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J Thorac Cardiovasc Surg 1997;114:501-503
© 1997 Mosby, Inc.

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PULMONARY ROOT TRANSLOCATION FOR BIVENTRICULAR REPAIR OF DOUBLE-OUTLET LEFT VENTRICLE WITH ABSENT SUBPULMONIC CONUS

San Francisco, Calif.

From the Division of Cardiothoracic Surgery, University of California, San Francisco, San Francisco, Calif.

Received for publication Jan. 23, 1997; accepted for publication March 4, 1997. Address for reprints: V. Mohan Reddy, MD, Division of Cardiothoracic Surgery, 505 Parnassus Ave., M593, San Francisco, CA 94143-0118.

Double-outlet left ventricle (DOLV) is a rare form of malpositioned great arteries in which the aorta and the main pulmonary artery arise from the left ventricle in the presence of a malalignment ventricular septal defect. DOLV occurs as a spectrum and has been found with a variety of associated lesions, most commonly pulmonic or subpulmonic stenosis (or both), tricuspid value anomalies, and hypoplasia of the right ventricle. ¹ Depending on the size and function of the right ventricle and tricuspid valve, along with the size and position of the ventricular septal defect, DOLV is usually amenable to biventricular repair. Traditionally, this has been accomplished by either an intraventricular beffle procedure or ventricular septal defect closure with conduit reconstruction of the right ventricular outflow tract (Rastelli repair). ^2,3 The former technique is not suitable for all forms of DOLV, especially those with a restrictive intraventricular communication or subpulmonic stenosis. The Rastelli approach is suboptimal, especially in children, because they will almost inevitably outgrow their conduit and require conduit replacement. ⁴ In addition, valve function deteriorates in valved tissue conduits used in the pulmonary circulation, and the right ventricle does not always tolerate chronic pulmonary insufficiency well. ⁵ Experience with the Ross procedure has shown that the pulmonary root can be excised and relocated without compromising valve integrity. ⁶ Given the success of this procedure, it is likely that DOLV can be repaired by translocating the pulmonary root from the left ventricle to the right ventricle with similar efficacy. In deed, two reports (a total of three patients) have described biventricular repair of DOLV with subpulmonic or bilateral conus by this technique. ^3,7

Since 1993, we have performed two-ventricle repair of DOLV in three patients. Demographic and diagnostic features are summarized in Table I.All patients had pulmonic or subpulmonic stenosis (or both), two normal-sized ventricles, and normal atrioventricular valve morphology and function. In the first of these patients (patient 1), who was referred for surgical treatment at 9 days of age with moderate-severe valvular/annular stenosis, the malalignment septal defect was closed to the aorta, the native pulmonary artery was oversewn, and a 10 mm pulmonary allograft conduit was used to establish right ventricle-pulmonary artery continuity. The other two patients, both of whom had no subpulmonic conus, underwent translocation of the pulmonary valve and artery from the left ventricle to the right ventricle (Fig. 1). Patient 2 was referred to us at 24 years of age after having undergone two previous systemic–pulmonary artery shunts and two Rastelli procedures, with both great arteries committed to the left ventricle and conduit pulmonary outflow tract reconstruction (4 and 6 years previously, with the second conduit placed in parallel with the first). Patient 3 was 7.6 years old at the time of the operation and had also undergone four previous procedures, including three systemic-pulmonary artery shunts and, 4 years earlier, a bidirectional cavopulmonary shunt. The pulmonary root was translocated and the bidirectional cavopulmonary shunt was left in place.

View larger version (229K): [in this window] [in a new window]   Fig. 1. A, The pulmonary root (PT), positioned to the left and slightly posterior to the aorta (Ao), is being excised. Through a right ventriculotomy and the ventricular septal defect (VSD), the aortic (AV) and mitral (MV) valves can be visualized in the left ventricle. B, Polytetrafluoroethylene patches (PP) have been used to close the ventricular septal defect and the defect in the left ventricle from excision of the pulmonary root. The posterior rim of the root (PT) is sutured to the superior aspect of the right ventriculotomy. C, The procedure is completed by creating a hood (PH) across the anterior aspect of the anastomosis.

Discussion.

