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Ann Thorac Surg 1999;68:976-981
© 1999 The Society of Thoracic Surgeons
a Division of Cardiovascular Surgery, Childrens Memorial Hospital, Chicago, Illinois, USA
Address reprint requests to Dr Mavroudis, Childrens Memorial Hospital, 2300 Childrens Plaza, M/C #22, Chicago, IL 60614
e-mail: c-mavroudis{at}nwu.edu
Presented at the Poster Session of the Thirty-fifth Annual Meeting of The Society of Thoracic Surgeons, San Antonio, TX, Jan 2527, 1999.
| Abstract |
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ventricular repair or in conjunction with other repairs of congenital heart defects. Methods. Between 1992 and 1998, 15 patients (mean age, 8.1 ± 7.9 years) had bidirectional Glenn shunt in association with repair of congenital heart defects. Eighty-seven percent had at least one previous operation. All patients had simultaneous or previous intracardiac repair and had bidirectional Glenn shunt to volume unload the small right ventricle (group A, n = 7), to unload the poorly functioning right ventricle (group B, n = 2), to redirect superior vena cavapulmonary venous atrial connection to treat cyanosis (group C, n = 2), or to unload the pulmonary left ventricle for residual intracavitary hypertension in patients with L-transposition of the great arteries, ventricular septal defect, and pulmonary stenosis (group D, n = 4). Intraoperative hemodynamic assessment was done in 2 patients in group A by selective use of inflow occlusion and flow probes.
Results. All patients survived. Four patients had successful, concurrent arrhythmia circuit cryoablation for Wolf-Parkinson-White syndrome (n = 1) or atrial reentry tachycardia (n = 3). Superior and inferior vena caval flow averaged 36% and 64% of cardiac output, respectively. Postoperative superior vena caval pressure (n = 13) was 13.7 ± 4.0 mm Hg with pulmonary arterial flow pattern contributed by the ventricle in systole (pulsatile) and the superior vena cava in diastole (laminar).
Conclusions. The bidirectional Glenn shunt is an effective adjunct to congenital heart repair to treat pulmonary ventricular pressure-volume problems and anomalous superior vena caval to left atrial connections.
| Introduction |
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Since our previous report on this subject [7], we have done selected intraoperative and postoperative hemodynamic studies, which led us to expanded use of the BDG (1
ventricular repair) in association with a wider spectrum of congenital heart defects. A recent symposium organized by Marcelletti and Iorio and published in this journal [26] comprehensively analyzed the 1
ventricular repair by several anastomists, cardiologists, and surgeons. The purposes of this study were to review our experience and to discuss additional issues of nomenclature, functional results, and expanded indications.
| Material and methods |
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ventricular repair). The average age at operation was 8.1 ± 7.9 years (range, 0.8 to 27.4 years). Eighty-seven percent (13 of 15 patients) had at least one previous operation. Data are reported as mean ± standard deviation.
Operative management and hemodynamic evaluation
All patients had uneventful sternotomy (n = 6) or resternotomy (n = 9) with aortobicaval cardiopulmonary bypass, mild to moderate systemic hypothermia (32°C to 28°C), and cold blood cardioplegia when appropriate. The intracardiac procedures were aimed at closure of septal defects, relief of obstructions, valvular repairs, and pulmonary artery augmentation to achieve complete repair with the exception of small or pressure-overloaded pulmonary ventricles. The BDG was performed to unload the targeted ventricle during the rewarming stage using interrupted polydioxanone (Ethicon, Somerville, NJ) suture for the entire anastomosis.
In 2 patients, hemodynamic evaluation was done using simultaneous pulmonary artery and IVC flow probes (Transonic Corp, Ithaca, NY) and intracavitary pressure measurements (Camino Catheters, San Diego, CA). Inflow occlusion to construct right ventricular function curves relating right atrial pressure to aortic, SVC, and IVC flows was done in 1 patient.
