Thoracoabdominal Aortic Bypass for Mid-Aortic Syndrome in a 16-Year-Old Girl—A Case Report

Objectives: We report a case of a 16-year-old girl with severe arterial hypertension, who was diagnosed with mid-aortic syndrome of the abdominal aorta. Additionally a 5 mm × 3 mm cerebral aneurysm was found. The aneurysm was successfully coiled. Since blood pressures remained elevated under the medication with amlodipine, she was scheduled for aortic surgery with a lag of 4 weeks after the cerebral intervention to minimize bleeding complications. At that time, she was transferred to our unit.

Methods: The girl suffered from claudication after short time walking. She presented with high blood pressures at both upper extremities (right arm: 159/102 mm Hg, left arm: 160/99 mm Hg) with a systolic pressure gradient of 30 mm Hg to the lower extremities. Echocardiography showed good LV function and only mild hypertrophy. Pulsed Doppler spectra of the abdominal aorta was biphasic with diastolic runoff. CT scan confirmed the severely stenotic aortic segment 4 cm in length just below the diaphragm with a minimal diameter of 3 mm at its narrowest point. Both renal arteries and the superior mesenteric artery originated from the stenotic aortic segment. The celiac trunk was completely occluded at its aortic offspring and was perfused via collaterals. The patient underwent surgery, an aortoaortic bypass with a Dacron's vascular graft was inserted between descending thoracic and abdominal aorta to bypass, the stenosis and the right renal artery was transferred to the aorta more distally.

Result: Postoperative CT scan revealed unrestricted perfusion of the graft and of both renal arteries. Due to removal of lymphatic tissue during surgery, she was kept on a low fat diet until chylus production stopped. Within 3 months, blood pressure normalized with low dose atenolol. No blood pressure gradient was observed.

Conclusion: Mid-aortic syndrome is a rare condition, causing hypertension in children. Diagnostic evaluation of symptoms should always include an examination of femoral pulses, blood pressure gradients, and Doppler examination of the abdominal aorta. Depending on the length and width of the aortic stenosis and the branching visceral arteries, a thoracoabdominal bypass graft is an option to normalize blood pressure, especially when cerebrovascular abnormalities are present. Medical treatment alone is mostly ineffective and transcatheter interventions can usually only be offered if the visceral branches are not involved.

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Artikel online veröffentlicht:
21. Februar 2021