P ersistent 5th aortic arch, an apparently rare congenital anomaly, is defined as a single aortic arch with separate superior and inferior parts.1 It was initially called a double-lumen aortic arch.2 The 5th aortic arch forms between the true aortic arch (a derivative of the 4th aortic arch) and the pulmonary artery (a derivative of the 6th aortic arch).3 Without additional coarctation of the existing aorta, this anomaly alone has no physiologic significance and does not necessitate surgical intervention.We report our catheter-based treatment of an infant who had aortic coarctation of a persistent 5th aortic arch with no associated cardiac defects.
Case ReportIn July 2012, a 7-month-old infant girl was referred to our department for evaluation of a heart murmur. The healthy-looking patient had a normal prenatal and postnatal history. Her blood pressure was normal, and her systemic oxygen saturation was 98% on room air. Auscultation revealed normal heart sounds and a grade 2/6 midsystolic murmur that was audible at the 3rd left sternal border and at the back. The patient's diminished femoral pulses raised the suspicion of aortic coarctation.Electrocardiographic results were normal. Echocardiograms showed left ventricular hypertrophy and an unusual double-lumen aortic arch. There was coarctation of the inferior part of the arch (Fig. 1), and mild obstruction was evident upon color-flow Doppler analysis. Continuous-wave Doppler images revealed a 50-mmHg peak instantaneous gradient at the coarctation site, with typical diastolic runoff. Conventional angiograms showed that the common origin of all brachiocephalic vessels from the ascending aorta ended after the left subclavian artery. A distinct vessel in the inferior part of the interrupted arch originated from the ascending aorta and connected with the descending aorta (Fig. 2). We concluded that this lower arch was a persistent 5th aortic arch. The definitive diagnosis was interrupted 4th arch with persistent 5th arch. The coarctation was at the distal end of the 5th arch.During catheterization, after consulting a cardiac surgeon, we performed balloon angioplasty to correct the coarctation. Afterwards, the peak-to-peak gradient decreased from 50 to 20 mmHg (Fig. 3). At age 1 year, the patient was normotensive. Echocardiograms revealed mild repeat coarctation at the distal end of the persistent 5th arch and a 30-mmHg peak instantaneous gradient across the coarctation site. As of June 2014, we continue to monitor the patient for hypertension and any increase in the gradient. Surgical intervention might be necessary in the future.