Group descriptions and operative history
We organized this disparate group of patients who had BDG (1
ventricular repair) according to the classification of Van Arsdell and associates [4], which segregated patients into those who had small right ventricles (group A), those who had preoperative right ventricular dysfunction (group B), and those who had BDG to facilitate a biventricular repair (group C). Most of our patients fit the inclusionary criteria for those groups. Van Arsdell and associates also described a group D, which consisted of patients who had BDG to treat postoperative right ventricular dysfunction. We did not have any patients who fit that group. We did, however, do BDG in patients with L-transposition of the great arteries (L-TGA), ventricular septal defect (VSD), and pulmonary stenosis (PS) to unload the pulmonary ventricle for pressure considerations. Those patients comprise group D in our series, which differs from that in the study by Van Arsdell and associates [4].
Group A (n = 7)
The unifying factor in this group of patients is a small right ventricle (52% ± 0.7% of normal; range, 40.0% to 59.5%). Right ventricular volume was calculated by Simpsons rule using hand planimetered biplane angiograms and expressed as percent of normal based on standard nomograms for age, size, and weight [27]. Based on previous reports [57], we determined that the right ventricular size should be between 35% and 65% of predicted normal for optimal results. The rationale for BDG in these patients is to unload the pulmonary ventricular volume to allow optimal physiologic ventricular function in concordance with ventricular size. All 7 patients had previous systemic to pulmonary artery shunts (in addition, 1 patient had a classic right Glenn procedure) for decreased pulmonary blood flow. Subsequent operations were modified to the pathologic anatomy but in all cases included systemic to pulmonary artery shunt takedown and BDG (1
ventricular repair). Two patients had right ventricular outflow tract reconstruction with atrial septal defect (ASD) closure for pulmonary atresia with intact ventricular septum. One patient had VSD and ASD closures in association with systemic to pulmonary artery shunt takedown and pulmonary artery band takedown. One patient had a series of operations resulting in tricuspid valvuloplasty, VSD and ASD closures, and right ventricle to pulmonary artery conduit. One patient had previously undergone an atriopulmonary Fontan procedure, diagnostic reevaluation, and was found to have tricuspid stenosis (not tricuspid atresia), inlet VSD, pulmonary venous baffle obstruction, and atrial reentry tachycardia. The patient had takedown of Fontan, enlargement of atrial pulmonary venous baffle, closure of VSD, right ventricle to pulmonary artery valved conduit, and modified right-sided Maze operation [28, 29] (Fig 1). Two patients with tricuspid atresia type IB had large enough right ventricles (48% and 50% of normal) to have a modified Bjork procedure (ASD closure, VSD closure, right atrial to right ventricular connection) with a no. 25 Carpentier-Edwards porcine valved conduit in 1 patient, and a no. 25 Carpentier-Edwards porcine prosthesis in the other). Both patients had right atrial to right ventricular obstruction based on porcine valved conduit stenosis in 1 and porcine valve calcification in the other. Both patients had subsequent uncomplicated right atrium to right ventricular valve replacement, 1 with a no. 25 Carpentier-Edwards porcine valved conduit and the other with a no. 27 Carpentier-Edwards porcine prosthesis. Both had BDG to complete the 1
ventricular repair. Subsequent valve stenosis in both patients resulted in total cavopulmonary Fontan conversion and modified right-sided Maze procedure in 1 patient and planned conversion in the other.
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ventricular repair) in association with tricuspid annuloplasty (no. 26 Carpentier-Edwards tricuspid ring) and modified right-sided Maze procedure [28, 29] for atrial reentry tachycardia. The other patient, who had a previous classic Glenn shunt for Ebsteins anomaly, small right ventricle (60% of normal), and Wolf-Parkinson-White syndrome, had pulmonary artery recontinuity, tricuspid anulovalvuloplasty, pericardial patch repair of obstructed left pulmonary veins, ASD closure, and right free wall dissection with cryoablation for a manifest accessory connection.
Group C (n = 2)
This group of patients had BDG to correct the cyanosis caused by a left superior vena cava connection to the left atrium. One patient had an associated patent foramen ovale. The other patient had dextrocardia, atrioventricular discordance, ventriculoarterial concordance, bilateral SVCs, incomplete atrioventricular canal, and large muscular VSD. Complete repair in the latter patient required VSD closure, intraatrial Mustard type baffle, atrioventricular valve repair, and BDG. As such, this is not part of a 1
ventricular repair (both of our patients had normal sized right and left ventricles) but an extracavitary caval diversion to avoid the use of complex interatrial baffles that have the attendant risks for atrial arrhythmias, baffle leaks, and baffle obstructions.
