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Ann Thorac Surg 2007;84:1619-1626. doi:10.1016/j.athoracsur.2007.05.074
© 2007 The Society of Thoracic Surgeons
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Original Articles: Cardiovascular

Excellent Midterm Outcome of Extracardiac Conduit Total Cavopulmonary Connection: Results of 126 Cases

Toshihide Nakano, MD*, Hideaki Kado, MD, Tsuyoshi Tachibana, MD, Kazuhiro Hinokiyama, MD, Akira Shiose, MD, Masaki Kajimoto, MD, Yusuke Ando, MD

Department of Cardiovascular Surgery, Fukuoka Children’s Hospital, Fukuoka, Japan

Accepted for publication May 29, 2007.

* Address correspondence to Dr Nakano, Department of Cardiovascular Surgery, Fukuoka Children’s Hospital, 2-5-1 Tojin-machi, Chuo-ku, Fukuoka 810-0063, Japan (Email: nakano.t{at}fukuoka-child.jp).

Presented at the Forty-third Annual Meeting of The Society of Thoracic Surgeons, San Diego, CA, Jan 29–31, 2007.


   Abstract
Top
 Abstract
Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 References
 
Background: Extracardiac conduit total cavopulmonary connection has shown good early results; however, its long-term outcome has yet to be reported.

Methods: Of 282 patients who underwent extracardiac conduit total cavopulmonary connection since 1994, 126 patients who have been followed up for more than 5 years were included in this retrospective study. Actuarial survival rate, incidence of late complications, hemodynamic variables, and results of exercise tolerance test were reviewed.

Results: Follow-up time was 96.4 ± 23.0 months. There was 1 operative death and 6 late deaths. Actuarial survival rate was 95.2% and 93.6% at 5 and 10 years, respectively. Seven patients had late complications including new-onset supraventricular arrhythmia in 3, protein-losing enteropathy in 2, thromboembolism in 1, and bleeding complication in 1. Seven patients underwent reoperation not related to the conduit. Freedom from Fontan-related events was 88.8% at 5 years and 84.3% at 10 years. Late cardiac catheterization in 119 survivors showed central venous pressure of 9.9 ± 2.9 mm Hg, cardiac index of 3.6 ± 0.8 L · min-1 · m-2 and arterial oxygen saturation of 94.5 ± 2.3%. No patient showed conduit stenosis. Plasma concentration of atrial and brain natriuretic peptide (pg/mL) were 28.9 ± 20.0 and 25.8 ± 44.5. Exercise test performed in 101 patients showed endurance time of 75.7 ± 12.9% of normal value, peak heart rate of 92.3 ± 14.4% of normal, and peak oxygen consumption of 90.0 ± 20.0% of normal. The latest echocardiogram showed ejection fraction of 60.4 ± 11.7%. Three patients had pacemaker rhythm, 1 had junctional rhythm, and 115 patients had sinus rhythm.

Conclusions: Midterm outcome of extracardiac conduit total cavopulmonary connection was satisfactory with low incidence of late mortality and morbidity, and excellent hemodynamic state.


   Introduction
Top
 Abstract
 Introduction
Patients and Methods
 Results
 Comment
 Discussion
 References
 
The surgical procedure for single-ventricle physiology has evolved for decades, and the current modifications in common use are intraatrial lateral tunnel total cavopulmonary connection (LT-TCPC) and extracardiac conduit total cavopulmonary connection (EC-TCPC). As many patients have survived the early postoperative period, the surgeon’s attention has been directed to the late functional outcome of the patients, and there is no agreement regarding which modification could provide patients with better late quality of life. Limited numbers of reports comparing these two modifications are available, and both of these modifications have been revealed to show equally satisfactory early postoperative results [1–3]. Stamm and coworkers [4] analyzed 220 patients with LT-TCPC and described an excellent long-term outcome with good survival rate and low incidence of atrial tachyarrhythmia; however, little information is available for long-term outcome of EC-TCPC mainly because of its relatively short history.

