Early and mid-term results of Fontan operation versus anatomic correction for congenitally corrected transposition of the great arteries: A retrospective cohort study in a single center / 中国胸心血管外科临床杂志

@#Objective     To compare the early and mid-term results between Fontan operation and anatomic correction for congenitally corrected transposition of the great arteries (ccTGA). Methods     The clinical data of 53 patients with ccTGA who underwent anatomic correction and Fontan operation from January 2009 to September 2021 in our hospital were reviewed, including 41 males and 12 females with a mean age of 55.02 (3-168) months. They were divided into an anatomic correction group (16 patients) and a Fontan operation group (37 patients) according to the operation. The hospitalization mortality, survival rate, postoperative complications, and free rate from re-intervention between the two groups were compared. Another 180 healthy children were recruited as a control group, and 14 children were matched with the propensity score matching method as a Fontan control group. The results of cardiopulmonary exercise testing (CPET) between the Fontan operation group and the Fontan control group were compared. Results     There were 2 (12.5%) early deaths and 3 (18.8%) early re-intervention in the anatomic correction group, while 1 death and 2 re-intervention in the Fontan operation group. In addition, there were 9 patients (56.3%) in the anatomic correction group and 6 (16.2%) patients in the Fontan operation group suffering from arrhythmia after operation, respectively. Compared with the anatomic correction group, cardiopulmonary bypass time, aortic cross-clamping time, intubation time and ICU stay were significantly shortened in the Fontan operation group (P<0.05). CPET results showed that, percent predicted max VO2 in the Fontan operation group was lower than that in the Fontan control group (0.84±0.11 vs. 0.99±0.12, P<0.05). The patients were followed up for 0.5-126.0 months. Two patients were lost in the Fontan operation group. There was no death and 1 re-intervention in the anatomic correction group, while no death or re-intervention in the Fontan operation group. The 1-year, 5-year and 10-year transplant-free survival rate of the anatomic correction group and the Fontan operation group was 87.5%, 87.5%, 87.5% and 97.3%, 97.3%, 97.3%, respectively (P>0.05). The 48 patients were classified as grade Ⅰ-Ⅱ in cardiac function in the last follow-up. Conclusion     There is no statistical difference in the transplant-free survival rate between the anatomic correction and the Fontan operation group. The postoperative complications in the Fontan operation group are decreased than those in the anatomic correction group. The Fontan operation is also a good choice, even though the patients with ccTGA meet the condition of the procedure of anatomic correction.

Prognosis and risk factors for mild to moderate or moderate atrioventricular valve regurgitation after Fontan operation / 中国胸心血管外科临床杂志

@#Objective    To investigate the prognosis and risk factors of mild to moderate or moderate atrioventricular valve regurgitation (AVVR) after Fontan operation. Methods    A total of 34 patients with mild to moderate or moderate AVVR who accepted Fontan operation and atrioventricular valve (AVV) repair between 2004 and 2018 in our center were selected as an AVV repair group. The patients in the same period were matched as a control group by the ratio of 1 : 1-2. Finally 99 patients were included into this study, including 64 males and 35 females, with an average age of 63.4±36.3 months and weight of 17.3±6.7 kg. Grades of AVVR decreased more than 1 was defined as significant improvement. Endpoints of the study were death, Fontan takedown, AVV replacement. Risk factors including Fontan procedures, AVV repair procedures, cardiac anatomy were analyzed. Results    Patients were followed up for 1.5 (0.3-4.0) years. Overall mortality was 15.2%. Most (82.4%) of AVV repair group accepted single AVV repair procedure while partial annuloplasty was the most common (52.9%). With the extension of follow-up, the degree of AVVR in the whole group showed a gradually increasing trend (r=0.352, P=0.000). Mild to moderate AVVR improved spontaneously after Fontan operation, while moderate AVVR did not. AVV repair could improve the degree of AVVR after moderate regurgitation, without increasing the surgical mortality, and regurgitation significantly decreased in 8.8% patients. AVV repair was not effective for mild to moderate AVVR and would increase surgical mortality. Conclusion    AVV function shows a gradual downward trend after Fontan operation. AVV repair is effective for moderate AVVR, does not increase mortality, but the degree of improvement is limited. AVV repair is not effective for mild to moderate AVVR and increases surgical mortality.

