(Circulation. 1995;92:294-297.)
© 1995 American Heart Association, Inc.
Articles |
From the Divisions of Cardiac Surgery and Cardiology, Children's Heart Institute, Children's Hospital-San Diego, Calif.
Correspondence to Richard D. Mainwaring, MD, 3030 Children's Way, Suite 310, San Diego, CA 92123.
| Abstract |
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Methods and Results Ninety-two patients have undergone BDG at our institute during the interval from 1986 through 1994. At the time of BDG, 40 patients had either a systemic-topulmonary artery shunt or patent right ventricular outflow tract as an additional source of pulmonary blood flow. Fifty-two patients had elimination of APBF. There were three operative deaths (two with and one without APBF) and four procedures (two in each group) that failed and required subsequent revision. Thus, there were 85 patients who underwent successful operation. Effusions (defined as chest tube drainage exceeding 7 days' duration) occurred in 8 of 85 patients; this complication was seen in 7 of 36 patients (19%) with APBF and 1 of 49 patients (2%) without APBF (P<.05). There were 11 deaths, including 6 patients (17%) with APBF, 2 patients (4%) without APBF, and 3 of the patients (75%) who had a failed BDG.
Conclusions The data suggest that morbidity and mortality are lower in patients in whom APBF is eliminated at the time of the BDG.
Key Words: shunts ventricles blood flow
| Introduction |
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Although many centers endorse the staged surgical approach to single ventricle patients, certain controversies still exist. There is considerable debate concerning the timing of the BDG, as well as the ideal interval between BDG and the Fontan procedure.10 In addition, there is a divergence of opinion as to the merits of leaving an accessory source of pulmonary blood flow (eg, a systemic-topulmonary artery shunt or a patent right ventricular outflow tract) at the time of the BDG. Proponents suggest that a dual source of pulmonary blood flow results in more satisfactory levels of arterial saturation, but opponents emphasize the merits of eliminating volume loads to the heart.11
At Children's Hospital-San Diego, we began performing the BDG in 1986.12 For several years, accessory sources of pulmonary blood flow were left in the majority of operations performed, whereas in more recent years we have favored eliminating these additional sources of blood flow. The present study reviews our experience to assess the impact of accessory pulmonary blood flow on outcome after the BDG.
| Methods |
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Forty patients had a shunt or patent right ventricular
outflow tract as an additional source of pulmonary blood flow
augmenting the BDG. The median age of these patients at operation
was 11 months (range, 2.5 to 195 months; 10th to 90th
percentile, 3 to 53 months) and the median weight was 7.4 kg
(range, 3.9 to 37 kg; 10th to 90th percentile, 4.8 to 13.1 kg). Thirty
of these patients (75%) had undergone a previous palliative procedure
(see Table 1
). Concomitant procedures performed in this
group are summarized in Table 2
.
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Fifty-two patients had no accessory pulmonary blood flow at
the time of the BDG. Median age was 8.6 months (range, 2.2 to 46
months; 10th to 90th percentile, 4 to 14 months) and the median weight
was 6.9 kg (range, 3.9 to 15.8 kg; 10th to 90th percentile, 5.2 to 10.3
kg). Thirty-two of the 52 patients had undergone a previous
palliative procedure (see Table 3
). Concomitant
procedures are summarized in Table 4
.
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The results of this study are reported as either the mean±SEM or, where appropriate, the median with the range and 10th to 90th percentile limits. Statistical comparison of the two groups was performed using a Mann-Whitney test. Kaplan-Meier survival curves were constructed using the product-limit method. To test for an association between survival and year of follow-up, a two-tailed Fisher's exact test was used. A value of P<.05 was considered significant.
| Results |
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There were three operative deaths (3.3%), two occurring in the group with accessory pulmonary blood flow and one without accessory pulmonary blood flow. Causes of operative death included failure to oxygenate, leading to myocardial dysfunction in two patients, and primary myocardial dysfunction in one.