Extracardiac conduit TCPC has theoretical advantages over LT-TCPC, including optimal laminar flow in the systemic venous pathway, minimal atrial surgery and cardiac ischemic time, and avoidance of pressure-related atrial stretch, which altogether could result in better preservation of ventricular function and lower incidence of atrial arrhythmia. In Fukuoka Children’s Hospital, EC-TCPC has been our procedure of choice for single-ventricle physiology since the mid 1990s. The purpose of this study is to investigate the late outcome of EC-TCPC by retrospectively reviewing the patients who have been followed up for more than 5 years.


   Patients and Methods
Top
 Abstract
 Introduction
 Patients and Methods
Results
 Comment
 Discussion
 References
 
This retrospective study was approved and individual consent was waived by the Institutional Ethics Committee of Fukuoka Children’s Hospital.

Of 282 patients with single-ventricle physiology who had undergone EC-TCPC since 1994, 126 consecutive patients who have been followed up for more than 5 years were included in this study. For the 156 patients who have been followed up for less than 5 years, follow-up was 100%, and follow-up time was 29.7 ± 17.5 months. Age at operation was younger than those who had earlier operation (4.2 ± 3.3 years versus 5.8 ± 3.6 years, p < 0.01). There was no operative death, and late death occurred in 2 patients. One patient died of ventricular failure 3 years after the operation, and the other patient died of infection 13 months after the operation. No reoperation has been performed so far, and 1 patient had protein-losing enteropathy. We excluded these 156 patients because we have already reported the good early results of EC-TCPC [1, 5], and we would like to focus on the late outcome of this modification in this study. Three patients who were converted to EC-TCPC from the other type of Fontan modification during the same period were excluded from this study. Follow-up was complete. Follow-up time was indicated by closing interval (elapsed time between the date of the surgery and the date of the most recent outpatient visit), and it was 96.4 ± 23.0 months (range, 60.4 to 147.6) in this group of patients.

Patient characteristics are summarized in Table 1. Thirty-nine patients had heterotaxy syndrome and accounted for 31.0% of the study group. All the patients had polytetrafluoroethylene (PTFE) graft for extracardiac conduit as an inferior vena cava–pulmonary artery pathway. The size of the graft was 16 mm in 39 patients, 18 mm in 56 patients, 20 mm in 30 patients, and 22 mm in 1 patient. The operation was performed with standard full flow cardiopulmonary bypass. and the heart was arrested when intracardiac procedure was required. Ninety-nine patients (78.6%) had undergone a preceding bidirectional Glenn operation. Concomitant procedures are listed in Table 2. In 2 patients, fenestration was created by interposing a PTFE graft (3 mm in the one and 8 mm in the other) between the extracardiac conduit and atrium. Indication for fenestration was intraoperative high central venous pressure in the one and small pulmonary artery index (100 mm2/m2) in the other. The patient with 8-mm fenestration underwent a subsequent surgical closure 5 months later; and in the other patient, fenestration closed spontaneously.


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  		Table 1 Patient Profile
 

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  		Table 2 Concomitant Procedures
 
The incidences of operative mortality, late mortality, and late complications were reviewed. Operative mortality was defined as a death occurring within 30 days of the operation or within the same hospitalization of the operation. Protein-losing enteropathy was diagnosed when a patient presented with decreased serum albumin level (< 3 g/dL); symptoms including chronic diarrhea, abdominal pain, and chronic edema in extremities or abdomen; and increased fecal {alpha}1 antitrypsin clearance more than 35 mL per day. Hemodynamic variables were obtained in the most recent cardiac catheterization. Results of echocardiogram, electrocardiogram (ECG), and plasma concentration of atrial natriuretic peptide (ANP) and brain natriuretic peptide (BNP) were also obtained from the last outpatient visit. Exercise tolerance tests using standard Bruce protocol were performed repeatedly in 101 patients. The results from the last test were reviewed and compared with age- and sex-matched control subjects (n = 653). Data are expressed by mean ± 1 SD. Actuarial survival and freedom from Fontan-related events were estimated by the Kaplan-Meier method.


