Ann Thorac Surg 2003;75:138-142
© 2003 The Society of Thoracic Surgeons
Original article: cardiovascular
Modified Rastelli procedure for double outlet right ventricle with left-malposition of the great arteries: report of 9 cases
Qingyu Wu, MDa,
Qibin Yu, MDa,
Xiubin Yang, MDa*
a Cardiac Surgery, Cardiovascular Institute and Fu Wai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China
Accepted for publication August 12, 2002.
* Address reprint requests to Dr Yang, Department of Cardiac Surgery, Cardiovascular Institute and Fu Wai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing 100037, China.
e-mail: xiubinyang{at}yahoo.com
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Abstract
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BACKGROUND: Double outlet right ventricle with L-malposition of the great arteries is a rare type of double outlet right ventricle. This article reviews our experience in treating this disease surgically.
METHODS: Between September 1995 and May 1999, 9 patients with double outlet right ventricle [S, D, L] underwent modified Rastelli procedure at Fu Wai Hospital. In all patients, the two great arteries originated completely from the right ventricle. The malformation was associated with subaortic conus and L-transposition of the great arteries. The ventricular septal defect (VSD) was subpulmonary in 3 patients, and non-committed in 6. All but one patient had either pulmonary valvular stenosis or subpulmonary stenosis. No patient had any type of palliative operation before. A right ventriculotomy was made to repair the VSD with a Dacron or vascular prosthesitic patch, and an intraventricular tunnel was made between the left ventricle and the aorta. The main pulmonary artery was divided and the proximal end was closed. A homograft conduit was implanted between the inlet of the right ventricle and the main pulmonary artery. In the Rastelli procedure, the conduit is usually positioned between the right ventricular outflow tract and the pulmonary artery.
RESULTS: All patients survived and recovered uneventfully. Echocardiography demonstrated that all intraventricular tunnels and valved conduits were functioning well. The results were satisfactory.
CONCLUSIONS: Modified Rastelli procedure is an optimal method for surgically treating double outlet right ventricle with left-malposition of the great arteries. It can completely correct the right ventricular outflow tract stenosis, and right to left shunt, and avoid injuring the right coronary artery.
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Introduction
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Double outlet right ventricle (DORV) with left-malposition of the great arteries is a rare type of congenital heart disease [1]. From September 1995 to May 1999, nine DORV patients with L-transposition of the great arteries were completely corrected using modified Rastelli procedure at Fu Wai Hospital. The results were satisfactory.
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Patients and methods
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Patient population
There were 6 male and 3 female patients with ages ranging from 2 to 21 years of age (mean, 10 years old). Eight of 9 patients had cyanosis of different degrees. The chest roentgenogram revealed 2 patients with increased pulmonary vascularity, and the other 7 with reduced pulmonary vascularity before the operation. In 8 of 9 patients, there existed pulmonary valvular or subvalvular stenosis of different degrees, and the aorta was leftward and anterior to the pulmonary artery. All patients had DORV [S, D, L], and concordant atrioventricular connection. The ventricular septal defect was subpulmonary in 3 patients and peri-membranous and non-committed in 6; DORV was associated with left superior venae cavae in 2 patients, with the left anterior descending branch originating from the right coronary artery in 2 patients, with atrial septal defect and patent ductus arteriosus (PDA) in 1 patient, and with Ebstein anomaly in 1 patient.
In all patients, echocardiogram was used for making the diagnosis. Seven patients had cardiac catheterization and angiocardiography (Fig 1).
No patients had any type of palliative operation before.
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Fig 1. Selective right ventriculogram. (A) Posteroanterior view. (B) Lateral view. White arrows indicate the anterior aorta and aortic valve and black arrows indicate the pulmonary artery and pulmonary valve.
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Operative findings and technique
The operation was performed using hypothermic cardiopulmonary bypass. A median sternotomy was made, and cardiopulmonary bypass was established through cannulation in the ascending aorta and both venae cavaes. In the patient with PDA, the PDA was dissected and ligated before cross-clamping the ascending aorta.
The main operative findings were as follows: in all patients, both the great arteries entirely originated from the right ventricle; there was no fibrous continuity between the mitral and aortic valves; the aorta was leftward and anterior to the pulmonary artery (Fig 2);
the subpulmonary conus was absent or poorly developed; the right atrium was usually small, and the right ventricle was enlarged and hypertrophied; the atrioventricular connection was concordant. Eight of 9 patients had pulmonary valvular or subpulmonary stenosis (Fig 3).
The VSD was non-committed in 6 patients, and subpulmonary in 3 patients.
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Fig 2. View of the preoperative heart. The aorta was leftward and anterior to the pulmonary artery, and entirely originated from the right ventricle. (Ao = aorta; RA = right atrium;RV = right ventricle.)
