Cardiac Case Study: March/April 2006
Weighing the Treatment Options for Aortic Coarctation
By Michael El-Chami, MD, Clinical Cardiology Fellow, Emory University School of Medicine
Technological advances have simultaneously made the diagnosis of coarctation quicker and more certain and the choice of a treatment more subtle and nuanced.
That was the case with a 43-year-old Hispanic male presented to a referring hospital with symptoms of congestive heart failure. He had a history of diastolic murmur; an echocardiogram revealed a bicuspid aortic valve with severe aortic insufficiency and an ejection fraction of 20 percent. Cardiac catheterization was attempted in preparation for aortic valve surgery, however, the wire would not advance above the descending aorta. The patient was referred to Emory Crawford Long Hospital for evaluation.
His physical exam revealed a grade II/VI diastolic murmur at the left upper sternal border, absent arterial pulses in the lower extremities but with normal pulses in the upper extremities. The diagnosis of aortic coarctation was suspected. Magnetic resonance angiography of the aorta revealed severe coarctation distal to the left subclavian with a gradient of 70 mmHg. The patient had an extensive collateral arterial network. Cardiac catheterization using the radial approach revealed normal coronary arteries.
Treatment options still evolving
What would be the next step in the management of this patient?
- Balloon angioplasty of the coarctation followed by aortic valve replacement
- Stenting of the coarctation followed by aortic valve replacement
- Aortic valve replacement and coarctation repair
Coarctation of the aorta constitutes 6 - 8 percent of all cardiac congenital defects. Most commonly, it appears in bicuspid aortic valve patients (30 - 40 percent) as well as those with Turner syndrome (10 percent).
Angioplasty is gaining wide acceptance as the first line of treatment of adult aortic coarctation. Procedural success, defined as a residual gradient of less than 20 mmHg, is more than 90 percent. In general, balloon angioplasty has been proven to be safe but with some potential complications: 5 - 25 percent incidence of recoarctation (especially in infants), paracoarctation aortic dissection or rupture and 5 - 7 percent incidence of aneurysm formation.
Stenting also is gaining wide acceptance in the treatment of coarctation. The success rate is up to 98 percent. In a comparison of stenting of discrete unoperatedcoarctation
to historically matched patients undergoing balloon angioplasty of native aortic coarctation, both modalities had similar satisfactory outcomes, with stenting being a better way to relieve stenosis. In the stent group, two patients required restenting, one due to aneurysm formation and the other due to stent fracture.
Surgery also has very low mortality with a rate of coarctation in the range of 5 - 10 percent. Given the high success rate and the much less invasive nature, however, angioplasty has become an attractive alternative to surgery in the treatment of adult aortic coarctation.
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Our patient had severe aortic insufficiency with depressed left ventricular ejection fraction. It was felt that his impaired left ventricular function made aortic valve replacement and surgical coarctation repair too risky. We considered percutaneous repair in order to decrease resistance to blood flow and improve forward stroke volume, hence reducing the degree of aortic insufficiency.
Our patient underwent balloon angioplasty and stenting of his coarctation for pre-angioplasty and for post-angioplasty using a 36 x 12 mm stent. The patient had a follow-up echo three months later revealing an improvement in ejection fraction to 35 percent but with persistence of severe aortic insufficiency. He subsequently underwent successful aortic valve replacement.
References:
- Gotzsche CO, Krag-Olsen B, Nielsen J, et al. Prevalence of cardiovascular malformations and association with karyotypes in Turner syndrome.
Arch Dis Child 1994;71:433.2. - Nihoyannopoulos P, Karas S, Sapsford RN, et al. Accuracy of two-dimensional echocardiography in the diagnosis of aortic arch obstruction. J Am CollCardiol 1987;10:1072.
- Fawzy ME, Awad M, Hassan W, et al. Long-term outcome (up to 15 years) of balloon angioplasty of discrete native coarctation of the aorta in adolescents and adults. J Am CollCardiol 2004;43:1062.
- Ledesma M, Alva C, Gomez FD, et al. Results of stenting for aortic coarctation.
Am J Cardiol 2001;38:1518. - Cohen M, Fuster V, Steele PM, et al. Coarctation of the aorta. Long-term follow up and prediction of outcome after surgical correction.Circulation 1989;80:840.
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