551.57 ± 452.77 (median 470) ml, and mean ICU stay was 2.27 ± 3.09 (median 1.5) days. Mean daily pleural drainage after discharge from the ICU was 163.7 ± 88.01 (median 140) ml, and mean time to removal of pleural tubes was 15.76 ± 8.4 (median 14) days. Total hospital stay was 17.03 ± 8.62 (median 15) days. At an early follow-up of 2-40 (median 25) months, all survivors (n = 69) had a patent Fontan circuit with normal ventricular function on echocardiography. There were no late deaths or thromboembolic complications. Off-pump ECF is a low-risk procedure that avoids the harmful effects of CPB. Post-operative course of these patients is predictable with substantial savings in costs.

Fontan Physiology: Anaesthetic Implications for Non-Cardiac Surgery: A Case Report

Patients with congenital heart diseases are a growing population and noncardiac surgeries will become an important health care issue. Modification of surgical techniques and medical advances has improved the survival of patients with complex congenital cardiac abnormalities, resulting in more adult patients with fontan physiology presenting for non-cardiac surgery. The older fontan patient is at particular risk of thromboembolism, arrhythmias, progressive ventricular dysfunction, protein losing enteropathy and plastic bronchitis. Understanding the physiology of the fontan circulation is essential for the successful anaesthetic management of these patients. Anaesthesia should be administered only in a centre where the relevant cardiology and intensive care expertise are available. We report a case of fontan physiology in a 24 year old female patient who underwent sternal keloid revision under general anaesthesia. Case Report The hospital ethics committee granted approval for the publication of this case report and waiver of informed consent (CIRB Ref No: 2014/391/D). We report the anaesthetic management of a 24 year old female weighing 44 kg with a history of fontan's procedure in childhood who underwent sternal keloid revision under general anaesthesia. She was previously seen by the plastic surgeon for sternal keloid with recurrent infection and pus discharge for one year duration, with inadequate response to medical therapy. She was born with Pulmonary Atresia with Intact Ventricular Septum (PAIVS), large Atrial Septal Defect (ASD) and a hypoplastic right ventricle. She underwent a left Blalock-Tausig (BT) shunt procedure as a neonate. At three years of age, a modified fontan's procedure with BT shunt dissection, anastomosis between superior vena cava to the right pulmonary artery, lateral tunnel using gortex and over sewing of the Main Pulmonary Artery (MPA) was performed. Conversion to extra-cardiac fontan was done at 11 years of age. She was currently on Aspirin 100mg once a day, and on 6 monthly follow-up with her cardiologist. Functionally, she was independent in her activities of daily living, was able to tolerate physical activities up to a maximal effort tolerance of 4 (metabolic equivalents) METS, and did not have any symptoms of cardiovascular compromise. On examination, she was alert but very anxious, afebrile with a blood pressure of 116/74mmHg and heart rate 95 beats per minute. She had a grade 2 systolic murmur while the rest of the systemic examination was unremarkable. A keloid was present extending from the suprasternal notch to the xiphisternum. Preoperative investigations were unremarkable. Electrocardiogram (ECG) showed sinus rhythm. She was reviewed in the Adult Congenital Heart Disease (ACHD) clinic prior to the operation. Her perioperative cardiac risk was estimated to be moderate, and a 2D-echo was arranged on the morning of the operation day as her last 2D-echo was performed more than 2 years ago. Her 2-D echo showed a large ASD with unrestricted flow, inferior vena cava (IVC) fontan pathway patent with low velocity flow, superior vena cava (SVC) connection to pulmonary artery (PA) patent with low velocity flow, hypoplastic right ventricle with right ventricular systolic pressure of

A new procedure for a trans-conduit puncture by grasping the dilator tip with a snare catheter: An alternative access method during catheter ablation of supraventricular tachycardias after an extracardiac Fontan operation

There have been 2 reports of catheter ablation (CA) of supraventricular tachycardias (SVTs) after extracardiac Fontan procedures (EC-Fs) via trans-conduit punctures. 1 Dave A.S. Aboulhosn J. Child J.S. Shivkumar K. Transconduit puncture for catheter ablation of atrial tachycardia in a patient with extracardiac fontan palliation. Heart Rhythm. 2010; 7: 413-416 Abstract Full Text Full Text PDF PubMed Scopus (42) Google Scholar , 2 Pass R.H. Nappo L. Eugenio P. Lopez L. A transbaffle approach to ablation in a child with an extracardiac fontan. Pacing Clin Electrophysiol. 2010; 33: 368-371 Crossref PubMed Scopus (13) Google Scholar However, it was difficult to stabilize the needle tip during the puncture. We report 2 consecutive cases undergoing CA of SVTs using a new trans-conduit puncture procedure.