Four patients (two from each group) underwent a BDG that failed to provide satisfactory palliation and subsequently required surgical revision. Each of these patients had significant hypoplasia of one branch pulmonary artery and underwent attempted pulmonary artery reconstruction at the time of the BDG. Two patients had acute thrombosis of the BDG managed by insertion of a systemic-topulmonary artery shunt on the side of the discontinuous pulmonary artery. One patient had late thrombosis and also underwent systemic-topulmonary artery shunt. The fourth patient on follow-up had severe stenosis of one pulmonary artery and underwent revision and patch enlargement. This attempt resulted in thrombosis, and subsequently a shunt was performed to the discontinuous pulmonary artery.
Eighty-five patients underwent successful BDG (36 with accessory
pulmonary blood flow and 49 without). Effusions (defined as
chest tube drainage exceeding 7 days) occurred in 7 patients (19%) in
the former group and in 1 (2%; P<.05) in the latter group
(Fig 2
).
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Length of hospital stay for the two groups is shown in Fig
3
. There were 3 patients (6%) who underwent BDG with no
accessory pulmonary blood flow whose hospital stay exceeded 14
days. In contrast, 11 patients (31%) in the group with accessory
pulmonary blood flow had a hospital stay in excess of 2 weeks
(P<.05). More than half of the prolonged hospitalizations
were attributable to the development of effusions. Other causes of
prolonged stay included poor oxygenation (n=2), low
cardiac output (n=2), sepsis (n=1), and sick sinus syndrome
requiring
pacemaker insertion (n=1).
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There were 11 late deaths including 6 patients (17%) with accessory
pulmonary blood flow, 2 patients (4%) without accessory
pulmonary blood flow (P<.05), and 3 of the patients
(75%) who had a failed BDG. Causes of late death are listed in Table
5
.
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Actuarial analysis was performed for patients undergoing BDG
with and without accessory pulmonary blood flow (Fig 4
). This
evaluation indicates 1-, 2-, and 3-year
survival rates of 92%, 84%, and 77% with accessory pulmonary
blood flow versus 98%, 91%, and 86%, respectively, without accessory
pulmonary blood flow.
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Fifty-three of the 85 patients who underwent successful BDG have subsequently undergone completion of their Fontan procedure. This includes 22 of 36 patients with accessory pulmonary blood flow and 31 of 49 patients without accessory pulmonary blood flow. There were 3 (6%) early and 3 (6%) late deaths after the Fontan procedure.
| Discussion |
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Effusions are one of the principal causes of extended hospital stay after BDG. This complication was seen in 9% of patients who underwent successful operation, an incidence comparable to previous reports.13 However, in our series, the incidence of effusions was ninefold higher in the group with accessory pulmonary blood flow. The cause of pleural effusions after BDG remains uncertain but clearly must be associated with an increased left-to-right shunt. The single patient who developed an effusion after BDG without accessory pulmonary blood flow had a large aortopulmonary collateral vessel that served as the major source of pulmonary blood flow preoperatively. When this patient developed an effusion postoperatively, catheter embolization was performed with resolution of this complication.
Combined early and late deaths occurred in 21% of patients with accessory pulmonary blood flow compared with 6% of patients without accessory pulmonary blood flow. Several deaths were likely unrelated to the BDG (eg, the patient who died of tracheal complications), although others may be more directly related (such as those after the Fontan procedure). The actuarial analysis does suggest a survival advantage for patients without accessory pulmonary blood flow. The reason for this difference is unknown, but potentially early relief from volume load could result in either improved preservation of ventricular function11 or a reduction in changes in pulmonary vascular resistance.4
Mendelsohn et al14 have demonstrated that the BDG may result in impaired growth of the pulmonary arteries. Potentially, this could have an adverse effect at the time of the Fontan, and some authors have emphasized the importance of pulmonary artery size in this operation.15 Others have found that pulmonary artery size does not predict survival after the Fontan procedure.16 17 Certainly, if pulmonary artery size were universally accepted as an important predictive factor, then one might speculate that the addition of accessory pulmonary blood flow to the BDG might facilitate pulmonary artery growth and improve outcome after the Fontan. From our data, one could infer that pulmonary artery growth does not appear to be a significant clinical problem because patients undergoing BDG without accessory pulmonary blood flow demonstrate a level survival curve, at least through 3 years of follow-up, with a significant number having completed the Fontan.
