Title: Middle Aortic Syndrome (MAS) –A Rare Cause Of Hypertension In A Child with Rheumatic Heart Disease
Authors: Nasreen Ali, Sunil Kumar Agarwalla, Muhamed Shabeer, Minakhi Sahu, Nawed Momin, Jagat Shreya Satapathy, Biswajit Patanaik
DOI: https://dx.doi.org/10.18535/jmscr/v5i6.26
Abstract
The estimated prevalence of hypertension(HTN) in paediatric age group is about 2.5%.These are mostly due to secondary causes,of which renovascular causes constitute (60-70%).Middle aortic syndrome(MAS) is a rare cause of HTN.
Here we present a case of 10year old girl child, a known case of rheumatic heart disease (RHD) under treatment for carditis. She was on steroids and penicillin. She was admitted in the emergency paediatric department with complains of continued fever for 7 days. On examination the blood pressure was 180/110mm in the right arm supine position and it was thought to be steroid induced HTN. Detailed clinical examination revealed absent B\L femoral pulsation.On further investigation by MR Aortogram, a diagnosis of MAS was confirmed.MAS is a rare disease with only 200 reported cases and this is the first case report citing co-presentation of RHD and MAS.
Keywords: Renovascular cause, carditis, MR Aortogram, co-presentation of RHD and MAS
References
1. Watson NA, Chalmers N, Naqvi N. Supradiaphragmatic middle aorta syndrome--MR and angiographic imaging. The British journal of radiology. 1998 Feb;71(842):213-6.
2. Daghero F, Bueno N, Peirone A, Ochoa J, Torres GF, Ganame J. Coarctation of the abdominal aorta. Circulation: Cardiova-scular Imaging. 2008 Jul 1;1(1):e4-6.
3. Connely JE, Wilson SE, Laxrence PL, Fujitani RM. Middle aortic syndrome: Distal thoracic and abdominal coarctation, a disorder with multiple etiologies. J Am Coll Surg 2002;194:774–781.
4. Stanley JC, Criado E, Eliason JL, et al. Abdominal aortic coarctation: Surgical treatment of 53 patients with thoracoabdo-minal bypass, patch aortoplasty, or interposition aortoaortic graft. J Vasc Surg 2008;48;1073–1082.
5. Petrak B, Bendova S, Seeman T, et al. Mid-aortic syndrome with renovascular hypertension and multisystem involvement in a girl with familiar neurofibromatosis von Recklinghausen type 1. Neuro Endocrinol Lett 2007;28:734–738.
6. Sumboonnanonda A, Robinson BL, Gedroye WMW, et al. Middle aortic syndrome: Clinical and radiological findi-ngs. Arch Dis Child 1992;67:501–505.
7. Messina LM, Goldstone J, Ferrell LD, et al. Middle aortic syndrome. Effectiveness and durability of complex arterial revascularization techniques. Ann Surg 1986;204:331–339.
8. Graham LM, Zelenock GB, Erlandson EE, et al. Abdominal aortic coarctation and segmental hypoplasia. Surgery 1979;86: 519–529.
9. Onat T, Zeren E. Coarctation of the abdominal aorta. Review of 91 cases. Cardiologia 1969;54:140–157.
10. Stanley JC, Zelenock GB, Messina LM, Wakefield TW. Pediatric renovascular hypertension: A thirty-year experience of operative treatment. J Vasc Surg 1995;21: 212–226.
11. Sumboonnanonda A, Robinson BL, Gedroyc WM et-al. Middle aortic syndrome: clinical and radiological findings. Arch. Dis. Child. 1992;67 (4): 501-5. Arch. Dis. Child. (citation) - Free text at pubmed - Pubmed citation.