Magnetic Resonance Imaging Assessment of Blood Flow Distribution in Fenestrated and Completed Fontan Circulation with Special Emphasis on Abdominal Blood Flow

OBJECTIVE: To investigate the regional flow distribution in patients with Fontan circulation by using magnetic resonance imaging (MRI). MATERIALS AND METHODS: We identified 39 children (18 females and 21 males; mean age, 9.3 years; age range, 3.3–17.0 years) with Fontan circulation in whom flow volumes across the thoracic and abdominal arteries and veins were measured by using MRI. The patients were divided into three groups: fenestrated Fontan circulation group with MRI performed under general anesthesia (GA) (Group 1, 15 patients; average age, 5.9 years), completed Fontan circulation group with MRI performed under GA (Group 2, 6 patients; average age, 8.7 years), and completed Fontan circulation group with MRI performed without GA (Group 3, 18 patients; average age, 12.5 years). The patient data were compared with the reference ranges in healthy controls. RESULTS: In comparison with the controls, Group 1 showed normal cardiac output (3.92 ± 0.40 vs. 3.72 ± 0.69 L/min/m2, p = 0.30), while Group 3 showed decreased cardiac output (3.24 ± 0.71 vs. 3.96 ± 0.64 L/min/m2, p = 0.003). Groups 1 and 3 showed reduced abdominal flow (1.21 ± 0.28 vs. 2.37 ± 0.45 L/min/m2, p < 0.001 and 1.89 ± 0.39 vs. 2.64 ± 0.38 L/min/m2, p < 0.001, respectively), which was mainly due to the diversion of the cardiac output to the aortopulmonary collaterals in Group 1 and the reduced cardiac output in Group 3. Superior mesenteric and portal venous flows were more severely reduced in Group 3 than in Group 1 (ratios between the flow volumes of the patients and healthy controls was 0.26 and 0.37 in Group 3 and 0.63 and 0.53 in Group 1, respectively). Hepatic arterial flow was decreased in Group 1 (0.11 ± 0.22 vs. 0.34 ± 0.38 L/min/m2, p = 0.04) and markedly increased in Group 3 (0.38 ± 0.22 vs. −0.08 ± 0.29 L/min/m2, p < 0.0001). Group 2 showed a mixture of the patterns seen in Groups 1 and 3. CONCLUSION: Fontan circulation is associated with reduced abdominal flow, which can be attributed to reduced cardiac output and portal venous return in completed Fontan circulation, and diversion of the cardiac output to the aortopulmonary collaterals in fenestrated Fontan circulation.

An investigation on the transcatheter closure of antegrade pulmonary blood flow with high-risk Fontan operations / 中华胸心血管外科杂志

Objective To investigate the therapeutic effect of the transcatheter closure of antegrade pulmonary blood flow with high-risk Fontan operations.Methods Retrospective analysis of 6 Fontan surgical patients, 4 males and 2 females, who received surgical treatment in the cardiothoracic surgery of Shanghai Children's Medical Center from May 2016 to August 2018;Age(5.7 ±2.9) years;(19.8 ±5.5) kg weight.All 6 patients were treated with primary pulmonary artery banding operation and secondary bilateral Glenn operation(BDG) and re-PAB.Before Fontan surgery, cardiac catheterization was performed in the department of cardiology to close the antegrade pulmonary blood flow .Results These six patients received interventional therapy in the department of cardiology.The mean pulmonary artery pressure(Pp) before occlusion was(17.0 ±0.8) mmHg (1 mmHg=0.133 kPa), the ventricular diastolic pressure(VEDP) was (11.2 ±0.9) mmHg, and the mean pulmonary artery pressure after occlusion was effectively decreased to(14.2 ±0.7) mmHg and VEDP to(9.7 ±0.7) mmHg.Fontan surgery was performed 6 to 12 months after the occlusion.No death was found after the operation, and all the clinical indicators were normal after the operation.Follow-up was conducted for 2-24 months, and the cardiac function and the function of each organ recov-ered well.Conclusion For some patients with high risk Fontan before operation , this method can safely and effectively reduce the average pulmonary artery pressure and reduce the ventricular and pulmonary vascular load , which is of positive help for the successful Fontan operation.

