Asymmetric Pulses in a 5-Year-Old Asian Female

Abstract

A 5-year-old female was seen in the pediatric cardiology clinic following her adoption from China 1 month prior. A cardiovascular assessment was requested due to her pertinent cardiac medical history of ventricular septal defect (VSD), patent ductus arteriosus (PDA), and right aortic arch. She underwent surgical closure of the VSD and ligation of the PDA at age of 2 years in China. Her postoperative echocardiogram in China showed excellent results with no residual lesions. In addition to establishing follow-up cardiology clearance was requested for a rectal biopsy to rule out Hirschsprung's because of chronic constipation.At the cardiology visit she was described as an active child who is able to keep up well with peers. She had a history of constipation and recurrent abdominal pain. A review of systems was negative for fevers, diaphoresis, emesis, shortness of breath, exercise intolerance, dizziness, syncope, or claudication. Her development was normal and her growth tracked the third percentile for height and weight and was felt to be due to social circumstances.On examination, the patient was found to have a heart rate of 90 beats/min, right upper extremity blood pressure of 130/84 mm Hg, and a right lower extremity blood pressure of 105/57 mm Hg with normal oxygen saturation. The upper extremity pulses were 2+ while both femoral artery pulses were absent, with acyanotic, warm, and well-perfused lower extremities. Auscultation yielded normal S1 and S2 heart sounds with no murmurs, and clear lung fields. Abdominal exam showed mild distension but otherwise was unremarkable. No bruit was appreciated. An electrocardiogram demonstrated normal sinus rhythm. Because of the weak femoral pulses and the significant blood pressure discordance between upper and lower extremities, an echocardiogram was obtained which demonstrated a right aortic arch without thoracic coarctation, a moderately dilated aortic root and ascending aorta, and a blunted abdominal aorta spectral Doppler profile suggestive of abdominal aortic obstruction; there was no residual shunting from the previously repaired VSD or the PDA. Because of these concerning findings, a magnetic resonance imaging and angiography (MRI/MRA) study of the chest and abdomen was obtained. The MRI/MRA demonstrated severe asymmetric luminal narrowing in the upper abdominal aorta just superior to the celiac and renal arteries and a moderately dilated ascending aorta. It also showed circumferential aortic wall thickening with increase contrast enhancement suggestive of aortic wall inflammation (Figure 1). These findings were most consistent with aortitis and the patient was admitted to the inpatient cardiology service. Hospital CourseRheumatology and infectious disease services were consulted. An extensive workup was initiated and yielded low inflammatory markers with an erythrocyte sedimentation rate of 4 mm/h and C-reactive protein of <0.29 mg/dL. Infectious work up, including tuberculosis and syphilis, was negative. Biomarkers suggestive of vasculitis were obtained and foun...