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(Circulation. 1995;92:228-232.)
© 1995 American Heart Association, Inc.

Articles

Use of the Bidirectional Glenn Procedure in the Presence of Forward Flow From the Ventricles to the Pulmonary Arteries

Presented at the 67th Scientific Sessions of the American Heart Association, Dallas, Tex, November 1994.

Hideki Uemura, MD; Toshikatsu Yagihara, MD; Yasunaru Kawashima, MD; Kenji Okada, MD; Tetsuro Kamiya, MD; Robert H. Anderson, MD

From the National Cardiovascular Center (H.U., T.Y., Y.K., K.O., T.K.), Suita, Osaka, Japan, and the National Heart and Lung Institute (H.U., R.H.A.), London, UK.

Correspondence to Hideki Uemura, MD, Department of Cardiovascular Surgery, National Cardiovascular Center, 5-7-1 Fujishirodai, Suita, Osaka 565, Japan.

	Abstract

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Background Relative regression of the pulmonary arterial size has been reported after a conventional bidirectional Glenn procedure. Maintaining a supplemental pulmonary flow could be of surgical value unless the option also militates against the efficacy of the partial right heart bypass.

Methods and Results Twenty-seven patients considered unsuitable for a Fontan-type procedure underwent a bidirectional Glenn procedure in the presence of forward flow from the ventricles to the pulmonary arteries, the flow being maintained through the pulmonary trunk in 22 or a systemic-to-pulmonary shunt in 5. There was one surgical death due to atrioventricular valvular regurgitation. Subsequently, 9 patients have successfully undergone a total cavopulmonary connection 2.6±1.9 years after the initial procedure. Preoperative and postoperative catheterizations revealed changes in arterial oxygen saturation (75±11% compared with 83±7%, P<.001) and end-diastolic volumes of the systemic ventricles (from 238±92% to 188±97% of the expected normal volume, P<.01), whereas no difference was detected in the mean cross-sectional area of the right and left pulmonary arteries compared with the expected normal value for the right pulmonary artery (from 76±21% to 81±20%) or in the ventricular ejection fraction (from 53±8% to 50±14%). The relative regression or growth of the pulmonary arterial size was statistically related to the size of the channel for forward flow.

Conclusions Maintenance of forward flow from the ventricle provides a feasible means, when performing a bidirectional Glenn procedure, of protecting against regression of pulmonary arterial size as well as off-loading the ventricles and improving arterial oxygen saturation.

Key Words: Glenn procedure • Fontan procedure • surgery • pulmonary arteries • heart defects, congenital

	Introduction

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In recent years, a bidirectional Glenn procedure^{1 2} has been used increasingly for palliation in patients initially considered unsuitable for a Fontan-type procedure.^{3 4 5 6 7} The procedure provides a means of reducing the volume load on the ventricles as well as of improving the arterial oxygen saturation. On the other hand, the quantity of pulmonary flow produced in this setting is much less than that of either the normal or the Fontan circulation. Development of the pulmonary arterial tree is known to be less than normal when the total amount of pulmonary flow is markedly reduced,^{8 9} and relative regression of the pulmonary arterial size has been reported after a bidirectional Glenn procedure.¹⁰ Persistent pulsatile pulmonary arterial flow, if coupled with the maintenance of an adequate total pulmonary perfusion, may promote growth of the pulmonary arteries. We have pursued, therefore, the option of creating a bidirectional cavopulmonary anastomosis in the presence of forward flow from the ventricles to the pulmonary arteries. This additional source of pulmonary blood perfusion, however, could negate the merits achieved by the conventional bidirectional Glenn procedure. We have analyzed our experience to evaluate this possibility.

	Methods

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Patients
Since 1989, 27 consecutive patients diagnosed at the National Cardiovascular Center, Osaka, Japan, as initially being unsuitable for either biventricular repair or a Fontan-type operation have undergone a bidirectional Glenn procedure while we preserved forward flow from the ventricles to the pulmonary arteries. Their major cardiac malformations are given in Table 1. Primary conditions ruling out a total right heart bypass were markedly impaired ventricular contraction in 10 patients, severe regurgitation of the atrioventricular valves in 9, excessive development of systemic-to-pulmonary collateral vessels likely to produce unfavorably high pulmonary resistances in 4, pulmonary hypertension in 2, moderate pulmonary arterial hypoplasia in 1, and prominent fistulous communications between the right ventricle and the right coronary artery in 1. Secondary conditions were atrioventricular valvular insufficiency in 6 patients, markedly developed systemic-to-pulmonary collaterals in 5, pulmonary hypertension in 5, hypoplastic pulmonary arteries in 5, impaired ventricular function in 4, and supraventricular tachyarrhythmias in 3. Of the 27 patients, 20 had previously undergone palliative operations; these consisted of construction of a systemic-to-pulmonary shunt in 14, banding of the pulmonary trunk in 2, both of these maneuvers in 2, replacement of a common atrioventricular valve in 1, and repair of totally anomalous pulmonary venous connection in 1.

