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Ann Thorac Surg 1998;65:1721-1725
© 1998 The Society of Thoracic Surgeons
a Department of Cardiovascular Surgery, Fukuoka Childrens Hospital, and Department of Cardiac Surgery, Kyushu University Hospital, Fukuoka, Japan
Accepted for publication December 30, 1997.
Address reprint requests to Dr Masuda, Department of Cardiac Surgery, Kyushu University Hospital, Maidashi 3-1-1, Higashi-ku, Fukuoka, 812 Japan
| Abstract |
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Methods. Between November 1991 and July 1996, the two-staged strategy was performed in 40 high-risk Fontan candidates with a mean interval of 17.2 months after introducing the bidirectional Glenn shunt (staged group). We considered a young age (<2 years), high mean pulmonary arterial pressure (
20 mm Hg), high pulmonary vascular resistance (
3 Wood units), small pulmonary artery (Nakata index <200 mm2/m2), atrioventricular valve incompetence (
moderate), distortion of pulmonary artery, anomalous pulmonary venous return, and poor ventricular function as risk factors for the successful completion of Fontan circulation. During the same pe-riod, 68 patients underwent the modified Fontan procedure in a one-step fashion (primary group).
Results. In the staged group after the bidirectional Glenn shunt, the mean pulmonary arterial pressure and ventricular end-diastolic pressure were both found to have decreased significantly to the same level as those in the primary group, whereas the pulmonary artery demonstrated a significantly smaller size than that in the primary group. Operative morbidity was similar in both groups. Operative mortality was also similar and low in both groups (1.5% in the primary group and 0% in the staged group).
Conclusions. A bidirectional Glenn shunt was found to be a useful interim palliation in high-risk Fontan candidates. This two-staged strategy may extend the operative indications for the Fontan procedure.
| Introduction |
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Although there are still no clearly accepted criteria regarding the risk factors for the Fontan procedure, we consider the following factors to be risk factors for the completion of Fontan circulation according to previous reports in the literature: age less than 2 years [1, 2], a mean pulmonary arterial pressure more than 20 mm Hg [3, 4], a pulmonary vascular resistance more than 3 Wood units [5, 6], a cross-sectional area of the pulmonary arteries (Nakata index [7]) less than 200 mm2/m2 [5], more than moderate atrioventricular valve regurgitation [3, 4, 6, 8, 9], a distortion of the pulmonary artery such as a nonconfluent or almost nonconfluent pulmonary artery [35, 9, 10], anomalous pulmonary venous return [6, 9], and a ventricular ejection function less than 0.40 [4]. When the pulmonary arterial pressure or pulmonary vascular resistance is unknown, we consider the patient to have one risk factor.
In this article, we compared the clinical results of primary Fontan cases (low-risk group) and staged Fontan cases (high-risk group) to evaluate the efficacy of our strategy for using the Fontan procedure.
| Patients and methods |
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The patients in the staged group underwent the bidirectional Glenn shunt at an average age of 4.9 ± 4.0 years (age range, 0.6 to 16.7 years), and the Fontan procedure was completed at an average age of 6.3 ± 4.0 years (age range, 1.5 to 18.6 years) with a mean interval of 17.2 ± 5.0 months after the bidirectional Glenn shunt. The average age at the time of undergoing the Fontan procedure in the primary group was 6.8 ± 3.9 years and was not significantly different from that in the staged group. The most common diagnoses were a single ventricle (39 in the primary group and 25 in the staged group), tricuspid atresia (9 and 6), and mitral atresia (5 and 3, respectively).
Seventy-two percent of the primary group patients and 80% of the staged group patients underwent previous operative procedures such as a systemic-to-pulmonary shunt or pulmonary artery banding at least once (maximum, three times) before either the Fontan operation or bidirectional Glenn shunt.
Before undergoing a bidirectional Glenn shunt, the patients in the staged group had more risk factors than the patients in the primary group (Table 1). Preoperative catheterization revealed a higher mean pulmonary arterial pressure, a higher pulmonary vascular resistance, a lower arterial oxygen saturation, and a higher ventricular end-diastolic pressure in the staged group compared with those in the primary group (Table 2). Before the Fontan procedure in the staged group, the mean pulmonary arterial pressure and the ventricular end-diastolic pressure decreased significantly to the same level of those in the primary group, whereas the pulmonary vascular resistance remained unchanged and was still higher than that in the primary group. The pulmonary artery showed a significantly diminished size after the bidirectional Glenn shunt (Fig 1) without any progression of arterial oxygen desaturation. One patient in the staged group underwent a replacement of the atrioventricular valve after a bidirectional Glenn shunt and before the completion of the Fontan procedure.
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All data were expressed as the mean ± 1 standard deviation, except for actuarial probability estimates represented as the mean ± 1 standard error. An analysis of differences was appropriately done by the paired or unpaired Students t test or the Mann-Whitney U test on continuous variable comparisons, or by the
2 contingency tables on discrete variable comparisons. An actuarial analysis was performed by the Kaplan-Meier method, and the generalized Wilcoxon test was used for comparisons of the actuarial rates. The statistical analysis was carried out using Stat View (Abacus Concepts, Inc, Berkely, CA) on a Macintosh computer. A p value less than 0.05 was considered significant.
| Results |
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After completing the Fontan circulation, almost half of the patients required catecholamine support in both groups (Table 4). Ninety-six percent of the primary group patients and 93% of the staged group patients were extubated 1 day after the operation. The mean duration of pleural drainage was similar in both groups (almost 2 weeks). The median value was also the same in both groups (10 days). The incidence of perioperative arrhythmia, such as junctional bradycardia, supraventricular tachycardia, and atrioventricular block, was also similar in both groups. Most of these arrhythmias were transient. There was one operative death within 30 days in the primary group (1.5%) and no death in the staged group (0%).
