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Ann Thorac Surg 1996;61:845-850
© 1996 The Society of Thoracic Surgeons

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Original Articles: Cardiovascular

Usefulness of Pulsatile Bidirectional Cavopulmonary Shunt in High-Risk Fontan Patients

Departments of Cardiovascular Surgery and Pediatric Cardiology, National Children's Hospital, Tokyo, Japan

Accepted for publication November 4, 1995.

	Abstract

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Background. A bidirectional cavopulmonary shunt has been performed for the high-risk Fontan patient. It is well known that in the presence of the bidirectional cavopulmonary shunt alone to secure pulmonary blood flow, the central pulmonary artery size decreases over time. We have performed pulsatile bidirectional cavopulmonary shunt (PBCPS), keeping pulmonary blood flow from the ventricle through the stenotic pulmonary valve, or a Blalock-Taussig shunt in patients who do not meet the criteria for the Fontan operation.

Methods. Eleven patients who underwent PBCPS between 1989 and 1993 were reviewed. We compared the results of cardiac catheterization immediately before PBCPS and during the postoperative observation period (310 ± 257 days).

Results. Pulmonary blood flow and arterial oxygen saturation increased significantly after PBCPS (p < 0.01). Pulmonary artery area index showed a tendency to increase (p = 0.11). The mean number of risk factors for the Fontan procedure decreased significantly from 1.8 ± 1.1 to 0.7 ± 0.8 after PBCPS (p < 0.05). Overall, 5 of the 11 patients (45.5%) met the criteria for the Fontan procedure, and a fenestrated Fontan procedure was carried out in 4 of them.

Conclusions. The PBCPS is useful for high-risk Fontan patients not only in the staged Fontan operation, but also as definitive palliation.

	Introduction

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
See also page 849.

With the increase in the range of indications in recent years, the Fontan procedure has been expanded gradually to include not only tricuspid atresia, but also complex cardiac anomalies associated with a functional single ventricle. Since Choussat and associates [1] published their criteria for the Fontan procedure in patients with tricuspid atresia, various criteria have been proposed [2–5]. Recent reports have shown the usefulness of the so-called staged Fontan operation, in which a bidirectional cavopulmonary shunt (BCPS) [6, 7] is created before conducting the Fontan procedure, if certain requirements are met in high-risk Fontan patients [8–10]. In the presence of BCPS alone to secure pulmonary blood flow, however, the central pulmonary artery size decreases over time [11]. To deal with this problem, we have performed a pulsatile bidirectional cavopulmonary shunt (PBCPS) in high-risk Fontan patients who do not completely meet the criteria of the Fontan procedure. In the PBCPS technique, pulmonary blood flow was supplied, in addition to the BCPS, from the ventricle through the stenotic pulmonary valve (PS) or from a Blalock-Taussig shunt (BTS) on the side contralateral to the BCPS. This study reviews the usefulness of PBCPS for this group of patients.

	Material and Methods

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Eleven patients who underwent PBCPS between 1989 and 1993 despite failure to meet the criteria for the Fontan procedure were included (Table 1). They had a mean (± standard deviation) age of 5.9 ± 3.2 years and weighed 16.3 ± 6.4 kg at the time of operation, and were observed for 310 ± 257 days after operation. The diagnosis was single ventricle of the right-ventricle type in 7 patients, including 4 with right isomerism heart, and single ventricle of the left-ventricle type in 4 patients, including 1 with tricuspid atresia. Previous operations included BTS in 8 patients and pulmonary artery banding in 1.

Operative Procedure
The BCPS procedures were performed using cardiopulmonary bypass in all patients. To maintain an adequate pulmonary blood flow, we decided whether both the PS and BTS should be left intact or if either the PS or BTS should be closed, using the data of pulmonary/systemic blood flow ratio. In patients with a pulmonary/systemic blood flow ratio greater than 1.0, the BTS was ligated; in patients with a value less than 1.0, both the PS and BTS were left open. Bilateral BCPS was performed in 3 patients, BTS ligation in 3, simultaneous annuloplasty in 1, and annuloplasty plus valvuloplasty in 1. In addition to BCPS, pulmonary blood flow was supplied from the PS in 6 patients, the BTS in 3, and the PS and BTS in 2 after operation.

Outcome Variables
We compared the results of cardiac catheterization immediately before PBCPS and during the postoperative observation period (310 ± 257 days) in the 11 patients. Pulmonary artery pressure and resistance before and after operation were compared in 9 patients because they could not be determined in 2 patients (patients 1 and 2) before operation. Single ventricular end-diastolic volume before and after operation was compared in 8 patients because the BTS was ligated in 3.

Statistical Analysis
The paired t test was used to compare the following before and after PBCPS: arterial oxygen saturation, pulmonary/systemic blood flow ratio, pulmonary vascular resistance, mean pulmonary artery pressure, single ventricular end-diastolic volume index, and ventricular ejection fraction. The Wilcoxon signed rank test was used to compare the number of risk factors for the Fontan procedure before and after PBCPS. Differences were regarded as statistically significant at p less than 0.05.