View this table: [in this window] [in a new window]   Table 1. Major Cardiac Malformations

Surgical Methods
The operation was performed at the age of 0.8 to 22.3 years (mean age, 6.2±5.2 years). The calculated body surface area ranged from 0.40 to 1.55 m² (mean, 0.73±0.32 m²). A bidirectional cavopulmonary anastomosis was constructed under cardiopulmonary bypass in 21 patients, and temporary bypass between the superior caval vein and the right atrium was used to achieve this procedure in the other 6 patients. Plasty of the atrioventricular valve and repair of totally anomalous pulmonary venous connection were concomitantly achieved in 12 and 5 patients, respectively. Forward flow from the ventricles to the pulmonary arteries was maintained through the pulmonary trunk in 22 and via a systemic-to-pulmonary shunt in 5, the side of the shunt being on the opposite side of the cavopulmonary anastomosis in 4 and on the same side in 1. Either banding of the pulmonary trunk (in 13 patients) or pulmonary valvotomy (in 2) was added as necessary to regulate the amount of the flow through the pulmonary trunk. The most narrow pathway provided for forward flow to continue from the ventricles to the pulmonary arteries was shown, based on measurements at postoperative angiography, to range from 1.5 to 10 mm (mean, 6.0±2.4 mm) internal diameter. This restriction was defined, on the anteroposterior and the lateral projections during systolic phases, as the smallest diameter at either the subpulmonary, pulmonary annular, or pulmonary truncal levels being produced by native stenosis or adjusted by surgical means. In patients in whom a systemic-to-pulmonary shunt was left open, the previously constructed prosthetic graft was a single-woven Dacron (Golaski) or expanded polytetrafluoroethylene (Gore-Tex) tube 5 mm in diameter.

Postoperative Evaluation
To investigate the efficacy of this modified option, we analyzed data derived from preoperative (27) and postoperative (18) catheterizations in addition to assessing clinical prognoses. The postoperative examinations were carried out at 9.9±7.3 months (range, 1 to 22 months) after the bidirectional Glenn procedure. In the patients undergoing a subsequent total cavopulmonary connection (see later), these postoperative examinations were made before the definitive surgery. The end-diastolic volume of the systemic ventricle was described as a percentage compared with the anticipated normal value calculated from body surface area.¹¹ The pulmonary arterial size was quantified by calculating a pulmonary arterial area index in which the mean cross-sectional area of the right and left pulmonary arteries was compared with the anticipated normal value for the right pulmonary artery.^{12 13}

Some comparable data were available for comparison from 4 patients undergoing bidirectional cavopulmonary anastomosis without construction of additional blood supply to the pulmonary arteries, the operations that were being performed before 1988.

	Results

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There was one surgical death—this patient died on the 25th postoperative day due to supraventricular tachyarrhythmias accompanied by recurrent atrioventricular valvular regurgitation and esophageal bleeding. Late deaths have occurred in 6 additional patients, the major causes being recurrent atrioventricular regurgitation with ventricular dysfunction in 4, progressive obstruction across the anastomosis for the repair of totally anomalous pulmonary venous connection in 1, and pneumonia in 1. In contrast, 9 patients have successfully undergone construction of a total cavopulmonary connection (Table 2).