The clinic report of 566 consecutive cases of Fontan operation and analysis early risk factors / 中华胸心血管外科杂志

Objective To summarize 566 consecutive cases of Fontan operation in the treatments of complex congenital heart diseases and to analyze the risk factors of early surgical outcome.Methods We reviewed all 566 consecutive patients who underwent a Fontan procedure in Shanghai Children's Medical Center from January 2006 to May 2014.Preoperative records of staged surgeries,perioperative data and imaging results were obtained from medical records.Results A total of 566 patients(M/F =345/221) underwent a Fontan operation,with 166 cases of single stage Fontan operation and 400 cases of multi-staged operation.The median age at the time of the Fontan procedure was 5 years,and the median weight was 15.4 kg.In our series,there were 278 extracardiac conduits,110 intracardiac conduits,114 lateral tunnels,36 intracardiac/extracardiac conduits,and 27 direct cavopulmonary conduits.There were 36 cases of early Fontan failure,with overall early postoperative survival rate being 93.6％.The median ventilation duration was 8 h,with the median postoperative intensive care unit stay being four days,the average oxygen saturation being 0.90 at the end of ICU stay,the median chest drainage time being 10 days,and the median postoperative hospital stay being 19 days.Besides the CICU oxygen saturation,the rest postoperative results had no obvious differences in five surgical groups.Heterotaxy syndrome,earlier age at the time of the Fontan procedure,bilateral Glenn shunt,moderate AV valve regurgitation and severe and right ventricular-type morphology were risk factors of early Fontan failure,P ＜ 0.05.Conclusion In order to improve the early outcome of Fontan operation,closely follow-up should be taken directly after Glenn operation in high-risk patients.Doctors should also have an in-depth understanding of the of postFontan physiological and pathological features,and select the operation time and Fontan type most suited to the of the individualized characteristics.

Effect of pulmonary arterial hypertension on early outcomes of children with functional single ventricle after Fontan operation: A case control study / 中国胸心血管外科临床杂志

@#Objective    To explore the effect of pulmonary arterial hypertension on the children with functional single ventricle in the early period after Fontan operation. Methods    Forty-three children with pulmonary arterial hypertension after Fontan operation were enrolled in our department between January 2015 and December 2016. There were 24 males and 19 females at a median age of 4.3 years ranging from 2.5 to 4.8 years. The pulmonary arterial pressure was evaluated by cardiac catheterization. There were 23 children diagnosed without pulmonary hypertension (a non-PAH group) including 16 males and 7 females, while 20 patients were diagnosed with pulmonary hypertension (a PAH group) including 8 males and 12 females. Postoperative parameters related to outcomes were compared between the two groups. Results    There was no death in the non-PAH group, but the mortality of children in the PAH group was 20.0% (4/20， χ2=5.34, P=0.02). The central venous pressure (t=–2.50, P=0.02), N-terminal prohormone of brain natriuretic peptide (NT-proBNP, Z=–3.50, P<0.01), peritoneal dialysis rate (χ2=5.40, P=0.02), incidence of arrhythmia (χ2=4.40, P=0.03) in the PAH group were significantly higher than those of the non-PAH group. The early postoperative utilization rate of pulmonary vascular targeting agents in the PAH group was significantly higher than that in the non-PAH group (χ2=6.30, P=0.04). Conclusion    Pulmonary arterial hypertension is one of the most important factors which influence the early postoperative prognosis of children with functional single ventricle after Fontan operation.

