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You are here: Contents > 2012 > Volume 21 Number 3 May 2012 > MISCELLANEOUS > Postoperative Cardiac Homograft Involvement in Erdheim-Chester Disease

Postoperative Cardiac Homograft Involvement in Erdheim-Chester Disease

Amulya T. Siram, Tasha Kouvatsos, Yvelisse Suarez, Alexander Wohler, Paul Stelzer, James Strauchen, Jeffrey I. Mechanick

Division of Endocrinology, Diabetes and Bone Diseases, Department of Pathology and Department of Cardiothoracic Surgery, Mount Sinai Medical Center, New York, NY, Department of Internal Medicine, Thomas Jefferson University Hospital, Philadelphia, PA, Department of Cardiothoracic Surgery, St. Joseph’s Regional Medical Center, Paterson, NJ, USA

Erdheim-Chester disease (ECD) is a rare multisystem disorder which is known to affect the skin, lungs, bone, pituitary gland, retroperitoneum and cardio-vascular system. The case is described of a patient with ECD who had previously undergone a Ross procedure for presumed endocarditis involving the aortic valve and aortic root. The patient subsequent-ly developed arthralgias, abdominal pain
(requiring an exploratory laparotomy) and polydip-sia. Furthermore, he developed progressive, sympto-matic stenosis of the pulmonic homograft.

A reoperative replacement of the homograft was required. The clinically suspected diagnosis of ECD was confirmed by a pathologic analysis of the explanted pulmonary homograft, and also (retrospectively) of previously resected mesenteric tissue. It is postulated that the patient may have developed ECD as a result of an immunologic reaction to the homograft tissue used for the
Ross procedure.­
The Journal of Heart Valve Disease 2012;21:401-404

Postoperative Cardiac Homograft Involvement in Erdheim-Chester Disease

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