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A complete follow-up was done in both groups. The mean follow-up duration was 20.3 ± 13.9 months (maximum, 51.4 months) in the primary group and 11.6 ± 6.7 months (maximum, 27.2 months) in the staged group. Three patients in each group died during the follow-up period. The actuarial survival rate was 93% ± 3% at 4 years in the primary group, and 95% ± 4% at 2 years in the staged group, and no significant differences were observed between the groups.
| Comment |
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During the study period, 82 bidirectional Glenn shunts were performed on high-risk patients in our hospital with an acceptable operative mortality rate (1.2%). Ten patients died before the completion of the Fontan procedure, and they had been considered to be unsuitable Fontan candidates. Forty patients have already completed the Fontan procedure successfully without early operative death (operative mortality, 0%). The remaining patients are still waiting for completion of the Fontan procedure. Norwood and Jacobs [12] reported an excellent early mortality rate of 7.6% in a staged completion of the Fontan operation followed by the hemi-Fontan operation, but a primary Fontan operation had quite a high early mortality rate of 16%. Similar series at both the University of Michigan [5] and the University of Utah [9] have also reported good results in performing the staged Fontan operation with an operative mortality rate of less than 8%. We believe that our results of an overall mortality of 0.9% including a primary Fontan operation may thus support our strategy for the Fontan procedure.
In our high-risk patients, a bidirectional Glenn shunt resulted in a significant decrease in the mean pulmonary arterial pressure and ventricular end-diastolic pressure. The reduction in the pulmonary blood flow and ventricular volume due to the bidirectional Glenn shunt might be the main cause of these favorable results during the waiting period for the completion of the Fontan procedure without any progression of arterial oxygen desaturation. Although the pulmonary artery showed a significantly diminished size after a bidirectional Glenn shunt, Mendelsohn and colleagues [13] reported a reduction in the pulmonary artery size more than 15 months after a bidirectional shunt, and our results are compatible with their findings. Although the changes in the pulmonary artery size in the staged group seemed to have almost no impact on the operative results of the Fontan procedure, we now intend to complete the Fontan procedure within 18 months after a bidirectional Glenn shunt, whenever possible.
For Fontan circulation, both pulmonary vascular resistance and the compliance of the ventricle are the most important factors in venous blood flowing through the pulmonary vasculature. Recent studies have focused on the impaired diastolic ventricular function early after the Fontan procedure [14, 15]. After the completion of Fontan circulation, the ventricular end-diastolic volume decreases suddenly and markedly with a constant ventricular muscle mass resulting in relative hypertrophy of the ventricle. This inappropriate ventricular hypertrophy is supposed to impair the diastolic ventricular filling that causes an increase in the pulmonary venous pressure and systemic venous pressure associated with a low cardiac output. This relative hypertrophy is reported to decrease by 2 months [14]. In the staged Fontan procedure, the bidirectional Glenn shunt as a first step operation alone also causes a similar volume reduction in the ventricle; however, the impact of impaired diastolic ventricular compliance is diminished only because the superior venous blood has to flow through the pulmonary vasculature. After completing the staged Fontan procedure, the diastolic ventricular volume remains constant. As a result, the staged Fontan procedure may avoid the deleterious effect of a sudden decrease in the diastolic ventricular volume, which might be fatal in some high-risk patients.
As a first-step operation for the staged Fontan procedure there are some alternatives including the classic Glenn shunt, a bidirectional Glenn shunt, and the hemi-Fontan procedure. A classic Glenn shunt introduces the superior venous blood directly into the right lung. Recently, hepatic venous blood has been considered to be an important factor in preventing pulmonary arteriovenous malformations [16]. In the classic Glenn shunt, the hepatic venous blood flows only through the left pulmonary vasculature even after the completion of Fontan circulation. The hemi-Fontan procedure requires cardiac arrest not only at the first stage but also at the second stage. Therefore, we selected a bidirectional Glenn shunt as a first step operation because it does not per se require cardiac arrest and the hepatic venous blood is distributed to both lungs after the completion of Fontan circulation. Although Manning and colleagues [17] reported a higher incidence of sinoatrial node dysfunction in the staged operation, the incidence rate of perioperative arrhythmia in the staged Fontan group was similar to that in the primary Fontan group. The frequent application of an extracardiac conduit might avoid any damage to the sinus node artery in our staged Fontan patients.
The extracardiac Fontan procedure is another topic of discussion regarding the Fontan operation [1820]. The extracardiac conduit technique has many advantages: (1) it avoids myocardial ischemia; (2) it avoids atriotomy and an intraatrial suture line, which may cause supraventricular arrhythmia; (3) it avoids atrial over- distention by high systemic venous pressure, which also may cause postoperative arrhythmia; and (4) it provides hemodynamic benefits due to the laminar flow. Although further investigation is required concerning the growth capability and incidence of thromboembolism, we consider an extracardiac conduit to be a useful option for the Fontan procedure in high-risk patients.
The long-term results of a total cavopulmonary connection in high-risk patients remains a matter of concern. However, there is still little information available on this subject [6]. Because the Fontan operation is a palliative procedure, a careful and close follow-up is required. In our hospital, warfarin is given to all patients with a total cavopulmonary connection to prevent any thromboembolism occurring in the pulmonary circulation, although the benefits of warfarin are still uncertain and therefore, further investigation is required.
In conclusion, the clinical results of a staged Fontan procedure in high-risk patients were excellent. The two-staged strategy may extend the operative indications for the Fontan procedure.
| Acknowledgments |
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| References |
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