   Results
Top
 Abstract
 Introduction
 Patients and Methods
 Results
Comment
 Discussion
 References
 
Postoperative Mortality
There was 1 operative death. A 2.9-year-old boy, with a single right ventricle, who underwent concomitant atrioventricular valve replacement died of ventricular failure and high pulmonary vascular resistance 1.2 months after the operation. Late deaths occurred in 6 patients. The first patient, with mitral atresia, who was readmitted for complete atrioventricular block and cerebral infarction subsequently died 1.3 months after the operation. The second patient, with hypoplastic left heart syndrome, had cardiac arrest during placement of chest tube for pleural effusion 3.4 months after the operation. The third patient, also with hypoplastic left heart syndrome, died of late onset fluminant hepatitis 4.3 months after the operation. The fourth patient, with tricuspid atresia, who underwent nonstaging TCPC had severe progressive ventricular dysfunction and died after 20 months. The fifth patient, with double-inlet ventricle, aged 12.6 years, died of congestive heart failure caused by progressive ventricular dysfunction 30 months after the operation. The last patient, with heterotaxy syndrome, died of pneumonia after 95.6 months. Actuarial survival rate for this group of patients was 95.2% at 5 years and 93.6% at 10 years (Fig 1). At the last follow-up, 3 patients were New York Heart Association class II, 1 patient was class III, and the remaining 115 survivors were class I.


Figure 1
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  		Fig 1. Kaplan-Meier estimated survival curve. Error bars indicate 95% confidence interval. Numbers of patients at risk are shown in parentheses.

 
Late Morbidity
Three patients, 2 of them were heterotaxy syndrome, had new-onset supraventricular arrhythmia including sick sinus syndrome in 2 and paroxysmal supraventricular tachycardia in 1 at an interval of 0.7, 9.6, and 42.2 months. Protein-losing enteropathy developed in 2 patients 49.3 and 49.9 months after the operation. One patient had cerebral infarction, possibly due to thromboembolism at 1.3 months postoperatively, and another patient had a minor subdural hematoma 3 months after the operation.

Reintervention
Seven patients underwent reoperation. Three patients had a permanent pacemaker implantation, for sick sinus syndrome in 1 patient and for advanced atrioventricular block in 2 patients. Ligation of large systemic venous-atrial collateral vessel was performed in 2 patients. Aortic valve replacement was done in 1 patient, and another patient had surgical closure of fenestration. Two patients underwent percutaneous transcatheter stent placement for short-segment pulmonary branch stenosis. No patient has required reintervention for extracardiac conduit so far. Freedom from Fontan-related events (including late mortality, morbidity, reoperation, and catheter reintervention) was 88.8% at 5 years and 84.3% at 10 years (Fig 2). The size of the conduit was not related to the freedom from Fontan-related events (79.4% in 16 mm, 89.1% in 18 mm, and 82.5% in 20 mm at 10 years; p = 0.33 by log-rank test).


Figure 2
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  		Fig 2. Kaplan-Meier estimated overall freedom from Fontan-related events, which include death, reoperation, and postoperative complications (supraventricular arrhythmias, protein-losing enteropathy, thromboembolism, and bleeding complication). Error bars indicate 95% confidence interval. Numbers of patients at risk are shown in parentheses.

 
Hemodynamic Assessments
All survivors had undergone postoperative cardiac catheterization at least once. The average number of postoperative catheterization was 2.4 times per patient. The mean interval from operation to the last cardiac catheterization was 39.4 ± 28.3 months. Hemodynamic variables obtained at the most recent catheterization are listed in Table 3. Thirty-one patients had cardiac catheterization with the interval of more than 5 years, and their hemodynamic variables were identical. Angiogram of the systemic venous pathway identified no thrombus formation or stenosis in the PTFE graft. The latest echocardiography was performed in 118 patients at an interval of 86.8 ± 25.2 months. Systemic ventricular function was normal in 96 (81.4%), mildly reduced in 19 (16.1%), moderately reduced in 2 (1.7%), and severely reduced in 1 (0.8%). The magnitude of atrioventricular valve regurgitation was none or trivial in 78 patients (66.1%), mild in 32 patients (27.1%), and moderate in 8 patients (6.8%).


