Ann Thorac Surg 2001;71:1347-1349
© 2001 The Society of Thoracic Surgeons
Case report
Pulmonary root translocation for biventricular repair of double-outlet left ventricle
Yoshio Ootaki, MDa,
Masahiro Yamaguchi, MDa,
Yoshihiro Oshima, MDa,
Naoki Yoshimura, MDa,
Shigeteru Oka, MDa
a Department of Cardiothoracic Surgery, Kobe Children’s Hospital, Kobe, Hyogo, Japan
Accepted for publication May 19, 2000.
Address reprint requests to Dr Ootaki, 1-1-1 Takakuradai, Suma-ku, Kobe, Hyogo, 654-0081, Japan
e-mail: y.ootaki{at}nifty.ne.jp
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Abstract
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Double-outlet left ventricle is conventionally repaired with an extracardiac conduit when pulmonary stenosis is present. We report the use of pulmonary root translocation to the right ventricle to construct the posterior wall with autologous tissue and a porcine pericardial monocusp ventricular outflow patch anteriorly for 2 patients with double-outlet left ventricle. This technique allows minimization of pulmonary insufficiency, avoids coronary artery ligation with infundibulotomy, and has a major theoretical advantage for growth potential.
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Introduction
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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 malaligned ventricular septal defect. There is a substantial incidence of tricuspid valve abnormalities and right ventricular hypoplasia in DOLV, similar to the high association of mitral valve abnormalities and hypoplasia of the left ventricle in patients with double-outlet right ventricle. The choice of biventricular repair of DOLV or use of Fontan-type procedures is based on the degree of right ventricular hypoplasia.
When biventricular repair is feasible, the surgical approach depends on the presence of pulmonary stenosis and the relationship of the ventricular septal defect to the great arteries. The intraventricular baffle procedure is required in the presence of a subaortic or subpulmonary ventricular septal defect [1–3]. However, this technique is not suitable for all forms of DOLV, especially those with restrictive interventricular communication or subpulmonary stenosis.
Generally, in the presence of pulmonary stenosis, regardless of severity, external right ventricle–pulmonary artery valved conduits have been used. These cannot grow and are subject to late conduit obstruction [4]. To correct DOLV when pulmonary stenosis is not severe, pulmonary root translocation and commissurotomy can be accomplished, thus minimizing the use of right-sided conduits and reoperation [5–7].
We report two cases of surgical correction of DOLV accompanied by severe pulmonary stenosis. Our objective in translocating the pulmonary artery to the right ventricle was to avoid the use of an extracardiac conduit and to avoid right ventricular dysfunction related to pulmonary insufficiency. Further, the longer the translocated pulmonary artery is, the better we can choose the site of right ventriculotomy that least interferes with the right coronary artery or conus branch. Demographic and diagnostic features are summarized in Table 1. Both patients had severe valvular stenosis, bicuspid pulmonary valves, two normal-sized ventricles, and normal atrioventricular valve.
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Case reports
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Patient 1
Patient 1 was first seen because of cyanosis when he was 25 days old. On the day of admission, he underwent cardiac catheterization and balloon atrioseptostomy. He was relatively well until the age of 8 years, when he started to become increasingly short of breath. At operation, the pulmonary artery and valve exited the left ventricle posteriorly and to the right of the aorta. The pulmonary valve was bicuspid, and the annulus of the valve measured 10 mm in diameter.
Patient 2
Patient 2 was first seen because of cyanosis when she was 3 years old, and she was then relatively well until the age of 6 years. At operation, the pulmonary artery and valve exited the left ventricle posteriorly and to the left of the aorta. The pulmonary valve was bicuspid, and the annulus of the valve measured 8 mm in diameter.
Repair was accomplished using mild hypothermic cardiopulmonary bypass. The pulmonary artery, complete with the stenotic valve, was uprooted from the base of the left ventricle; extreme care was taken to avoid injury to the left anterior descending coronary artery and the left circumflex coronary artery. The main pulmonary artery and its branches were dissected out to each hilum so that the main pulmonary artery could swing rightward in front of the aorta to join the right ventricular outflow tract. The atrial septal defect was closed with a Dacron patch through a right atriotomy in patient 1, and the ventricular septal defect was closed through a right infundibulotomy in both patients. The residual defect from the main pulmonary artery in the left ventricle was directly sutured in patient 1 and was closed with a Dacron patch lined with porcine pericardium in patient 2, with care taken to avoid injury to or kinking of the coronary arteries. The main pulmonary artery and annulus were excised anteriorly, and the posterior rim of the roots was sutured to the cephalic margin of the infundibular incision. A porcine pericardial monocusp ventricular outflow patch (Polystan) was sewn over the pulmonary outflow tract (Fig 1).
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Fig 1. (Patient 1.) (A) The pulmonary artery (PA) and valve exited the left ventricle posteriorly and to the right of the aorta (Ao). Incision for right infundibulotomy (broken line) is shown. (B) The ventricular septal defect is closed with a Dacron patch (DP). The residual defect from the main pulmonary artery in the left ventricle is directly sutured. The main pulmonary artery and annulus are excised anteriorly, and the posterior rim of the root is sutured to the superior aspect of the right infundibulotomy, thereby avoiding the right coronary artery. (C) A porcine pericardial monocusp ventricular outflow patch (Polystan) is sewn over the pulmonary outflow tract.
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No early deaths or complications occurred. Catheterization 4 weeks after the operation showed normal biventricular function and no stenosis or insufficiency of the right ventricular outflow tract except trivial pulmonary insufficiency in patient 2. Both patients were doing well 26 months and 55 months postoperatively and were in New York Heart Association class I.
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Comment
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Double-outlet left ventricle is an anomalous ventriculoarterial connection in which both great arteries arise wholly or in large part from the morphologic left ventricle. If there is no pulmonary stenosis, an intraventricular tunnel repair may be possible to allow the right ventricle to eject into the pulmonary artery [1–3]. Experience with the Ross procedure has shown that the pulmonary root can be excised and relocated without compromising valve integrity [8]. Given the success of this procedure, it was likely that DOLV could be repaired by translocating the pulmonary root from the left ventricle to the right ventricle with similar efficacy. Indeed, three reports [5–7] have described biventricular repair of DOLV without pulmonary stenosis.
When pulmonary stenosis is present, biventricular repair of DOLV has been achieved using right ventricle–main pulmonary artery external conduits regardless of the commitment of the ventricular septal defect to the great arteries [4]. However, external conduits cannot grow and are subject to late conduit obstruction and replacement. Valveless conduits have been placed, and though initially well tolerated, they often result in progressive right ventricular dysfunction [9]. Chiavarelli and colleagues [5] suggested that translocation of the pulmonary artery to correct DOLV is feasible despite coexisting pulmonary stenosis with moderate obstruction across the pulmonary valve because this obstruction will decrease further with time.
In the presence of severe pulmonary stenosis, our modification of pulmonary root translocation with a porcine pericardial monocusp ventricular outflow patch is applicable. With this technique, the pulmonary artery can grow, and the risk of late conduit obstruction may be decreased. Further, we can take longer pulmonary roots than those excised above a stenosed pulmonary valve. A longer pulmonary root is freely available for infundibulotomy, thereby avoiding the right coronary artery or conus branch.
In conclusion, we believe that the present technique allows minimization of the pulmonary insufficiency, avoids coronary artery ligation with infundibulotomy, and has a major theoretical advantage in growth potential. Long-term follow-up is needed to assess the late results of this approach.
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References
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Abstract
Introduction