Group D (n = 4)
This group is slightly different from the others and is composed of 3 patients with L-TGA, VSD, and PS; and 1 patient with L-TGA and PS. All 4 patients had a normally functioning tricuspid valve with systemic and suprasystemic left ventricular (pulmonary ventricular) pressures and normal sized left ventricles [27]. These patients can be treated by a number of options, including VSD closure, left ventricle to pulmonary artery conduit (not a good option [30]), and combined Mustard-Rastelli operation [15] (obligatory conduit change, potential high incidence of atrial arrhythmias). We chose to avoid ventriculotomies, conduits, and potential atrial arrhythmias by transatrial VSD closure, pulmonary valvotomy, and BDG to unload the left ventricle, not for volume considerations as in group A, but for left (pulmonary) ventricular pressure considerations (Fig 2). We therefore unloaded the pulmonary ventricles in group A for volume considerations, group B for contractility issues, group C to correct cyanotic lesions, and group D for pressure considerations.
| Results |
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ventricular repair to extracardiac total cavopulmonary artery Fontan 6
years postoperatively in a patient with tricuspid atresia type 1B (group A). Two patients are awaiting reoperations for a small baffle leak (group C) and conversion from 1
ventricular repair to extracardiac cavopulmonary artery Fontan in the other patient with tricuspid atresia type 1B (group A). Intraoperative hemodynamic studies in 2 group A patients (Table 1) showed average differential SVC caval (36% of cardiac output) and IVC (64% of cardiac output) flows. Function curves showed the change in cardiac output and SVC and IVC flows relative to changing right atrial pressure in 1 patient (Fig 3).
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For the 4 patients who had an operation for arrhythmia (1 with Wolf-Parkinson-White syndrome and 3 with atrial reentry tachycardia), the mean follow-up time was 2.8 years (range, 1.3 to 6.1 years). Three are free of arrhythmia and receiving no antiarrhythmic medications. One patient is taking sotalol for one episode of recurrent atrial reentry tachycardia.
| Comment |
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ventricular repair [33]. The name, "1
ventricular repair" has become more common, especially because the operation is used mostly for patients with small or poorly functioning pulmonary ventricles. There are instances however, when "BDGS in association with congenital repairs" is more descriptive, especially when used as an adjunct to repair in patients with two ventricles. What this operation will eventually be called is clearly of lesser importance than its possible impact on patients with small right ventricles, poorly functioning right ventricles, inflow cyanotic lesions, such as left SVC to left atrial connection, and pressure-loaded pulmonary ventricles who have less favorable options for treatment.
The BDG has been used most frequently to include the small right ventricle in the pulmonary circulation thereby establishing pulsatile pulmonary artery blood flow and avoiding the laminar flow of a Fontan operation [4, 7, 17, 26]. All of our patients with small (35% to 65% of normal) tripartite right ventricles had excellent long-term outcomes with relatively low SVC pressures and favorable functional status. Our attempts to include 2 patients with type IB tricuspid atresia without tripartite right ventricles in this group by establishing right atrial to right ventricular valved conduits were unsuccessful in the long term because of conduit failure. We agree with other authors who emphasize that a functioning tripartite ventricle is necessary for success of the 1
ventricular repair [16, 17].
Patients with poorly functioning right ventricles can be good candidates for BDG (1
ventricular repair) by volume unloading the right ventricle and thereby shifting the physiologic function curve downward and to the left, which will allow the right ventricle to function more efficiently at a diminished preload capacity. We had 2 patients in this group with Ebstein and Uhl anomalies who responded favorably to this repair. Van Arsdell and associates [4] have used BDGS in this setting in 11 patients, 9 of whom had Ebsteins anomaly and 2 had pulmonary atresia with intact ventricular septum. There were two deaths in their group (11% mortality rate), which emphasizes the degree of ventricular dysfunction and associated conditions in these patients.
Two patients in our series and 4 patients in the series of Van Arsdell and associates [4] had BDG to correct the cyanosis caused by a SVC connection to the left atrium. The BDG was accomplished without SVC hypertension and without the potential risks of atrial arrhythmias and baffle complications that attend complex interatrial baffle reconstructions. As noted by others [4, 33], this strategy can be used to facilitate more complex repairs in patients with borderline pulmonary ventricles.
