This Article Full Text Full Text (PDF) Data Supplement Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Google Scholar Articles by Yan, B. P. Articles by de Moor, M. PubMed PubMed Citation Articles by Yan, B. P. Articles by de Moor, M. Related Collections Coronary imaging: angiography/ultrasound/Doppler/CC Pediatric and congenital heart disease, including cardiovascular surgery

(Circulation. 2009;120:181-182.)
© 2009 American Heart Association, Inc.

Images in Cardiovascular Medicine

Dynamic Left Main Coronary Artery Obstruction Associated With Williams Syndrome

Bryan P. Yan, MBBS, FRACP; Thomas J. Kiernan, MD, MRCPI; Kenneth Rosenfield, MD; Joseph M. Garasic, MD; Michael de Moor, MBBCh

From the Cardiology Division, Section of Vascular Medicine, Massachusetts General Hospital, Harvard Medical School (B.P.Y., T.J.K., K.R., J.M.G.), and Division of Pediatric Cardiology, Massachusetts General Hospital for Children, Harvard Medical School (M.d.M.), Boston, Mass.

Correspondence to Michael de Moor, MBBCh, FACC, MassGeneral Hospital for Children, CRP510, 175 Cambridge St, Boston, MA 02114. E-mail mdemoor@partners.org

An extract of the first 250 words of the full text is provided, because this article has no abstract.

A 5-year-old boy with Williams syndrome (WS) presented with a history of diffuse recurrent obstructive vascular disease. At the age of 12 months, he underwent pericardial patch augmentation of the ascending aorta and pulmonary arteries. He subsequently required Dacron patch repair of the ascending aorta and aortic arch at 3 years of age. In the interim, he had undergone percutaneous transluminal angioplasty and stenting for recurrent peripheral pulmonary artery stenoses and aortic recoarctation. On routine follow-up, he was found to have worsening right ventricular hypertension associated with disparate lung perfusion on a nuclear perfusion scan. He was brought to the catheterization laboratory for further investigation. Angiography showed recurrent pulmonary artery in-stent restenosis and aortic recoarctation. Coronary angiography demonstrated a 30% ostial right coronary stenosis and a 75% dynamic left main coronary artery (LMCA) stenosis (Figure 1 and Movie I of the online-only Data Supplement). Intravascular ultrasound was performed to further evaluate the LMCA stenosis before anticipated revascularization. After administration of intracoronary nitroglycerin, intravascular ultrasound was performed with a 2.5F Atlantis SR Pro 40-MHz catheter (Boston Scientific, Natick, Mass) by automatic pullback at 0.5 mm/s. Intravascular ultrasound revealed dynamic contraction of the LMCA without evidence of fixed obstruction (Figure 2 and Movie II of the online-only Data Supplement). Quantitative intravascular ultrasound analysis demonstrated a reduction in arterial lumen cross-sectional area from 14.8 mm² in diastole to 3.7 mm² in systole, thus indicating a 75% dynamic obstruction. Although there is no consensus on the cross-sectional area at which LMCA obstruction . . . [Full Text of this Article]