UnrepairedTetralogyofFallotinAdulthood成人未修复的法洛四联症_NEJM音频听写

A29-year-oldmanwhohadrecentlyimmigratedtotheUnitedStatespresentedtothecardiologyclinicwithworseningexertionaldyspnea,visualblurring,andheadaches.Thesesymptomshadbeenpresentsincechildhood,buthehadnotreceivedregularmedicalcareandtheconditionhadnotbeendiagnosed.Thepatienthadfoundthathissymptomslessenedwhenhesquattedorafter30minutesofrest.Theoxygensaturationwas92%whilethepatientwasbreathingambientair.Thephysicalexaminationwasnotableforaharshholosystolicmurmurattheleftsternalborderwithasternalheave.Therewasnoevidenceofdigitalclubbing.Electrocardiographyshowedsinusrhythmwitharightbundle-branchblock.Laboratorystudiesshowedahemoglobinlevelof20gperdeciliter.Echocardiographyshowedtheaortaoverridingalargeventricularseptaldefectandrightventricularhypertrophy,alongwithsubpulmonicstenosis,allofwhichconfirmedthediagnosisoftetralogyofFallot.Thecharacteristicmurmurisduetorightventricularoutflowobstruction.Squattingmaneuversincreasesystemicvascularresistance,resultinginreversalofshuntingattheventricularseptaldefectandthereforeareductioninsymptoms.Thepatientunderwentsurgicalrepairincludingpulmonaryvalvotomy,closureoftheseptaldefect,resectionofmusclebundlesintherightventricularoutflowtract,andpatchaugmentationoftheinfundibulumandmainpulmonaryartery.Twomonthslater,hewasrecoveringwellwithoutfurtherepisodesofdyspnea,visualdisturbance,orheadache.

一名新近移民美国的29岁男子因劳累性呼吸困难、视力模糊和头痛就诊。患者自童年起已存在上述症状，但从未诊治。病人下蹲或休息30分钟后症状减轻。呼吸空气时，患者血氧饱和度为92％。体格检查发现位于胸骨左缘粗糙的全收缩期杂音伴有胸骨隆起。无杵状指。心电图示窦性心律、右束支传导阻滞。实验室检查显示血红蛋白20g/dl。超声心动图示主动脉骑跨、室间隔缺损和右心室肥大、及肺动脉瓣下狭窄，这些均支持法洛四联症的诊断。其特征性杂音是由右心室流出道阻塞所致。下蹲会增加血管阻力，导致室间隔缺损处的分流逆转，从而减轻症状。该患者进行了外科手术修复，包括肺动脉瓣切开术、室间隔缺损闭合术、右心室流出道肌束切除术及右心室漏斗部和主要肺动脉修补扩张术。两个月后，患者恢复良好，无呼吸困难、视力障碍或头痛发作。

资料选自

JohnDickey,M.D.,andChristopherPhelan,M.D.NEnglJMed;:e97

NEJM

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