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Fig 3. View of the intraoperative heart. The aorta was leftward and anterior, while the pulmonary artery was rightward and posterior; there existed pulmonary valve stenosis. (Ao = aorta; PVO = pulmonary valve orifice; RV = right ventricle.)
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After cross-clamping the ascending aorta, the right atrium was incised to explore the size and location of the VSD. In 3 patients, the VSD was small so as to be enlarged by 1–2 cm beneath the aorta. A right ventriculotomy was made to repair the VSD with a tailored Dacron or vascular prosthesis patch (Fig 4).
Three to four interrupted mattress stitches were made in the base of the septal leaflet of the tricuspid valve. The remaining VSD’s margin was sutured with a 4/0 or 5/0 prolene running suture. An intraventricular tunnel was constructed to direct the blood from the left ventricle to the aorta. Another right ventriculotomy was made rightward to the previous incision, which was longitudinally 10-mm away from the right coronary artery and the right atrioventricular groove. The length of the incision depended on the patient’s body weight. The main pulmonary artery was divided at the supravalvular level, the proximal end was closed, and the distal end was connected with a valved homograft pulmonary artery in 5 patients, and with a valved homograft aorta in the other 4 patients. The other side of the homograft conduit was anastomosed directly with the second right ventricular incision in 2 patients, and was connected with the incision by an artificial graft in 7. A conduit was implanted between the inlet of the right ventricle and the main pulmonary artery. All the right ventricular anastomotic stomas were beneath the annulus of the tricuspid valve in the right ventricle (Fig 5).
So the conduit was located on the right side of the heart.
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Fig 4. View of the intraoperative heart. The VSD can be well exposed through a subaortic right ventriculotomy. (RVI = right ventricular incision; VSD = ventricular septal defect.)
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Fig 5. View of the postoperative heart. The external conduit was lying on the right side of the heart and the right ventricular anastomotic stoma was located beneath the tricuspid annulus in the inlet of the RV. (Ao = aorta; EC = external conduit; RV = right ventricle.)
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In the patient associated with Ebstein anomaly, the downward displaced posterior leaflet of the tricuspid vavle was cut off from the annulus and sutured to the normal position. The atrial septal defect was repaired simultaneously.
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Results
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All patients survived and recovered well uneventfully. The gradients between the left ventricle and the aorta, and between the right ventricle and the pulmonary artery, were less than 25 mmHg in all patients, which was measured immediately after repairs. The average postoperative hospitalization time was 23 days.
The postoperative echocardiogram demonstrated that the intraventricular tunnel and the external conduit functioned well in all patients (Fig 6),
and indicated no murmur or only grade 1/6 murmur.
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Fig 6. View of the postoperative echocardiogram. (A) Illustration that the intraventricular tunnel was clear. (B) Illustration that the external conduit was clear. (AO = aorta; LA = left atrium; LVOT = outflow tract of left ventricle; RA= right atrium;RVOT = outflow tract of right ventricle.)
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Comment
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Double outlet right ventricle with left-malposition of the great arteries is a rare type of DORV [1]. Anatomically, both great arteries originated entirely from the right ventricle, and the VSD was the only outlet of the left ventricle; there was no fibrous continuity between mitral and aortic valves; the aorta was leftward and anterior, and directly anterior to the pulmonary artery; a conus existed beneath the aorta; the right ventricular outflow tract was extremely hypoplastic or even absent, the pulmonary artery originated from the right ventricle behind the aorta; the right atrium was usually small; and the right ventricle was enlarged and hypertrophied. The subpulmonary conus was extremely poorly developed or even absent, and the course of the coronary artery changed correspondingly, because the aorta was leftward and anterior to the pulmonary artery. The right coronary artery ran across the right atrioventricular groove, and the left anterior descending branch might originate from the right coronary artery. The aorta lying anteriorly to the pulmonary artery caused the VSD location to be remote from the aortic outlet. In our series, six VSDs were non-committed, and three were subpulmonary. In a literature review, the VSD was subaortic in DORV with left-malposition of the great arteries, and the right ventricular outflow tract developed, so it could be enlarged [2]. But in our series, 6 VSDs were remote from the aorta, and usually there was no outflow tract in the right ventricle, because the pulmonary artery was stenotic just behind the aorta and opening directly in the right ventricle. Right ventricular angiocardiography could provide accurate diagnosis of DORV with left-malposition of the great arteries. It could also indicate the origination and position of great arteries as well as the size and location of a VSD.
