Terauchi Yasunobu, Tashiro Miwa, Kubo Toru, Nakashima Yasuteru, Ueda Motoko, Baba Yuichi, Yamasaki Naohito, Matsumura Yoshihisa, Furuno Takashi, Kitaoka Hiroaki, Doi Yoshinori
Department of Medicine and Geriatrics, Kochi Medical School, Kohasu, Oko-cho, Nankoku-shi, Kochi 783-8505, Japan.
Postgraduate Clinical Training Center, Kochi Medical School, Kochi, Japan.
J Cardiol Cases. 2012 Jun 23;6(3):e81-e83. doi: 10.1016/j.jccase.2012.05.009. eCollection 2012 Sep.
A 76-year-old female taking oral medications for chronic atrial fibrillation and hypertension was admitted to our hospital for examination of bilateral leg edema. Transthoracic echocardiography showed an enlarged coronary sinus of about 40 mm. Contrast-enhanced computed tomography revealed absence of the left innominate vein, a persistent left superior vena cava, and absence of the hepatic segment of the inferior vena cava. The interrupted inferior vena cava connected with the hemiazygos vein. The left superior vena cava also continued to the hemiazygos vein with reflux to the enlarged coronary sinus. A diagnosis of heterotaxy syndrome associated with polysplenia was made. No other congenital cardiac abnormalities were found. The patient was also diagnosed as having right cardiac failure due to atrioventricular valve regurgitation associated with chronic atrial fibrillation. The bilateral leg edema improved rapidly with oral medications. Although complex venous malformations such as those described above are rarely observed in the elderly, absence of the hepatic segment of the inferior vena cava, as in this case, is a particularly pathognomonic malformation in left isomerism in heterotaxy syndrome.
一名76岁女性因慢性心房颤动和高血压正在服用口服药物，因双侧腿部水肿入院接受检查。经胸超声心动图显示冠状窦扩大，约40毫米。增强计算机断层扫描显示左无名静脉缺如、永存左上腔静脉以及下腔静脉肝段缺如。中断的下腔静脉与半奇静脉相连。左上腔静脉也延续至半奇静脉并反流至扩大的冠状窦。诊断为与多脾相关的内脏异位综合征。未发现其他先天性心脏异常。该患者还被诊断为因慢性心房颤动相关的房室瓣反流导致右心衰竭。口服药物后双侧腿部水肿迅速改善。虽然上述复杂的静脉畸形在老年人中很少见，但如此病例中的下腔静脉肝段缺如，是内脏异位综合征左位异构中一种特别具有诊断意义的畸形。
