Overview Snapshot A 7-year-old-boy is seen for the first time by a primary care physician. His parents report that he tires easily and often complains of weakness in his legs. Physical exam shows a healthy boy with a blood pressure of 141/91 mmHg. His lower extremities are slightly atrophic with a mottling appearance. Introduction A congenital heart defect characterized by narrowing of the lumen of the aorta usually occurs just distal to subclavian artery at the juntion of the ductus arteriosus and aortic arch leads to decreased flow distal to the constriction and increased flow proximal to it Risk factors include Turner syndrome affects males > females associated with bicuspid aortic valve (~70%) A cause of secondary HTN Presentation Symptoms patients present in childhood with asymptomatic HTN due to decreased blood flow to kidneys when symptomatic may present with headaches or blurred vision dyspnea on exertion syncope systemic hypoperfusion (shock state) without cyanosis Physical exam shows systolic BP in the upper extremities greater than in the lower extremities right arm BP may be greater than left arm BP with weak femoral and distal pulses continuous murmur over collateral vessels in the back may have machine-like murmur due to a patent ductus arteriosus lower extremity skin mottling Evaluation CXR may reveal: "reverse 3 sign" due to dilatation of the proximal and distal segments surrounding the coarctation "rib notching" due to collateral circulation through intercostals Diagnosis made by CT or aortogram Echo shows left ventricular hypertrophy (can also be seen sometimes on EKG) increased incidence of bicuspid aortic valves (70%) Differential Primary HTN, pheochromocytoma, aortic stenosis, renal artery syndrome, renal disease, Cushing's, and hyperaldostronism Treatment Surgical correction or balloon angioplasty +/- stent placement repair ASAP to prevent heart failure, CAD, and intercerebral hemorrhage Endocarditis prophylaxis indicated prior to dental work before and after surgical repair of defect Prognosis, Prevention, and Complications 25% continue to have HTN even after repair Condition often recurs