Gomase Shantanu, Kuthe Sachin, Sonkusale Manish
Dept of Pediatrics, Jawaharlal Nehru Medical College, DMIMS, Sawangi (M), India.
Department of Cardiovascular and Thoracic Surgery, Jawaharlal Nehru Medical College, DMIMS, Sawangi (M), India.
Int J Surg Case Rep. 2022 Aug;97:107438. doi: 10.1016/j.ijscr.2022.107438. Epub 2022 Jul 21.
Myxoma is the commonest cardiac tumor in adults but is very rare in a newborn. The majority of myxoma arises from the interatrial septum. 75% of myxomas are found in the left atrium and 20% are found in the right atrium. Myxomas can be single or multiple. Multiple myxomas are usually associated with familial syndromes. Myxomas are generally not associated with another congenital heart defect except atrial septal defect. As myxomas are rare in newborns most of the knowledge is through various case reports.
We report a rare case of an 8-day-old neonate presenting with cyanosis and respiratory distress. Transthoracic echocardiography was suggestive of right atrial myxoma which was arising from the atrioventricular junction on the right side of an interatrial septum. It was associated with the total anomalous pulmonary venous connection. The patient underwent successful excision of myxoma and rerouting of the common venous chamber to the left atrium with an uneventful immediate postoperative course.
Cardiac myxomas are rare in newborns and their association with TAPVC even rare. Our patient had single atrial myxoma with TAPVC. The significance of this association is not clear. This association is mostly by chance. Probably it is the first case reported of its kind.
As myxomas are very rare in neonates, it is highly possible to miss other associated structural heart defects. Diagnosis of atrial myxoma can be suggested by echocardiogram but a definite diagnosis can only be confirmed by histology.
黏液瘤是成人最常见的心脏肿瘤,但在新生儿中极为罕见。大多数黏液瘤起源于房间隔。75%的黏液瘤位于左心房,20%位于右心房。黏液瘤可为单发或多发。多发黏液瘤通常与家族综合征相关。黏液瘤一般不与除房间隔缺损以外的其他先天性心脏缺陷相关。由于黏液瘤在新生儿中罕见,大部分知识来源于各种病例报告。
我们报告一例罕见的8日龄新生儿,表现为发绀和呼吸窘迫。经胸超声心动图提示右心房黏液瘤,起源于房间隔右侧的房室交界处。它与完全性肺静脉异位连接相关。患者成功切除黏液瘤,并将共同静脉腔改道至左心房,术后即刻过程平稳。
心脏黏液瘤在新生儿中罕见,其与完全性肺静脉异位连接的关联更为罕见。我们的患者为单发心房黏液瘤合并完全性肺静脉异位连接。这种关联的意义尚不清楚。这种关联大多是偶然的。这可能是首例此类报告病例。
由于黏液瘤在新生儿中非常罕见,很有可能漏诊其他相关的结构性心脏缺陷。心房黏液瘤的诊断可通过超声心动图提示,但明确诊断只能通过组织学证实。