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  		Table 3 Cardiac Catheterization
 
Plasma ANP and BNP Concentration
Plasma concentration of ANP and BNP obtained at the most recent follow-up was 28.9 ± 20.0 pg/mL and 25.8 ± 44.5 pg/mL (Fig 3). The majority of patients (80.9%) showed an ANP level within normal range (< 40 pg/mL), and 61.9% of patients had normal BNP level (< 18.4 pg/mL). Eight patients who showed moderate atrioventricular valve regurgitation at the last echocardiogram were revealed to have significantly higher levels of ANP (50.8 ± 28.9 pg/mL) and BNP (92.8 ± 123.8 pg/mL).


Figure 3
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  		Fig 3. Plasma concentrations of atrial natriuretic peptide (ANP) and brain natriuretic peptide (BNP). The values were within the normal range in 80.9% for ANP (< 40 pg/mL) and 61.9% for BNP (18.4 pg/mL). The solid circle indicates the mean value, and the error bar indicates one upper standard deviation.

 
Exercise Tolerance Test
A series of treadmill exercise tests with the standard Bruce protocol were performed regularly in 101 patients (84.9% of survivors), and data were collected from the last test in each patient for the analysis. Age at the test was 12.7 ± 4.4 years, and the interval from the operation was 82.7 ± 22.3 months (range, 32.9 to 143.6). The results are summarized in Table 4. When compared with age- and sex-matched control subjects, all the indices were significantly lower in the patients group. When percentage predicted value of more than 85% was defined as normal, only 22.8% of patients showed normal exercise endurance time, whereas 80.0% of patients had a normal heart rate response and 55.4% of patients showed normal peak oxygen consumption (VO2). The relationship between percentage predicted values and age at operation is shown in Figure 4. Younger age at operation was significantly correlated with higher peak VO2 (p < 0.001, R2 = 0.27); however, peak VO2 had no correlation with the duration of follow-up. During exercise, 8 patients showed sporadic atrial (n = 2) or ventricular (n = 6) premature beats, and 1 patient showed transient atrioventricular block. The remaining 92 patients showed no dysarrhythmia during the test.


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  		Table 4 Results of Treadmill Test
 

Figure 4
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  		Fig 4. Relation between age at operation and (A) exercise endurance time (EET), (B) peak heart rate (HR), and (C) peak VO2 in individual patients for the treadmill exercise test. There was a significant negative linear correlation between peak VO2 and age at operation (C); however, there was no correlation between peak VO2 and the duration of follow-up (D).

 
Cardiac Rhythm
In the latest follow-up ECG, 115 patients (96.6% of the 119 survivors) maintained sinus rhythm, 1 patient showed junctional rhythm, and 3 patients had pacemaker rhythm. Thirty-six patients had 24-hour Holter monitoring at the interval of 58.2 ± 31.5 months. Two patients were discovered to have sick sinus syndrome, 6 patients showed sporadic atrial or ventricular premature beats, and the remaining 28 patients showed no dysarrhythmia. One of the patients with sick sinus syndrome underwent permanent pacemaker implantation afterward.

Medication
The majority of the patients (98.3%) have been receiving low-dose warfarin sodium with the target International Normalized Ratio between 1.5 and 2.0, and 79.2% of patients have been taking aspirin (1 mg/kg daily) for prophylaxis of thrombus formation. The contraindication for warfarin in 2 patients was repeated history of hemoptysis associated with major pulmonary arteriovenous malformation combined with pulmonary venous stenosis in the one and history of minor subdural hematoma in the other. Angiotensin-converting enzyme inhibitor or angiotensin receptor blocker has been prescribed for 84.2% of our patients for its afterload reducing as well as cardiovascular protective effects. A ß-antagonist, mainly carvedilol, has been given to 25.0% of the patients who had any episode of tachyarrhythmias, decreased ventricular function, or elevated plasma BNP level. The other medications included diuretics (10.8%) and digoxin (5.0%).


