Correspondence

Aortic Pseudocoarctation Causing Refractory Hypertension

N Engl J Med 2002; 346:784-785March 7, 2002

Article

To the Editor:

We report a case of pseudocoarctation of the aorta caused by an intraluminal calcific mass. A 49-year-old woman who smoked and had had hypertension for eight years was evaluated for an intramural aortic hematoma seen on magnetic resonance imaging (MRI). She had recently been examined because of uncontrollable hypertension and transient acute renal dysfunction. Magnetic resonance angiography and captopril-augmented renal scanning excluded the presence of renal-artery stenosis, a renal biopsy excluded the presence of parenchymal disease, and tests of urine and plasma excluded the presence of pheochromocytoma, Cushing's syndrome, and Conn's syndrome. The patient also had intermittent claudication. She presented to the referring hospital with low back pain and severe hypertension (220/100 mm Hg). MRI suggested the presence of an intramural hematoma at the thoracic level.

Transferred for further evaluation, she had a brachial blood pressure of 230/70 mm Hg; occasionally, her blood pressure was 305/135 mm Hg. She had weak femoral pulses and a continuous murmur in the interscapular region. Her blood pressure was controlled with esmolol and nitroprusside. Transesophageal echocardiography revealed a complex, calcified, and fixed mass limited to the lumen of the descending aorta that was approximately 10 cm in length; it began as a shelf-like projection and became more complex, with ridges and furrows and areas of severe luminal narrowing (90 percent of the cross-sectional area). Doppler ultrasonography revealed a gradient of 145 mm Hg. There were prominent paraaortic collateral vessels that fed back into the aorta distal to the mass, consistent with a chronic obstruction. A computed tomographic scan confirmed that the mass was overtly calcific and solely intraluminal. This was confirmed at surgery by the normal appearance of the external aortic wall (Figure 1Figure 1Gross Pathological Specimen of the Excised Portion of the Descending Thoracic Aorta, Showing the Smooth External Aortic Wall and the Complex Intraluminal Mass.). Surgical excision with interposition of a tube graft was performed.

Microscopical examination showed no evidence of an intimal tear or adventitial disease. The mass had the appearance of a calcific organized thrombus. On gross inspection and histologic examination, there were no atherosclerotic changes in the specimen, no evidence of neoplasia, and no sign of any major intimal damage that might have caused the lesion. An evaluation for thrombophilia revealed that the patient was heterozygous for the common prothrombin mutation. She had had a postpartum deep venous thrombosis years earlier.

The constellation of signs and symptoms correlated entirely with the location of and physiological disturbances associated with the lesion — the syndrome of aortic pseudocoarctation. The amenability of the lesion to surgery, the complete correction of the hypertension, and the unsuspected thrombophilia were striking aspects of the case.

Majo Joseph, M.B., B.S.
Yves Leclerc, M.D.
Stuart J. Hutchison, M.D., C.M.
St. Michael's Hospital, Toronto, ON M5B 1W8, Canada
stuart.hutchison@utoronto.ca

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