There are a number of options to treat patients with L-TGA, VSD, and PS. VSD closure and left ventricle to pulmonary artery conduits have been associated with unfavorable results [30]. More avant garde operations, such as the double switch [34] or Mustard-Rastelli [15] operations, are effective in the short term, but future problems relating to conduit changes and arrhythmias are unknown. We decided to approach this clinical problem by minimizing any future obligatory operations and avoiding any atrial suture lines that would contribute to future debilitating arrhythmias. The clinical basis for our surgical scheme is derived from our experience with patients who had a successful atrial baffle operation for dextro (D)-transposition of the great arteries (D-TGA) and residual PS. Those patients had increased left ventricular pressure, left ventricular hypertrophy, and a stabilized interventricular septum, all of which help stabilize the tricuspid valve apparatus and assist right ventricular function. In further support of these findings, some patients with tricuspid regurgitation and right ventricular dysfunction after atrial baffle operations for D-TGA improved with pulmonary artery banding, which is done for induction of left ventricular hypertrophy en route to staged arterial switch conversion [34]. These patients generally had right ventricular dysfunction and tricuspid regurgitation, presumably caused by a shift of the interventricular septum toward the left ventricle (pancaked left ventricle) thereby stretching the tricuspid valve chordae. Pulmonary artery banding in those patients caused a shift of the interventricular septum toward the right ventricle and improved the mechanical configuration and function of the tricuspid valve. The resultant left ventricular hypertrophy is thought to assist right ventricular function thereby improving overall hemodynamic conditions. The same conditions existed in our group D patients who had VSD closure, pulmonary valvulotomy with subpulmonic resection, and BDG to volume unload the left ventricle to decrease pressure. The goal was to shift the intercept of the pressure-volume curve downward and to the left. The end result is a left (pulmonary) ventricle with a higher but not suprasystemic pressure that can stabilize the interventricular septum and assist the right ventricle for sustained ventricular function. The advantages of such a repair are (1) the patient might not need further operations such as conduit changes and atrial baffle revisions, and (2) arrhythmogenic suture lines are avoided. Robicsek and colleagues [21] reported a 27-year follow-up in a patient with L-TGA, VSD, and PS in whom a unilateral cavopulmonary artery anastomosis was done at 4 years of age without any intervening operations. Cardiac catheterization in that patient disclosed the following pressure measurements: aorta (100/50 mm Hg), left ventricular pulmonary ventricle (90/05 mm Hg), right pulmonary artery via cavopulmonary anastomosis (18 mm Hg), and left pulmonary artery via left ventricle (30/10, 20 mm Hg). Oxygen saturation was 92% at rest and 75% at maximum exercise. Whether this patient will benefit from VSD closure and pulmonary reconnection is debatable, but it could increase exercise tolerance without destabilizing the beneficial effects of the primary operation. Clearly, not all patients with L-TGA are candidates for this strategy. Patients with poorly functioning tricuspid valves (eg, Ebsteins anomaly) are probably better treated by a double switch option resulting in left ventricular systemic connection. However, for patients with L-TGA, with or without VSD, and PS with a properly functioning tricuspid valve, the BDG with VSD closure and pulmonary outflow tract enlargement offers an attractive alternative that has the potential advantages of surgical finality and avoidance of troubling arrhythmias.
No patient in our series required BDG takedown for SVC hypertension. Van Arsdell and associates [4] reported shunt takedown in 1 patient for SVC hypertension and atrial septal defect creation in another for high right atrial pressure. These complications reflect a patient population with greater preoperative hemodynamic compromise than our patients. The BDG is an adjunct to repair and will not favorably influence hemodynamic problems relating to the systemic circulation to any great degree.
Our experience with BDG in association with other repairs for congenital heart defects in four disparate groups of patients resulted in no deaths and a low complication rate. BDG can be an important therapeutic adjunct when used selectively to solve pulmonary ventricular pressure-volume problems and anomalous SVC to left atrium connections.
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ventricular repair. Ann Thorac Surg 1998;66:673-677.
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