   Comment
Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
Discussion
 References
 
Factors that could deteriorate the long-term outcome of post-Fontan patients are widely known and include atrial dysarrhythmias, protein-losing enteropathy, thromboembolism, and ventricular dysfunction [6–8]. Total cavopulmonary connection had been introduced to reduce the incidence of atrium-related complications and provide improved hemodynamic efficiency [9]. Currently, two modifications in common use are intra-atrial lateral tunnel TCPC (LT-TCPC) and extracardiac conduit TCPC (EC-TCPC). There is no agreement regarding the superiority of one type of modification over the other in terms of long-term outcome. Experimental investigations emphasized the importance of surgical design that minimizes energy loss in the systemic venous pathway to achieve an efficient Fontan circulation. Lardo and coworkers [10] demonstrated that EC-TCPC could provide superior hemodynamics compared with LT-TCPC in terms of fluid energy dissipation; on the other hand, Bove and coworkers [11] suggested using computational fluid dynamics that LT-TCPC following hemi-Fontan procedure could provide the superior hydraulic performance over the other modifications in the particular group of patients with hypoplastic left heart syndrome. Thus, whether EC-TCPC is the optimal palliation for all single-ventricle physiology is still controversial. Clinically, both modifications have shown good early and midterm outcomes [1–3]; however, our previous report [1] and others [2] demonstrated the advantages of EC-TCPC in terms of the postoperative morbidity, although the follow-up period was relatively short for the definite conclusion.

Extracardiac conduit TCPC has been performed with increasing frequency in patients with single-ventricle physiology. The good early to midterm outcome, low incidence of postoperative complications [1, 2, 12–14], and the functional and durable status of the conduit [15, 16] encourage the continuous usage of this procedure. One of the theoretical advantages of EC-TCPC is its low incidence of postoperative atrial arrhythmias, which would significantly deteriorate late outcome and quality of life of the post-Fontan population. The incidence of late-onset atrial arrhythmias ranges from 2.5% to 8% [1, 2, 12–14] after EC-TCPC, which is lower than that of LT-TCPC, ranging from 13.6% to 17% [1, 4, 17]. Our previous report [1] as well as that of others [2] found the LT-TCPC was an independent predictor of postoperative atrial arrhythmias; however, Kumar and coworkers [3] described the incidence of postoperative sinus node dysfunction in EC-TCPC as not negligible. The cause of postoperative supraventricular arrhythmias should be multifactorial, including patient-related factors as well as procedure-related factors. In fact, 2 of the 3 patients with supraventricular arrhythmia in our series were heterotaxy syndrome patients.

Cardiopulmonary response to exercise in post-Fontan patients was subnormal, with reported peak heart rate ranging from 76.6% to 80.6%, and peak VO2 ranging from 60.5% to 76.1% in types of Fontan modifications other than EC-TCPC [18–20]. Ventricular filling capacity is recognized to be a major determinant during exercise and is influenced by ventricular compliance, pulmonary vascular resistance, and efficiency of the systemic venous pathway [21]. In our series, mean peak VO2 was 90.0% of age-and sex-matched predicted value, which was better than the result of other investigators. We assume this is possibly because EC-TCPC provides more efficient systemic venous blood flow during exercise than other types of Fontan modifications, along with our aggressive surgical approach for pulmonary distortion at any time of staging operations to achieve unobstructed pulmonary vasculature. In addition, the higher systemic oxygen saturation in our series (94.5%) due to our nonfenestrating policy may play a part in our good results. Diller and coworkers [22] described that a lower heart rate response in exercise was associated with a greater risk of death in Fontan operation. The reason for the impaired heart rate response to exercise in Fontan population is unknown, but it should be multifactorial and include sinus node dysfunction. Our result of near-normal heart rate response to exercise (92.3% of predicted value) may be attributable to better preservation of sinus node function in EC-TCPC patients, which could provide another rationale for using this modification. Indeed, our previous report revealed that peak heart rate at exercise was significantly higher in EC-TCPC compared with LT-TCPC [1]. Comparable with other investigators, we also found that earlier completion of Fontan circulation was associated with better exercise performance [18, 19], and of note, exercise performance was not decreased with the duration of follow-up so far. Senzaki and coworkers [23] suggested, however, that Fontan patients have an intrinsic abnormality in both ventricular systolic and diastolic function in response to elevated heart rate; therefore, careful observation is required in the longer follow-up.

