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

Pediatr Nephrol. 2013 Oct;28(10):2023-33. doi: 10.1007/s00467-013-2514-8. Epub 2013 Jun 18.

Abstract

Background: Midaortic syndrome is often associated with refractory hypertension. The aim of our study 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 years (birth to 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 the 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.

MeSH terms

  • Adolescent
  • Adult
  • Antihypertensive Agents / adverse effects
  • Antihypertensive Agents / therapeutic use*
  • Aorta, Abdominal / drug effects*
  • Aorta, Abdominal / physiopathology
  • Aorta, Abdominal / surgery*
  • Aortic Diseases / diagnosis
  • Aortic Diseases / drug therapy
  • Aortic Diseases / physiopathology
  • Aortic Diseases / surgery
  • Aortic Diseases / therapy*
  • Arterial Occlusive Diseases / diagnosis
  • Arterial Occlusive Diseases / drug therapy
  • Arterial Occlusive Diseases / physiopathology
  • Arterial Occlusive Diseases / surgery
  • Arterial Occlusive Diseases / therapy*
  • Arterial Pressure / drug effects
  • Boston
  • Child
  • Child, Preschool
  • Combined Modality Therapy
  • Constriction, Pathologic
  • Endovascular Procedures* / adverse effects
  • Female
  • Humans
  • Hypertension / diagnosis
  • Hypertension / drug therapy
  • Hypertension / physiopathology
  • Hypertension / surgery
  • Hypertension / therapy*
  • Infant
  • Infant, Newborn
  • Kaplan-Meier Estimate
  • Male
  • Retrospective Studies
  • Syndrome
  • Time Factors
  • Treatment Outcome
  • Vascular Surgical Procedures* / adverse effects
  • Young Adult

Substances

  • Antihypertensive Agents