A 9-year-old boy was referred for cardiac evaluation as part of screening for his dermatomyositis. He had no history of headache, nosebleeds, aching calves, or exercise intolerance. On examination, a 3/6 low frequent systolic murmur was present at the apex and left second intercostal space with radiation to the carotic arteries. Arterial femoral pulsations were absent. Systolic blood pressure in the right arm and right leg was 170 and 100 mm Hg, respectively.
Chest x-ray revealed marked rib notching (arrows in Figure 1). An ECG revealed sinus rhythm without features of left ventricular hypertrophy. Surface echocardiography confirmed the lack of left ventricular hypertrophy because the interventricular septum diameter at end diastole and left ventricular posterior wall thickness at end diastole measured 5.5 mm (p36) and 5.5 mm (p35), respectively. The maximum peak flow velocity in the left ventricular outflow tract measured 1.7 m/s (10.9 mm Hg), indicating no significant left ventricular outflow tract gradient. The short-axis and parasternal long-axis views revealed a tricuspid aortic valve with normal excursions. An increased peak flow velocity of 3.5 m/s (comparable to a Doppler gradient of 49 mm Hg), together with the typical Doppler pattern, indicated severe stenosis of the descending aorta (Figure 2).
Magnetic resonance angiography confirmed the coarctation just beyond the left subclavian artery (black arrow in Figure 3) and several pronounced collaterals (white arrows in Figure 3). We performed a coarctectomy with end-to-end anastomosis. Two months after surgery, the patient’s systolic blood pressure was 110 mm Hg under β-blockade.
In severe and late-diagnosed aortic coarctation, numerous collaterals can develop for the arterial blood supply to the lower half of the body. As a result of pronounced intercostal collateral arteries, bone erosion may be noted as rib notching in the lower margin of the third to sixth or ninth ribs. Although a tendency exists to perform a balloon angioplasty and/or stent implantation as the primary treatment of aortic coarctation, there is currently no definite proof of a profound benefit for balloon angioplasty or surgery as the primary treatment of aortic coarctation. This is due to the existence of relatively limited prospective randomized controlled trials, a lack of long-term follow-up, and/or the existence of data reflecting only a single institution’s preferences for treatment. To date, only 2 prospective randomized controlled trials are available.1,2 Both studies demonstrated similar success rates (reduction in peak systolic pressure gradient across the coarctation) between treatment groups. Shaddy et al2 and Cowley et al3 showed a significantly higher risk of aneurysm formation, recurrent coarctation, and iliofemoral artery injury during follow-up in the angioplasty group compared with the surgery group. Despite criticism,4 Wong et al5 outlined the major and minor complications for balloon angioplasty and surgical repair. They concluded that balloon angioplasty is slightly preferable over surgery as the initial treatment for native aortic coarctation in children.
In our patient, the surgeons decided to perform a coarctectomy with end-to-end anastomosis, considering the excellent results of this surgical intervention in our center. However, at the moment, the approach is changing, favoring primary percutaneous balloon dilatation as the initial treatment for aortic coarctation in our hospital, particularly in older children.
Hernandez-Gonzalez M, Solorio S, Conde-Carmona I, Rangel-Abundis A, Ledesma M, Munayer J, David F, Ortegon J, Jimenez S, Sanchez-Soberanis A, Melendez C, Claire S, Gomez J, Teniente-Valente R, Alva C. Intraluminal aortoplasty vs. surgical aortic resection in congenital aortic coarctation: a clinical random study in pediatric patients. Arch Med Res. 2003; 34: 305–310.
Shaddy RE, Boucek MM, Sturtevant JE, Ruttenberg HD, Jaffe RB, Tani LY, Judd VE, Veasy LG, McGough EC, Orsmond GS. Comparison of angioplasty and surgery for unoperated coarctation of the aorta. Circulation. 1993; 87: 793–799.
Cowley CG, Orsmond GS, Feola P, McQuillan L, Shaddy RE. Long-term, randomized comparison of balloon angioplasty and surgery for native coarctation of the aorta in childhood. Circulation. 2005; 111: 3453–3456.