Title:Mid-Aortic Syndrome: A Rare Cause of Renovascular Hypertension in Childhood Treated Percutaneously with an Unusual Vascular Access
Volume: 16
Issue: 4
Author(s): Emma Diletta Stea, Giovanni Meliota, Vincenza Carbone, Diletta Torres, Luisa Santangelo, Giovanni Piscopo, Paolo Giordano, Giuseppina Annicchiarico, Ugo Vairo and Mario Giordano*
Affiliation:
- Pediatric Nephrology and Dialysis Unit, Azienda Ospedaliero Universitaria Policlinico, Pediatric Hospital Giovanni XXIII, Bari,Italy
Keywords:
Renovascular, hypertension, middle-aortic syndrome, PTRA, Anterograde access.
Abstract:
Introduction: Mid-Aortic Syndrome (MAS) is a rare vascular malformation characterized
by segmental narrowing of the abdominal aorta and stenosis of its principal branches. Patients
affected by MAS typically present malignant renovascular hypertension, with variable clinical
symptoms like claudication, abdominal angina, and headache. Moreover, they can develop other
complications, such as hypertensive encephalopathy, congestive heart failure and vascular brain accidents.
Hypertension with MAS is often resistant to multidrug therapy, requiring a surgical approach
to treat the clinical symptoms, prevent or block organ damage and normalize the blood
pressure.
Case report: Here, the case of a 4-year-old boy showing elevated blood pressure with left ventricular
hypertrophy leading to idiopathic MAS, who was successfully treated with percutaneous transcatheter
renal angioplasty (PTRA) using an unusual, anterograde access, is reported.
Discussion and Conclusion: In children and adolescents, vascular malformations like MAS must
be considered as a possible cause of hypertension. PTRA is a successful therapeutic strategy in
children with severe renovascular hypertension. Anterograde access, using an axillary artery, can
be a valid approach for PTRA when femoral access is difficult to achieve.