Because DORV [S, D, L] with L-position of the great arteries is anatomically peculiar, the operative maneuver is different from other types of double outlet right ventricle. The aorta was anterior to the pulmonary artery, the right coronary artery ran across the right atrioventricular groove, and the VSD was subpulmonary or not-committed. So if an interventricular tunnel was constructed between the left ventricle and the aorta, the obstruction of the right ventricular outflow tract was inevitable afterwards. Therefore, an incision had to be made beneath the tricuspid annulus in the inlet of the right ventricle, and then was anastomosed with the proximal of the conduit. In order to avoid injuring the large branches of the right coronary artery and the chordal structure of the tricuspid valve, first we found a place near the right atrioventricular groove where no large vessels crossed on the surface of the right ventricle, and then we observed the intraventricular structure through the orifice of the tricuspid valve with a right-angle clamp. Thus we could also keep the pulmonary outflow tract patent. Another incision beneath the aorta might be needed for exposure and repair of VSD.
In a literature review, DORV with L-position of the great arteries was usually corrected by constructing an intraventricular tunnel between the left ventricle and the aorta, removing the subpulmonary conal musculature, and enlarging the right ventricular outflow tract with a patch [3, 4]. This was done because the VSD was subaortic and the pulmonary artery was more rightward and anterior than that of the patients in our series. There was still a muscular pulmonary cone beneath the pulmonary annulus, and a space for releasing stenotic RVOT. So the intraventricular tunnel didn’t cause the obstruction of the right ventricular outflow tract [5]. When no muscular cone presents, and the stenotic pulmonary annulus is posterior to the aorta and between the mitral and tricuspid annuli, the abnormality can not be corrected by enlarging the right ventricular outflow tract with a patch, because RVOT doesn’t really exist. Switch procedure was considered optimal for patients with anteroposterior great arteries and subpulmonary VSD [6–8]. But we didn’t perform Switch procedure in our series, because the pulmonary valve was abnormal, and the pulmonary annulus was hypoplastic and opening totally in the right ventricle. It was not Taussig-Bing abnormality.
Modified Rastelli procedure could completely correct the pulmonary valvular or subvalvular stenosis, and avoid obstruction of the right ventricular outflow tract caused by the intraventricular tunnel. It differed from the traditional method, because all the right ventricular anastomotic stomas of the conduit were located beneath the tricuspid annulus in the inlet of the right ventricle. To our knowledge, this procedure has not been performed on patients with DORV and left-malposition of the great arteries previously. The incision didn’t affect the blood flow of the right ventricle, and did no obvious harm to the right ventricle function. The early operative results were satisfactory.
The key to this procedure is to ensure a clear intraventricular tunnel and external conduit without residual shunt postoperatively. More importantly, the conduit should be placed in a proper position, so as to avoid twisting or compression, which could lead to right heart failure.
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References
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- Stewart S. Double-outlet right ventricle: a collective review with a surgical viewpoint. J Thorac Cardiovasc Surg 1976;71:355-365.[Abstract]
- Van Praagh R., Perez-Trevino C., Reynolds J.L., et al. Double outlet right ventricle [S.D.L] with subaortic ventricular septal defect and pulmonary stenosis. Am J Cardiol 1975;35:42-53.[Medline]
- Lincoln C. Total correction of D-loop double-outlet right ventricle with bilateral conus, L-transposition, and pulmonaic stenosis. J Thorac Cardiovasc Surg 1972;64:435-440.[Medline]
- Yamaguchi M., Horikoshi K., Toriyama A., et al. Successful repair of double outlet right ventricle with bilateral conus, l-transposition of great arteries [S.D.L], and subpulmonary ventricular septal defect. J Thorac Cardiovasc Surg 1976;71:366-370.[Abstract]
- de Oliveira S.A., Atik E., Fibho G.P., Galiano N., Macruz R., Zerbini E.J. Successful correction of double outlet right ventricle with a ventricular D-loop and l-malposition of the great arteries, bilateral conus, pulmonary stenosis and subaortic ventricular septal defect. Scand J Thorac Cardiovasc Surg 1976;10:209-213.[Medline]
- Mavroudis C., Backer C.L., Muster A.J., Rocchini A.P., Rees A.H., Gevitz M. Taussig-Bing anomaly: arterial switch versus Kawashima intraventricular repair. Ann Thorac Surg 1996;61:1330-1338.[Abstract/Free Full Text]
- Takeuchi K., McGowan F.X., Jr, Moran A.M., et al. Surgical outcome of double-outlet right ventricle with subpulmonary VSD. Ann Thorac Surg 2001;71:49-52.[Abstract/Free Full Text]
- Kawahira Y., Yagihara T., Uemura I., et al. Ventricular outflow tracts after Kawashima intraventricular rerouting for double outlet right ventricle with subpulmonary ventricular septal defect. Eur J Cardiothorac Surg 1999;16:26-31.[Abstract/Free Full Text]
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Abstract
Introduction