View this table: [in this window] [in a new window]   Table 2. Outcomes of Patients

Preoperative and postoperative catheterization revealed changes in arterial oxygen saturation (75±11% compared with 83±7%, P<.001 by paired t test) and end-diastolic volume of the systemic ventricles (238±92% compared with 188±97% of the expected normal value, P<.01, paired t test), whereas no difference was detected in the standardized pulmonary size (76±21% and 81±20% for the pulmonary arterial area index) or in ventricular ejection fraction (53±8% and 50±14%) (Fig 1). Relative regression of the pulmonary arterial sizes tends to be minimized when the channel for maintained forward flow is greater (Fig 2). Postoperative pressures within the superior caval vein or the pulmonary arteries ranged from 8 to 18 mm Hg (mean, 11.7±3.3 mm Hg), the values being unrelated to the narrowest internal diameter for forward flow from the ventricles to the pulmonary arteries (Fig 2). No correlation was detected between end-diastolic volumes of the ventricle and the diameter of the channel for forward flow, but recurrent atrioventricular valvular regurgitation appeared to significantly affect the value of end-diastolic volume (Fig 2). There also was no significant difference in either the pattern of the channel for forward flow or the side on which the shunt was left open.

View larger version (33K): [in this window] [in a new window]   Figure 1. Preoperative and postoperative catheterization. End-diastolic volume of the systemic ventricle was described as a percentage compared with the anticipated value calculated from the patient's body surface area.10 The pulmonary arterial area index, which was calculated as the mean cross-sectional area of the right and left pulmonary arteries compared with the anticipated normal value for the right pulmonary artery,12 13 was also described in a percentage. Black dots in the graph for end-diastolic volume represent the presence of atrioventricular valvular regurgitation. Statistical evaluations were done in 18 patients undergoing both preoperative and postoperative catheterizations with a paired t test. Closed triangles represent the values in the 4 patients undergoing bidirectional cavopulmonary anastomosis without additional forward flow. Pre indicates preoperative catheterization; post, postoperative catheterization.

View larger version (18K): [in this window] [in a new window]   Figure 2. Influence of sizes of the channel for forward flow. To describe changes in the pulmonary arterial sizes, the postoperative value for the pulmonary arterial area index was divided by the preoperative value. Index for the size of forward flow was calculated from the cross-sectional area of the channel divided by body surface area. Crossed circle in the graph concerning end-diastolic volume demonstrates the presence of postoperative recurrence of atrioventricular valvular regurgitation. Closed triangles represent the values in the 4 patients undergoing bidirectional cavopulmonary anastomosis without additional forward flow. Pre indicates preoperative; post, postoperative; PA, pulmonary arterial; SCV, superior caval vein.

	Discussion

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Well-controlled supplemental flow to the pulmonary arteries has proved to be an option when a bidirectional Glenn procedure is performed.^{7 14} Regarding hemodynamic physiology, there are differences between patients undergoing this modification and those who have undergone a conventional bidirectional Glenn procedure or a Fontan-type operation, with the quantity of the pulmonary flow and the volume load on the ventricle varying in accordance with the amount of additional blood supplied to the pulmonary arteries (Fig 3). Such additional pulmonary perfusion may have, as was suggested by Triedman et al,¹⁵ either a beneficial influence on pulmonary vascular development or a deleterious impact by imposing a volume load on the ventricles. The additional flow in their patients, however, was via collateral vessels. In our protocols, the partial right heart bypass operation achieved by bidirectional cavopulmonary anastomosis has been regarded as a palliative procedure on the path toward a functionally definitive operation, ie, a total cavopulmonary connection. Excessive perfusion through such collateral vessels can influence surgical outcomes after a Fontan-type operation.^{16 17 18} It seems sensible to us, therefore, to maintain adequate pulmonary vascular development after the bidirectional Glenn procedure with less contribution from systemic-to-pulmonary collateral arteries. Of course, it is controversial whether the size of the proximal pulmonary arteries influences surgical results after a Fontan-type procedure.¹⁹ Because the indexed pulmonary arterial area could represent the status of the pulmonary vascular bed in the absence of any distortion of the proximal pulmonary arterial tree⁹ and because hypoplasia of the pulmonary arteries could be an undesirable risk factor,^{18 19 20} we argued that avoidance of pulmonary arterial regression after the bidirectional Glenn procedure would improve further the outcome after a subsequent Fontan-type operation for the group of high-risk patients in whom functional repair was initially considered inapplicable.

View larger version (40K): [in this window] [in a new window]   Figure 3. Physiological differences between options for right heart bypass operations. The additional forward flow can produce inadequate reduction of the overload placed on the ventricle compared with the circulation after either the conventional bidirectional Glenn procedure or a Fontan-type procedure.

