Ann Thorac Surg 2003;76:1283-1286
© 2003 The Society of Thoracic Surgeons
Case report
Staged biventricular repair of taussig-bing anomaly with subaortic stenosis and coarctation of aorta
Hong Gook Lim, MDa,
Woong-Han Kim, MD, PhD*a,
Young Tak Lee, MDb,
Jae Jin Han, MDa,
Soo-Cheol Kim, MDa,
Cheong Lim, MDa,
Chan-Young Na, MDa
a Departments of Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute, Bucheon, Kyungki-do, South Korea
b Department of Thoracic and Cardiovascular Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea
Accepted for publication February 24, 2003.
* Address reprint requests to Dr W. H. Kim, Department of Cardiovascular Surgery, Sejong General Hospital, 91-121 Sosa Bon 2-dong, Sosa-ku, Bucheon-shi, Kyungki-do, 422-232, South Korea
e-mail: woonghan{at}korea.com
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Abstract
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We present successful procedures for 2 infants who had the Taussig-Bing anomaly with subaortic stenosis and coarctation of the aorta. The initial procedure was coarctoplasty and the Damus-Kaye-Stansel procedure with modified Blalock-Taussig shunt. The second procedure was intraventricular repair (Kawashima procedure), Damus-Kaye-Stansel take-down and the reuse of native aortic and pulmonary valves 19 and 25 months later. Both patients survived the operations and postoperative hemodynamics were excellent at both 28 and 59 months follow-ups.
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Introduction
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Surgical repair of the Taussig-Bing anomaly with subaortic stenosis and coarctation of the aorta (CoA) is required in the neonatal period because of marked cyanosis and congestive heart failure, but the size discrepancy of the great arteries, hypoplastic aorta and aortic valve (AV), and subaortic stenosis make the total correction technically difficult. Growth of the AV and regression of subaortic stenosis and ventricular hypertrophy can be expected after initial Damus-Kaye-Stansel (D-K-S) procedure. We present the successful staged biventricular repair for 2 infants who had the Taussig-Bing anomaly with subaortic stenosis and CoA.
A 5-month-old male infant (patient 1) weighing 6.1 kg, and a 1-month-old female infant (patient 2) weighing 3.3 kg, were admitted to the hospital because of cyanosis and congestive heart failure. Echocardiographic findings were double-outlet right ventricle with subpulmonary ventricular septal defect and CoA. The great arteries were side by side. Coarctation of the aorta was isthmic hypoplasia in the first patient and diffuse long segment in the second patient (Fig 1)
. Cardiac catheterization showed 30 mm Hg of pressure gradient at CoA and 15 mm Hg at the subaortic area, and pulmonary vascular resistance was 9 Wood units/m2 in patient 1. Both patients demonstrated subaortic stenosis, not only as pressure gradient in cardiac catheterization, but also morphologically (Fig 1). The final diagnoses were Taussig-Bing anomaly, CoA, patent ductus arteriosus (PDA), subaortic stenosis, and severe pulmonary hypertension.
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Fig 1. Angiogram showing pulmonary artery from right ventricle and right modified Blalock-Taussig shunt (RMBT) before second stage operation. The proximal portion of the right pulmonary artery is absent.
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The operative procedure in the first stage was D-K-S procedure with modified Blalock-Taussig shunt using 5 mm (patient 1) and 4 mm (patient 2) Gore-Tex vascular grafts (W. L. Gore and Associates, Flagstaff, AZ), respectively, coarctoplasty with extended end-to-end anastomosis, PDA division, and atrial septectomy. Aortic cross-clamp time was 28 minutes (patient 1) and 37 minutes (patient 2), and cardiopulmonary bypass time was 160 minutes (patient 1) and 188 minutes (patient 2), respectively. Coarctoplasty was performed with regional perfusion without circulatory arrest. Arterial perfusion was done through the innominate artery shunt (Fig 2).
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Fig 2. Schematic presentations showing the first operative technique. (A) Arterial perfusion was done through the innominate artery shunt. (B) The Damus-Kaye-Stansel procedure with modified Blalock-Taussig shunt.
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In patient 2, the size of aortic annulus increased from 5.8 mm to 9.6 mm, the pulmonic annulus decreased from 12.7 mm to 10.4 mm, and the subaortic stenosis and ventricular hypertrophy regressed 14 months after the first stage operation (Fig 1).
