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A rare and treatable cause of hypertension in pregnancy

Dear Editor A 25yearold pregnant patient in the 24th gestational week was referred for evaluation of resistant hypertension. There was significant obstetric history of one premature delivery with neonatal death and two instances of intrauterine fetal loss. The patient was asymptomatic except for occasional headaches. Hypertension, diagnosed during the first gestation 4 years ago, was poorly controlled in spite of the use of multiple antihypertensive agents. Clinical examination revealed a significant blood pressure difference in the upper and lower extremities (upper limb 178/106 mm Hg vs lower limb 120/94 mm Hg) with weak lower limb pulses. As part of the standard workup for secondary hypertension, echocardiography with color Doppler was performed, which revealed concentric left ventricular hypertrophy with good biventricular function. Interrogation of the abdominal aorta revealed marked narrowing with turbulent flow (Figure A and B). Levels of Creactive protein and erythrocyte sedimentation rate were both within the normal range. A provisional diagnosis of aortoarteritis involving the abdominal aorta was made—the socalled middle aortic syndrome.1 Cardiac catheterization with aortography was performed at the 28th gestational week with adequate measures to minimize fetal radiation exposure, confirming the presence of long segment narrowing of the abdominal aorta (Figure C) with pressure gradients of 86 mm Hg. A selfexpandable stent of 14 mm×70 mm (Epic, Boston Scientific) was deployed at the site of maximum narrowing (Figure D). Immediately following the procedure, the aortic pressure gradient decreased by more than half to 40 mm Hg. Thereafter, hypertension could be adequately controlled with monotherapy and the patient had an uneventful caesarean section at term with good outcome. To our knowledge, this is the first case describing interventional therapy for middle aortic syndrome during pregnancy with excellent fetomaternal outcome. Middle aortic syndrome may be secondary either to a congenital anomaly or acquired conditions such as Takayasu arteritis, infection, obliterative panarteritis, neurofibromatosis, retroperitoneal fibrosis, fibromuscular dysplasia, and mucopolysaccharidosis.2 Changes in the aortic wall during pregnancy, coarctationassociated aortopathy, and longstanding hypertension combine to increase the risk of aortic rupture/dissection. Also, poorly controlled hypertension leads to adverse fetal (growth retardation, placental abruption, and premature delivery) and maternal (renal failure and hypertensive crisis) outcomes.3 Given the unsatisfactory response to multiple antihypertensive agents and their adverse effect, intervention with stenting is preferred over a conservative approach.4 The procedure was carried out during the 28th week of gestation to minimize the effect of radiation on fetal organogenesis as well as the risk of preterm labor. The procedure is generally associated with low risk and subsequently enables adequate blood pressure control with good pregnancy outcome.