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Ann Thorac Surg 1995;60:213-216
© 1995 The Society of Thoracic Surgeons

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Current Reviews

Alternatives in Biventricular Repair of Double-Outlet Left Ventricle

Departments of Cardiothoracic-Vascular Surgery and Pediatrics, Loyola University Medical Center, Maywood, Illinois and Heart Institute for Children, Oak Lawn, Illinois

	Abstract

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Wide variation in morphology of double-outlet left ventricle allows numerous surgical alternatives that require sorting out to develop a more organized approach. There is a high association between tricuspid abnormalities and right ventricular hypoplasia with double-outlet left ventricle that calls for either Fontan-type procedure or biventricular repair. With pulmonic stenosis, biventricular repair has been accomplished using right-sided conduits. When pulmonic stenosis is mild or absent, repair techniques without conduits depend on the commitment of the ventricular septal defect (VSD). With subaortic VSD and mild pulmonic valvar stenosis, we successfully performed translocation of the main pulmonary artery and valve to the right ventricle on 2 patients (ages 32 and 8 months). Both patients are doing well 2 years and 1 year postoperatively. Others have successfully connected the right ventricle to the pulmonary artery with intraventricular baffle by enlarging a subaortic VSD or when the VSD is either subpulmonic or doubly committed. With subaortic VSD, although it has not been reported, biventricular repair can also be accomplished using a right ventricle-to-aorta baffle combined by either atrial or arterial switch. We believe that a simplified management plan can be formed in double outlet left ventricle based on the size of the right ventricle, presence of pulmonic stenosis, and commitment of the VSD. Whenever possible, translocation of the main pulmonary artery and valve or intraventricular repair should be accomplished in double-outlet right ventricle minimizing the use of right-sided conduits and reoperation.

	Introduction

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
The wide variation in morphology of double-outlet left ventricle makes its surgical treatment challenging. Bharati and colleagues [1] and VanPraagh and associates [2] have classified double-outlet left ventricle independently, and based on these classifications, surgical techniques were either referred to or suggested. However, these classifications, though comprehensive, appear quite complex for surgeons' practical use.

There is a significant incidence of tricuspid abnormalities and right ventricular hypoplasia in double-outlet left ventricle similar to the high association of mitral valve abnormalities and hypoplasia of the left ventricle with double-outlet right ventricle. Biventricular repair of double-outlet left ventricle or use of Fontan-type procedures will depend on the degree of right ventricular hypoplasia.

When biventricular repair is feasible, the surgical approach depends on the presence of pulmonic stenosis and the relationship of the ventricular septal defect to the great arteries. Generally, in the presence of pulmonic stenosis, regardless of severity, external right ventricular-to-pulmonary artery valve conduits have been used [3–7]. Such conduits, however, will need replacement. Valveless conduits have been used and though initially well-tolerated, they often result in progressive right ventricular dysfunction.

In our desire to perform a potentially curable biventricular repair of double-outlet left ventricle, we uprooted and translocated to the right ventricle the main pulmonary artery and valve. To determine the attractiveness of this repair, we analyzed our experience.

	Material and Methods

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
We performed a biventricular repair using the uprooted main pulmonary artery and valve on 2 patients with double outlet left ventricle on April 20, 1992 and then on April 13, 1994. We reviewed their clinical profiles, surgical technique, and results. We also reviewed the reported classifications and biventricular repair techniques in double outlet left ventricle. Based on this review, we adopted a simplified surgical classification and we discuss here the role of the technique we used in the management scheme for double-outlet left ventricle.

Definition and Classification
The rule used in defining double-outlet right ventricle of one-and-a-half of the great arteries coming from the right ventricle is also applied in double outlet left ventricle [2]. Embryologically, the fault may be related to absorption or failure of development of the subpulmonic conus [2].

