Ann Thorac Surg 2004;77:319-321
© 2004 The Society of Thoracic Surgeons
Case report
Composite aortoplasty for recurrent coarctation after neonatal repair in Williams syndrome
Jeni L. Marks, MDa,
Max B. Mitchell, MDa*,
David N. Campbell, MDa,
Warren H. Toews, MDb
a Department of Surgery, University of Colorado Health Sciences Center, Denver, Colorado, USA
b Department of Pediatric Cardiology, Presbyterian Saint Luke’s Medical Center, Denver, Colorado, USA
Accepted for publication March 25, 2003.
* Address reprint requests to Dr Mitchell, 1056 E. 19th Ave, B200, Denver, CO 80218, USA
e-mail: mitchell.max{at}tchden.org
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Abstract
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Supravalvar aortic stenosis is the most frequent operation required for Williams syndrome; however, coarctation repair is more common in patients requiring surgery in the first few months of life. We report on a child with Williams syndrome in whom extensive reoperation was required 5 months after neonatal aortic coarctation repair. A composite left subclavian artery flap and allograft patch aortoplasty of the aortic arch and descending aorta was performed through a left thoracotomy using cardiopulmonary bypass and circulatory arrest. Detailed anatomic evaluation of the aortic arch and descending aorta is recommended before initial coarctation repair in neonates with Williams syndrome.
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Introduction
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Elastin arteriopathy is the underlying cause of the cardiovascular manifestations of Williams syndrome [1, 2]. Presentation requiring surgery in the neonatal period is rare, and usually involves aortic coarctation [3]. Limited data concerning coarctation repair in neonates with Williams syndrome indicate that recurrent arch obstruction is common [3]. Recently, we repaired aortic coarctation in a neonate with Williams syndrome. Recurrent arch obstruction necessitated radical reoperation. Advance recognition of the magnitude of arteriopathy in similar patients may allow more effective initial surgical intervention.
A 2.7-kg male with elfin facies and absent femoral pulses was suspected to have Williams syndrome at birth. Echocardiogram revealed aortic coarctation, a closed ductus arteriosus, and poor pulsatility in the descending aorta. The supravalvar aorta was narrow without measurable gradient. PGE1 infusion was initiated to open the ductus arteriosus. On day of life 3, an extended end-to-end arch repair was performed advancing the descending aorta up under the origin of the left carotid artery. After excising the coarctation and juxtaductal aorta, marked thickening of the aortic wall up to the limits of the vascular clamps was discovered, and the repair was performed with abnormal vessel walls. After surgery, femoral pulses were diminished but palpable. Genetic evaluation confirmed Williams syndrome. Renal sonography and magnetic resonance angiography revealed renovascular involvement of the right kidney. The child was discharged home after 4 days on oral propranolol. At 5 months, hypertension was poorly controlled with multidrug treatment, and femoral pulses were not palpable. At catheterization, femoral artery access could not be achieved. Transvenous angiography demonstrated severe aortic narrowing with extensive descending thoracic aortic disease (Fig 1).
Moderate stenosis of the left carotid origin suggested transverse arch involvement.
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Fig 1. Angiogram demonstrating recurrent arch obstruction with extensive descending aortic involvement. Comparison with the 4F catheter (1.3-mm diameter) reveals the severity of aortic obstruction.
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Reoperation was performed through extended left posterolateral thoracotomy in the fourth intercostal space. The arch and descending aorta were widely exposed. Left subclavian artery branches were divided up to the thoracic outlet. Through anterior pericardial incision, cardiopulmonary bypass was initiated with cannulas in the right atrial appendage and ascending aorta. After cooling to 18°C, the left carotid artery was snared, and clamps were placed distal to the innominate artery and on the descending aorta. Flow was lowered to maintain an arterial pressure of 40 mm Hg measured in the right arm. The left subclavian artery was divided at the thoracic outlet and opened longitudinally into its origin. The aortic wall was approximately 3 mm thick, and the severity of transverse arch and descending aortic disease was much greater than suggested at angiography. Aortotomy was extended down the descending aorta to the eighth intercostal space, where wall thickness was more normal (Fig 2A).
The transverse arch was opened toward the proximal clamp, but the arterial wall remained markedly thickened. The posterior suture line of an extended subclavian artery flap was constructed. Next, the circulation was arrested, the innominate artery was snared, and the ascending aorta was clamped proximal to the aortic cannula. Aortotomy was extended into the more normal appearing ascending aorta. A pulmonary artery allograft was used to augment the aortic arch to the origin of the subclavian flap. The width of the allograft patch was approximately equal to the width of the opened subclavian artery flap. The arch was de-aired, and flow was restored to the heart and head after the distal arch was clamped. Composite allograft and subclavian flap augmentation of the descending aorta was then completed (Fig 2B). Full pump flow was established, and the patient was weaned from bypass after rewarming. Circulatory arrest was 21 minutes. Cardiopulmonary bypass time was 86 minutes.