	Results

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Operative Results of Pulsatile Bidirectional Cavopulmonary Shunt
The results are presented in Table 1. There were no operative deaths. One patient with right isomerism heart died (late death) of heart failure and arrhythmia caused by aggravation of atrioventricular valve regurgitation. As shown in Figure 1, right pulmonary artery pressure curves showed a pulsatile pattern. The arterial oxygen saturation increased significantly, from 75.4% ± 6.6% before operation to 83.4% ± 3.8% after operation (p < 0.01) (Fig 2). The pulmonary/systemic blood flow ratio increased significantly, from 0.87 ± 0.45 before PBCPS to 1.11 ± 0.42 after PBCPS (p < 0.01). The PAI increased in 8 patients, but did not change or decreased slightly in the remaining 3. Overall, The PAI showed a tendency to increase, from a mean of 270 ± 62 mm²/m² before operation to a mean of 297 ± 65 mm²/m² afterward, although this increase was not significant (p = 0.11). The PAI was less than 250 mm²/m² in 4 patients before and in 2 patients after operation (Fig 3). The pulmonary vascular resistance was similar before (2.2 ± 0.7 U•m²) and after (1.8 ± 0.7 U•m²) operation (p = 0.17). It was greater than 2.0 U•m² in 7 patients before and in 3 patients after operation (see Fig 3). The mean pulmonary artery pressure showed a tendency to increase, from 12.9 ± 2.1 mm Hg to 14.7 ± 4.2 mm Hg, although this increase was not significant (p = 0.15). The single ventricular end-diastolic volume index remained virtually unchanged, from 134 ± 62 mL/m² to 118 ± 33 mL/m² (p = 0.38). The ventricular ejection fraction also remained unchanged before and after PBCPS. The ventricular ejection fraction was less than 0.5 in 3 patients before and in 2 patients after operation. Annuloplasty and valvuloplasty were performed in 2 patients with atrioventricular valve regurgitation. The atrioventricular valve regurgitation was improved or remained unchanged (grade 1 or lower) in all patients, except for patient 5 with right isomerism heart.

View larger version (13K): [in this window] [in a new window]   Fig 1. . Right pulmonary artery pressure curves after pulsatile bidirectional cavopulmonary shunt in patients 1 and 5. Patient 1 (left) had Blalock-Taussig shunt and pulsatile bidirectional cavopulmonary shunt; patient 5 (right) had pulsatile bidirectional cavopulmonary shunt in the presence of pulmonary stenosis and bidirectional cavopulmonary shunt. Right pulmonary artery flows are pulsatile in both cases.

View larger version (19K): [in this window] [in a new window]   Fig 2. . Changes in arterial oxygen saturation index (SaO2; left) and the pulmonary/systemic blood flow ratio (Qp/Qs; right). Both indices showed significant increases after operation (p < 0.01). The paired t test was used for statistical analysis.

View larger version (19K): [in this window] [in a new window]   Fig 3. . Changes in pulmonary artery area index (PAI; left) and pulmonary vascular resistance (Rp; right). The PAI showed a tendency to increase, but not significantly (p = 0.11). The PAI was less than 250 mm2/m2 in 4 patients before operation, but this number decreased to 2 after pulsatile bidirectional cavopulmonary shunt. The pulmonary vascular resistance remained virtually unchanged before and after operation (p = 0.17). However, it was greater than 2.0 U•m2 in 7 patients before operation and 2 afterward. The paired t test was used for statistical analysis.

	Comment

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
The criteria for the Fontan procedure have changed over time, and the outcome of this procedure has improved through strict adherence to these criteria. Recently, a staged Fontan operation using a BCPS was reported to be useful in patients who do not completely meet the criteria for the Fontan procedure because of distortion of the pulmonary artery or atrioventricular valve regurgitation [8–10]. If a BCPS is created without leaving pulmonary blood flow through the PS or BTS [11], however, the central pulmonary artery size decreases over time because of decreases of pulmonary blood flow. To deal with this problem, since 1989 we have performed PBCPS in high-risk Fontan patients who do not completely meet the criteria for the Fontan procedure. The PBCPS increases pulmonary blood flow without increasing ventricular volume load, whereas BCPS does not necessarily increase pulmonary blood flow because of ligation of the BTS or closure of the PS. In fact, both pulmonary blood flow and arterial oxygen saturation increased significantly in all 11 patients in our study. Ventricular end-diastolic volume remained virtually unchanged after PBCPS, indicating that ventricular volume load did not increase. As a result of all these changes, atrioventricular valve regurgitation was aggravated only in the patient with right isomerism heart.

Mendelsohn and colleagues [11] reported that a significant decrease in PAI [12] occurred in patients with a BCPS in place for more than 15 months. In our patients with PBCPS, PAI showed a tendency to increase during the postoperative observation period (310 ± 257 days). The PAI was less than 250 mm²/m² in 4 patients before and 2 patients after operation. Our study revealed that in the central pulmonary artery growth, PBCPS was a very efficient procedure without reducing PAI. Pulmonary vascular resistance was greater than 2.0 U•m² in 7 patients and was not determined in 2 patients before operation. After PBCPS in these 9 patients, there were only 3 patients whose pulmonary vascular resistances were greater than 2.0 U•m². As a result, the number of risk factors for the Fontan procedure decreased significantly after PBCPS. Overall, 5 of the 11 patients (45.5%) became candidates for the Fontan operation. A fenestrated Fontan operation [13] has already been performed in 4 of these patients with good results, and the other patient is now awaiting this operation. Two of these 4 patients underwent closure of the fenestration using a clamshell device by cardiac catheterization, and 1 is now waiting for this procedure.

