Complex aortic coarctation and PHACE syndrome.
In PHACE syndrome, the acronym PHACE stands for the association of posterior fossa malformations, cervicofacial hemangiomas, arterial anomalies, coarctation and eye anomalies. We report our findings in four patients with this syndrome, in whom it was characterized by complex aortic coarctation that required not only preoperative echocardiographic investigation, but also the use of techniques such as magnetic resonance imaging and angiography. Surgical treatment was also complex. Prognosis in this condition depends primarily on cardiovascular and cerebral artery complications associated with the syndrome.
Servicio de Cardiología, Hospital Sant Joan de Déu, Esplugues de Llobregat, Barcelona, Spain. fprada@hsjdbcn.org
Fredy
F
Prada
Carlos
C
Mortera
Joaquim
J
Bartrons
Miguel
M
Rissech
Lorenzo
L
Jiménez
Juan
J
Carretero
Carlota
C
Rovira
María A
MA
Vicente
D001017:Aortic Coarctation / Q000150:complications / Q000175:diagnosis / Q000209:etiology*; D005124:Eye Abnormalities / Q000150:complications / Q000175:diagnosis; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D020752:Neurocutaneous Syndromes / Q000150:complications / Q000175:diagnosis; D012189:Retrospective Studies