儿童期出现严重肺动脉高压的弯刀综合征成人型
Adult Form of Scimitar Syndrome Presenting as Severe Pulmonary Hypertension in a Child.
作者信息
Kalpana Sivasambo, Balaji B Sarath, Elilarasi Selladurai, Solomon Neville A G
机构信息
Department of Pulmonology, Institute of Child Health and Hospital for Children; and *Department of Pediatric Cardiac Surgery, Apollo Childrens Hospital; Chennai, India. Correspondence to: Dr Sivasambo Kalpana, Department of Pulmonology, Institute of Child Health and Hospital for Children, Halls road, Egmore, Chennai.
出版信息
Indian Pediatr. 2015 Oct;52(10):889-90. doi: 10.1007/s13312-015-0738-3.
BACKGROUND
Scimitar syndrome is a rare association of congenital cardiopulmonary anomalies; the adult form is not usually is associated with pulmonary hypertension.
CASE CHARACTERISTICS
6-year-old girl with recurrent episodes of cough and breathlessness, along with features of right heart enlargement. Computed tomography angiogram revealed right pulmonary veins draining into inferior vena cava with dextroposition of heart.
OUTCOME
Successfully managed with surgical correction.
MESSAGE
Scimitar syndrome should be considered in any child with unexplained pulmonary hypertension and dextroposed heart.
背景
弯刀综合征是一种罕见的先天性心肺异常组合；成人型通常不伴有肺动脉高压。
病例特征
一名6岁女孩，反复出现咳嗽和呼吸急促，伴有右心扩大的表现。计算机断层血管造影显示右肺静脉汇入下腔静脉，心脏右位。
结果
通过手术矫正成功治疗。
启示
对于任何患有不明原因肺动脉高压和心脏右位的儿童，都应考虑弯刀综合征。
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