Pulmonary arteriovenous (AV) fistulas after the BDG procedure have been a theoretical concern. The development of pulmonary AV fistulas has been noted after the classic Glenn shunt18 and after the Kawashima operation.19 20 Both of these operations exclude hepatic venous circulation to the lungs on first pass, suggesting a "hepatic factor" in the etiology of pulmonary AV fistulas.21 Since the BDG also excludes hepatic circulation to the lungs, it is plausible that pulmonary AV fistulas could develop in this setting. This rationale has been put forth as an argument in favor of including a source of accessory pulmonary blood flow.21 However, we have not observed the development of pulmonary AV fistulas after BDG except in the specific circumstance of interrupted inferior vena cava with azygous continuation. There were 4 patients included in our series with this systemic venous anatomy, 2 of whom have subsequently developed fistulas. In contrast, none of the 88 patients with a continuous inferior vena cava have developed pulmonary AV fistulas. Thus, the argument to leave accessory pulmonary blood flow to prevent pulmonary AV fistulas does not seem to be well founded for the vast majority of patients. Whether a source of accessory pulmonary blood flow would prevent pulmonary AV fistulas after the Kawashima operation is unknown.
The BDG procedure failed in 4 of 92 patients; each of these patients had severe stenosis and hypoplasia of the left pulmonary artery. Attempts at reconstruction of the pulmonary arteries may increase the risk of thrombosis after BDG secondary to the low pressure venous flow. Each of these patients subsequently had a shunt placed to the discontinuous pulmonary artery. One patient developed pleural effusions after this procedure and eventually died of sequelae of the effusion. Two other patients in this group died, resulting in an overall mortality rate of 75% for failed procedures. On the basis of this experience, it is our current recommendation to address severe pulmonary artery stenosis or hypoplasia well in advance of the BDG using either surgical or stent techniques.22
In summary, patients undergoing the BDG and having no accessory pulmonary blood flow had lower morbidity and mortality than patients with accessory pulmonary blood flow. It is our belief that relief from volume loading will aid in preserving both pulmonary and ventricular function. Further study is required to determine whether this strategy will improve long-term outlook after the Fontan procedure.
| Acknowledgments |
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| References |
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2. Mazzera E, Corno A, Picardo S, Di Donato R, Marino B, Costa D, Marcelletti C. Bidirectional cavo-pulmonary shunts: clinical applications as staged or definitive palliation. Ann Thorac Surg. 1989;47:415-420. [Abstract]
3. Baffa JM, Rychik J, Gullquist SD, Barber G, Jacobs ML, Norwood WI, Murphy JD. Outcome following bidirectional cavopulmonary anastomosis prior to modified Fontan procedure. J Am Coll Cardiol. 1991;17:33A. Abstract.
4. Mietus-Synder M, Lang P, Mayer JE, Jones RA, Castaneda AR, Lock JE. Childhood systemic-pulmonary shunts: subsequent suitability for Fontan operation. Circulation. 1987;76(suppl III):III-39-III-44.
5. Coles JG, Kielmanowicz S, Freedom RM, Benson LN, Moes F, Olley PM, Rabinovitch M, Rosenberg H, Sherret H, Rowe RD, McLaughlin PR, Trusler GA, Williams WG. Surgical experience with the modified Fontan procedure. Circulation. 1987;76(suppl III):III-61-III-66.