Based on the presence of hypoplasia of the right ventricle, double-outlet left ventricle is classified surgically into type I, without hypoplasia (where biventricular repair could be performed) and type II, with hypoplasia (where Fontan-type operations are performed; Table 1). Type I is further subdivided into those with pulmonic stenosis on whom external conduits are generally used and those with absent or resectable pulmonic stenosis, on whom numerous possible techniques of repair could be performed depending on the commitment of the ventricular septal defect to the great arteries.

Patient Profiles
Both patients were male, and they were 32 months (patient 1) and 8 months (patient 2) of age, respectively, at the time of corrective operation. They presented with cyanosis in the newborn period and had different diagnoses. The first patient was thought to have tetralogy of Fallot based on echocardiography and the second patient, transposition of the great arteries with ventricular septal defect and subpulmonic stenosis on cardiac catheterization. Both patients had modified Blalock-Taussig shunts in the newborn period. Patient 1 initially had a right-sided shunt that failed, followed by a left-sided shunt. Patient 2 had a balloon atrial septostomy and right-sided shunt.

In both patients, subsequent cardiac catheterizations established the correct diagnosis of double-outlet left ventricle with subaortic ventricular septal defect and subpulmonic stenosis. The aorta was to the right and posterior. Streaming was noted and was quite significant in Patient 1, who had an intact atrial septum. In this patient, the pulmonary blood flow was increased with the main pulmonary artery pressure of 40 mm Hg and yet significant arterial desaturation was present (78%).

Surgical Technique
Cardiopulmonary bypass was instituted through an ascending aorta cannula and bicaval cannulation through the right atrial wall. Moderate hypothermia (26°C and 28°C, respectively) and antegrade multidose cardioplegia were used.

In each patient there was a short but distinct subpulmonic chamber (Fig 1). The pulmonary valve was bicuspid and thin. Mild supravalvar pulmonic stenosis was also present.

View larger version (8K): [in this window] [in a new window]   Fig 1. . (A) Intraoperative photograph of patient 1 showing the aorta (Ao) to the right of and somewhat posterior to the main pulmonary artery (MPA). (B) The MPA and valve had been uprooted from the left ventricle and the outflow closed with pledgeted sutures (arrows). (C) The MPA with valve (arrows) was anastomosed to the right ventricle (RV) after closure of the ventricular septal defect. The MPA also was opened to perform valvotomy and enlarge the supravalvar area. (D) Polytetrafluoroethylene patch (PP) was used to make a hood proximal to the pulmonary valve (arrows) to complete the anastomosis of the MPA to the RV. Another patch (PP) was used distal to the valve to enlarge the supravalvar area.

	Results

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Their hospital courses were uneventful and both patients were doing well at 2 years and 1 year postoperatively. Cardiac catheterization in patient 1 performed 4 months postoperatively showed a normal-looking right ventricle-to-main pulmonary artery and left ventricle-to-aorta connection. The right ventricle/left ventricle ratio was low (45 mm Hg/90 mm Hg). A small muscular ventricular septal defect that was not closed at the time of corrective operation was present.

	Comment

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
In the presence of pulmonic stenosis, biventricular repair of double-outlet left ventricle has been accomplished with the use of right ventricle-to-main pulmonary external conduits regardless of the commitment of the ventricular septal defect to the great arteries [3–7].

In the absence of pulmonic stenosis or when the pulmonic stenosis could be relieved, numerous possibilities exist in terms of biventricular repair without the use of right-sided conduits. In the presence of subaortic ventricular septal defect, enlargement of the ventricular septal defect toward the pulmonary valve has been accomplished and an intraventricular baffle or ``boomerang patch'' fashioned to connect the right ventricle to the main pulmonary artery [8]. Although the patch has been called a ``boomerang patch'', its configuration is quite different from the boomerang patch described by McGoon [9] in the repair of transposition of the great arteries.

