To the Editor:
We read with interest the article, “Familial Cardiac Myxoma,” written by Mahilmaran and colleagues,. 1 which described Carney's complex, which involves cutaneous pigmentation, fibromyxoid tumors of the skin, and myxomas of the heart. The syndrome has the characteristic feature of a high recurrence rate (up to 21%).
Recently, during a heart-harvesting procedure, we were evaluating the heart of a 39-year-old woman with a left atrial myxoma. The woman died of cerebral ischemia due to embolism. Echocardiography showed a left atrial myxoma (area, 6 cm2) attached to the roof of the left atrium (Fig. 1). It was clear that the woman's heart was outside the standard acceptance criteria for donation, but the organ was nonetheless considered for an urgent transplantation.
Fig. 1 Apical 4-chamber view shows a myxoma attached to the left atrial roof.
Inspection of the body showed numerous fibromyxoid tumors on the back, chest, and abdomen. The localized myxoma could have been removed easily from the heart, because the atrial roof would have been trimmed out. However, considering the presence of Carney's syndrome, we determined the risk to be too great for the heart recipient. Consequently, the heart was not harvested.
Worldwide, the waiting list for organs is long, and the criteria for acceptable donors continue to be expanded. Older donors' hearts (> 60 years) have been transplanted with results and outcomes comparable to those of grafts from younger patients. 2,3 Upon inspection, the heart is not harvested for transplantation in only 12% of multiple-organ donors. 4.
A donor heart is not considered acceptable for transplantation when malignancy with the possibility of metastasis is present. Myxomas, like other benign cardiac tumors, are associated with embolic episodes leading to neurologic symptoms and also to sudden death. In the present case, despite the elevated risk of coronary artery embolism during harvesting maneuvers, the donor heart was considered for an urgent case—but only after a frozen section of the tumor confirmed the echocardiographic diagnosis of myxoma.
There is 1 previous report in the medical literature. 5 of a donor heart that was rejected for transplantation because of a myxoma: the potential donor was a 30-year-old woman who was brain-dead as a result of an embolus originating from a mitral valve myxoma. The tumor was discovered on the anterior leaflet of the mitral valve thorough direct inspection of the heart.
In conclusion, our report confirms that careful investigation should be performed in all donors sustaining brain death from cerebral infarction, in order to determine the source of the embolus. Furthermore, in case of Carney's syndrome in a donor, the heart should be judged unsuitable for transplantation because of the risk of tumor recurrence.
Footnotes
Letters to the Editor should be no longer than 2 double-spaced typewritten pages and should contain no more than 4 references. They should be signed, with the expectation that the letters will be published if appropriate. The right to edit all correspondence in accordance with Journal style is reserved by the editors.
References
- 1.Mahilmaran A, Seshadri M, Nayar PG, Sudarsana G, Abraham KA. Familial cardiac myxoma: Carney's complex. Tex Heart Inst J 2003;30:80–2. [PMC free article] [PubMed]
- 2.Mulvagh SL, Thornton B, Frazier OH, Radovancevic B, Norton HJ, Noon GP, Young JB. The older cardiac transplant donor. Relation to graft function and recipient survival longer than 6 years. Circulation 1989;80(5 Pt 2):III126-32. [PubMed]
- 3.Ibrahim M, Masters RG, Hendry PJ, Davies RA, Smith S, Struthers C, et al. Determinants of hospital survival after cardiac transplantation. Ann Thorac Surg 1995;59:604–8. [DOI] [PubMed]
- 4.Eurotransplant International Foundation. Leiden, The Netherlands. Annual report; 1996.
- 5.de Boer HD, de Jonge N, de Boer WJ. Mitral valve myxoma: an unusual reason for rejecting a donor heart. J Heart Lung Transplant 1999;18:924–5. [DOI] [PubMed]