The Early- and Med-term Outcomes of Modified Fontan Operation in Patients With Functional Single Ventricle and Separate Hepatic Venous Drainage / 中国循环杂志

Objective: To summarize the early- and med-term outcomes of modified Fontan operation in patients with functional single ventricle and separate hepatic venous (SHV) drainage. Methods: A total of 39 patients with functional single ventricle and SHV drainage received modified Fontan operation in our hospital from 2009-01 to 2015-12 were retrospectively analyzed. There were 26 (66.7%) male, the average age was (6.98±3.62) years, body weight was (20.79±9.66) kg and pre-operative mean pulmonary artery pressure was (9.77±2.51) mmHg. Based on if SHV combining Apicocaval Juxtaposition (ACJ), the patients were divided into 2 groups: SHV group,n=29 and SHV+CAJ group,n=10. The early (3 months) - and med (5 years)-term post-operative outcomes were analyzed. Results: General clinical data was similar between 2 groups,P>0.05. There were 20/39 patients with one-stage operation, 19 with two-stage operation (18 after Glenn operation and 1 after B-T operation). Modified Fontan operation method was mainly used extracardiac conduit-common open technique (30 patients, 76.9%). 19 patients had CPB with aorta clamping, CPB time was 72-446 (189.74±84.96) min and aorta clamping time was 26-171 (89.05±43.96) min; the other 19 patients had CPB without aorta clamping, CPB time was 60-209 (109.99±98.86) min. 10 patients received re-surgery due to early post-operative complications. 3 patients died at early post-operation with the mortality at 7.7%. The average follow-up time was 3.9 (0.83-7.17) years, 1 patient had cardiac function at NYHA II and the others at NYHA I; the 5-year survival rate was 100%. Conclusion: The early- and med-term outcomes of modified Fontan operation were good in patients with functional single ventricle and SHV drainage. Selection of modified Fontan operation method should be based on the distance form inferior vena cava to SHV and presence of ACJ.

"Air embolism during fontan operation".

In patients with a right to left intracardiac shunt, air embolism results in an obligatory systemic embolization. Nonembolization of entrained air is described in a child with a single ventricle physiology who had earlier undergone bidirectional Glenn shunt construction and Damus–Kaye–Stansel anastomosis. The air entrainment was detected by intra-operative transesophageal echocardiography. The combined effect of a “diving bell” phenomenon and mild aortic valve regurgitation are suggested as the reasons for the confi nement of air into the ventricle preventing catastrophic systemic embolization.

Inhaled iloprost for the treatment of patient with Fontan circulation / 소아과

Decreased exercise capacity after Fontan surgery is relatively common and the failure of the Fontan state gradually increases with age. However, there is no further treatment for patients with Fontan circulation. Pulmonary vasodilation therapy is an effective method to solve this problem because pulmonary vascular resistance is a major factor of the Fontan problem. Inhaled iloprost is a chemically stable prostacyclin analogue and a potent pulmonary vasodilator. We experienced two cases of Fontan patients treated with inhaled iloprost for 12 weeks. The first patient was an 18-year-old female with pulmonary atresia with an intact ventricular septum, and the second patient was a 22-year-old male with a double outlet right ventricle. Fifteen years have passed since both patients received Fontan surgery. While the pulmonary pressure was not decreased significantly, improved exercise capacity and cardiac output were observed without any major side effects in both patients. The iloprost inhalation therapy was well tolerated and effective for the symptomatic treatment of Fontan patients.