What is a Modern Fontan?

The Fontan procedure has undergone multiple modifications since its original description in 1971 [1]. However, all modifications before 1988 focused on somehow connecting the right atrial appendage to the pulmonary arteries. This forced the caval blood to take a curved path to the pulmonary arteries, with the inferior caval return following an S-shaped route and the superior caval return taking a 180 turn. Consequently, the caval return lost significant kinetic energy [2]. To improve this, another modification of Fontan was described in 1988 whereby the caval return was ‘‘streamlined’’ (the caval veins took a straight path to the pulmonary arteries), with significantly better kinetic energy and outcome. This was the lateral tunnel total cavopulmonary connection (lateral tunnel TCPC) [2]. Then, 2 years later, an alternative form of streamlined Fontan was described, the extracardiac conduit [3]. Streamlining the Fontan circuit was very significant and marked the transition from the classic to the modern Fontan. The description of modern Fontan seemed simple: if the caval return follows a straight path to the pulmonary arteries, it is modern; otherwise it is classic. Both lateral tunnel TCPC and extracardiac Fontans are therefore modern. The rare cases of intra-atrial tube Fontans, as may be used in the setting of isomerism, also would be considered modern because the inferior caval blood takes a straight path toward the pulmonary arteries. However, this description is incomplete. Another important difference between classic and modern Fontans is worth noting because it may have contributed to the improvement in outcome. In modern Fontans, the coronary sinus is not included in the Fontan circuit. Rather, it drains into the atrium, where the pressure is 7 or 8 mmHg lower than in the Fontan circuit. If the coronary sinus drains into the Fontan circuit (classic Fontan), it may subject the myocardium to the same deleterious effects of chronic venous hypertension seen in other organs (e.g., proteinlosing enteropathy, hepatic and renal dysfunction, ascites). It is interesting that, at least in my experience, cardiac dysfunction usually is a prominent component in the clinical picture of a failing classic Fontan, whereas the clinical picture of a failing modern Fontan tends to be dominated by peripheral complications (e.g., protein-losing enteropathy, renal and hepatic dysfunction) with relatively wellpreserved cardiac function. Perhaps the definition of modern Fontan should consist of both criteria: streamlining and maintaining the drainage of the coronary sinus within the atrium.

EXTRACARDIAC CONDUIT FONTAN PROCEDURE WITHOUT THE USE OF CARDIOPULMONARY BYPASS: EARLY OUTCOMES

We compared intensive care unit (ICU) and overall length of stay (LOS), chest tube (CT) duration, days of mechanical ventilation and mortality for non-fenestrated, extracardiac Fontans (NFEF) performed on and off cardiopulmonary bypass (CBP). A retrospective review identified 135 patients who

Is the Extracardiac Conduit the Preferred Fontan Approach for Patients With Univentricular Hearts?

With improvements in technology, operative technique, and critical care medicine, more and more children with congenital heart disease are surviving into adulthood. As they age, these patients are providing physicians with new challenges, and we are realizing the long-term consequences of our previous interventions. This oftentimes has changed the mode of treatment for subsequent children. Response by Khairy and Poirier on p 2515 Fontan palliation for single-ventricle patients was first described in 1971.1 The original approach was in the form of a classic atriopulmonary Fontan connection, bypassing the nonfunctional right ventricle. After recognizing the long-term complication of an atriopulmonary connection (right atrial dilation, arrhythmias, and thrombus formation), modifications have been made. The atriopulmonary connection has been abandoned in favor of modified Fontan anatomy and physiology. The modified Fontan can be performed by the use of an extracardiac conduit approach or a lateral tunnel approach. The extracardiac Fontan uses an external conduit to anastomose the inferior vena cava into the pulmonary arteries, whereas a lateral tunnel Fontan uses a baffle within the right atrium (Figure 1). Because the systemic venous pathway is completely separated from the right atrium with the extracardiac Fontan, it is more challenging for the electrophysiologist to access the heart for ablation. My aim is to demonstrate why “the extracardiac conduit is the preferred Fontan approach for patients with univentricular hearts.” Figure 1. Left , Lateral tunnel Fontan shows the Fontan pathway inside of the right atrium. Right , Extracardiac Fontan shows the conduit completely separated from the right atrium. The extracardiac Fontan has several advantages over the lateral tunnel Fontan. Minimizing cardiopulmonary bypass and ischemia may optimize early postoperative outcomes. In multiple studies, both prolonged cardioplegic arrest and extended cardiopulmonary bypass times have been associated with an increased risk of early postoperative death or Fontan failure.2– …

