Medical Mystery of the Week

      You have recieved your COVID19 immunizations but still participate in Telemedicine.

You are shown the chest x-ray of a 53-year-old man (see below) but given no other information. 






      What is your diagnosis, what test(s) would you order to confirm your suspicions, and what treatment, if any, do you recommend?

 DIAGNOSIS: Coarctation of the aorta (CoA) as evidenced by the "Figure of 3" as seen on the PA chest x-ray - the aorta, the constriction, and the post-constriction dilation.

                                                      ↓  dilated aortic knob 
                                                      3 ← constriction
                                                      ↑   post-stenotic dilation

      Also present on the chest x-ray are signs suggestive of pulmonary embolism (wedge-shaped infiltrates (Hampton's humps) at both lung bases, a small left pleural effusion, an elevated left hemidiaphram, and a Westermark sign (sudden cutoff) of the left main pulmonary artery). The aorta is also atherosclerotic as evidenced by the calcified plaque in the aortic knob.
      Regardless of age, the possibility of CoA should be considered in hypertensive patients with evidence of diminished/delayed femoral pulses and diminished leg blood pressures (taken with thigh blood pressure cuffs); these patients may have claudication and/or lower extremity fatigue. Additional clues to the diagnosis of CoA may include pulsatile intercostal arteries and inferior notching of ribs 3-8 on chest x-ray; a sytolic bruit over the left internal mammary artery; and/or an interscapular systolic bruit. The diagnosis of CoA should also be entertained in any patient with a bicuspid aortic valve (present in 75% of patients) or an unexplained dissection of the aorta.
      The treament of CoA is either surgical correction of the defect (most constrictions occur at the junction of the left subclavian and ligamentum arteriosum and the aortic isthmus; about 40-80% of patients have associated transverse arch hypoplasia) or percutaneous correction with covered stents or stent graphs. Strict control of hypertension is also indicated, noting that the commonest cause of death in adult patients with CoA is ischemic heart disease (there is a 75% mortality by age 43). The upper extremity hypertension is thought to result from increased left ventricular afterload coupled with diminished lower body perfusion and consequent activation of the renin-angiotension-aldosterone system.

      Suggested reference: P. Agasthi, S.H. Pujari, A. Tseng, J.N. Graziano, F. Marcotte, D. Majdalany, at. al. Management of adults with coarctation of the aorta. World J. Cardiol. 2020. 26;12(5): 167-191.