Ann Thorac Surg 2001;72:2119-2121
© 2001 The Society of Thoracic Surgeons
Case report
Double switch operation for superior-inferior ventricles
Narutoshi Hibino, MD*a,
Yasuharu Imai, MDa,
Mitsuru Aoki, MDa,
Toshiharu Shin'oka, MDa,
Takeshi Hiramatsu, MDa
a Department of Pediatric Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women’s Medical College, Tokyo, Japan
Accepted for publication February 3, 2001.
* Address reprint requests to Dr Hibino, Department of Pediatric Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women’s Medical College, 8-1 Kawada-cho Shinjuku-ku, Tokyo, 162-8666 Japan
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Abstract
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Superior-inferior ventricles are a rare cardiac malformation characterized by the two ventricles lying one above the other instead of side by side. Consequently, the interventricular septum that separates such ventricles is horizontal, and anomalies of the atrioventricular valves and the ventriculoarterial relations are almost always present. This complex anomaly is difficult to manage with an operation, so few cases have been reported. We describe a successful experience in which we performed a double switch operation, consisting of the Senning and Jatene procedures, for this rare malformation accompanied by double-outlet right ventricle {S,L,L}. This is the first report we have been able to locate of a double switch operation for superior-inferior ventricles.
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Introduction
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Superior-inferior ventricles are a rare anomaly characterized by a horizontal ventricular septum with large defect and a hypoplastic right ventricular sinus localized anterosuperiorly to the left ventricle. The results of this complex lesion have been poor. We report a successful experience for the repair of such a malformation using double switch operation.
The patient was a 14-year-old boy who was born with double-outlet right ventricle {S,L,L}, superior-inferior heart, ventricular septal defect (VSD), and coarctation of the aorta. At 3 months of age, he underwent subclavian flap and pulmonary artery banding for coarctation of the aorta. At 13 years of age, cardiac catheterization revealed a pulmonary vascular resistance (Rpu) of 3.7 w.u:m2, and transvenous atrial septostomy was performed. Medication with prostacycline was commenced. At 14 years of age, repeated cardiac catheterization reproduced the above diagnosis, as well as tricuspid regurgitation (2 of 4), Rpu of 2.7, flow ratio of pulmonary blood flow/systemic blood flow (Qp/Qs) of 1.2, right ventricular end diastolic volume of 72% with an ejection fraction of 55%, and left ventricular end diastolic volume of 113% with an ejection fraction of 59% (Fig 1). Because tricuspid regurgitation was worsening and Rpu was slightly high, we decided to perform a double switch operation consisting of the Senning and Jatene procedures, despite moderately low right-ventricular volume.
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Fig 1. Preoperative angiograms (lateral projection). (A) Injection into the right ventricle (RV). (B) Injection into the left ventricle (LV). These figures show both great arteries (Ao = aorta; PA = pulmonary artery) and the right ventricle through the ventricular septal defect (arrow) in the horizontal septum.
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A median sternotomy was performed under general anesthesia. After dissection of the great arteries and division of the previously ligated ductus, pump perfusion was established through the ascending aortic and bicaval cannulation. The main pulmonary artery was divided at the banded site. During cold fibrillation, the right atrium was opened and the Senning procedure was performed. The tricuspid valve showed prolapse of the anterior leaflet, and a Reed’s annuloplasty was performed. After cardiac arrest was induced, the aorta was divided and both coronary arteries were removed. The VSD extended toward the great arteries, with the tricuspid chordae inserted into the short conus septum. These chordae were divided and later reimplanted onto the patch. The aortic and pulmonary valves were at the same levels, and bilateral conus were present. Interventricular rerouting was performed with a composite patch of equine xenopericardium and Dacron velour (C. R. Bard, Inc, Haverhill, MA). Starting from the pulmonary artery, the patch was sutured from the subpulmonary conus, running around the posterior ridge of the pulmonary artery and down to the posterior margin of the VSD, along the ventriculoinfundibular fold. Next, from the aorta, the patch closure was completed by suturing from the anterior margin of the VSD up to the conus septum in a spiral manner. Then both coronary arteries were reimplanted into the main pulmonary artery sinuses. After the French maneuver, the arterial switch was completed. Weaning from cardiopulmonary bypass was uneventful (Fig 2).
