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PEDIATRIC CARDIAC Impact of Postoperative Hemodynamics in Patients With Functional Single Ventricle Undergoing Fontan Completion Before Weighing 10 Kg Noritaka Ota, MD, Yoshifumi Fujimoto, MD, Masaya Murata, MD, Yuko Tosaka, MD, PhD, Yujiro Ide, MD, Maiko Tachi, MD, Hiroki Ito, MD, Ai Sugimoto, MD, and Kisaburo Sakamoto, MD Department of Cardiovascular Surgery, Mt. Fuji Shizuoka Children’s Hospital, Shizuoka, Japan Background. Although the Fontan procedure is now being performed in younger patients, the influence of the early timing of Fontan on midterm to long-term results remains unclear. We investigated whether the timing of Fontan completion affects subsequent hemodynamics in patients with functional single ventricle followed for more than 3 years. Methods. Between January 1997 and December 2008, 163 patients with functional single ventricle underwent extracardiac total cavopulmonary connection (TCPC) at a single institution. The survivors routinely underwent postoperative catheterization at 1 year and then every 5 years after TCPC and were divided into group A (weight < 10 kg; n ⴝ 65) and group B (weight > 10 kg; n ⴝ 97), and retrospectively reviewed. Results. Mean follow-up was 6.6 ⴞ 3.7 years. Mean weight and conduit size were 8.5 ⴞ 0.8 kg and 17.1 ⴞ 1.2 mm for group A versus 20.2 ⴞ 13.1 kg and 18.8 ⴞ 1.9 mm for group B, respectively (p < 0.001). There were 3 hospital deaths (group A, n ⴝ 1; group B, n ⴝ 2) and 5 late deaths (group A, n ⴝ 3; group B, n ⴝ 2). No TCPC was taken down. There were no thromboembolic events in either group. The end-diastolic volume of ventricle (eDV) (% of normal) was (232.7 ⴞ 91.4 before TCPC versus 139.3 ⴞ 57.2 5 years after TCPC; p < 0.001) in group A and (209.6 ⴞ 77.7 before TCPC versus 136.7 ⴞ 61.4 5 years after TCPC; p < 0.001) in group B. Ventricular ejection fraction and cardiac index at 5 years were similar in both groups. The end-diastolic pressure of ventricle (eDP) (mm Hg) at 1 year (p ⴝ 0.0037) and at 5 years (p ⴝ 0.047) was significantly lower in group A compared with group B. Conclusions. TCPC can be performed earlier with good intermediate results. Earlier unloading of a univentricular heart by means of TCPC might be advantageous for preservation of future ventricular function. (Ann Thorac Surg 2012;94:1570 –7) © 2012 by The Society of Thoracic Surgeons S gate whether or not the timing of Fontan completion affects the postoperative hemodynamics more than 3 years after Fontan completion. We would like to determine whether or not Fontan operations can be done as safely and well in babies weighing less than 10 kg (about 1 year old) as can be accomplished later. ince the first report of a successful Fontan operation in 1971, this maneuver has been performed with various modifications in patients with single-ventricle physiology [1, 2]. Among these modifications, total cavopulmonary connection (TCPC), introduced by de Leval and colleagues [3] in 1988, is widely accepted because it improves late mortality and morbidity, especially in atrial arrhythmia. The Fontan procedure is now being safely performed in younger patients [4], although how the timing of Fontan completion affects long-term outcome and hemodynamics remains unclear. In a previous study examining the timing of the Fontan operation, our group found that younger patients experienced longer peritoneal drainage and were in the hospital longer [5]. The present study was undertaken to extend the observation of the initial cohort and investi- Accepted for publication June 4, 2012. Presented at the Forty-eighth Annual Meeting of The Society of Thoracic Surgeons, Fort Lauderdale, FL, Jan 28 –Feb 1, 2012. Address correspondence to Dr Sakamoto, Department of Cardiovascular Surgery, Mt. Fuji Shizuoka Children’s Hospital, 860 Urushiyama Aoi-ku Shizuoka, Shizuoka, 420-8660 Japan; e-mail: [email protected]. shizuoka.jp. © 2012 by The Society of Thoracic Surgeons Published by Elsevier Inc Material and Methods Study Design We conducted a single-center retrospective review of the medical records of 163 consecutive patients (83 boys and 80 girls) with functional single