Ann Thorac Surg 2006;81:1512-1514
© 2006 The Society of Thoracic Surgeons
Case report
Bland-White-Garland Syndrome in Pregnancy: Reoperation of ALCAPA With an Internal Thoracic Radial Artery "Y"-Graft
Cliff K. Choong, FRACS,
Carlo Martinez, MD,
Hendrick B. Barner, MD
*
,
Phillip A. Ludbrook, MD
Division of Cardiothoracic Surgery, Washington University School of Medicine, St. Louis, Missouri
Accepted for publication April 18, 2005.
* Address correspondence to Dr Barner, 6125 Clayton Ave, Suite 430, St. Louis, MO 63139 (Email: hendrick.barner{at}tenethealth.com).
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Abstract
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Anomalous origin of the left coronary artery from the pulmonary artery is rare and more so in the adult. Reimplantation of the left main coronary into the aorta is successful in early life, but it may be more difficult in the adult who had a previous repair. We report a successful reoperation of anomalous origin of the left coronary artery from the pulmonary artery in an adult patient using a left internal thoracic and radial artery "Y"-graft. Composite arterial grafting has become an established and straight forward technique in coronary surgery, and it is appropriate and recommended for congenital lesions in adults and at any age if necessary.
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Introduction
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Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is rare and more so in the adult, because the majority of those afflicted die in the first year without surgical correction. Reimplantation of the left main coronary into the aorta is successful in early life, but it may be more difficult in the adult who had a previous repair. We report the reoperation of ALCAPA in an adult patient using a left internal thoracic and radial artery "Y"-graft.
A 28-year-old woman, with a history of Bland-White-Garland syndrome and ligation of the ALCAPA at 4 months of age, presented with effort angina during her pregnancy. She had a dobutamine stress that showed marked anterolateral hypokinesis with electrocardiographic ischemia. Cardiac catheterization revealed a large dominant right coronary with multiple collaterals supplying the small circumflex well, but poorly to the small left anterior descending artery. The left ventriculogram revealed moderate global hypokinesis and severe anterolateral hypokinesis with an estimated ejection fraction of 35% and 1+ mitral regurgitation. She tolerated the pregnancy and delivered the fetus by Cesarean section. She continued to experience angina and underwent elective operation. At surgery the left ventricle was hypokinetic and dilated without scarring. The left anterior descending artery was 1.5 mm, and the first marginal branch was 1.0 mm, and these were grafted. The left internal thoracic artery was grafted to the mid-left anterior descending artery and the radial artery to the side of the mid-left internal thoracic artery and to the obtuse marginal branch. The "Y" anastomosis was first constructed by making a 4-mm incision in the internal thoracic artery at the level of the left atrial appendage on the smooth side of the internal thoracic artery pedicle and opening the radial artery for 2–3 mm (fish mouth) to create an end-to-side anastomosis with continuous 7-0 Prolene (Ethicon, Somerville, NJ).
The distal anastomoses were created with 3 to 4 mm coronary arteriotomies, fish mouthing of the conduit and anastomoses using a continuous 7-0 Prolene (Ethicon, Somerville, NJ). The conduits were secured to the epicardium with 6-0 Prolene (Ethicon) to relieve tension and prevent angulation. A standard technique was routinely utilized to prevent spasm of the arterial conduits. Blood containing papaverine 2 mg/mL was injected intraluminally after completion of the "Y" anastomosis with subsequent exposure to arterial blood pressure for a duration of 10 minutes, which dilates the conduits. Her postoperative course was uneventful, and she was discharged on postoperative day 5.
Three months later she complained of chest pain and cardiac catheterization, revealed patent grafts with small left coronary vessels that were attenuated distally. Ventricular function was similar to preoperative function, but there was no mitral regurgitation. Three years later, an echocardiography stress test demonstrated no inducible dysfunction and only a mild global hypokinesis. Four years postoperatively a catheterization revealed patent grafts with enlargement of the native left coronary arteries and retrograde filling of the distal circumflex system that was not present at 3 months (Figs 1, 2). The right coronary was of normal size without evident collaterals to the left coronary system. Five years postoperatively, a stress echocardiography revealed a normal left ventricular function with no segmental wall motion abnormalities and termination of the stress test at 9 minutes, 45 seconds for shortness of breath.
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Fig 1. (A, B) Cardiac catheterization 4 years after surgery showing patent left internal thoracic and radial artery "Y"-graft supplying the left coronary system.