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Fig 2. Composite allograft and subclavian flap aortoplasty. (A) The aortotomy extends from the distal ascending aorta to the mid descending thoracic aorta and is connected to the left subclavian artery flap incision. (B) The left subclavian artery flap is turned down into the descending thoracic aorta and the aorta is augmented with a homograft patch.
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Postoperatively, femoral pulses were easily palpable. The child was neurologically intact and was extubated the next day. The patient was discharged postoperative day 4. Right nephrectomy was performed 6 weeks later. At 1-year follow-up the aortic arch is widely patent without aneurysm, pulses remain easily palpable, and hypertension is controlled with propranolol and nifedipine.
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Comment
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Williams syndrome is characterized by varying degrees of mental retardation, dysmorphic facies, growth deficiency, cardiovascular disease, urogenital defects, and hypercalcemia [2, 4]. Fluorescence in situ hybridization demonstrating a microdeletion of chromosomal region 7q11.23 is diagnostic. Hypertension may result from coarctation of the aorta, renovascular disease, or noncompliant arterial vessels. Cardiovascular findings commonly lead to diagnosis that can be established in up to 50% of patients before 1 year of age [3]. Although one-third of Williams syndrome patients exhibit cardiovascular signs or symptoms as newborns, surgical intervention in the neonatal time frame is rare [3, 5]. Although surgery for supravalvar aortic stenosis is the most frequent operation required for Williams syndrome, coarctation repair is more common in patients requiring surgery in the first few months of life [3]. There are little data concerning results of coarctation repair in young infants with Williams syndrome, but recurrence appears common. In a series of 75 Williams syndrome patients, only 5 required initial surgical intervention before 5 months of age [3]. Of the 3 patients undergoing coarctation repair at this age, all developed recurrence. The abnormal nature of our patient’s aorta was noted at initial repair, and was likely related to the recurrent obstruction. This finding and the presence of early renovascular disease suggest that diffuse arteriopathy was present at birth. We underestimated the severity of this patient’s arteriopathy at initial echocardiographic evaluation. Considering our experience and the apparent increased risk of recurrent coarctation in young babies with Williams syndrome, detailed imaging of the aortic arch and descending aorta with either conventional or magnetic resonance angiography should be considered before initial surgical intervention. More extensive initial correction may prevent recurrent arch obstruction.
Several aspects of this case warrant mention. The magnitude of aortic disease was greater than suggested by angiography due to the extreme thickness of the aortic wall. The extended subclavian flap provided growth potential in the severely diseased descending aorta. Our approach permitted single-stage aortic arch and descending aortic reconstruction beyond what could have been achieved by median sternotomy while optimizing both brain and spinal cord protection. Venous cannulation for cardiopulmonary bypass with left thoracotomy is usually achieved through the left femoral vein or main pulmonary artery. In a child weighing 4.5 kg, femoral or iliac vein cannulation would have been suboptimal, and the size of the pulmonary artery in this child was inadequate for venous cannulation. Extending the posterolateral thoracotomy anteriorly allowed right atrial and ascending aortic cannulation. Although exposure of the right atrial appendage is limited, cannulation is feasible provided that there are no pericardial adhesions. Longer follow-up is required to exclude delayed aneurysm formation or recurrent obstruction. Residual hypertension requiring medication is most likely due to poor arterial compliance.
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References
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- Ewart A.K., Jin W., Atkinson D., et al. Supravalvular aortic stenosis associated with a deletion disrupting the elastin gene. J Clin Invest 1994;93:1071-1077.
- Williams J.P.C., Barratt-Boyes B.G., Lowe J.B. Supravalvar aortic stenosis. Circulation 1961;24:1311-1318.[Abstract/Free Full Text]
- Eronen M., Peippo M., Hiippala A., et al. Cardiovascular manifestations in 75 patients with Williams syndrome. J Med Genet 2002;39:554-558.[Abstract/Free Full Text]
- Wessel A., Pankau R., Kececioglu D., et al. Three decades of follow-up of aortic and pulmonary vascular lesions in the Williams-Beuren syndrome. Am J Med Genet 1994;52:297-301.[Medline]
- Stamm C., Friehs I., Ho S.Y., et al. Congenital supravalvar aortic stenosis: a simple lesion?. Eur J Cardiothorac Surg 2001;19:195-202.[Abstract/Free Full Text]
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Abstract
Introduction