ventricle who underwent TCPC at Mt. Fuji Shizuoka Children’s Hospital in Shizuoka, Japan from January 1997 to December 2008. The institutional review board of the hospital approved this study, and individual consent for the study was waived. One patient who came from a foreign country was not included in the study. In accordance with our institutional policy, Fontan candidates underwent cardiac catheterization before operation (n ⫽ 162 patients) and this was repeated in survivors at 1 (n ⫽ 156), 5 (n ⫽ 84), and 10 (n ⫽ 22) years 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2012.06.022 Ann Thorac Surg 2012;94:1570 –7 OTA ET AL FONTAN OPERATION IN INFANTS LESS THAN 10 KG 1571 Variable Sex (F/M) Age at Fontan operation (y) Median Mean Body weight at Fontan operation (kg) Median Mean Size of conduit (mm) Long follow-up (catheter study ⬎ 5 y) Main diagnosis Heterotaxy HLHS DORV Pulmonary atresia Tricuspid atresia Single ventricle Others Type of ventricle (RV/LV) Concomitant anomaly CAVV TAPVR (extracardiac type) MAPCA Hernia Group A (⬍ 10 kg, n ⫽ 65) Group B (⬎ 10 kg, n ⫽ 97) p Value 34/31 46/51 0.63 1.5 (0.8–3.2) 1.6 ⫾ 0.5 5.1 (1.2–25) 7.3 ⫾ 6.0 ⬍ 0.0001 8.7 (5.7–10) 8.5 ⫾ 0.8 17.1 ⫾ 1.2 32 (49.2%) 14.9 (10.1–66) 20.18 ⫾ 13.13 18.79 ⫾ 1.9 52 (53.6%) ⬍ 0.0001 ⬍ 0.0001 0.42 16 (24.6%) 20 (30.8%) 5 7 3 7 7 45/20 30 (30.9%) 3 (3.1%) 12 4 12 19 17 53/44 0.48 ⬍ 0.0001 0.43 0.20 0.11 0.18 0.43 0.072 21 20 11 0 1 37 23 6 2 0 0.505 0.365 0.037 0.52 0.40 CAVV ⫽ common atrioventricular valve; DORV ⫽ double-outlet right ventricle; HLHS ⫽ hypoplastic left heart syndrome; MAPCA ⫽ major aortopulmonary collateral artery; RV/LV ⫽ right ventricle/left ventricle; TAPVR ⫽ total anomalous pulmonary venous return. after TCPC to assess Fontan circulation. Patients also underwent additional cardiac catheterization if clinically indicated. The central venous pressure and end-diastolic pressure of the systemic ventricle were measured at the time of catheterization. The cardiac index was also measured using the Fick principle from the oxygen consumption and oxygen content in the systemic and pulmonary blood. The ventricular volume was calculated using Simpson’s rule. The ventricular ejection fraction was calculated as the ratio of the stroke volume to the end-diastolic volume (%). The end-diastolic volume was divided by the body surface area for standardization, and the values are presented as ratios (% of normal value). Patients From January 1997 to December 2009, 162 patients with functional single ventricle underwent TCPC at Mt. Fuji Shizuoka Children’s Hospital. The survivors routinely underwent postoperative catheterization at 1 year and then every 5 years after TCPC and were divided based on body weight at TCPC into group A (⬍ 10 kg; n ⫽ 65— heterotaxy, n ⫽ 16 [24.6%]; hypoplastic left heart syndrome [HLHS], n ⫽ 20 [30.8%]; and others, n ⫽ 29 [44.6%]) and group B (⬎ 10 kg; n ⫽ 97— heterotaxy, n ⫽ 31 [32.0%]; HLHS, n ⫽ 3 [3.1%]; and others, n ⫽ 63 [64.9%]). The timing of the TCPC procedure was at the discretion of the attending cardiologist and at the convenience of the family of the patient. Anomalies associated with these patients were a common atrioventricular valve in 59 of the 162 patients (36.4%), total anomalous pulmonary venous drainage in 43 of the 162 (26.5%) patients, major atriopulmonary collateral arteries in 2 of the 162 (1.2%) patients, and hiatal hernia in 1 of the 162 patients (Table 1). Operative Procedure Our standard maneuver has been to establish TCPC consisting of expanded polytetrafluoroethylene tubing (Gore-Tex, WL Gore & Associates, Flagstaff, AZ) with cardiopulmonary bypass. Aortobicaval cardiopulmonary bypass with moderate hypothermia (32–34°C) was used on the beating heart. Cardioplegic arrest was used for short periods if intracardiac repair and unusual rerouting for patients with separated hepatic vein [6] was needed. In patients who underwent cross-clamping, myocardial protection was achieved with antegrade cold crystalloid cardioplegia. Conduit diameter was based on patient weight, morphologic relationships, and inferior vena cava (IVC) diameter. The clamp was applied to the atrium-IVC junction and