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 common 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.
Carney Complex* ; Cyclic AMP-Dependent Protein Kinases ; Follow-Up Studies ; Heart* ; Humans ; Myxoma ; Polymorphism, Single Nucleotide ; Recurrence

BACKGROUND: This study aimed to develop the models for regional cardiac surgery centers, which take regional characteristics into consideration, as a policy measure that could alleviate the concentration of cardiac surgery in the metropolitan area and enhance the accessibility for patients who reside in the regions. METHODS: To develop the models and set standards for the necessary personnel and facilities for the initial management plan, we held workshops, debates, and conference meetings with various experts. RESULTS: After partitioning the plan into two parts (the operational autonomy and the functional comprehensiveness), three models were developed: the ‘independent regional cardiac surgery center’ model, the ‘satellite cardiac surgery center within hospitals’ model, and the ‘extended cardiac surgery department within hospitals’ model. Proposals on personnel and facility management for each of the models were also presented. A regional cardiac surgery center model that could be applied to each treatment area was proposed, which was developed based on the anticipated demand for cardiac surgery. The independent model or the satellite model was proposed for Chungcheong, Jeolla, North Gyeongsang, and South Gyeongsang area, where more than 500 cardiac surgeries are performed annually. The extended model was proposed as most effective for the Gangwon and Jeju area, where more than 200 cardiac surgeries are performed annually. CONCLUSION: The operation of regional cardiac surgery centers with high caliber professionals and quality resources such as optimal equipment and facility size, should enhance regional healthcare accessibility and the quality of cardiac surgery in South Korea.
Delivery of Health Care ; Education ; Gangwon-do ; Health Facilities ; Health Services Accessibility ; Humans ; Korea ; Quality of Health Care ; Thoracic Surgery*