Rapid progression of long-segment coarctation in a patient with Williams' syndrome.
Over a period of 6 days, a three-week-old male developed a long-segment coarctation, with sub-total obstruction of the descending aorta, immediately distal to the left subclavian artery. On the 24th day of life, the stenotic region was repaired by placement of a pulmonary allograft patch measuring 3 centimetres in length. Severe diffuse vascular medial thickening was discovered at the operation. Subsequent fluorescence in-situ hybridization proved positive for Williams' syndrome. To our knowledge, this is the first report of rapidly progressive infantile arteriopathy in the setting of Williams' syndrome.
Arrington
Cammon
C
University of Utah, Department of Pediatric Cardiology, Salt Lake City, UT, USA.
Tristani-Firouzi
Martin
M
Puchalski
Michael
M
D001017:Aortic Coarctation / Q000209:etiology*; D001024:Aortic Valve Stenosis / Q000209:etiology; D018450:Disease Progression; D006801:Humans; D017380:Hypertrophy, Right Ventricular / Q000209:etiology; D007231:Infant, Newborn; D008297:Male; D018980:Williams Syndrome / Q000150:complications* / Q000235:genetics