Status:
Ready to upload
Record number:
1790
Adverse Occurrence type:
MPHO Type:
Estimated frequency:
Very rare occurrence in a transplanted heart
Time to detection:
8 years after heart transplant
Alerting signals, symptoms, evidence of occurrence:
Routine transthoracic echocardiography revealed an entirely asymptomatic tumor (4.8 x 2.5 x 2.0 cm) in the left atrium attached to the atrial roof. Myxoma was suspected and confirmed after surgical extirpation one month later. Total tumor size was 6 x 4 cm. In the postoperative course, the patient developed an aortic dissection which spread to both iliac arteries, resulting in impaired perfusion of the visceral organs. Despite early re-operation, the patient died of hepatorenal failure.
Demonstration of imputability or root cause:
DNA analysis (recipient´s venous blood vs. tumor tissue) with two different PCRs was performed: recipient was heterozygotic for insertion/deletion variant within the ACE gene but tumor tissue lacked this insertion band. Second PCR with primers specific for the insertion confirmed the presence of an insertion allele in the recipient blood and showed this to be missing in the tumor tissue. Result confirmed a donor origin of the myxoma.
Imputability grade:
3 Definite/Certain/Proven
Groups audience:
References:
Suggest new keywords:
case report
heart transplant
DNA typing
Cardiovascular
atrial myxoma
tumor
Malignancy
Reference attachment:
Suggest references:
Dufková B, Málek I, Vymetalová Y, Hosková L, Skalský I, Pirk J, et al. Myxoma of donor origin in a transplanted heart. J Heart Lung Transplant. 2007 Aug;26(8):865–7.
Note:
first review done on August 1, 2018 (Kerstin)
Second review 8/5/18 (Mike)
Expert comments for publication:
8 years after transplant this myxoma would be classified as donor-derived but not donor-transmitted by standard approach, although it is admitted that the presence of a small lesion at the time of transplant cannot be rigorously excluded unless one knows the growth rate of this tumor. Since myxoma can lead to serious complications, such as embolization, strokes, valve obstruction etc., resection was strongly indicated in this heart recipient. The patient´s death was not caused by the myxoma itself but by complications of the resection. The authors discuss whether to accept or reject a donor with known myxoma prior to organ donation and refer to 2 reports in the literature. They conclude that it might be acceptable for urgent recipients in special cases when easy extirpation of the myxoma is possible (without valve connection, without signs of Carney complex) pretransplant.