title: Midaortic syndrome: 30 years of experience with medical, endovascular and surgical management.
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.
authors.affiliation: Department of Cardiology, Boston Children's Hospital and Harvard Medical School, Boston, MA 02115, USA. diego.porras@childrens.harvard.edu
authors.forename: Diego
authors.initials: D
authors.lastname: Porras
authors.forename: Deborah R
authors.initials: DR
authors.lastname: Stein
authors.forename: Michael A
authors.initials: MA
authors.lastname: Ferguson
authors.forename: Gulraiz
authors.initials: G
authors.lastname: Chaudry
authors.forename: Ahmad
authors.initials: A
authors.lastname: Alomari
authors.forename: Khashayar
authors.initials: K
authors.lastname: Vakili
authors.forename: Steven J
authors.initials: SJ
authors.lastname: Fishman
authors.forename: James E
authors.initials: JE
authors.lastname: Lock
authors.forename: Heung B
authors.initials: HB
authors.lastname: Kim
mesh_terms: D000293:Adolescent; D000328:Adult; D000959:Antihypertensive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D001012:Aorta, Abdominal / Q000187:drug effects* / Q000503:physiopathology / Q000601:surgery*; D001018:Aortic Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology / Q000601:surgery / Q000628:therapy*; D001157:Arterial Occlusive Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology / Q000601:surgery / Q000628:therapy*; D062186:Arterial Pressure / Q000187:drug effects; D001900:Boston; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003251:Constriction, Pathologic; D057510:Endovascular Procedures / Q000009:adverse effects; D005260:Female; D006801:Humans; D006973:Hypertension / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology / Q000601:surgery / Q000628:therapy*; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D012189:Retrospective Studies; D013577:Syndrome; D013997:Time Factors; D016896:Treatment Outcome; D014656:Vascular Surgical Procedures / Q000009:adverse effects; D055815:Young Adult
chemical_list: D000959:Antihypertensive Agents