Carney Complex: Eleven Open Heart Operations in a Single Family

Herein, we report on a family with Carney complex. Four members of the family underwent a total of 11 open heart operations as well as 9 other operations to treat extrathoracic masses. All the family members met at least 2 major clinical criteria and 1 supplemental criterion. We analyzed their genom...

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Bibliographic Details
Main Authors: Sung Joon Han, Woosik Han, Min-Woong Kang, Jae Hyeon Yu, Shinkwang Kang, Myung Hoon Na
Format: Article
Language:English
Published: Korean Society for Thoracic and Cardiovascular Surgery 2017-12-01
Series:Korean Journal of Thoracic and Cardiovascular Surgery
Subjects:
Online Access:http://www.kjtcvs.org/journal/download_pdf.php?doi=10.5090/kjtcs.2017.50.6.471
Description
Summary:Herein, we report on a family with Carney complex. Four members of the family underwent a total of 11 open heart operations as well as 9 other operations to treat extrathoracic masses. All the family members met at least 2 major clinical criteria and 1 supplemental criterion. We analyzed their genomic loci, including the protein kinase A regulatory subunit 1 gene. The results revealed no specific mutations, except for a com-mon single nucleotide polymorphism. This case series of Carney complex emphasizes the importance of close longitudinal follow-up because of the high rate of tumor recurrence irrespective of the site. Clinicians should not overlook the specific features of familial myxoma.
ISSN:2233-601X
2093-6516