• Journal of Chest Surgery
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• v.31 no.8
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• pp.816-819
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• 1998

Myxomas are the most common form of intracardiac tumors and are found primarily in the left atrium. In rare cases, Carney and associates have described a syndrome called "the complex of myxoma" consisting of cardiac myxoma, which characteristically is familial, in assocation with two or more of the follow conditions: myxomatous masses (cardiac myxoma, cutaneous myxosma, and mammary myxoid fibroademoma), spotty pigmented lesions of the skin, and endocrine disorders. We report a case of familial atrial myxoma with Carney's complex in a 19-year old woman who has spotty pigmentations on her face, and left atrial myxomas, and myxoma on the right nipple. Her mother and sister share the left atrial myxoma. The myxomas originated in the septum of the left atrium and the anterior leaflet of the mitral valve were successfully excised. In conclusion, family members of affected patients should be screened periodically with echocardiography in an attempt to identify asymptomatic cardiac myxomas. Complete excision and postoperative follow up are necessary to rule out the muticentricity and high rate of recurrent lesions.

• Journal of Chest Surgery
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• v.50 no.6
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• pp.471-473
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• 2017

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.

• Journal of Chest Surgery
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• v.49 no.2
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• pp.119-121
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• 2016

Although cardiac myxoma is the most commonly encountered benign cardiac tumor in cardiac surgery practice, recurrent cardiac myxoma is very rare, is most commonly related to the Carney complex, and usually requires multiple cardiac operations with specific requirements in terms of perioperative management. In this report, we describe a patient who experienced the fourth recurrence of cardiac myxoma and review the diagnostic criteria of the Carney complex. This is the first report of such a case in Korea.

• The Korean journal of internal medicine
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• v.33 no.6
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• pp.1256-1257
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• 2018

• Journal of Chest Surgery
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• v.56 no.1
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• pp.42-48
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• 2023

Background: With recent advances in cardiac surgery through minimal access, mini-thoracotomy has emerged as an excellent alternative for cardiac myxoma resection. This study analyzed the surgical results of this approach, focusing on postoperative cerebral embolism and tumor recurrence. Methods: We retrospectively reviewed 64 patients (mean age, 56.0±12.1 years; 40 women) who underwent myxoma resection through mini-thoracotomy from October 2008 to July 2020. We conducted femoral cannulation and antegrade cardioplegic arrest in all patients. Patient characteristics and perioperative data, including brain diffusion-weighted magnetic resonance imaging (DWI) findings, were collected. Medium-term echocardiographic follow-up was performed. Results: Thirteen patients (20.3%) had a history of preoperative stroke, and 7 (11.7%) had dyspnea with New York Heart Association functional class III or IV. Sixty-one cases (95.3%) had myxomas in the left atrium. The mean cardiopulmonary bypass and cardiac ischemic times were 69.0±28.6 and 34.1±15.0 minutes, respectively. Sternotomy conversion was not performed in any case, and 50 patients (78.1%) were extubated in the operating room. No early mortality or postoperative clinical stroke occurred. Postoperative DWI was performed in 32 (53%) patients, and 7 (22%) showed silent cerebral embolisms. One patient underwent reoperation for tumor recurrence during the study period; in that patient, a genetic study confirmed the Carney complex. Conclusion: Mini-thoracotomy for cardiac myxoma resection showed acceptable clinical and neurological outcomes. In the medium-term echocardiographic follow-up, reliable resection was proven, with few recurrences. This approach is a promising alternative for cardiac myxoma resection.

• Journal of Chest Surgery
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• v.43 no.1
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• pp.67-72
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• 2010

Ten percent of all myxomas are the familial form. Familial myxomas appear to have autosomal dominant transmission. We experienced two siblings with familial myxomas. A left atrial myxoma was surgically removed in a 21-year-old woman. Six years later, other myxomas were found in the right atrium and the left atrium and these were also surgically removed. Right ventricular and right atrial myxomas were surgically excised in her brother. The two siblings were found to have frame-shift mutations in the PRKAR1A gene (c.537delA; p.Gly180GlufsX26), which is the causative gene for Carney complex. Obtaining the genetic diagnosis makes it possible to prepare more effective therapeutic strategies for these patients and the gene carriers. Complete excision, ruling out multicentricity and proper postoperative follow up are all necessary to avoid recurrence of myxoma.