Ngai Calvin, Freedberg Robin S, Latson Larry, Argilla Michael, Benenstein Ricardo J, Vainrib Alan F, Donnino Robert, Saric Muhamed
The Leon H Charney Division of Cardiology, New York University Langone Health, New York City, New York.
Department of Radiology, New York University Langone Health, New York City, New York.
Echocardiography. 2018 Oct;35(10):1684-1691. doi: 10.1111/echo.14124. Epub 2018 Aug 23.
Partial anomalous pulmonary venous return (PAPVR) comprises a group of congenital cardiovascular anomalies associated with pulmonary venous flow directly or indirectly into the right atrium. Scimitar syndrome is a variant of PAPVR in which the right lung is drained by right pulmonary veins connected anomalously to the inferior vena cava. Surgery is the definitive treatment for scimitar syndrome. However, it is not always necessary as many patients are asymptomatic, have small left-to-right shunts, and enjoy a normal life expectancy without surgery. We report multimodality imaging in four adults with scimitar syndrome and the implications for management of this rare syndrome.
部分性肺静脉异位引流（PAPVR）包括一组先天性心血管异常，其特征是肺静脉血流直接或间接流入右心房。弯刀综合征是PAPVR的一种变体，其中右肺由异常连接至下腔静脉的右肺静脉引流。手术是弯刀综合征的确定性治疗方法。然而，并非总是有必要进行手术，因为许多患者无症状，存在小的左向右分流，且未经手术也能享有正常的预期寿命。我们报告了4例患有弯刀综合征的成人的多模态成像及其对这种罕见综合征管理的意义。
