OBJECTIVES: To report the clinical features, nature of vasculopathy observed and the management instituted in three cases of PHACE syndrome. RESULTS: All three patients were noted to have diffuse vasculopathy and aortic arch hypoplasia in addition to aortic coarctation adding substantial surgical risk or making surgery palliative. CONCLUSION: PHACE syndrome should be considered in the differential diagnosis of aortic coarctation associated with aortic arch hypoplasia.
OBJECTIVES: To report the clinical features, nature of vasculopathy observed and the management instituted in three cases of PHACE syndrome. RESULTS: All three patients were noted to have diffuse vasculopathy and aortic arch hypoplasia in addition to aortic coarctation adding substantial surgical risk or making surgery palliative. CONCLUSION: PHACE syndrome should be considered in the differential diagnosis of aortic coarctation associated with aortic arch hypoplasia.
Authors: Michelle L Bayer; Peter C Frommelt; Francine Blei; Johannes M P J Breur; Maria R Cordisco; Ilona J Frieden; Deborah S Goddard; Kristen E Holland; Alfons L Krol; Mohit Maheshwari; Denise W Metry; Kimberly D Morel; Paula E North; Elena Pope; Joseph T Shieh; James F Southern; Orli Wargon; Dawn H Siegel; Beth A Drolet Journal: Am J Cardiol Date: 2013-09-28 Impact factor: 2.778