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J Thorac Cardiovasc Surg 2004;128:946-948
© 2004 The American Association for Thoracic Surgery

Brief Communications

Dysphagia: An unusual presentation of giant aneurysm of the right coronary artery and supravalvular aortic stenosis in Williams syndrome

Carmelo Mignosa, MD, FETCSa,*, Salvatore Agati, MDa, Salvatore Di Stefano, MDa, Giovanni Pizzimenti, MDb, Enrico Di Maggio, MDc, Dario Salvo, MDd, Giuseppe Ciccarello, FEBCPe

a Pediatric Cardiac Surgery Unit, Azienda U.S.L. 5, Ospedale San Vincenzo, Taormina (Messina), Italy
b Cardiology Unit, Azienda U.S.L. 5, Ospedale di Milazzo, Messina, Italy
c Radiology Department, Azienda U.S.L. 5, Ospedale San Vincenzo, Taormina (Messina), Italy
d Pediatric Cardiac Intensive Care Unit, Azienda U.S.L. 5, Ospedale San Vincenzo, Taormina (Messina), Italy
e Perfusion Service Edwards Lifesciences, Milan, Italy

Received for publication April 16, 2004; accepted for publication May 13, 2004.

* Address for reprints: Carmelo Mignosa MD, FETCS, Unità Operativa di Cardiochirurgia, Presidio Ospedaliero "San Vincenzo," Contrada Sirina, 98039 Taormina (Messina), Italy
carmignosa@tiscali.it

The first 20% of the full text of this article appears below.


Dr Mignosa


Supravalvular aortic stenosis (SVAS) is typically associated with Williams syndrome. We describe the case of a mentally retarded 56-year-old man with Williams syndrome in whom giant aneurysm of the right coronary artery was an incidental finding during investigations for dysphagia.


   Clinical summary
 
A 56-year-old patient with dysphagia was admitted to our hospital for evaluation. On medical history, he was found to have neurodevelopmental delay and was receiving antihypertensive therapy. An extrinsic stenosis on the midportion of the esophagus did not allow us to complete full gastroenteral screening. Computed tomographic scanning showed the presence of a mediastinal mass 11 cm in diameter compressing the esophagus (Figure 1), which was diagnosed as an isolated aneurysm of the right sinus of Valsalva. The echocardiogram showed the presence of an SVAS with a gradient of 70 mm Hg, and the pulmonary trunk and bifurcation were normal. Angiography showed normal peripheral pulmonary vascularization and normal left coronary artery anatomy. The right coronary artery at its origin from the aorta opened up in a huge dilatation . . . [Full Text of this Article]






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