Our study illustrated that the pulmonary arterial area index of our patients, which is a standardized indicator of pulmonary arterial size, did not decrease as a whole; in 12 patients, it increased (Fig 2). If adequate growth of the pulmonary arterial size is to be achieved, a significant channel is needed for forward flow. In addition to the effect of an adequate amount of pulmonary flow, two supplemental contributions can be considered. The first is the presence of pulsatile flow within the pulmonary arteries. It has been suggested that such pulsatile patterns might also be instrumental in promoting the growth of the pulmonary arterial tree. Such pulsatile flow could protect against the development of pulmonary arteriovenous fistulas. As has been discussed, the contribution of pulsatile flow, and even its existence, remains controversial.^{21 22 23} In our series, however, the pulsatile nature of flow was unequivocally demonstrated by angiography and Doppler echocardiography in several patients who had a relatively large channel constructed for forward flow. The second benefit is based on avoidance of the potential deleterious effect on the pulmonary vasculature of exclusion of blood draining from the liver.²⁴ Although further biochemical or pharmacological investigations are obviously necessary on this subject—the still unknown "hepatic factors"—we believe that the additional forward flow also provides a circulation in which part of the hepatic venous effluent joins the pulmonary arterial perfusate. In this respect, it could be noteworthy that so far, no pulmonary arteriovenous fistulas have been recognized in our patients.

In contrast, the surgeon must be aware of potentially deleterious aspects of this option. The forward flow joining the pulmonary perfusion might be the cause of insufficient reduction of the volume overload to the ventricles (Fig 3). This, of course, will depend on the amount of the supplemental flow. When postoperative atrioventricular valvular regurgitation is no more than mild, the greater sizes of the channel are not related to an excessively large end-diastolic volume. We should consider, nevertheless, that postoperative recurrence of atrioventricular valvular regurgitation can be associated with an insufficient reduction of volume overload to the ventricle. The preoperative end-diastolic volume of the ventricle was more than 240% of the expected normal values in 14 patients. Among these 14, concomitant valvular surgery was necessary for treating significant atrioventricular valvular regurgitation in 10 (Fig 1). Postoperative recurrence of regurgitation occurred in 7 of these 10 patients (5 with isomeric right appendages and 2 with usual atrial arrangement). This compounding factor was related to death in either the short or the long term in 5 patients. That is, a finding of more than 240% of end-diastolic volume in patients necessitating surgery to the regurgitant atrioventricular valve was an unequivocal risk factor. In such circumstances, any amount of additional pulmonary flow could become excessive in terms of avoiding recurrent insufficiency and obtaining adequate volume reduction. Much more restricted channels for forward flow may well have been suitable in those patients whose deaths were related to recurrence of regurgitation across the atrioventricular valve. Its significance would be particularly greater when treating patients with a common atrioventricular valve associated with isomeric atrial appendages ("visceral heterotaxy").^{7 25}

Another possible problem associated with the presence of additional flow is the increased postoperative pressure within the pulmonary arteries,¹⁴ this being equal to that within the superior caval vein. (This fact was emphasized by Dr Roger B.B. Mee (Cleveland, Ohio) during discussion at the Cardiology in the Young Symposium held at the Great Ormond Street Hospital for Sick Children, London, UK, in April 1994.) In our series, pressures within the superior caval vein were considered to be within a reasonable range. Furthermore, pressure studies during the operation in several patients revealed no significant differences between superior caval venous pressures with the forward flow open as designed and those with the flow temporarily closed. If patients had critically high pulmonary resistances, however, such an additional amount passing through the optional pathway would have been recognized as excessive as it would have induced the rise in systemic venous pressure in the upper body.

The obvious limitation of the present study is its lack of a control group. We used this option in a consecutive series of patients. Because of this, we cannot conclude whether the additional flow is advantageous for either clinical prognosis or further definitive repair. The indications for this surgical strategy of providing and regulating the forward flow, therefore, have yet to be clarified. In another respect, this also reflects the complicated initial clinical condition, as the patients had significant risk factors that ruled out an immediate definitive repair. The results of the present study showed, as a whole, that the presence of the additional flow prevented regression of pulmonary arterial size as well as off-loading the ventricle and improving arterial oxygen saturation. Excessive additional flow, however, can negate the benefits sought from partial bypass of the right heart, particularly in the setting of severe regurgitation across the atrioventricular valve. If constructed, therefore, the size of the channel for forward flow is crucial. This size must be determined on the basis of its beneficial and deleterious aspects in each individual patient.

	References

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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 Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Uemura, H. Articles by Anderson, R. H. Search for Related Content PubMed Articles by Uemura, H. Articles by Anderson, R. H.