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Fig 2. (A) Anteroposterior view of the cardiac anatomy. The horizontally oriented ventricular septum separates a moderate hypoplastic superior right ventricle (RV) and a well-developed inferior left ventricle (LV). There is situs solitus with atrioventricular discordance, ventricular L-loop, and L-malposition of the great arteries {S,L,L}. Both great arteries arise from the RV (double outlet right ventricle). The solid lines indicate the direction of flow from ventricles to arteries. (B) The ventricular septal defect (VSD) was closed with a spiral patch, as indicated by the heavy black line. A double switch operation was performed with the Senning and Jatene procedures. The solid line shows the direction of flow from ventricles to arteries. (Ao = aorta; PA = pulmonary artery; RA = right atrium.)
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The postoperative course was uneventful. After 4 weeks the patient was discharged from the hospital. Postoperative echocardiography showed no VSD leakage, no pressure gradient between the pulmonary vein and left atrium, and good cardiac function (Fig 3).
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Fig 3. Postoperative echocardiography. No pressure gradient was found between the pulmonary vein (PV) and left atrium (LA); this is the new connection created by the Senning procedure. There is no ventricular septal defect leakage. (LV = left ventricle; MV = mitral valve; RA = right atrium; RV = right ventricle; RVOT = right ventricle outlet tract; TV = tricuspid valve.)
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Comment
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The superior-inferior ventricles, as named by Van Praagh and colleagues [1], consist of a right ventricle located superior and anterior to the left ventricle, with the ventricular septum in a horizontal plane. Transposition or malposition of the great arteries is usually present, and the subpulmonary conus is often restrictive [2].
From a surgical perspective, two points are particularly important and should be evaluated preoperatively. The first is the degree of underdevelopment of the right ventricle and the size of the tricuspid valve. Although the infundibulum of the right ventricle is well developed, the right ventricular sinus most often shows a variable degree of underdevelopment with marked hypertrophy of its free wall [3]. The size of the tricuspid valve is in accordance with the size of the right ventricular sinus. If a small left ventricle is found and there is no elevation of Rpu, a Fontan type repair would be more suitable, whereas a moderate degree of hypodevelopment of the right ventricle may be handled by biventricular repair [3]. In our case, we were also able to perform the double switch operation for biventricular repair despite a moderately hypoplastic right ventricle. The double switch operation has the advantage of creating a nearly normal atrioventricular/ventriculoarterial relationship, in which the anatomical left ventricle serves as a systemic ventricle, thus preventing the long-term deterioration of systemic right ventricular function that usually follows a conventional repair [4]. The second point concerns the degree of malalignment and exertion of the VSD. Malalignment of VSD is often found in the patient with double outlet of the right ventricle. The VSD is usually large and located in the inlet portion of the ventricle, extending toward the atrioventricular annulus. In our case, for better visualization of the VSD ridge, the defect was approached through both the aorta and the pulmonary artery.
In conclusion, we successfully performed a double switch operation consisting of Senning and Jatene procedures for superior-inferior ventricles with one double outlet of the right ventricle, using a spiral VSD patch closure.
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References
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Van Praagh S., LaCorte M., Fellows K.E., et al. Superio-inferior ventricles: anatomic and angiocardiographic findings in ten postmortem cases. In: Van Praagh R., Takao A., eds. Etiology and morphogenesis of congenital heart disease. New York: Futura Publishing Company, 1980:317-378.
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Freedom R.M., Culham G., Rowe R.D. The criss-cross and superioinferior ventricular heart: an angiocardiographic study. Am J Cardiol 1978;42:620.[Medline]
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Galinanes M., Chartrand C., van Doesburg N.H., et al. Surgical repair of surerioinferior ventricles: experience with 3 patients. Ann Thorac Surg 1985;40:353-359.[Abstract/Free Full Text]
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Imai Y. . Double-switch operation for congenitally corrected transposition. Advances in cardiac surgery. St. Louis: Mosby-Year Book, Inc, 1997:65-86.
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Abstract
Introduction
