Related Experiment Videos
Middle aortic syndrome: distal thoracic and abdominal coarctation, a disorder with multiple etiologies
John E Connolly1, Samuel E Wilson, Peter L Lawrence
1Department of Surgery, University of California, Irvine, USA.
Insights
Surgical reconstruction effectively treats middle aortic syndrome, a rare form of coarctation. Vascular reconstruction relieved severe hypertension and claudication in all eight patients studied, with long-term graft patency.
Area of Science:
- Cardiovascular Surgery
- Vascular Surgery
- Pediatric Cardiology
Background:
- Middle aortic syndrome, a rare coarctation variant (0.5%-2.0%), affects the distal thoracic or abdominal aorta.
- Causes include vasculitis (Takayasu's disease), congenital factors, and von Recklinghausen's disease.
- Distinct from common thoracic coarctation, it presents unique surgical challenges.
Observation:
- Eight patients with middle aortic coarctation experienced severe hypertension or claudication.
- Aortograms were used for diagnosis, followed by vascular reconstruction procedures.
- Follow-up ranged from 4 to 9 years post-surgery.
Findings:
- Vascular reconstructions included bypass, resection with grafting, and renal artery reanastomosis.
- All eight patients maintained graft patency.
- Successful relief of hypertension and claudication was achieved in all patients.
Implications:
- Aggressive surgical treatment is indicated for symptomatic middle aortic coarctation, even with potential disease progression.
- Revascularization offers long-term correction of renovascular hypertension and claudication.
- Despite surgical complexity due to fibrotic aortic walls, successful outcomes are achievable.
Background:
Congenital coarctation of the thoracic aorta at the ligamentum arteriosum or the aortic arch is well recognized. But a much less common variety (0.5% to 2.0%) of aortic coarctation is located in the distal thoracic aorta, or abdominal aorta, or both and is often called "middle aortic syndrome" or "mid-aortic dysplastic syndrome." These types of aortic coarctation are most often secondary to a form of granulomatis vasculitis commonly known as Takayasu's disease in this country or aortitis syndrome in Japan. No single genesis explains every case and beside vasculitis as a cause, some are thought to be congenital in origin and others are associated with von Recklinghausen's disease.
Study Design:
Eight patients with severe hypertension or claudication secondary to middle aortic coarctation were studied with aortograms and subsequently treated by vascular reconstruction procedures.
Results:
Vascular reconstructions consisted of aortoaortic bypass, aortic resection with interposed grafting, reanastomotic resection of renal arteries into prosthetic grafts, and renal artery bypass with autogenous material. All eight patients' grafts have remained patent, with followups of 4 to 9 years, with relief of hypertension and claudication. Although Takayasu's disease can be progressive, aggressive surgical treatment in eight patients followed for 4 to 8 years postoperatively demonstrates that severe hypertension, claudication, or both are important indications for revascularization.
Conclusions:
Whatever the cause, assuming that active aortic inflammation has been medically treated and is in a burned-out state, patients with abdominal coarctation who have symptomatic renovascular hypertension, claudication, or both are good candidates for revascularization. Although surgical repair is more difficult than with congenital thoracic coarctation, because aortic walls are fibrotic and often also involve the renals, all eight of our patients had successful longterm correction of their hypertension and coarctation.