The Clinical Outcomes of Damus-Kaye-Stansel Procedure According to Surgical Technique

BACKGROUND: The Damus-Kaye-Stansel (DKS) procedure is a method for mitigating the risk of systemic ventricular outflow tract obstruction (SVOTO). However, there have been few reports on which surgical technique shows a better outcome. The objective of this study was to compare the outcome of the DKS procedure according to the surgical technique used. METHODS: We retrospectively reviewed 12 consecutive patients who underwent the DKS procedure from March 2004 to April 2013. When the relationship of the great arteries was anterior-posterior, the double-barrel technique (group A) was performed. If the relationship was side-by-side, the ascending aortic flap technique (group B) was performed. RESULTS: There was no early mortality and 1 late mortality in group B. There was no statistically significant difference in the median peak pressure gradient of preoperative subaortic stenosis in both groups: 14 mmHg (range, 4 to 53 mmHg) in group A and 15 mmHg (range, 0 to 30 mmHg) in group B (p=0.526). Further, a significant postoperative pressure gradient was not observed in either group A or group B. More than moderate postoperative neoaortic regurgitation was observed in 1 patient of group B; this patient underwent neoaortic valve replacement 66 months after the DKS procedure. No one had a recurrent SVOTO during follow-up. CONCLUSION: The DKS procedure is an effective way to minimize the risk of SVOTO, and there is little difference in the outcomes of the DKS procedure according to the surgical technique used.

Acute ischemic stroke in a child with cyanotic congenital heart disease due to non-compliance of anticoagulation / 世界急诊医学杂志（英文）

BACKGROUND: Stroke is a common presentation in geriatric patients in emergency department but rarely seen in pediatric patients. In case of acute ischemic stroke in pediatric age group, management is different from that of adult ischemic stroke where thrombolysis is a good option.METHODS: We report a case of a 17-year-old male child presenting in emergency with an episode of acute ischemic stroke causing left hemiparesis with left facial weakness and asymmetry. The patient suffered from cyanotic congenital heart disease for which he had undergone Fontan operation previously. He had a history of missing his daily dose of warfarin for last 3 days prior to the stroke.RESULTS: The patient recovered from acute ischemic stroke without being thrombolyzed.CONCLUSION: In pediatric patients, acute ischemic stroke usually is evolving and may not require thrombolysis.

Application of inhaled nitric oxide in the early period after Fontan operation / 中国小儿急救医学

Objective To evaluate the effects of inhaled nitric oxide(INO) in the early period after Fontan operation. Methods Twenty children receiving INO [(10 ～ 25) × 10 -6] after fight heart bypass for congenital heart disease were evaluated. The changes of hemodynamic and respiratory parameters were observed at two different intervals. The levels of methemoglobin (MetHb)and nitrogen dioxide(NO2)were monitored. Results In INO therapy group,CVP decreased from (20.8±4.8) mm Hg to (14.3±1.8) mm Hg,TPG decreased from (16.3 ±3.0) mm Hg to (8.8±2. 1) mm Hg,PaO2/FiO2 increased from (70.3±16.9) mm Hg to (120.5 ±14.0) mm Hg,and arterial oxygen saturation increased from 0. 78 ±0. 14 to 0. 91 ±0. 09 ,blood lactic acid level decreased from (3.98 ± 0. 86) mmol/L to (1.29 ± 1.60) mmol/L. No toxic side effect was observed. Conclusion INO has no significant long-term effect on patients after Fontan operation, but it can improve respiratory function, and reduce the incidence of low cardiac output syndrome and high cava pressure caused by reactive elevation of pulmonary vascular resistance in the early postoperative period after Fontan operation.