the latter was transected with the intention to preserve a sizable atrial cuff at the IVC stump to allow a longer anastomosis and potential for growth. Conduit-to-IVC anastomosis was completed PEDIATRIC CARDIAC Table 1. Subgroup Comparison 1572 OTA ET AL FONTAN OPERATION IN INFANTS LESS THAN 10 KG Ann Thorac Surg 2012;94:1570 –7 Fig 1. Actual survival. PEDIATRIC CARDIAC with running sutures of 5-0 or 6-0 Prolene (Ethicon, Somerville, NJ). The inferior pulmonary artery wall was opened longitudinally and extended to the level of the main pulmonary artery on 1 side and the takeoff of the right lower lobe pulmonary artery on the others. In small patients, the pulmonary artery incision was extended into the anterior wall of the superior vena cava to obtain enough space for anastomosis. The pulmonary artery end of the conduit was beveled with the medial end longer and the pulmonary conduit anastomosis was completed with a 6-0 Prolene running sutures. All patients underwent modified ultrafiltration. In patients in whom Fontan pressure greater than18 mm Hg developed after modified ultrafiltration, the fenestration was created in the form of side-to-side anastomosis between the conduit and the atrial wall using a 4-mm aortic puncher. In both patient groups, we routinely administered 5 mg/kg ticlopidine as antiplatelet therapy. Warfarin was not routinely used. Statistical Analysis Data are presented as means ⫾ standard deviation as indicated. Differences between the 2 groups (as shown in Table 1) were determined with Fisher’s exact test, the Mann-Whitney rank-sum test, or the Student’s t test, as appropriate. The rate of freedom from reoperation and survival were estimated using the Kaplan-Meier method, and differences in these rates between the groups were assessed using the log-rank test. All analyses were conducted with Prism software, version 5.0.0 (GraphPad Software Inc, LaJolla, CA). All values of p less than 0.05 were considered statistically significant. of TCPC. The major cardiac defects were heterotaxy syndrome in 46 patients (28.4% [46/162]) (n ⫽ 16 in group A, n ⫽ 30 in group B), HLHS in 23 patients (14.2% [23/162]) (n ⫽ 20 in group A, n ⫽ 3 in group B), and others in 93 patients (Table 1). All 162 patients underwent TCPC. One hundred forty of them had previously undergone bidirectional cavopulmonary Glenn anastomosis as a staged approach (group A, n ⫽ 60; group B, n ⫽ 80) before TCPC. The median interval from initial palliation to the time of the bidirectional Glenn operation (BDG) was 6.1 months (range, 2.0 –27.3 months) in group A and 16.9 months (range, 3.4 –165 months) in group B (p ⬍ 0.0001). The median interval from BDG to Fontan operation was 11.7 months (range, 4.4 –30.5 months) in group A and 23.2 months (range, 5.4 –204 months) in group B (p ⬍ 0.0001). There were 3 hospital deaths (group A, n ⫽ 1; group B, n ⫽ 2) and 5 late deaths (group A, n ⫽ 3; group B, n ⫽ 2). The causes of death in these patients were supraventricular tachyarrhythmia in 2 patients, noncardiac infection in 4 patients, and indeterminate issues in 2 patients. The Table 2. Surgical Procedure at the Initial Palliation Procedure Group A Group B (⬍ 10 kg, n ⫽ 65) (⬎ 10 kg, n ⫽ 97) p Value Patients Fontan Modified Norwood Shunt type B-T shunt Central shunt RV-PA conduit PAB PVS repair AVV repair Groups A and B were aged 1.6 ⫾ 0.5 years and 7.3 ⫾ 6.0 years, respectively, (p ⫽ 0.0001) and weighed 8.5 ⫾ 0.8 kg and 20.18 ⫾ 13.1 kg, respectively, (p ⫽ 0.0001) at the time AVV ⫽ atrioventricular valve; B-T shunt ⫽ Blalock-Taussig shunt; PAB ⫽ pulmonary artery banding; PVS ⫽ pulmonary valve stenosis; RV-PA conduit ⫽ right ventricle-pulmonary artery conduit. Results 2 22 3 5 1.0 ⬍ 0.0001 23 2 11 16 3 2 45 3 18 31 3 1 0.2 1.0 0.4 0.4 0.9 0.5 Ann Thorac Surg 2012;94:1570 –7 1573 OTA ET AL FONTAN OPERATION IN INFANTS LESS THAN 10 KG Variable Cardiopulmonary bypass (min) Aortic clamp (n) Clamp time (min) Concomitant procedure Atrioventricular valve plasty Atrioventricular replacement Repair of pulmonary vein stenosis Damus-Kaye-Stansel operation Subaortic stenosis repair Septostomy Fenestration Pulmonary artery plasty Pacemaker related Postoperative