Mid aortic syndrome is rare cause of hypertensive urgency in children with poor outcome if left untreated, high index of suspicion with prompt management is the key to survival with good outcome. A 12-year-old boy was presented with fever, puffiness of face, and breathing difficulty. Clinically, he had hypertension with differential pulsation and BP in upper and lower limbs. He had peak systolic gradient of 80 mm Hg between upper and lower limb. His echocardiography and CT angiography was suggestive of significant isolated 80% narrowing of abdominal aorta without involvement any other large vessels. Percutaneous balloon dilatation of aorta was done considering multiple parameters. Post procedure, there was significant improvement in BP and we could wean his multiple anti-hypertensive drugs to keep his blood pressures below 95th centile. His BP remained control with minimum upper and lower limb gradient on follow up of almost 1 year.
Key message: Mid aortic syndrome is most uncommon amongst them. With prompt diagnosis and proper selection of therapeutic options like balloon dilatation or surgical correction, it has good prognosis. Aortic narrowing because of different diseases is an uncommon cause of HT urgency in children.
D'Souza SJ, Tsai WS, Silver MM, Chait P, Benson LN, Silverman E, et al. Diagnosis and management of stenotic aorto-arteriopathy in childhood. J Pediatr. 1998; 132 Suppl 6:1016–1022.
Graham LM, Zelenock GB, Erlandson EE, Coran AG, Lindenauer SM, Stanley JC. Abdominal aortic coarctation and segmental hypoplasia. Surgery. 1979; 86 Suppl 4:519–529.
Onat T, Zeren E. Coarctation of the abdominal aorta. Review of 91 cases. Cardiologia. 1969; 54 Suppl 3:140–157.
Senning A, Johansson L. Coarctation of the abdominal aorta. J Thorac Cardiovasc Surg. 1960; 40:517–523.
Ellis D, Shapiro R, Scantlebury VP, Simmons R, Towbin R. Evaluation and management of bilateral renal artery stenosis in children: a case series and review. Pediatr Nephrol Berl Ger. 1995; 9 Suppl 3:259–267.
Delis KT, Gloviczki P. Middle aortic syndrome: from presentation to contemporary open surgical and endovascular treatment. Perspect Vasc Surg Endovasc Ther. 2005; 17 Suppl 3:187–203.
Cohen JR, Birnbaum E. Coarctation of the abdominal aorta. J Vasc Surg. 1988; 8 Suppl 2:160–164.
Mickley V, Fleiter T. Coarctations of descending and abdominal aorta: long-term results of surgical therapy. J Vasc Surg. 1998; 28 Suppl 2:206–214.
Connolly JE, Wilson SE, Lawrence PL, Fujitani RM. Middle aortic syndrome: distal thoracic and abdominal coarctation, a disorder with multiple etiologies. J Am Coll Surg. 2002; 194 Suppl 6:774–781.
Sharma S, Bahl VK, Saxena A, Kothari SS, Talwar KK, Rajani M. Stenosis in the aorta caused by non-specific aortitis: results of treatment by percutaneous stent placement. Clin Radiol. 1999; 54 Suppl 1:46–50.
Sharma S, Shrivastava S, Kothari SS, Kaul U, Rajani M. Influence of angiographic morphology on the acute and longer-term outcome of percutaneous transluminal angioplasty in patients with aortic stenosis due to nonspecific aortitis. Cardiovasc Intervent Radiol. 1994; 17 Suppl 3:147–151.
Fava MP, Foradori GB, García CB, Cruz FO, Aguilar JG, Kramer AS, et al. Percutaneous transluminal angioplasty in patients with Takayasu arteritis: five-year experience. J Vasc Interv Radiol JVIR. 1993; 4 Suppl 5:649–652.
Rao SA, Mandalam KR, Rao VR, Gupta AK, Joseph S, Unni MN, et al. Takayasu arteritis: initial and long-term follow-up in 16 patients after percutaneous transluminal angioplasty of the descending thoracic and abdominal aorta. Radiology. 1993; 189 Suppl 1:173–179.
Tyagi S, Kaul UA, Nair M, Sethi KK, Arora R, Khalilullah M. Balloon angioplasty of the aorta in Takayasu's arteritis: initial and long-term results. Am Heart J. 1992; 124 Suppl 4:876–882.
Siwik ES, Perry SB, Lock JE. Endovascular stent implantation in patients with stenotic aortoarteriopathies: early and medium-term results. Catheter Cardiovasc Interv Off J Soc Card Angiogr Interv. 2003; 59 Suppl 3:380–386.