Outcome after Fontan Operation in Korea

PURPOSE: Despite extracardiac conduit Fontan had many advantages, long-term results related to longevity of conduit, and anticoagulation were not proven. This study was to evaluate the long-term outcome of hospital survivors with extracardiac Fontan circulation. METHODS: Between 1996 and 2006, 200 patients underwent extracardiac conduit Fontan operation. Median age at the Fontan operation was 3.4 years (range:16 months-35.7 years). All patients (89.5 %) except 21 patients of one stage Fontan operation underwent bi-directional cavopulmonary shunt. Fenestration was required in 85 patients (42.5%). RESULTS: There has been 6 hospital mortalities (3.0%), and 7 late mortalities (3.6%) at a mean follow-up of 52.4+/-32.2 months (range; 18days-120 months). Overall 10-years survival was 92.4 2.1%. Multivariate analysis identified severe infection at early postoperative periods (hazard ratio =12.439, P=0.001), and high pulmonary arterial pressure at preoperative period (hazard ratio=3.445, P=0.038) as risk factors for mortality. Reoperation was performed in 24 patients (12.0%), and freedom from reoperation was 82.4+/-4.1% at 10 years. Arrhythmia occurred in 32 patients (16.0%) after Fontan operation, and freedom from arrhythmia was 85.14.4% at 10 years. Risk factors for arrhythmia were heterotaxy syndrome (P=0.001), Follow up duration (P=0.027) and the age at Fontan operation (P=0.001). Freedom from thromboembolism was 92.91.9% at 10 years. The conduit cross- sectional area decreased by 14%, and the extent of decrease of the conduit cross-sectional remained stable irrespective of the follow-up duration. 95.2% of patients had New York Heart Association class I. CONCLUSIONS: After 10 years of follow-up, the overall survival, and the functional status of survivors of the extracardiac Fontan procedure are satisfactory. Fenestration has a beneficial effect on the results of high-risk Fontan patients. The incidence of late deaths, reoperations, obstructions of the cavopulmonary pathway, arrhythmias and thromboembolisms is low.

20 Years Surgical Experiences for Ebstein's Anomaly / 대한흉부외과학회지

BACKGROUND: We retrospectively evaluated the clinical results of surgically managing patients with Ebstein's anomaly. MATERIAL AND METHOD: Between Feb. 1984 and June 2006, 50 patients who underwent surgical treatment for Ebstein's anomaly at Yonsei Cardiovascular Center were retrospectively reviewed. The mean age of the patients was 26.9 years and 19 patients were male. Associated anomalies included atrial septal defect (33), patent ductus arteriosus (2), ventricular septal defect (1), and pulmonary stenosis (4), and 90% (45/50) of the patients had more than a moderate degree of tricuspid regurgitation. Carpentier type A was present in 6 patients, type B in 26, type C in 14 and type D in 4. Ten patients were associated with WPW syndrome. Conservative surgery was possible in 31 patients (tricuspid annuloplasty, plication of the atrialized RV), Fontan's operation was performed in 4 patients, tricuspid valve replacement was done in 12 and palliative surgery was done in 2 patients. Thirteen patients were associated with bi-directional cavopulmonary shunt (BCPS: one and a half ventricular repairs); 10 patients with WPW syndrome and 4 patients with atrial fibrillation underwent concomitant ablation. RESULT: The postoperative median NYHA functional class (3-->1) and the mean cardio-thoracic ratio (0.65-->0.59) were decreased significantly (p94.1%), and median TR grade (4-->1) were also significantly improved (p=0.004, p<0.001). For comparison of BCPS and conservative surgery, the preoperative right ventricular pressure (33.0 vs. 41.3 mmHg), the ICU stay (2.86 vs. 1.89 days), the hospital say (10.6 vs. 16.8 days), and the left ventricular ejection fraction (64.3 vs. 72.8%) were statistically different. Postoperative mortality occurred in 3 patients (6%) due to biventricular failure in 2 patients and sepsis in the other patient. The mean follow up duration was 101.5 months, and one patient died of Fontan failure and 6 patients required reoperation (bioprosthetic degenerative change (2) and Fontan conversion (4)). The overall survival rate at 10 years was 90.2%; the freedom from reoperation rate and rate of cardiac related events were 78.9% and 49.2%, respectively. CONCLUSION: Surgical management of Ebstein's anomaly can be performed safely, and the associated BCPS may be helpful for high-risk patients. Adequate application of surgical management may increase the long-term survival with a reduced rate of reoperation.