DOLV has been shown to occur with all forms of conal arrangement: subpulmonic, subaortic, bilaterally present, and bilaterally absent. One of the ways in which our two patients who underwent pulmonary root translocation differ from previously reported cases is that a subpulmonic conus was absent in both, whereas a subpulmonic or bilateral conus has been present in all three of the previous patients. ^3,7 Although the subpulmonic conus may be shorter than usual in DOLV, pulmonary valve excision in this setting is technically similar to the approach used in the Ross procedure. Absence of a subpulmonic conus makes for a more difficult procedure than aortic valve replacement with a pulmonary autograft, primarily because it necessitates careful dissection of the deeply set pulmonary valve from the atriventricular valve(s) with which it is in continuity.

Although results of right ventricular outflow tract conduit reconstruction in DOLV have been favorable, ² we believe that pulmonary root translocation from the left ventricle to the right ventricle is a preferable approach. The primary advantage of this technique is that native tissue is used for right ventricular outflow tract reconstruction. On the basis of outcomes after pulmonary autograft aortic valve replacement, ⁶ translocating the native root from the left ventricle to the right ventricle will most likely translate into growth potential and preserved valve function. These factors will contribute to a decreased likehood of reoperation related to the right ventricular outflow tract and right ventricular dysfunction related to pulmonary insufficiency. ^4,5 In cases of mild pulmonary valve/anulus hypoplasia, such as in patient 2 in the present series, relief from obstruction can be achieved with a limited transanular patch or with a supplementary bidirectional cavopulmonary shunt to unload the right ventricle. When more moderate pulmonary stenosis is present,pulmonary root translocation may still be the optimal approach if the outflow obstruction can be eliminated by placing a bidirectional cavopulmonary shunt with or without a limited transanular patch. One of the unique aspects of our two cases of pulmonary root translocation for DOLV is that additional means were taken to relieve pulmonary outflow obstruction in both patients, including a limited transanular patch in one and maintenance of a bidirectional cavopulmonary shunt in the other. These modifications, neither of which has been previously described, demonstrate that pulmonary root translocation can be undertaken in patients with pulmonary outflow obstruction or a right heart that may be incapable of supporting an entire cardiac output. However, in patients with severe outflow tract obstruction, such as our patient 1, who underwent surgery at 9 days of age, a standard Rastelli-type procedure may still be the best option.

Regarding the timing of pulmonary root translocation for DOLV the best option is probably to perform primary repairing infancy, as has been the case in two of the previously reported cases. ^3,7 However, as we have demonstrated in this report, pulmonary root translocation may also be performed successfully in patients who have undergone multiple previous operations, including Rastelli repair, in which case the pulmonary root may be translocated to the previous conduit anastomosis site.

References

1. Van Praagh R, Weinberg PM, Srebro JP. Double-outlet left ventricle. In: Adams FH, Emmanouilides GC, Riemenschneider TA, editors. Heart disease in infants, children and adolescents. 4th ed. Baltimore: Williams & Wilkins; 1989. p. 461-85.
   
2. Bharathi S, Lev M, Stewart R, McAllister HA, Kirklin JW. The morphologic spectrum of double outlet left ventricle and its surgical significance. Circulation 1978;58:558-65. [Free Full Text]
   
3. DeLeon SY, OW EP, Chiemmongkoltip P, Vitullo DA, Quinones JA, Fisher EA, et al. Alternatives in biventricular repair of double-outlet left ventricle. Ann Thorac Surg 1995;60:213-6. [Abstract/Free Full Text]
   
4. Reddy VM, Rajasinghe HA, McElhinney DB, Hanley FL. Performance of right ventricle to pulmonary artery conduits after repair of truncus arteriosus: a comparison of Dacron housed porcine valves and cryopreserved allografts. Semin Thorac Cardiovasc Surg 1995;7:133-8. [Medline]
   
5. Ilbawi MN, Idriss FS, DeLeon SY, Muster AJ, Gidding SS, Berry TE, et al. Factors that exaggerate the deleterious effects of pulmonary insufficiency on the right ventricle after tetralogy repair. J Thorac Cardiovasc Surg 1987;93:36-44. [Abstract]
   
6. Reddy VM, Rajasinghe HA,McElhinney DB, van Son JAM, Black MD, Silverman NH, et al. Extending the limits of the Ross procedure. Ann Thorac Surg 1995;60:S600-3.
   
7. Chiavarelli M, Boucek MM, Bailey LL. Arterial correction of double outlet left ventricle by pulmonary artery translocation. Ann Thorac Surg 1992;53:1098-100.[Abstract]
   

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