Transhepatic approach to create stent fenestration in the extracardiac fontan conduit in a child with dextrocardia and interrupted inferior vena cava with azygos continuation

Plastic bronchitis is a rare life-threatening complication of Fontan operation. When medical treatment is ineffective in the setting of high systemic venous pressures, Fontan fenestration may be considered to decompress venous pressures and improve cardiac output by creation of the right-to-left shunting. However, transcatheter approach can be difficult in patients with complex venous anatomy. We report a 4-year-old girl born with hypoplastic left ventricle and heterotaxy syndrome, who developed plastic bronchitis following extracardiac Fontan procedure. Her venous anatomy was complex with dextrocardia and interrupted inferior vena cava with azygos continuation. Stent fenestration was successfully performed via transhepatic approach, which was selected based on the anatomical relationship (between extracardiac conduit, left atrium, and hepatic veins) delineated by pre-catheterization cardiac MRI. Simultaneous transesophageal echocardiography guided the intervention. Her plastic bronchitis improved significantly in 3 months but slowly progressed after the stent fenestration. At her 8-month follow-up, stent fenestration remains open and she is currently under heart transplantation evaluation due to persistent plastic bronchitis. Treatment of plastic bronchitis can be undertaken with Fontan fenestration, with pre-procedural MRI playing an essential role in patients with complex venous anatomy.

Abstract 2212: Computational Model of Exercise Effects on Fontan Hemodynamics Demonstrates Favorable Energetics In Extracardiac Fontans When Compared to Lateral Tunnel

Background: Previous computational experiments using anatomically correct Fontan geometries have suggested that extracardiac Fontans (EC) have lower power loss compared to lateral tunnel intracardiac (IC) Fontans. Differences in exercise performance may also be important in the long term morbidity of Fontan patients. We have previously developed a computational model for studying exercise conditions. Hypothesis: It is our hypothesis that the EC total cavopulmonary connection (TCPC) is more hemodynamically favorable and demonstrates significantly lower resistance at exercise. Methods: We performed steady flow CFD simulations on 3-D anatomical reconstructions of TCPC from 9 Fontan patients using cardiac magnetic resonance imaging (CMR) data, 5 with EC and 4 with IC. Caval flows were determined using CMR velocimetry and outlet pulmonary artery pressures tuned to obtain flow splits corresponding to equal vascular lung resistance (EVLR) of 2 Woods units for each lung. Simulated exercise conditions of twice (2x) and three times (3x) baseline MRI flow were performed by increasing IVC flow. Power loss calculations using control volume analysis were performed for each TCPC and each flow condition studied. Power losses were normalized to calculate a resistance index and the two groups compared using the Student t-test for independent groups. Results: Resistance index was significantly greater in IC compared to EC Fontans for all three flow conditions. This difference was magnified at exercise flow conditions. At baseline flow, EC TCPC resistance index was 0.14 compared to 0.49 Woods units for IC (p = 0.001). This rose to 0.29 and 0.81 for EC and IC TCPC respectively for the 2x exercise condition (p = 0.002), and to 0.44 and 1.2 for the 3x condition (p = 0.008). There was no significant difference in cardiac output between EC and IC pts, but the EC pts were significantly younger. Conclusions: CFD modeling of 3-D anatomical reconstructions demonstrates significant differences in power loss between EC and IC TCPC which are magnified at exercise conditions. The differences are hemodynamically significant, as high as 0.76 Woods units at the highest exercise flow condition, and are thus likely important in the long-term survival and quality of life of patients with TCPC.

Pulmonary and caval blood flow patterns in patients with intracardiac and extracardiac Fontan: a magnetic resonance study

We compared in vivo blood flow and pulsatility after different types of Fontan operation using magnetic resonance imaging. A total of 37 consecutive patients (mean age 19+/-7.9 years, 7.3+/-3.2 years after Fontan operation), 7 with atriopulmonary anastomosis (APC), 18 with intra-atrial lateral tunnel (LTFO) and 12 with extracardiac Fontan (ECFO) were studied using magnetic resonance phase-contrast velocity mapping. Blood flow (volume flow) in the superior vena cava (SVC), inferior vena cava (IVC) and both pulmonary arteries were measured and a pulsatility index was calculated for each vessel. For all modifications, the blood flow distribution between the SVC and IVC was normal (1:2). Patients with APC had a normal pulsatility, a dilated right atrium, partial backward flow in the IVC and physiological blood flow distribution between the pulmonary arteries. LTFO and ECFO patients had no retrograde flow in the IVC, equal blood flow distribution between the pulmonary arteries and very low or absent pulsatility. MRI allows hemodynamic quantification and characterization of various types of Fontan modifications and may be a valuable tool to predict Fontan failure. Despite showing normal pulsatility, patients with APC have right atrial dilatation and partial backward flow in the IVC, demonstrating suboptimal Fontan circulation. LTFO and ECFO both produce unidirectional antegrade flow in the IVC but pulsatility is very low or absent, which may promote poor pulmonary artery growth and increase of pulmonary vascular resistance contributing to late Fontan failure.