Results of Extracardiac Pericardial-flap Lateral Tunnel Fontan Operation / 대한흉부외과학회지

BACKGROUND: Extracardiac pericardial-flap lateral tunnel Fontan operation has theoretical advantage of growth potentiality of the extracardiac tunnels. The mid-term results of this technique and morphologic change of the lateral tunnel were studied. MATERIAL AND METHOD: Clinical data was reviewed in 42 patients who underwent extracardiac pericardial-flap lateral tunnel Fontan operation between November 1993 and December 2004. The age was 2.8+/-1.5 years and the body weight was 12.3+/-3.2 kg. Extracardiac tunnel was constructed using the pedicled pericardium with the base undetached. By reviewing the follow-up cardiac angiograms, the diameter and the cross-sectional area of the lateral tunnel was compared to those of inferior vena cava. RESULT: There were four operative mortality cases (9.8%) and the causes of death were low cardiac output for all four cases. Postoperatively, five patients had prolonged pleural effusion longer than two weeks and one patient required a permanent pacemaker due to complete heart block. Follow-up was possible in 37 patients and the follow up duration was 3.8+/-2.2 years. During that period, one patient died, of upper gastrointestional bleeding combined with heart failure and one patient died a sudden death of unknown cause. Two patients required reoperation due to subaortic stenosis and anastomosis site stenosis between inferior vena cava and lateral tunnel. In one patient, bradyarrhythmia was anew but there was no thromboembolic complication. The lateral tunnel showed growth in proportion to the size of the inferior vena cava. CONCLUSION: Extracardiac pericardial-flap lateral tunnel Fontan operation is relatively simple and safe. The mid-term result was favorable and the extracardiac tunnel showed potential for growth.

N-terminal Pro-B-type Natriuretic Peptide as a Predictive Risk Factor in Fontan Operation / 소아과

PURPOSE: This study aimed to investigate the correlation between the plasma level of N-terminal pro-B-type natriuretic peptide (pro-BNP) and several known risk factors influencing outcomes after Fontan operations, and to assess whether pro-BNP levels can be used as predictive risk factors in Fontan operations. METHODS: Plasma pro-BNP concentrations were measured in 35 patients with complex cardiac anomalies before catheterization. Cardiac catheterization was performed in all subjects. Mean right atrium pressure, mean pulmonary artery pressure (PAP), and ventricular end-diastolic pressure (EDP) were obtained. Cardiac output and pulmonary vascular resistance were calculated by Fick method. RESULTS: Plasma pro-BNP levels exhibited statistically significant positive correlations with mean PAP (r=0.70, P< 0.001), pulmonary vascular resistance (r=0.57, P< 0.001), RVEDP (r=0.63, P< 0.001), LVEDP (r=0.74, P< 0.001), and cardiothoracic ratio (r=0.71, P< 0.001). The area under the ROC curve using pro-BNP level to differentiate risk groups in Fontan operations was high: 0.868 (95 percent CI, 0.712-1.023, P< 0.01). The cutoff value of pro-BNP concentrations for the detection of risk groups in Fontan operations was determined to be 332.4 pg/mL (sensitivity 83.3 percent, specificity 82.7 percent). CONCLUSION: These data suggest that plasma pro-BNP levels may be used as a predictive risk factor in Fontan operations, and as a guide to determine the mode of therapy during follow-up after Fontan operations.

The Results of Extracardiac Fontan Operation in the Patients with Heterotaxy Syndrome / 대한흉부외과학회지

