CAJ | 학술논문

目的探讨弥漫性肺动静脉瘘(PAVFs)的临床表现、诊断和治疗方法.方法回顾性分析2例弥漫性PAVFs的病例资料,并结合国内外相关报道进行文献复习.结果 2例弥漫性PAVFs患者临床均表现为劳累性呼吸困难、紫绀和杵状指/趾等三联征.例1为女性,基础疾病为遗传性毛细血管扩张症,临床表现为反复鼻衄及面部毛细血管扩张等.例2为男性,基础疾病为肝硬化,临床表现为肝肺综合征.2例患者超声心动图均未见明显异常,超声心动图声学造影左心房显影.胸部CT显示双肺血管影增粗,胸膜下多发小结节影和磨玻璃影.肺动脉造影显示肺循环时间缩短,例2患者左下叶背段动脉分支与肺静脉直接连通,经导管行栓塞术,术后氧合无明显改善.随访5年,例1病情平稳,例2死于肝硬化,二者均无脑部并发症.结论弥漫性PAVFs临床罕见,常表现为劳累性呼吸困难、紫绀和杵状指/趾等三联征.肺动脉造影和超声心动图声学造影有助于早期诊断.
목적 탐토미만성폐동정맥루(PAVFs)적림상표현、진단화치료방법.방법 회고성분석2례미만성PAVFs적병례자료,병결합국내외상관보도진행문헌복습.결과 2례미만성PAVFs환자림상균표현위로루성호흡곤난、자감화저상지/지등삼련정.례1위녀성,기출질병위유전성모세혈관확장증,림상표현위반복비뉵급면부모세혈관확장등.례2위남성,기출질병위간경화,림상표현위간폐종합정.2례환자초성심동도균미견명현이상,초성심동도성학조영좌심방현영.흉부CT현시쌍폐혈관영증조,흉막하다발소결절영화마파리영.폐동맥조영현시폐순배시간축단,례2환자좌하협배단동맥분지여폐정맥직접련통,경도관행전새술,술후양합무명현개선.수방5년,례1병정평은,례2사우간경화,이자균무뇌부병발증.결론 미만성PAVFs림상한견,상표현위로루성호흡곤난、자감화저상지/지등삼련정.폐동맥조영화초성심동도성학조영유조우조기진단.
Objective To describe the clinical characteristics of diffuse pulmonary arteriovenous fistulas (PAVFs) and to evaluate the methods of diagnosis and treatment.Methods Retrospective analysis of two patients with diffuse PAVFs who were diagnosed in Peking University First Hospital and the literatures on the subject were reviewed.Results Both patients presented with the triad of dyspnea on exertion,cyanosis,and clubbing.Case 1 was a woman with hereditary telangiectasia disease (HHT),who also presented with repeated epistaxis and facial telangiectasia.Case 2 was a man with hepatic cirrhosis,who presented with hepatopulmonary syndrome.Echocardiographies of 2 patients were both normal,peripheral vein contrast echocardiography showed abnormal filling of the left atrium with echo contrast material.Chest CT showed bilateral intrapulmonary artery dilatation,subpleural small nodular,reticular and patchy opacities.Pulmonary angiography showed that pulmonary circulation time was both shortened.Pulmonary angiography also showed that lower left dorsal segment artery and pulmonary vein were directly connected in case 2,and then tanscatheter embolization was carried out.However,oxygenation of this patientwasn't improved.Case 1 was alive and case 2 died of liver cirrhosis after 5-year follow up,neither of them had brain complications.Conclusions Diffuse PAVFs are clinically rare and present with the triad of dyspnea on exertion,cyanosis,and clubbing.Pulmonary angiography and contrast echocardiography are helpful for diagnosis.