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Fig 2. (A, B) Cardiac catheterization 4 years after surgery showing patent left internal thoracic and radial artery "Y"-graft supplying the left coronary system.
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Comment
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Establishment of a two coronary system with arterial conduits in ALCAPA is unnecessary in early life because of the successful results of reimplantation of the left main coronary into the aorta and the limitations of the small conduit and coronary arteries. There have been very few reports utilizing arterial grafting in the treatment of ALCAPA. Mavroudis and colleagues [1] reported the use of the left internal thoracic artery in a 5.6-year-old child before reimplantation was a proven technique. Kitamura and coauthors [2] reported bilateral internal thoracic artery grafting with ligation of the left main coronary in an adult. Four other reports, all in adults, involved left anterior descending artery grafting to the left anterior descending artery that provided perfusion to the circumflex system retrogradely, with concomitant ligation of left main artery [3–6]. If the left main had been previously ligated as in our case, the confluence of the two arteries may not be preserved, and for this reason we chose two conduits. Our experience with composite radial artery grafting led to this choice, and our 4-year angiogram combined with 10-year patency data of the radial artery support this decision [7]. One case utilizing the radial artery in the treatment of ALCAPA was reported by Brodman and colleagues [8] who successfully used a single aortocoronary radial graft in a 15-month-old girl with ALCAPA.
Although recovery of left ventricular function and regression of mitral insufficiency has been excellent in pediatric patients 6 to 10 months after surgery, such information is limited in adults. Our patient had little change at the 3-month catheterization, other than resolution of mild mitral insufficiency; however, the stress test at 3 years revealed improved left ventricular function, and at 5 years the ejection fraction was normal. The 5 adults having repair of ALCAPA with arterial conduits were followed-up at 1 to 8 months, and 3 had postoperative catheterization, but only 1 gave the ejection fraction, which increased from 30% to 40% [2–6]. Four had perfusion stress testing with good perfusion and absence of ischemia [2–4, 6]. These observations, including ours, indicate that recovery of the ventricle in adults is similar to that observed in infants and children, although other reported cases have some persistent anterior perfusion abnormalities that may be related to coronary hypoplasia or congenital obstructive lesions.
Although experience with arterial conduits in adults with ALCAPA is small, experience with arterial conduits for coronary revascularization in the adult population without ALCAPA is large and long-term results of these patients support this approach in adults. It is also useful in children and even neonates when the usual techniques have failed [1, 8]. Reimplantation is feasible in some adults, but not those with prior ligation, and the Takeuchi pulmonic tunnel is complex and has been associated with pulmonary obstruction and conduit failure. Composite arterial grafting has become an established and straightforward technique in coronary surgery, and it is appropriate and recommended for congenital lesions in adults and at any age if necessary [1, 2, 8].
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References
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- Mavroudis C, Backer CL, Duffy CE, et al. Pediatric coronary artery bypass for Kawasake, congenital, post arterial switch and iatrogenic lesions Ann Thorac Surg 1999;68:506-512.[Abstract/Free Full Text]
- Kitamura S, Kawachi K, Nishii T, et al. Internal thoracic grafting for congenital coronary malformations Ann Thorac Surg 1992;53:513-516.[Abstract]
- Francois K, Provenier F, Jordaens L, et al. Anomalous origin of the left coronary artery from the pulmonary artery Ann Thorac Surg 1993;56:1168-1170.[Abstract]
- Chan RKM, Hare DL, Buxton BF. Anomalous left main coronary artery arising from the pulmonary artery in an adulttreatment by internal mammary artery grafting. J Thorac Cardiovasc Surg 1995;109:393-394.[Free Full Text]
- Niino T, Shiono M, Inoue T, et al. A reoperation of adult-type Bland-White-Garland syndrome Ann Thorac Surg 2003;76:267-269.[Abstract/Free Full Text]
- Kattach H, Anastasiadis K, Jin XY, Pillai R. Two-conduit repair for anomalous origin of the left coronary artery from the pulmonary artery in an adult J Thorac Cardiovasc Surg 2004;128:641-642.[Free Full Text]
- Barner HB, Sundt III TM, Bailey M, Zang Y. Intermediate term results of complete arterial revascularization in over 1,000 patients using an internal thoracic artery/radial artery T-graft Ann Surg 2001;234;:447-452.[Medline]
- Brodman RF, Issenberg HJ, Glickstein JS, Frame R. Use of a free radial artery graft for correction of Bland-White-Garland syndrome Ann Thorac Surg 1996;62:1525-1526.[Abstract/Free Full Text]
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Abstract
Introduction
