Author + information
- Diego Porras,
- Debroah Stein,
- Michael Ferguson,
- Gulraiz Chaudry,
- Ahmad Alomari,
- Khashayar Vakili,
- Steven J. Fishman,
- James Lock and
- Heung B. Kim
Midaortic syndrome (MAS) is associated with severe hypertension, which can be refractory to medical management and associated with significant morbidity and mortality.
We describe our 30-year experience with multidisciplinary management of patients with MAS.
Fifty-three patients were included with a median age at presentation of 6.7 (birth – 28.7) years. All patients underwent medical management and 35 patients (66%) underwent invasive management (percutaneous techniques: 21; surgical techniques: 5; both: 9). At most recent follow-up, the majority of the patients (67%) were normotensive. The median duration between the 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). Percutaneous interventions were acutely successful in decreasing the gradient across the obstruction and % luminal stenosis. However, freedom from reintervention was only 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.
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 the majority of patients with MAS.
Poster Sessions, Expo North
Sunday, March 10, 2013, 3:45 p.m.-4:30 p.m.
Session Title: Congenital Cardiology Solutions: Aortic Disease
Abstract Category: 13. Congenital Cardiology Solutions: Pediatric
Presentation Number: 1246-120
- 2013 American College of Cardiology Foundation