Magnetic Resonance Flow Analysis of Classic and Extracardiac Fontan Procedures: The Seesaw Sign

Energy inefficiencies lead to Fontan procedure failures. Our purpose was to quantify energy inefficiencies of various Fontan procedures using flow analysis. The study included 12 patients with Fontan operations; 7 with classic trans-atrial flow and five extra-cardiac Fontans. Flow analysis was used to determine the systolic and diastolic flow in the superior vena cava and inferior venous circulations (IVC, right atrium or conduit). Retrograde flow fractions were calculated. Inferior venous flow analysis was obtained in 12 patients and superior vena cava in 9 patients. A seesaw pattern (augmented inferior venous flow during diastole and augmented superior vena cava flow in systole) was present in five of seven patients with trans-atrial Fontan procedures, but no patient with an extra-cardiac Fontan procedure. Significant retrograde flow occurred in the superior vena cava in three children with trans-atrial Fontan procedures (retrograde flow fractions of 15-22%). Inferior venous flow occurred predominantly during diastole in five of seven children with trans-atrial Fontan procedures. Retrograde flow occurred in all seven children with the retrograde flow fractions ranging from 9 to 37% (mean 25%). Extracardiac Fontan flow was characterized by continuous balanced flow during systole and diastole. The association of the seesaw pattern with trans-atrial Fontan procedures was statistically significant (p = 0.028). Classic trans-atrial Fontan procedures are characterized by energy inefficiency creating a seesaw flow pattern of forward and reverse flow. Extra-cardiac Fontan procedures are more energy efficient. Magnetic resonance imaging is useful in detecting flow inefficiencies in patients palliated by the Fontan procedure.

Factors related to pleural effusions after Fontan procedure in the era of fenestration.

Significant pleural effusions after the Fontan operation prolong hospital stay, may increase the risk of infection, and may necessitate a pleurodesis procedure. From February 1991 to April 2000, 98 consecutive patients under the age of 18 years underwent the fenestrated Fontan procedure at Children's Hospital of Wisconsin. Ninety-four patients who survived at least 30 days after surgery were retrospectively evaluated for the following factors: age, ventricular morphology (right single ventricle, left single ventricle [RV/LV]), fenestration open (FO) or closed (FC) at end of operation, intracardiac Fontan (IF) or extracardiac Fontan (EF), days with chest tube output per day >5, 10, and/or 20 mL. kg(-1). d(-1) (CTO5, CTO10, and CTO20, respectively), need for pleurodesis, length of hospital stay (LOS), operation during winter respiratory viral season of November through March (ReVS+, ReVS-), and pre-Fontan mean pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR). In univariate analysis, the ReVS+ patients had prolonged LOS, greater chest tube output, and more pleurodesis (P<0.05), and PAP was related to CTO5 and CTO10 but not to CTO20 or LOS. No significant differences were found in LOS, CTO5, CTO10, CTO20, and need for pleurodesis between patients in RV/LV, FO/FC, IF/EF, or PVR groups. Patients <4 years of age had more instances of CTO20 (P<0.05). When we used ordinary least squares regression analysis with age, FO or FC, RV or LV, PAP, and ReVS+ or ReVS- to predict each of CTO5, CTO10, CTO20, and LOS, only ReVS+ or ReVS- and age were statistically significant in all models. Use of the Fontan procedure during the respiratory viral season appeared to be related to significant, prolonged pleural effusions and longer hospitalizations.

Factors Related to Pleural Effusions After Fontan Procedure in the Era of Fenestration

