• Journal of Chest Surgery
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• 제31권8호
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• pp.816-819
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• 1998

점액종은 심장 종양의 가장 흔한 형태이며 주로 좌심방에서 발견된다. Carney 등에 의하면가 족성을 가지 며 점액종(심장, 피부, 유방), 반점상의 피부 색소침착와 내분비성 질환이 동반된 증후군을 점액종 증후군 이라 명명하였다. 저자들은 얼굴과 좌완에 피부병변이 있고 좌심방과 우측 유두에서 점액종이 발견되고 가 족력이 있는 19세 여자를 Carney 증후군을 가진 가족성 심방 점액종으로 진단하여 좌심방 중격과 승모판에 부착된 점액종을 성공적으로 절제하였다. 심장 점액종의 가족성이 있는 동일 가족 구성원은 증상이 발현되 지 않더라도 심장 점액종을 발견하기 위해 선별적 심방초음파 검사가 주기적으로 실시되어야하며 재발과 다 발성병변이므로 수술시 종양의 완전 절제와 수술후 추적검사가 필요하다.

• Journal of Chest Surgery
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• 제50권6호
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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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• 제49권2호
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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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• 제33권6호
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• pp.1256-1257
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• 2018

• Journal of Chest Surgery
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• 제56권1호
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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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• 제43권1호
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• pp.67-72
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• 2010

전체 심장 점액종의 10% 정도가 가족성이며 상염색체 우성 유전을 보인다. 저자들은 21세에 좌심방 점액종 제거술을 받고 6년 후에 우심방과 좌심방에 점액종이 재발한 여자 환자와 26세에 우심실과 우심방에 점액종이 발생하여 제거술을 받은 남동생에서 가족성 점액종의 원인이 되는 PRKAR1A 유전자의 돌연변이(c.537delA; p.Gly180GlufsX26)를 확인하였다. 유전자 검사를 통한 가족성 점액종의 진단은 효과적인 치료계획을 세우는데 도움이 된다. 가족성 점액종의 경우 처음부터 다발성으로 존재할 가능성과 잦은 재발을 고려하여 수술 시 종양의 완전 절제와 수술 후 재발에 대한 추적검사가 필수적이다.