The second operation was done 25 months later in patient 1 and 19 months later in patient 2. The operative procedure was intraventricular repair (Kawashima procedure), D-K-S take-down, reuse of the AV and pulmonic valve, and closure of the atrial septal defect. The defects of D-K-S sites of the AV and pulmonic valve were reconstructed by bovine pericardiums (Fig 3).
Aortic cross-clamp time was 148 minutes (patient 1) and 201 minutes (patient 2), and cardiopulmonary bypass time was 212 minutes (patient 1) and 301 minutes (patient 2), respectively. Circulatory arrest was used in the first patient and not in the second patient. Arterial perfusion was done through the innominate artery cannulation with regional perfusion in patient 2. Both extubations were done on postoperative day 1 and postoperative courses were uneventful.
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Fig 3. Schematic presentations showing the second operative technique. Reuse of the native aortic valve (A) and the pulmonic valve (B). The defects of the Damus-Kaye-Stansel take-down sites were reconstructed by bovine pericardiums.
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Follow-up was done for 59 months in patient 1 and 28 months in patient 2. All patients were in the New York Heart Association functional class I in clinical status, chest roentgenograms showed mild cardiomegaly, and electrocardiograms showed sinus rhythm. Echocardiograms showed no left ventricular outflow tract obstruction, no right ventricular outflow tract obstruction, no aortic regurgitation, trivial pulmonic regurgitation, and trivial tricuspid regurgitation (Fig 4).
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Fig 4. Echocardiographic view. The postoperative apical four-chamber view of patient 2 showed neither left ventricular outflow tract obstruction nor ventricular septal defect leakage in the Kawashima procedure. Kawashima intraventricular baffling was well demonstrated. (AO = aorta; LV = left ventricle; LVOT = left ventricular outflow tract; RA = right atrium; TV = tricuspid valve.)
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Comment
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Current corrective surgical approaches for the Taussig-Bing heart include arterial switch with ventricular septal defect closure and Kawashima intraventricular repair [1]. Neonatal arterial switch operation demonstrated several morbidities such as neo-aortic regurgitation and residual right ventricular outflow tract obstruction [1]. The Kawashima intraventricular repair for side-by-side great arteries seems to be an attractive method, because it preserves the native AV and avoids coronary dissection [2]. The D-K-S repairs can be done in patients who had the Taussig-Bing anomaly with potential left ventricular outflow tract obstruction as an initial procedure [3, 4]. The D-K-S repair allows growth of the AV and regression of subaortic stenosis as in these patients. We cannot find the exact mechanism, but we suppose that the AV grows by increased forward flow and that subaortic stenosis regresses to a dramatic degree by afterload reduction. Due to the growth of the AV and regression of the subaortic stenosis after D-K-S repair, Kawashima intraventricular repair with resection of the conal septum produces more relief of the left ventricular outflow tract obstruction, and therefore we could reconstruct the great arteries and reuse the native aortic and pulmonic valves, avoiding the Rastelli procedure. After an initial D-K-S procedure in the Taussig-Bing anomaly with left ventricular outflow tract obstruction, a Rastelli procedure was usually done [3, 4]. This is the first report of a repair saving the native aortic and pulmonic valves without the Rastelli procedure after an initial D-K-S procedure in the Taussig-Bing anomaly with left ventricular outflow tract obstruction. The results recommend staged biventricular repair such as the Kawashima intraventricular repair by D-K-S repair for patients who have the Taussig-Bing anomaly with subaortic stenosis and CoA.
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References
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- 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]
- Serraf A., Lacour-Gayet F., Bruniaux J., et al. Anatomic repair of Taussig-Bing hearts. Circulation 1991;84(Suppl III):III200-205.
- Aoki M., Forbess J.M., Jonas R.A., Mayer J.E., Jr, Castaneda A.R. Result of biventricular repair for double-outlet right ventricle. J Thorac Cardiovasc Surg 1994;107:338-350.[Abstract/Free Full Text]
- Brown J.W., Ruzmetov M., Okada Y., Vijai P., Turrentine M.W. Surgical results in patients with double outlet right ventricle: a 20-year experience. Ann Thorac Surg 2001;72(5):1630-1635.[Abstract/Free Full Text]
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Abstract
Introduction