Although the usefulness of PBCPS based on the concept of a staged Fontan operation has not yet been reported, our study reveals acceptable results of PBCPS for the high-risk Fontan patient. In the remaining 6 patients, who were not suitable for the Fontan operation, pulmonary blood flow and arterial oxygen saturation increased significantly without increases in ventricular volume load or aggravation of atrioventricular valve regurgitation. The quality of life of these patients has improved. In 1991, Kobayashi and associates [14] performed PBCPS in patients who did not meet the criteria for the Fontan procedure and reported good results as definitive palliation. Our study also reveals that PBCPS is very useful for patients who are not suitable for a definitive Fontan procedure.

	Footnotes

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Address reprint requests to Dr Miyaji, Department of Cardiothoracic Surgery, Faculty of Medicine, University of Tokyo, Hongo 7-3-1, Bunkyo-Ku, Tokyo 113, Japan.

	References

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 

1. Choussat A, Fontan F, Besse P, Vallot F, Chauve A, Bricaud H. Selection criteria for Fontan's procedure. In: Anderson RH, Shinebourne EA, eds. Pediatric cardiology. Edinburgh: Churchill-Livingstone, 1978:559–65.
2. Mayer JE, Helgason H, Jonas RA, et al. Extending the limits for modified Fontan procedure. J Thorac Cardiovasc Surg 1986;92:1021–8.[Abstract]
3. Humes RA, Feldt RH, Porter CJ, Julsrud PR, Puga FJ, Danielson GK. The modified Fontan operation for asplenia and polysplenia syndromes. J Thorac Cardiovasc Surg 1988;96:212–8.[Abstract]
4. Fontan F, Fernandez G, Costa F, et al. The size of the pulmonary arteries and the results of the Fontan operation. J Thorac Cardiovasc Surg 1989;98:711–24.[Abstract]
5. Mair DD, Hagler DJ, Puga FJ, Schaff HV, Danielson GK. Fontan operation in 176 patients with tricuspid atresia. Results and a proposed new index for patient selection. Circulation 1990;82(Suppl 4):164–9.[Abstract/Free Full Text]
6. Mazzera E, Corno A, Picardo S, et al. Bidirectional cavopulmonary shunt: clinical applications as staged or definitive palliation. Ann Thorac Surg 1989;47:415–20.[Abstract]
7. Hopkins RA, Armstrong BE, Serwer GA, Peterson RJ, Oldham HN. Physiological rationale for a bidirectional cavopulmonary shunt: a versatile complement to the Fontan principle. J Thorac Cardiovasc Surg 1985;90:391–8.[Abstract]
8. Bridges ND, Jonas RA, Mayer JE, Flanagan MF, Keane JF, Castaneda AR. Bidirectional cavopulmonary anastomosis as interim palliation for high-risk Fontan candidates. Early results. Circulation 1990;82(Suppl 4):170–6.
9. Pridjian AK, Mendelsohn AM, Lupinetti FM, et al. Usefulness of the bidirectional Glenn procedure as staged reconstruction for the functional single ventricle. Am J Cardiol 1993;71:959–62.[Medline]
10. Hawkins JA, Shaddy RE, Day RW, Stuwtevant JE, Orsmond GS, McGough EC. Mid-term results after bidirectional cavopulmonary shunts. Ann Thorac Surg 1993;56:833–7.[Abstract]
11. Mendelsohn AM, Bove EL, Lupinetti FM, Crowley DC, Lloyd TR, Beekman RH III. Central pulmonary artery growth patterns after the bidirectional Glenn procedure. J Thorac Cardiovasc Surg 1994;107:1284–90.[Abstract/Free Full Text]
12. Nakata S, Imai Y, Takanashi Y, Kurosawa H, Tezuka K, Nakazawa M. A new method for quantitative standardization of cross-sectional areas of the pulmonary arteries in congenital heart diseases with decreased pulmonary blood flow. J Thorac Cardiovasc Surg 1984;88:610–9.[Abstract]
13. Bridges ND, Lock JE, Castaneda AR. Baffle fenestration with subsequent transcatheter closure: modification of the Fontan operation for patients at increased risk. Circulation 1990;82:1681–9.[Abstract/Free Full Text]
14. Kobayashi J, Matsuda H, Nakano S, et al. Hemodynamic effects of bidirectional cavopulmonary shunt with pulsatile pulmonary flow. Circulation 1991;84(Suppl 3):219–25.

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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Kagami Miyaji Akihiko Sekiguchi Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Miyaji, K. Articles by Isoda, T. Search for Related Content PubMed PubMed Citation Articles by Miyaji, K. Articles by Isoda, T. Related Collections Related Article