As we and Chiavarelli and colleagues [10] have demonstrated independently, uprooting or translocation of the main pulmonary artery and valve avoiding the use of external conduits can be successfully performed in the presence of subaortic ventricular septal defect and mild or resectable pulmonic stenosis. This technique should be superior to the valveless connection recommended by Lecompte and colleagues [11] in transposition with pulmonic stenosis.

Kreutzer and colleagues [12] have successfully uprooted the anatomic unit consisting of the main pulmonary artery and valve in the performance of right atrial appendage to main pulmonary artery connection in tricuspid atresia repair. This has also been accomplished successfully in the root replacement techniques of the Ross operation [13]. These uprooting procedures, however, have been done and facilitated in patients with normally related great arteries because of the presence of the infundibular chamber.

In certain patients with double-outlet left ventricle, as seen in both of our patients, there is a subpulmonic chamber that facilitates uprooting of the main pulmonary artery and valve. The presence of a subpulmonic stenosis that is more significant than the valvar and supravalvar stenosis allows the construction of the main pulmonary artery and use of a more competent pulmonary valve. Although valvar stenosis was present and commissurotomy had to be performed, good long-term outcome is expected, as seen in other patients with pulmonic valvar stenosis.

Although a PTFE tube graft had to be used in 1 patient because of the presence of the left anterior descending coronary artery across the right ventricle infundibulum, we believe that only one reoperation will be needed. In retrospect, use of a native pericardial tube instead of a PTFE tube graft could have eliminated such reoperation.

In the presence of subaortic ventricular septal defect, Rivera and colleagues [14] suggested repair by baffling of the right ventricle to the aorta, converting the pathophysiology into transposition, and performing an atrial switch procedure (Mustard or Senning). Another possible repair is combining an arterial switch procedure and baffling of the right ventricle to the neo–main pulmonary artery. To our knowledge, these last two procedures have not been performed.

In the presence of subpulmonic ventricular septal defect, Sakakibara and associates [15] have performed biventricular repair successfully by simply baffling the right ventricle to the main pulmonary artery. In the presence of doubly committed ventricular septal defect, repair has been accomplished using a ``boomerang patch'' [14].

In the early management of double-outlet left ventricle, with subaortic ventricular septal defect, balloon atrial septostomy should be done because of presence of unfavorable mixing, as seen in our first patient. In retrospect, the systemic pulmonary artery shunts probably could have been avoided had atrial septostomy been undertaken in this patient.

	Footnotes

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Address reprint requests to Dr DeLeon, Loyola University Medical Center, 2160 South First Ave, Maywood, IL 60153.

	References

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 

1. Bharati 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]
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8. Murphy DA, Gillis DA, Sridhara KS. Intraoperative repair of double outlet left ventricle. Ann Thorac Surg 1981;31:364–9.[Abstract]
9. McGoon DC. Intraventricular correction of transposition of the great arteries. J Thorac Cardiovasc Surg 1972;64:430–3.[Medline]
10. 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]
11. Lecompte Y, Neveux JY, Leca F, et al. Reconstruction of the pulmonary outflow tract without prosthetic conduit. J Thorac Cardiovasc Surg 1982;84:727–33.[Abstract]
12. Kreutzer G, Galindez E, Bono H, dePalma C, Laura JP. An operation for the correction of tricuspid atresia. J Thorac Cardiovasc Surg 1973;66:613–21.[Medline]
13. Elkins RC. Pulmonary autograft—the optimal substitute for the aortic valve. N Engl J Med 1994;330:59–60.[Free Full Text]
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15. Sakahibara S, Takao A, Arai T, Hashimoto A, Nogi M. Both great vessels arising from the left ventricle. Bull Heart Inst Japan 1967;11:66–86.

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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): Serafin Y. DeLeon Michel N. Ilbawi Roque Pifarré Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by DeLeon, S. Y. Articles by Pifarré, R. Search for Related Content PubMed PubMed Citation Articles by DeLeon, S. Y. Articles by Pifarré, R.