Surgical management of midaortic syndrome in children with renovascular hypertension: Over a quarter of century experience

Abstract

Background

If left untreated, mid-aortic syndrome with renovascular hypertension can lead to life-threatening complications due to refractory hypertension. We aimed to analyze the medium- and long-term outcomes in a pediatric population that underwent surgery over the past 25 years.

Methods

A review of eight children with mid-aortic syndrome resulting in renovascular hypertension due to renal arterial stenosis has been conducted over the past 25 years. Children diagnosed with Takayasu arteritis were excluded. Primary outcomes were survival, reoperations, and interventional procedures.

Results

Eight children, with a median age of 12.5 years (ranging from 4 to 17 years old), were included in the study period. One patient had Neurofibromatosis type I, while another was diagnosed with Williams-Beuren syndrome. Seven children had involvement of one or both renal arteries, and most of them were affected by visceral abdominal aortic branches: celiac trunk (n = 3) and/or superior mesenteric artery (n = 2), which required reimplantation. The procedure to alleviate the stenotic abdominal aortic segment employed a lateral-to-end aorto-aortic bypass tube graft, with or without renal or visceral arterial bypass. There were no early or late deaths during the follow-up period. One patient experienced a postoperative thrombosis of the venous graft between the aortic bypass graft and the right renal artery, necessitating a thrombectomy. One patient required dilation of the right renal artery due to stenosis in the reimplanted artery 18 months after the initial repair, while another patient needed dilation of the superior mesenteric artery at the anastomosis with the saphenous vein graft after 19 months. In the remaining patients, imaging revealed no significant residual stenosis, and all patients were tapered off anti-hypertensive medications by the last follow-up appointment.

Conclusions

Surgical repair of the mid-aortic syndrome can be performed with low operative and late morbidity in experienced hands. Long-term follow-up is associated with complete resolution of renal hypertension. Patients require regular follow-up because of the risk of visceral aortic branch and renal artery anastomotic restenosis. However, revascularization using short saphenous vein graft segments is durable, and if stenosis occurs, it can be easily managed percutaneously.