Increased atrial wall tension is the dominant stimulus for ANP release [24], and atrial distension causes development of atrial arrhythmias [25]. We previously reported a significant relationship between the degree of atrial wall dilatation, plasma ANP concentration, and development of supraventricular arrhythmias in LT-TCPC patients [1]. In this study, the majority of patients showed normal range of ANP level, indicating absence of pressure-related atrial stretch in EC-TCPC patients. This finding could provide a reasonable explanation for the low incidence of late-onset atrial dysarrhythmias in our patients. In contrast, BNP is released mainly from the ventricle during pressure overload and myocardial stretch, and plasma concentration of BNP is elevated in patients with ventricular hypertrophy or congestive heart failure [24]. Law and coworkers [26] reported that plasma BNP is elevated in post-Fontan patients with systemic ventricular dysfunction, and severity of heart failure was significantly associated with plasma BNP level. They also implied the potential value in using BNP to monitor the ventricular function in post-Fontan patients. The present study revealed 61.9% of our patients showed the normal range of BNP, and we consider strengthening the medication, including ß-antagonist, in patients with elevated plasma BNP levels.

Because of the palliative nature of the surgery as well as the presence of prosthetic material, it is our institutional policy that patients who have undergone EC-TCPC should be treated with cardiovascular protective and anticoagulant medicines. Post-Fontan patients manifest the increased systemic vascular resistance in response to low cardiac output status [27]. The importance of afterload reduction therapy has been acknowledged, and we prescribe angiotensin-converting enzyme inhibitors or angiotensin receptor blockers for most of our patients for this purpose [28]. Recently, Jin and coworkers [29] demonstrated that post-Fontan patients presented significantly impaired peripheral vascular function partly attributable to the preoperative chronic hypoxia, and they also implied the protective effects of angiotensin-converting enzyme inhibitor on endothelial function in post-Fontan patients.

The optimal anticoagulant prophylaxis after EC-TCPC is unknown [30, 31]; however, it has been well known that post-Fontan patients have abnormalities in both procoagulant and anticoagulant factors and the imbalance between these factors potentially favors thrombus formation [32]. Previous reports showed an incidence of 20% to 23% of thrombus formation in the extracardiac conduit if anticoagulants were not given [33, 34]. Our policy for anticoagulant prophylaxis is lifelong treatment with warfarin in combination with aspirin. The result of the present study revealing no thrombus formation in the conduit, and only one episode of possible thromboembolism and one bleeding complication, could justify the effectiveness of our anticoagulant protocol. In addition, low Fontan pressure obtained in the late cardiac catheterization in our patients could possibly attributable to the low risk of clinically silent small pulmonary emboli with our anticoagulant strategy.

The main concern for the conduit is the absence of the longitudinal growth potential because the diameter of the conduit (17.9 ± 1.5 mm in our series) could be considered as big enough for the adult body size [15, 16]. It has been reported that, in children 2 to 4 years of age, the distance between the inferior vena cava and the undersurface of the right pulmonary artery is approximately 60% of that in adults [16]. Therefore, it is possible that the conduit may cause distortion of the right pulmonary artery, compression of the pulmonary vein, or flow disturbance in the conduit as patient grows. Although we and other investigators have not observed either such problems or longitudinal torsion of the conduit in the midterm follow-up so far [15], further investigations are necessary for the long-term outcome of the conduit and the circumference.

