Heart, Lung and Circulation

Primary Hypoplasia of Aortic Arch (phaa)—An Update

      Primary Hypoplasia of Aortic Arch (phaa) has been linked with hypertension (hbp) in the young adult through the association of Ohm's and Poiseuille's law relating resistance to flow in the aorta. Four patients were described last year. This is an update.
      Aim: To investigate phaa in young adults presenting with hbp.
      Method: Four new patients with hbp over the last 12 months were investigated by CT/MRI of aortic arch (aa) after exclusion of renal causes. Segmental measurements of aa were indexed to BSA as follows: (doi:10.1016/j.hlc.2008.05.474)
      Normalised Arch=14.56BSA0.543

      Z score(s.d.)=11.49(ln[arch]mm0.543ln[BSA]2.678)

      where Z < −2 = phaa and Z > −2 = normal arch.
      BP > 130 mm Hg systolic = hbp.
      Results: Two new patients with phaa were identified and are currently being observed before any decision is made for management.
      Of the four original patients described: 1. Eighteen years previous double arch in infancy had aa stenting (Z −2.5 to −0.5) with settling of hbp 140/80 to 120/80 followup two years. 2. Fourteen years Turners syndrome with hbp 140/80 stented (Z −3 to 0) followup 12 months bp 120/70. 3. Fifteen years with asymptomatic vascular ring noted phaa (Z −4.3) early in childhood and developed hbp 174/71 at puberty. Awaiting treatment. 4. Twelve years post pda coil occlusion noted hbp 160/80 (Z −3.2) awaiting management strategy.
      • 1.
        Primary Hypoplasia of Aortic Arch indexed has been recognised in 6/8 young adults with hbp. Arch measurements should be undertaken in young adults with hbp.
      • 2.
        Aortic arch stenting has resulted in drop of hbp in two patients. this appears to be a potentially good method of treatment.
      • 3.
        Observation of patients is necessary to ascertain best outcome strategies.