Kocabeyoğlu Sinan Sabit, Özyüksel Arda, Çetin Erdem
Yüksek İhtisas Cardiovascular Education and Research Hospital, Ankara, Turkey.
Department of Cardiovascular Surgery, Medipol University, Istanbul, Turkey.
J Tehran Heart Cent. 2014;9(4):191-3. Epub 2014 Jul 6.
Carney Syndrome (CS) is an autosomal dominant multiple neoplasia syndrome that includes cardiac, endocrine, cutaneous, and neural tumors. Cardiac myxomas can be seen in the course of CS. A 46-year-old female patient was admitted to our clinic with palpitation, cough, and exertional dyspnea. Physical examination revealed pigmented lesions, especially scattered in the neck, chest, and extremities. Echocardiography revealed a left atrial mass, 6 × 4 cm in size, originating from the interatrial septum that was protruding into the left ventricle through the mitral valve during diastole. The tumor was excised from the interatrial septum with its pedicle. The patient was discharged at the fifth postoperative day without any complication. Cardiac myxomas are a cornerstone of this disease since cardiac manifestations and related complications account for the majority of mortality seen in the course of CS.
卡尼综合征(CS)是一种常染色体显性遗传的多发性肿瘤综合征,包括心脏、内分泌、皮肤和神经肿瘤。心脏黏液瘤可见于CS病程中。一名46岁女性患者因心悸、咳嗽和劳力性呼吸困难入院。体格检查发现色素沉着病变,尤其散在于颈部、胸部和四肢。超声心动图显示左心房有一大小为6×4 cm的肿块,起源于房间隔,舒张期通过二尖瓣凸入左心室。肿瘤从房间隔带蒂切除。患者术后第5天出院,无任何并发症。心脏黏液瘤是该病的一个关键特征,因为心脏表现及相关并发症是CS病程中所见死亡的主要原因。