Complex aortic coarctation and PHACE syndrome
Fredy Prada1, Carlos Mortera, Joaquim Bartrons
1Servicio de Cardiología, Hospital Sant Joan de Déu, Esplugues de Llobregat, Barcelona, Spain. fprada@hsjdbcn.org
Revista Espanola De Cardiologia
|November 13, 2010
Summary
PHACE syndrome involves multiple anomalies, including complex aortic coarctation. Early diagnosis and advanced imaging are crucial for managing cardiovascular and arterial complications, impacting patient prognosis.
Area of Science:
- Medical Genetics
- Pediatric Cardiology
- Neuroradiology
Background:
- PHACE syndrome is a complex disorder characterized by posterior fossa malformations, cervicofacial hemangiomas, arterial anomalies, coarctation of the aorta, and eye anomalies.
- Cardiovascular involvement, particularly aortic coarctation, is a significant feature requiring thorough investigation.
Observation:
- This report details findings in four patients diagnosed with PHACE syndrome.
- Complex aortic coarctation was a prominent feature in all reported cases.
- Diagnostic workup involved echocardiography, magnetic resonance imaging (MRI), and angiography.
Findings:
- The identified aortic coarctations were complex, necessitating advanced imaging techniques beyond standard echocardiography.
- Surgical interventions for these cardiovascular anomalies were intricate.
- Prognosis is strongly linked to the management of cardiovascular and cerebral artery complications.
Implications:
- Highlights the importance of multidisciplinary assessment for PHACE syndrome.
- Emphasizes the need for advanced imaging in diagnosing and managing cardiovascular manifestations.
- Underscores the critical role of addressing arterial anomalies for improved patient outcomes.
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