Background Significant pleural effusions after the Fontan operation prolong hospital stay, may increase the risk of infection, and may necessitate a pleurodesis procedure. Methods and Results From February 1991 to April 2000, 98 consecutive patients under the age of 18 years underwent the fenestrated Fontan procedure at Children’s Hospital of Wisconsin. Ninety-four patients who survived at least 30 days after surgery were retrospectively evaluated for the following factors: age, ventricular morphology (right single ventricle, left single ventricle [RV/LV]), fenestration open (FO) or closed (FC) at end of operation, intracardiac Fontan (IF) or extracardiac Fontan (EF), days with chest tube output per day &gt;5, 10, and/or 20 mL · kg −1 · d −1 (CTO5, CTO10, and CTO20, respectively), need for pleurodesis, length of hospital stay (LOS), operation during winter respiratory viral season of November through March (ReVS+, ReVS−), and pre-Fontan mean pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR). In univariate analysis, the ReVS+ patients had prolonged LOS, greater chest tube output, and more pleurodesis ( P &lt;0.05), and PAP was related to CTO5 and CTO10 but not to CTO20 or LOS. No significant differences were found in LOS, CTO5, CTO10, CTO20, and need for pleurodesis between patients in RV/LV, FO/FC, IF/EF, or PVR groups. Patients &lt;4 years of age had more instances of CTO20 ( P &lt;0.05). When we used ordinary least squares regression analysis with age, FO or FC, RV or LV, PAP, and ReVS+ or ReVS− to predict each of CTO5, CTO10, CTO20, and LOS, only ReVS+ or ReVS− and age were statistically significant in all models. Conclusions Use of the Fontan procedure during the respiratory viral season appeared to be related to significant, prolonged pleural effusions and longer hospitalizations.

Early experience with extracardiac Fontan operation

Background. The extracardiac Fontan (ECFO) operation has gained more widespread use over last decade. In this article we analyze our early experience with ECFO. Methods. Thirty-three patients underwent ECFO at median age 4.1 years. Normothermic perfusion with the heart beating was used in 24 patients and cardioplegia in 7. Fenestration was not performed in the last 16 patients who underwent surgery without cardioplegia. Results. The hospital mortality was 6% (2 patients). Necessity or duration of inotropic support, duration of mechanical ventilation, intensive care unit and hospital stay, and incidence of prolonged pleural effusions of patients operated on without cardioplegia were shorter and the rate of complications in these patients was lower than in patients who underwent cardioplegia. Conclusions. Our preliminary experience shows that ECFO can be performed in many patients with normothermic cardiopulmonary bypass and without cardioplegia and fenestration. Avoidance of cardioplegia seems to decrease postoperative morbidity. Incidence of early postoperative arrhythmias is low. Despite encouraging early results, longer follow-up is necessary to prove the real advantages of ECFO.

Transcatheter closure of an extracardiac fontan fenestration

As the extracardiac Fontan operation evolves, a reliable method for creating and subsequently closing communications between the systemic and pulmonary venous chambers would be useful. We describe a surgical technique for creating this “fenestration” and a complementary transcatheter technique that allows safe and reliable closure of these communications regardless of size and position.

Intermediate results of the extracardiac fontan procedure

Fourteen children (ages 2 to 14 years) and 1 adult (32 years) have undergone a modification of the Fontan procedure in which an extracardiac lateral tunnel or conduit is used in combination with staged or simultaneous bidirectional Glenn shunt(s). Extracardiac lateral tunnels (n = 9) were constructed using a polytetrafluoroethylene patch (n = 7), pericardial patch (n = 1), or in situ pericardial flap (n = 1). Extracardiac lateral conduits (n = 6) were constructed using nonvalved homografts (n = 2) or polytetrafluoroethylene tube grafts (n = 4). Fenestrations were created in 4 patients (2 each in extracardiac lateral tunnel and extracardiac lateral conduit patients). Aortic cross-clamping was completely avoided in 12/15 patients (aortic cross-clamping in 2 patients for atrial septal defect enlargement and 1 for Damus-Kaye-Stansel procedure). There have been no operative deaths. Prolonged postoperative chest tube drainage (> 2 weeks) has been rare (n = 1). At follow-up (range, 6 to 54 months; mean, 27.5 months), all patients are in New York Heart Association class I or II and remain in normal sinus rhythm. Late protein-losing enteropathy was seen in 1 patient and was successfully treated by percutaneous creation of a stented fenestration from the extracardiac tunnel to the systemic atrium. Late catheterizations reveal unobstructed extracardiac lateral tunnel function and low pulmonary pressures (range, 11 to 13 mm Hg). Advantages of the extracardiac Fontan include (1) avoidance of aortic cross-clamping in most patients, (2) the hemodynamic benefits of total cavopulmonary connection, (3) avoidance of atriotomy and intraatrial suture lines, (4) preservation of sinus rhythm and no arrhythmias at 2 year follow-up, (5) drainage of the coronary sinus to low pressure atrium, (6) allowance for early/late fenestrations, (7) prevention of baffle leaks and intraatrial obstruction, and (8) allowance for growth (tunnel procedures only). We recommend this extracardiac procedure for all suitable patients undergoing surgical conversion to the Fontan circulation.