Course Mechanical ventilation (h) (median) Duration of chest tube (d) (mean) Length of ICU stay (d) (median) Length of hospital stay (d) (median) Group A (⬍ 10 kg, n ⫽ 65) Group B (⬎ 10 kg, n ⫽ 97) p Value 141.1 ⫾ 45.1 26 (40%) 58.8 ⫾ 27.1 (24) 36.9% 3 2 2 0 0 3 3 10 1 165.1 ⫾ 48.7 62 (63.9%) 71.5 ⫾ 38.8 (41) 46.3% 8 0 2 4 2 5 5 11 4 0.01 0.004 0.1 0.5 12 (2–338.5) 7.9 ⫾ 3.3 4 (1–45) 28.5 (14–87) 7.3 (1.8–1385) 9.8 ⫾ 4.4 4 (1–35) 29.5 (5–143) 0.73 0.029 0.85 0.79 ICU ⫽ intensive care unit. overall survival rates (%) in group A versus group B at 1, 5, and 10 years were 96.9 versus 96.9, 93.8 versus 96.9, and 93.8 versus 94.5, respectively (p ⫽ 0.41, log-rank test) (Fig 1). Surgical Treatment Twenty-seven (16.7%) of 162 patients underwent a modified Norwood procedure (group A versus group B: 33.8% [22/65] versus 5.2% [5/97]; p ⬍ 0.001) at the first palliative operation. Additionally, 102 of the 162 patients (63.0%) had a systemic-to-pulmonary shunt (Blalock-Taussig) [n ⫽ 68], a central shunt [n ⫽ 5], a ventricle-to– pulmonary artery conduit [n ⫽ 29]), 47 patients (29.0%) had a pulmonary artery band, 3 patients had atrioventricular valve repair, and 6 patients had repair of total anomalous pulmonary venous drainage at the initial palliative operation (Table 2). Operative factors, including concomitant procedures (n ⫽ 45 [group A, n ⫽ 12; group B, n ⫽ 33]) performed at the time of Fontan operation are summarized in Table 3. The intrapulmonary artery septation procedures, which consist of unilateral cavopulmonary anastomosis, aortopulmonary shunt, and septation between 2 blood sources [7], were applied in both patient groups (group A, n ⫽ 8; group B, n ⫽ 12) to promote growth in small pulmonary arteries before the Fontan operation. The diameter of the extracardiac graft ranged from 14 to 24 mm. Fenestration was reconstructed in 8 patients (group A, n ⫽ 3; group B, n ⫽ 5) at the time of TCPC (Table 3). The older Fontan patients had a longer bypass time. However the crossclamp time was similar in both groups (Table 3). Aortic cross-clamping was used in 88 patients, including 26 (40%) in group A and 62 (63.9%) in group B. There were a greater number of patients undergoing an intracardiac concomitant procedure and patients with heterotaxy syn- drome who had separated hepatic vein drainage in group B compared with group A. The duration of chest tube drainage was significantly longer for group B patients than for group A patients. However there were no differences in the duration of mechanical ventilation and the length of stay in the intensive care unit between the 2 groups (Table 3). Twenty-seven patients required reoperation after Fontan completion: repair of the atrioventricular valve in 5 patients, repair of pulmonary vein stenosis in 1 patient, creation of a fenestration in 1 patient, rerouting for pulmonary arteriovenous malformation in 1 patient, ligation of collateral arteries in 2 patients, repair of pulmonary arteries in 2 patients, ligation of aortopulmonary collateral arteries in 2 patients, permanent pacemaker– Table 4. Reoperation Variable Atrioventricular valve repair Valve replacement Pulmonary vein stenosis repair Pulmonary artery plasty Ligation of systemic-topulmonary collateral Pacemaker related Fenestration Rerouting Others Takedown Total Group A (⬍ 10 kg, n ⫽ 65) Group B (⬎ 10 kg, n ⫽ 97) 3 2 2 1 0 0 2 2 0 0 1 1 0 0 0 12 9 0 1 3 0 15 PEDIATRIC CARDIAC Table 3. Surgical Data at Fontan Procedure 1574 OTA ET AL FONTAN OPERATION IN INFANTS LESS THAN 10 KG Ann Thorac Surg 2012;94:1570 –7 Fig 2. Freedom from reoperation. PEDIATRIC CARDIAC related complications in 10 patients, and noncardiacrelated issues in 3 patients (Table 4). The rate of freedom from reoperation at 10 years after TCPC was 80.1% in group A and 83.5% in group B (p ⫽ 0.39) (Fig 2). Catheterization Data The mean pulmonary artery pressure, pulmonary artery resistance, and pulmonary artery index immediately before TCPC did not significantly differ between the 2 groups. The number of interventions for systemic-topulmonary collateral arteries was higher in group A (group A versus group B: 66.2% [43/65] versus 30.9% [30/97]; p ⬍ 0.001) (Table 