Primary hypoplasia of aortic arch - an unrecognised cause of hypertension in the juvenile

Abstract

Introduction: Hypoplasia of aorta[HPA] is usually described in association with aortic coarctation and managed accordingly . With growth secondary HPA and arch distortion can occur needing therapy. Primary HPA [PHPA] has not been fully documented before. We describe 15 patients with PHPA. Materials and methods: 15 patients Age 6-24yrs, m16.yrs, 8 M,8 F, Fu 1-24yrs. Med.13.5yrs. 10 patients with previous cardiac diagnosis 5 patients normal. All patients well, echo DAO velocity ∼2m/s or more, no clinical pressure gradients, mild hypertension[HBP] at puberty, exercise HBP response. HBP consistently noted . Cta /mri arch demonstrate Localised or multiple HPA segments. Zscores arch normograms were plotted from Kaiser et al., Jour CVMR 2008.10.56. Zscore < - 2 or less of arch segments was diagnostic of PHPA. Results: PT.1 double ao arch surgery, HBP aged 14, stented age 18yr bsa1.9m2 z score – 3-> +0.8 HBP resolved fu 6 yrs. PT 2.18yr Turners Syndrome, HBP at puberty bsa.1.6m2age 13yr stented Zscore – 3 -> 0 with hypertension resolved. Fu 5yrs. PT 3 diffuse HPA with vascular ring stented Zscore -5 → -3 with partial improvement fu 4yrs. 12 remaining patients are on observation. Conclusion: 15 patients with documented PHPA 3 have undergone stenting with good outcome [fu 4-6yr]. 12 remain well, asymptomatic. Further studies and discussions needed to document the condition further and to plan management strategies.