6. Mainwaring RD, Lamberti JJ, Uzark K. The bidirectional Glenn: palliation of the univentricular heart. In: Karp RB, Laks H, Wechsler AS, eds. Advances in Cardiac Surgery. St Louis, Mo: Mosby-Year Book Inc; 1994;5:115-140.
7. Zellers TM, Driscoll DJ, Humes RA, Feldt RH, Puga FJ, Danielson GK. Glenn shunt: effect on pleural drainage after modified Fontan operation. J Thorac Cardiovasc Surg. 1989;98:725-729. [Abstract]
8. Castaneda AR, William WL. Glenn lecture: from Glenn to Fontana continuing evolution. Circulation. 1992;86(suppl II):II-80-II-84.
9. 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 IV):IV-170-IV-176.
10.
Jonas RA. Indications and timing for the
bi-directional Glenn shunt versus the fenestrated Fontan
circulation. J Thorac Cardiovasc Surg. 1994;108:522-524.
11.
Farrell PE Jr, Chang AC, Murdison KA, Baffa JM, Norwood
WI, Murphy JD. Outcome and assessment after the modified Fontan
procedure for hypoplastic left heart syndrome.
Circulation. 1992;85:116-122.
12. Lamberti JJ, Spicer RL, Waldman JD, Grehl TM, Thomson D, George L, Kirkpatrick SE, Mathewson JW. The bidirectional cavopulmonary artery shunt. J Thorac Cardiovasc Surg. 1990;100:22-30. [Abstract]
13. Douville EC, Sade RM, Fyfe DA. The hemi-Fontan operation in surgery for single ventricle: a preliminary report. Ann Thorac Surg. 1991;51:893-900. [Abstract]
14. Mendelsohn AM, Bove EL, Lupinetti FM, Crowley DC, Beekman RH. Central pulmonary artery growth patterns after the bi-directional Glenn procedure. J Am Coll Cardiol. 1994;107:1284-1290.
15. Nakata S, Yasuharu I, Yoshinori T, Kurosawa H, Tenzuka K, Nakazawa M, Ando M, Takao A. 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;88:610-619. [Abstract]
16. Girod DA, Rice MJ, Mair DD, Julesrud PR, Puga FJ, Danielson GK. Relationship of pulmonary artery size to mortality in patients undergoing the Fontan operation. Circulation. 1985;72(suppl II):II-93-II-96.
17. Bridges ND, Farrell PE Jr, Pigott JD III, Norwood WI, Chin AJ. Pulmonary artery index: a non-predictor of operative survival in patients undergoing modified Fontan repair. Circulation. 1989;80(suppl I):I-216-I-221.
18. Kopf GS, Laks H, Stansel HC, Hellenbrand WE, Kleinman CS, Talner NS. Thirty year follow-up of superior vena cava-pulmonary artery (Glenn) shunts. J Thorac Cardiovasc Surg. 1990;100:662-671. [Abstract]
19. Matsuda H, Kawashima Y, Hirose H, Nakano S, Kishimoto H, Sano T. Evaluation of total cavopulmonary shunt operation for single ventricle with common atrioventricular valve and left isomerism. Am J Cardiol. 1986;58:180-182. [Medline] [Order article via Infotrieve]
20. Moore JW, Kirby WC, Madden WA, Gaither NS. Development of pulmonary arteriovenous malformations after modified Fontan operations. J Thorac Cardiovasc Surg. 1989;98:1045-1050. [Abstract]
21.
Knight WB, Mee RBB. A cure for pulmonary
arteriovenous fistulas? Ann Thorac Surg. 1995;59:999-1001.
22. Mendelsohn AM, Bove EL, Lupinetti FM, Crowley DC, Lloyd TR, Fedderly RT, Beekman RH. Intraoperative and percutaneous stenting of congenital pulmonary artery and vein stenosis. Circulation. 1993;88:210-217.
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