5). The eDV (% of normal) was 232.7 ⫾ 91.4 versus 139.3 ⫾ 57.2 before TCPC versus 5 years after TCPC, respectively; p ⬍ 0.001) in group A and (209.6 ⫾ 77.7 versus 136.7 ⫾ 61.4, respectively; p ⬍ 0.001) in group B (Fig 3). Although ejection fraction and cardiac index (before TCPC versus 5 years after TCPC) did not differ significantly (p ⫽ 0.116 and p ⫽ 0.266, respectively) in group A, ejection fraction and cardiac index in group B at 5 years were significantly lower than before TCPC (p ⫽ 0.0057 and p ⫽ 0.0358, respectively) (Fig 4). The eDP (mm Hg) at 1 year (4.8 ⫾ 0.4 versus 6.8 ⫾ 0.4; p ⫽ 0.0037) and eDP at 5 years (6.5 ⫾ 0.4 versus 8.2 ⫾ 0.6; p ⫽ 0.047) were significantly lower in group A (Fig 1). The Fontan pressure (group A versus group B) at 1 year and 5 years after TCPC were (11.9 ⫾ 2.1 mm Hg versus 12.0 ⫾ 2.3 mm Hg, respectively; p ⫽ 0.8) and (11.6 ⫾ 2.1 mm Hg versus 11.1 ⫾ 2.7 mm Hg, respectively; p ⫽ 0.4). Comment Better understanding of the natural history and anatomic morphologic features of functional single ventricle, advances in surgical techniques and intraoperative myocardial protection, and comprehensive postoperative management have contributed to a remarkable improvement in early and late mortality before and after Fontan operations [4, 8 –10]. However elimination of cyanosis and ventricle unloading from the systemic and pulmonary circulation by Fontan operation at an earlier age remains a controversial topic. However volume unloading can be evident when there is collateral accessory flow between the systemic circulation and the pulmonary circulation [11]. More recent approaches to Fontan completion include volume-unloading operations at a younger age to reduce the adverse effects of prolonged ventricular volume overload on ventricular function. Furthermore, recent reports have demonstrated safe Fontan completion during the first year of life independent of anatomic diagnosis without additional morbidity or mortality Table 5. Catheter Study Before Fontan Operation Variable Pulmonary artery index Resistance of pulmonary artery PAP Atrial Pressure Transpulmonary pressure gradient Ejection fraction Cardiac index Intervention for collateral arteries PAP ⫽ pulmonary artery pressure. Group A (⬍ 10 kg, n ⫽ 65) Group B (⬎ 10 kg, n ⫽ 97) p Value 219.6 ⫾ 108.1 1.8 ⫾ 0.9 11.8 ⫾ 2.7 6.3 ⫾ 2.4 5.3 ⫾ 2.1 58.4 ⫾ 9.7 3.2 ⫾ 0.6 43 (66.2%) 245.8 ⫾ 115.8 1.8 ⫾ 0.9 11.9 ⫾ 3.2 6.8 ⫾ 2.2 5.2 ⫾ 2.8 58.8 ⫾ 9.4 3.4 ⫾ 0.9 30 (30.9%) 0.1 0.8 0.8 0.2 0.8 0.8 0.1 ⬍ 0.001 OTA ET AL FONTAN OPERATION IN INFANTS LESS THAN 10 KG 1575 Fig 3. Changes in ventricular end-diastolic volume (eDP) (% of normal volume) and ventricular end-diastolic pressure (eDP) (mm Hg) before and at 1, 5, and 10 years after Fontan operation in group A (solid circle) and group B (solid triangle). The eDP was significantly lower in group A than in group B at 5 and 10 years after total cavopulmonary connection. (A) eDV. (B) eDP. [4, 12]. We found no significant difference in long-term mortality and morbidity rates between the 2 groups, indicating that the Fontan procedure can be safely completed without increasing these rates over the long term. Initially, we used a 16-mm graft in 52 patients and a 14-mm graft in 2 patients during the extracardiac Fontan procedure. After gaining experience, we recently increased the use of 18-mm conduits. None of these grafts has required revision. Therefore we do not consider that the Fontan operation in younger patients is disadvantageous from this perspective. To eliminate cyanosis earlier in life in our patients, in 1997 we started to perform Fontan operations when patients weighed about 10 kg. Similar to other series [4, 8, 9, 13–16], there was extremely low late mortality. In our series, age at TCPC had no effect on mortality. Earlier Fontan completion has several theoretical advantages in terms of minimizing the effect of persistent cyanosis, the potential for paradoxical embolization, and the duration of chronic volume overload of the single ventricle [17]. Long-standing cyanosis and volume overload to the ventricle are believed to cause progressive ventricular fibrosis by inducing histopathologic changes, which