BACKGROUND: Historically the Fontan operation in patients with single ventricle and heterotaxy syndrome has been associated with high mortality because of systemic or pulmonary anomalous venous drainage, incompetent common atrioventricular valve, right ventricle type univentricular heart, and arrhythmia. MATERIAL AND METHOD: A retrospective review of 62 patients (age: 54.79+/-33.97 months) with heterotaxy syndrome who underwent a extracardiac Fontan operation between 1996 and 2005 was performed. Twenty one patients had left atrial isomerism, and 41 had right isomerism. The Fontan procedure was staged in all but 2 patients, and a fenestration was less placed in left isomerism. RESULT: Left isomerism was associated more with interrupted inferior vena cava and pulmonary arteriovenous fistula, and right isomerism was associated more with anomalous pulmonary venous drainage, common atrioventricular valve and morphologic right ventricle. There were 3 hospital deaths (4.8%), and 3 late deaths (5.2%) with a follow-up duration of 48.8+/-31.0 months. Eight-year survivals were 90.5+/-6.4% in left isomerism and 88.6+/-5.4% in right isomerism (p=0.94). At 8 years, freedom from reoperation was 73.9+/-11.3% in left isomerism, and 82.3+/-6.7% in right isomerism (p=0.87). Atrioventricular valve regurgitation progressed after Fontan operation in heterotaxy syndrome, and reoperation for pulmonary arteriovenous fistula and permanent pacemaker implantation for sinus node dysfunction were required more in left isomerism. CONCLUSION: The extracardiac Fontan operation can now be performed in patients with heterotaxy syndrome with excellent survival. However, morbidity in terms of postoperative atrioventricular valve regurgitation, arrhythmia, and pulmonary arteriovenous fistula remains significant.

Arrhythmia Surgery in Fontan Operation / 대한흉부외과학회지

BACKGROUND: Refractory atrial arrhythmias in patients late after the Fontan operation result in significant morbidity and mortality. We reviewed our experience with arrhythmia surgery in patients who had Fontan operation. MATERIAL AND METHOD: Between July 1986 and December 2003, 275 early survivors after Fontan operation were reviewed. Fourteen patients underwent arrhythmia surgery at reoperation after Fontan operation, and mean age at reoperation was 16.8+/-7.1 (range: 4.5~30.6) years. Mechanisms of arrhythmia included atrial flutter in 8 patients, and atrial fibrillation in 2. Arrhythmia surgery has evolved from isthmus cryoablation in 12 patients to right-sided maze in 2 patients. Thirty-two patients underwent prophylactic isthmus cryoablation concomitantly at initial Fontan operation. RESULT: Postoperative arrhythmias occurred in 68 patients (24.7%) among 275. There was no early and late mortality after the arrhythmia surgery. After redo Fontan operation, all patients maintained normal sinus rhythm. Atrial flutter recurred in 3 patients who had sinus conversion with medication and 7 required permanent pacemakers with a mean follow-up of 26.5+/-29.1 (range: 2~73) months. All patients have improved to NYHA class I or II. After prophylactic cryoablation at initial Fontan operation, 29 patients (90.6%) had sinus rhythm, 1 patient had junctional tachycardia, 1 patient had sinus nodal dysfunction, and 1 patient had AV block with a mean follow-up of 51.3+/-19.8 (range: 4~80) months. CONCLUSION: Redo Fontan operation, and concomitant arrhythmia surgery reduced atrial arrhythmias and improved NYHA functional classification.

Fontan Procedure for Functional Single Ventricle with Major Aortopulmonary Collateral Arteries / 대한흉부외과학회지

Not only morphology of pulmonary artery or pulmonary artery resistance, but major aortopulmonary collateral arteries are risk factors of Fontan procedure. We report a successful Fontan procedure after rehabilitation of pulmonary arteries by unifocalization and systemic to pulmonary shunt in a high risk Fontan candidate with functional single ventricle combined with hypoplastic pulmonary arteries and major aortopulmonary collateral arteries supplying most of the bilateral lung field.

Anaesthetic Management of an Adult with a Modified Fontan Procedure in Noncardiac Surgery: A case report / 대한마취과학회지

Given improvements in the diagnosis and treatment of congenital heart disease, more patients with heart disease reach adolescence or adulthood and require noncardiac surgery. Since 1971, the Fontan operation has been widely used for the management of tricuspid atresia and a variety of univentricular heart malformations. Although the life expectancy of these patients has increased, few reports are available on their anaesthetic management when a noncardiac operation is indicated. We report on the anaesthetic management of a 40-years-old male patient scheduled for permanent pacemaker insertion who underwent a modified Fontan procedure at the age of 27 years for pulmonary stenosis, common atrioventricular cushion defect, and for corrected transposition of the great arteries