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A Young Hypertensive Woman With a Rare Diagnosis of Middle Aortic Syndrome

CASE HISTORY A 20-year-old Asian woman presented with an 8-month history of headache. She was found to be hypertensive and started on antihypertensive medications. During follow-up, her symptoms persisted, and the hypertension was difficult to control with 2 antihypertensive drugs. She was referred to a cardiologist. In addition to headache, the patient noted some dizziness and dyspnea on exertion. There was no significant past medical history. Her blood pressure was reported to be controlled, at 130 ⁄80 mm Hg, with 3 antihypertensive medications: a b-blocker, an angiotensin-converting enzyme (ACE) inhibitor, and a calcium channel blocker. The patient’s father and mother were both hypertensive and receiving treatment; she was a nonsmoker and did not consume any alcohol. On examination, her heart rate was 70 beats ⁄ min and regular with weak femoral, posterior tibial, and dorsalis pedis pulses. There was a marked radial-femoral delay. Her blood pressure was equal in both arms, at 160 ⁄100 mm Hg. Lower limb blood pressure was unrecordable. Cardiovascular examination findings were unremarkable, with no clinical evidence of heart failure. Abdominal examination revealed a central supraumbilical systolic bruit, but no bruit over the areas of the kidneys. Electrocardiography showed borderline left ventricular hypertrophy. Echocardiography showed left ventricular hypertrophy with good systolic function. Funduscopy revealed grade I hypertensive changes. Results of radiography and computed tomography of the chest were normal. Computed tomography of the abdomen showed diffuse narrowing of the abdominal aorta. Later, the patient underwent 3-dimensional contrast-enhanced magnetic resonance angiography of the abdominal aorta (Figure), which showed that there was a long segmental narrowing of the abdominal aorta that was most marked in the upper portion (where it measures about 8 mm in diameter). A large, prominent superior mesenteric artery was noted, but the coeliac axis was not well seen. A long right renal artery with its origin at the superior mesenteric artery origin and a thin left renal artery with another long accessory artery arising from the lower aorta above the bifurcation supplying the lower pole of the kidney were noted. Results of complete blood count were normal, and the C-reactive protein level was normal, with an elevated erythrocyte sedimentation rate. All blood test findings, including an autoimmune and antibody screening, were normal. From the Department of Cardiology, Grantham and District Hospital, Grantham, UK Address for correspondence: Nimit C. Shah, MBBS, MRCP, Department of Cardiology, Grantham and District Hospital, Grantham NG31 8DG, UK E-mail: drnimitcshah@yahoo.co.uk Manuscript received April 28, 2007; revised June 4, 2008; accepted July 2, 2008