must result in both systolic and diastolic dysfunction. Therefore earlier elimination of cyanosis and volume overload can enhance cardiac performance in patients undergoing Fontan operations. We found that the ventricular enddiastolic pressure was significantly lower 5 years after the Fontan procedure had been completed in the younger patient group. Although the cardiac index and ventricular ejection fraction were significantly lower at 5 years after the Fontan procedure compared with those measurements before the Fontan operation in the older patient group, in the younger group we found there were no significant differences between cardiac index and ejection fraction before the Fontan procedure and at 5 years after the Fontan procedure. These results suggest that earlier Fontan completion helps to maintain ventricular contractility, prevent the progression of ventricular dysfunction, and maintain a good ejection fraction and cardiac index. Earlier unloading of a univentricular heart by means of Fontan completion in patients with collateral accessory flow between the systemic circulation and the pulmonary circulation might be advantageous for future ventricular function preservation [18, 19]. Other authors have recognized that patients with high systemic-to-pulmonary collateral flow were more likely to be older at the time of the Fontan operation [19]. It is possible that an increasing systemic oxygen saturation after the Fontan operation removes the stimulus for further growth of systemic-topulmonary collateral flow. In particular, it might be postulated that the systolic and diastolic function of the Fig 4. (A) Changes in ventricular ejection fraction (%) and (B) changes in cardiac index (L/min/m2) before total cavopulmonary connection (TCPC), at 1, 5, and 10 years after TCPC. There are no significant differences in group A; however, cardiac index and ejection fraction at 5 and 10 years after TCPC were lower than before TCPC in group B. PEDIATRIC CARDIAC Ann Thorac Surg 2012;94:1570 –7 1576 OTA ET AL FONTAN OPERATION IN INFANTS LESS THAN 10 KG PEDIATRIC CARDIAC single ventricle would be preserved by early reduction of volume loading. In addition, relief from early cyanosis might alleviate deleterious effects from a prolonged cyanotic state. Furthermore, the hemodynamics in Fontan circulation depend not only on cardiac contractility but also on pulmonary circulation and function, and the growth of respiratory and skeletal muscles is considered to be an important factor affecting Fontan circulation. For these reasons, we postulate that earlier elimination of cyanosis allows better growth of respiratory and skeletal muscles, which would subsequently preserve the cardiac index, especially at 5 and 10 years after the Fontan procedure. Our experience shows that in our current series, the TCPC achieves excellent survival. In our series of 162 patients, the hospital survival was 98.1% (159/162). There was little mortality during the follow-up period, with a 10-year actual survival of 93.5%. These outcomes were achieved in a broad spectrum of single-ventricle anatomies, including HLHS and heterotaxy syndrome, in a total of 43% of the patients. Study Limitations The study described here covered a long period, and its retrospective nature and inadequate number of patients for meaningful subgroup analysis should be recognized as limitations of the study, although the data in our database are collected prospectively for all operations. The effect of general improvement in surgical technique and perioperative care with increasing experience could not be analyzed in this study. In conclusion, earlier TCPC can be performed with good intermediate results. Earlier unloading of a univentricular heart by means of TCPC might be advantageous for future preservation of ventricular function. References 1. Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax 1971;26:240 – 8. 2. Kreutzer G, Galindez E, Bono H, De Palma C, Laura JP. An operation for the correction of tricuspid atresia. J Thorac Cardiovasc Surg 1973;66:613–21. 3. de Leval MR, Kilner P, Gewillig M, Bull C. Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. Experimental studies and early clinical experience. J Thorac Cardiovasc Surg 1988; 96:682–95. Ann Thorac Surg 2012;94:1570 –7 4. Pizarro C, Mroczek T, Gidding SS, Murphy JD, Norwood WI. Fontan completion in infants. Ann Thorac Surg 2006;81: 2243– 8; discussion 2248 –9. 5. Ikai A, Fujimoto Y, Hirose K, et al. Feasibility of the extracardiac conduit Fontan procedure in patients weighing less than 10 kilograms. J Thorac Cardiovasc Surg 2008;135:1145–52. 6. Nakata, T, Fujimoto Y, Hirose L, et al. Fontan completion in patients with atrial isomerism and separate hepatic venous drainage. Eur J Cardiothorac Surg 2010;37:1264 –70. 7. Sakamoto K, Ikai A, Fujimoto Y, Ota N. Novel surgical approach ’intrapulmonary-artery septation’ for Fontan candidates with unilateral pulmonary arterial hypoplasia or pulmonary venous obstruction. Interact Cardiovasc Thorac Surg 2007;6:150 – 4. 8. Tweddell JS, Nersesian M, Mussatto KA, et al. Fontan palliation in the modern era: factors impacting mortality and morbidity. Ann Thorac Surg 2009;88:1291–9. 9. Jaquiss RD, Siehr SL, Ghanayem NS, et al. Early cavopulmonary anastomosis after Norwood procedure results in excellent Fontan outcome. Ann Thorac Surg 2006;82:1260 –5; discussion 1265– 6. 10. Hosein RB, Clarke AJ, McGuirk SP, et al. Factors influencing early and late outcome following the Fontan procedure in the current era. The ’Two Commandments’? Eur J Cardiothorac Surg 2007;31:344 –52; discussion 353. 11. Sathanandam S, Polimenakos AC, Blair C, El Zein C, Ilbawi MN. Hypoplastic left heart syndrome: feasibility study for patients undergoing completion fontan at or prior to two years of age. Ann Thorac Surg 2010;90:821– 8; discussion 828 –9. 12. Weber HS, Gleason MN, Myers JL, et al. The Fontan operation in infants less than 2 years of age. J Am Coll Cardiol 1992;19:828 –33. 13. Petrossian E, Reddy VM, Collins KK, et al. The extracardiac conduit Fontan operation using minimal approach extracorporeal circulation: early and midterm outcomes. J Thorac Cardiovasc Surg 2006;132:1054 – 63. 14. Fiore AC, Turrentine M, Rodefeld M, et al. Fontan operation: a comparison of lateral tunnel with extracardiac conduit. Ann Thorac Surg 2007;83:622–9; discussion 629 –30. 15. Anderson PA, Sleeper LA, Mahony L, et al. Contemporary outcomes after the Fontan procedure: a Pediatric Heart Network multicenter study. J Am Coll Cardiol, 2008;52: 85–98. 16. Gaynor JW, Bridges ND, Cohen MI, et al. Predictors of outcome after the Fontan operation: is hypoplastic left heart syndrome still a risk factor? J Thorac Cardiovasc Surg 2002;123:237– 45. 17. Camposilvan S, Milanesi O, Stellin G, et al. Liver and cardiac function in the long term after Fontan operation. Ann Thorac Surg 2008;86:177– 82. 18. Banka P, Sleeper LA, Atz AM, et al. Practice variability and outcomes of coil embolization of aortopulmonary collaterals before Fontan completion: a report from the Pediatric Heart Network Fontan Cross-Sectional Study. Am Heart J 2011;162: 125–30. 19. Prakash A, Rathod RH, Powell AJ, et al. Relation of systemicto-pulmonary artery collateral flow in single ventricle physiology to palliative stage and clinical status. Am J Cardiol 2012;109:1038 – 45. Epub 2012 Jan 3. DISCUSSION DR JAMES JAGGERS (Aurora, CO): Let me ask you just 1 quick thing about your conduit size, that 16 or 17 mm I believe was your standard conduit size in either group. When you did your postoperative catheterizations at 5 years, was there any evidence that that was an inadequate size? Was there any level of obstruction in the smaller conduit? It is my personal bias that that is actually a very reasonable size, but that is contrary to others’ opinion. DR OTA: Actually, we did not find conduit obstruction from the catheter study which was done at 5 years after the Fontan operation, and some paper said that mean diameters of the IVC were changed 17 mm in the left lateral position to 23 mm in the right lateral position in patients with right atrial pressures greater than 8 mm Hg. Those findings might suggest that a conduit of 18 or 20 mm diameter might be acceptable I think. DR CARL LEWIS BACKER (Chicago, IL): This was a really good analysis with a considerable amount