Limitations
The limitation of this study is the retrospective nature of data collection. It may be that we underestimate the incidence of postoperative arrhythmias. The availability of data from the Holter study was limited because the test was conducted only for those in whom arrhythmia was suspected. However, findings from repeated measurements of exercise ECG (84.2%) in conjunction with serial ECG at every outpatient visit (100%) and Holter studies (30.0%) in our series could possibly cover the overall prevalence of arrhythmias.

In conclusion, the midterm outcome of patients who had undergone EC-TCPC was satisfactory, with a low incidence of late mortality and morbidity and excellent hemodynamic state. However, the long-term benefits and late incidence of conduit-related complications remain to be determined in the future follow-up studies.


   Discussion
Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
References
 
4DR JEFFREY P. JACOBS: (St. Petersburg, FL): While we’re waiting, I was just wondering, is the strategy in your program, then, just that they’re going to stay on coumadin for a long-term period, or do you have any plans at any time to take any of the patients off of the coumadin?

DR NAKANO: It’s our policy to treat the patients with anticoagulant therapy after Fontan operation. They should take lifelong treatment with coumadin and aspirin, because as you know, many literatures reported that the patients who have undergone Fontan operation have abnormalities in both procoagulant and anticoagulant factors, and the imbalance between these factors potentially favors thrombus formation. So in order to maintain good Fontan circulation, we think we need to put patients on a lifelong regimen of coumadin and aspirin. In addition, our good Fontan pressure, as I showed you in the slide, which was less than 10 mm Hg, could be attributable to the very low risk of clinically silent pulmonary thromboembolism with our anticoagulant strategy.

DR MARSHALL L. JACOBS (Philadelphia, PA): I want to compliment you on terrific results and a very, very clear, nicely organized presentation. My questions are about the very, very low incidence of late rhythm disturbances. You had only 3 patients with late arrhythmias, and I think even other large series of extracardiac conduit Fontans after bidirectional Glenn still seem to show an incidence of sinus node dysfunction that begins to have an increasing hazard function not in the first 5 years after surgery but in the interval between 5 and 10 years.

I wondered with only 36 of the patients having Holter monitors, on the one hand, do you think you might be underestimating the incidence of sinus node dysfunction? And if not, which is certainly a possibility and you have this very, very rare instance, could you tell us whether there’s something unique or innovative about the surgical techniques you’ve used that account for this much lower incidence of sinus node dysfunction in this series than in others?

DR NAKANO: Thank you very much for your comment, Dr Jacobs. It’s possible that we underestimate the incidence of atrial arrhythmias. However, we performed an exercise test on a regular basis, and we get an exercise ECG in every exercise test along with the series of regular ECGs at every single outpatient visit. Although Holter monitoring is very limited, I think we can cover the overall incidence of atrial arrhythmia in this group of patients.

With regard to the operation, we don’t perform any different procedures. What I can say is we dissect around the sinus node area very, very carefully, and we haven’t had any case in which we surgically injured the sinus node.

DR GERHARD ZIEMER (Tuebingen, Germany): In our experience with lateral tunnel or extracardiac conduit Fontans, which was published last year in the Journal of Thoracic and Cardiovascular Surgery, we do not routinely give coumadin. However, there were not extracardiac Gore-Tex tubes. These were extracardiac non–Gore-Tex tube tunnels, namely, constructed with pedicled in-situ pericardium sutured to the epicardial surface.

But my question is, if you really insist on the efficiency of coumadin plus aspirin, what would you reply to the notion that 40% of these patients may have aspirin resistance? So how can you say that it works in these patients?

DR NAKANO: We haven’t had such a problem at present. We use the coumadin in conjunction with the aspirin based on a study from our cardiologist. We use low doses of aspirin, which was 1 mg/kg per day, a very small dose of aspirin, and the target number of INR was 1.5 up to 2.0 for coumadin, so it’s not an aggressive anticoagulation strategy we are doing.

DR ZIEMER: But if there was aspirin resistance, you may not need to give it, and you cannot say it’s worthwhile giving it at all.


   References
Top
 Abstract
 Introduction
 Patients and Methods
 Results
 Comment
 Discussion
 References
 

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