Midaortic syndrome: 30 years of experience with medical, endovascular and surgical management

Abstract

Background Midaortic syndrome is often associated with refractory hypertension. Our aim was to better understand the short and medium-term outcomes in this patient population utilizing a multidisciplinary management approach. Methods We conducted a review of patients with midaortic syndrome treated at our institution over the past 30 years. Results Fifty-three patients presented at a median age of 6.7 (birth – 28.7) years. Thirty-five patients (66%) underwent invasive management (percutaneous techniques: 21; surgical techniques: 5; both: 9). Percutaneous interventions were acutely successful in decreasing the gradient across the obstruction and degree of luminal stenosis. However, freedom from reintervention was 58% at 1 year and 33% at 5 years. Freedom from reintervention after a surgical procedure was longer: 83% at 1 year and 72% at 10 years. At most recent follow-up, the majority of patients (69%) were normotensive. The median duration between time of presentation and achievement of blood pressure control was 5.7 (0.4 – 21.1) years. The median number of anti-hypertensive medications was 1 (0 – 5). Conclusions A multidisciplinary management strategy, which couples comprehensive medical management with catheter-based and surgical interventions, can lead to adequate blood pressure control and preservation of end-organ function in patients with midaortic syndrome.

The fate of a thoracoabdominal aortic bypass graft 10 years after surgery in a child with the middle aortic syndrome

“…[1][2][3][4] Our case was normotensive without antihypertensive medication through a period of nine years. Thus, the current case emphasized the long-term effectiveness of the thoracoabdominal bypass grafting on the control of hypertension, which led to a substantial period to pass in an asymptomatic and normotensive state.…”

“…[2,4] Additionally, while freedom from reintervention was reported to be 33% for the endovascular group at five years, it was 72% in the surgery group for 10 years. [2] In the article of Hetzer et al, [1] 14 children with a mean age of 6.7 years with the diagnosis of MAS were operated through extra-anatomic bypass grafting (descending-to-abdominal aortic bypass in two patients and ascending-to-abdominal aortic bypass in 12 patients). There was no reoperation or endovascular intervention for graft-related issues.…”

“…[1][2][3][4] In comparison to surgery, the long-term success of medical and/or endovascular treatment has of little value in which, freedom from reintervention for the endovascular and surgery groups for one year were 58% and 83%, respectively. [2,4] Additionally, while freedom from reintervention was reported to be 33% for the endovascular group at five years, it was 72% in the surgery group for 10 years.…”

“…[1][2][3][4] In comparison to surgery, the long-term success of medical and/or endovascular treatment has of little value in which, freedom from reintervention for the endovascular and surgery groups for one year were 58% and 83%, respectively. [2,4] Additionally, while freedom from reintervention was reported to be 33% for the endovascular group at five years, it was 72% in the surgery group for 10 years. [2] In the article of Hetzer et al, [1] 14 children with a mean age of 6.7 years with the diagnosis of MAS were operated through extra-anatomic bypass grafting (descending-to-abdominal aortic bypass in two patients and ascending-to-abdominal aortic bypass in 12 patients).…”

“…[1][2][3][4] In this article, we discuss the long-term durability of a thoracoabdominal aortic bypass graft in a child with MAS in which endovascular stenting showed a partial benefit due to the outgrowth.…”