of data on a substantial number of patients. My question relates to the finding of the decreased ejection fraction and increased end-diastolic pressure at that final measured point 5 years after the Fontan operation. The group that had the “early” Fontan was about 1.5 years of age at the time of their Fontan procedure. The group that had the “delayed” Fontan was about 5 years of age at the time of their Fontan procedure. The question I have is whether the change in ejection fraction and end-diastolic pressure could be related more to the age of the patient rather than to the time of follow-up since the Fontan because the “delayed Fontan” group was almost 4 years older at the time of the Fontan operation. Did your analysis take the age of the patient into account, not just the time period since the Fontan operation? DR OTA: Thank you for your good question. Regarding of ejection fraction, we can compare the number of group A at the 5 years after the Fontan with the number of group B at 1 year after the Fontan. It is likely to be age-adjusted comparison, and those age-adjusted comparison shows still the same trend as I showed in the previous slide. OTA ET AL FONTAN OPERATION IN INFANTS LESS THAN 10 KG 1577 Regarding of ventricular end-diastolic pressure, the timing at the Fontan operation is an important factor I think. There are slight differences between 2 groups before Fontan operation as I showed the previous slide. Some other paper said that Fontan patients in the highest amount of systemic to pulmonary collateral flow underwent the Fontan operation at an older age. It is possible that an increase in systemic oxygen saturation after the Fontan operation removes the stimulus for further growth of systemic-to-pulmonary collaterals. These findings support this hypothesis. Longstanding cyanosis and volume overload to the ventricle are believed to cause progressive ventricular fibrosis by inducing histopathologic changes, which must result in both systolic and diastolic dysfunction. In theory, systemic-to-pulmonary collateral flow causes volume overload of the systemic ventricle, which could contribute to adverse outcomes, and again I think the timing of Fontan operation is important factor for end-diastolic pressure. Thank you again for your great comments and questions. DR BACKER: I will ask you more about this after the session. Thank you again for your excellent paper. The Society of Thoracic Surgeons: Forty-Ninth Annual Meeting Mark your calendars for the Forty-Ninth Annual Meeting of The Society of Thoracic Surgeons (STS) to be held at the Los Angeles Convention Center, Los Angeles, California, from January 26–30, 2013. Visit Los Angeles to learn from the experts, network with colleagues from around the world, and prepare for whatever your future may hold. This preeminent educational event in cardiothoracic surgery is open to all physicians, residents, fellows, engineers, perfusionists, physician assistants, nurses, or other interested individuals who work with cardiothoracic surgeons. Meeting attendees will be provided with the latest scientific information for practicing cardiothoracic surgeons. Attendees will benefit from traditional Abstract Presentations and Invited Lectures, as well as Surgical Forums, Early Morning Sessions, Surgical Motion Pictures, and Procedural Hands-On Courses. Parallel sessions on Monday and Tuesday will focus on specific subspecialty interests. © 2012 by The Society of Thoracic Surgeons Published by Elsevier Inc An advance program with a registration form, hotel reservation information, and details regarding spouse/ guest activities will be mailed to STS members this Fall. Nonmembers may contact the Society’s Secretary, David A. Fullerton, MD, to receive a copy of the advanced program; however, detailed meeting information will be available on the STS website at www.sts.org. David A. Fullerton, MD Secretary The Society of Thoracic Surgeons 633 N Saint Clair St, Ste 2320 Chicago, IL 60611-3658 Telephone: (312) 202-5800 Fax: (312) 202-5801 email: [email protected] website: www.sts.org Ann Thorac Surg 2012;94:1577 • 0003-4975/$36.00 PEDIATRIC CARDIAC Ann Thorac Surg 2012;94:1570 –7