CAJ | 학술논문

目的：探讨婴儿型左冠状动脉异常起源于肺动脉（ALCAPA）的外科治疗经验。方法2006年1月至2011年12月，23例经心脏超声诊断为婴儿型ALCAPA患者得到手术治疗，年龄82～360 d，体重4.5～9.5 kg。左心室射血分数（EF 值）23%～45%，临床出现心功能不全症状。合并二尖瓣轻度反流8例，中度反流8例，中-重度反流7例。手术中将左冠状动脉从肺动脉游离后，直接将左冠状动脉移植到升主动脉4例，用部分肺动脉干和主动脉壁或心包片作左冠状动脉延伸与升主动脉吻合16例，应用肺动脉内通道术（Takeuchi方法）3例。5例合并二尖瓣重度反流者同期作瓣环环缩整形。术毕3例安装左心室辅助装置（LVAD）。结果术毕经食道超声检查，左冠状动脉血流通畅。左心室明显增大，二尖瓣中度反流2例，轻-中度12例，轻度9例。18例患者（包括3例术中安装LVAD者），经治疗在循环稳定后出院。死亡5例（死亡率21.7%），1例在术后48 h发生室颤，经心肺复苏后入手术室安置LVAD，因弥散性血管内凝血导致多脏器功能衰竭死亡。3例分别在术后12 h、32 h和48 h因突发心室颤动猝死。1例术后72 h二尖瓣反流加重，并出现多脏器衰竭而死亡。18例出院者术后随访2～5年，左冠状动脉血流均通畅。1例Takeuchi方法术后4年因肺动脉干右心室流出道梗阻而再次手术。超声提示二尖瓣轻度反流7例，轻-中度反流9例，中度反流2例。心功能EF值47%～65%，活动良好。结论婴儿型ALCAPA远期预后良好，但术后早期死亡率较高。即使有术中良好的心肌保护措施，术后仍易发生严重心律失常、心功能衰竭。早期确诊并尽早手术以免EF值明显下降，全面评估心功能并及时安置LVAD是降低死亡率的有效方法。
목적：탐토영인형좌관상동맥이상기원우폐동맥（ALCAPA）적외과치료경험。방법2006년1월지2011년12월，23례경심장초성진단위영인형ALCAPA환자득도수술치료，년령82～360 d，체중4.5～9.5 kg。좌심실사혈분수（EF 치）23%～45%，림상출현심공능불전증상。합병이첨판경도반류8례，중도반류8례，중-중도반류7례。수술중장좌관상동맥종폐동맥유리후，직접장좌관상동맥이식도승주동맥4례，용부분폐동맥간화주동맥벽혹심포편작좌관상동맥연신여승주동맥문합16례，응용폐동맥내통도술（Takeuchi방법）3례。5례합병이첨판중도반류자동기작판배배축정형。술필3례안장좌심실보조장치（LVAD）。결과술필경식도초성검사，좌관상동맥혈류통창。좌심실명현증대，이첨판중도반류2례，경-중도12례，경도9례。18례환자（포괄3례술중안장LVAD자），경치료재순배은정후출원。사망5례（사망솔21.7%），1례재술후48 h발생실전，경심폐복소후입수술실안치LVAD，인미산성혈관내응혈도치다장기공능쇠갈사망。3례분별재술후12 h、32 h화48 h인돌발심실전동졸사。1례술후72 h이첨판반류가중，병출현다장기쇠갈이사망。18례출원자술후수방2～5년，좌관상동맥혈류균통창。1례Takeuchi방법술후4년인폐동맥간우심실류출도경조이재차수술。초성제시이첨판경도반류7례，경-중도반류9례，중도반류2례。심공능EF치47%～65%，활동량호。결론영인형ALCAPA원기예후량호，단술후조기사망솔교고。즉사유술중량호적심기보호조시，술후잉역발생엄중심률실상、심공능쇠갈。조기학진병진조수술이면EF치명현하강，전면평고심공능병급시안치LVAD시강저사망솔적유효방법。
Objective To review our experience with the surgical repair of infant-type anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). Methods From Jan. 2006 to Dec. 2011, there were 23 patients with ALCAPA, mean age was 193 days (range, 82-360 days), mean weight was 6.2 kg (range, 4.5-9.5 kg), underwent surgical repair at our medical center. Diagnosis was established by echocardiography in all patients. All were clinical symptomatic of the severe heart dysfunction and left ventricle ejection fraction(EF) were 23%-45%. Mitral regurgitation was present in all patients:mild in 8 patients, moderate in 8 patients, and moderate to severe in 7 patients. Surgical methods were selected based on the location of the anomalous left coronary artery origin from the pulmonary trunk and the distance between the left coronary artery and the aorta. Major operative methods included the intrapulmonary tunnel (Takeuchi procedure) in 3 cases, direct reimplantation of the left coronary artery onto the aorta in 4 cases and reimplantation by pericardiac patch enlarge in 16 cases. Valvuloplasty were performed in 5 patients with mitral severe regurgitation. Concomitant left ventricular assistance device(LVAD) was used in 3 patients at the end of surgical repair. Results Postoperatively, transesophageal echocardiography demonstrated that blood flow of the left coronary artery were fluently and left ventricular were also enlargement. Mitral regurgitation were mild in 9 patients, mild to moderate in 12 patients and moderate in 2 patients. Hemodynamic were stable and discharged through therapy in 18 patients (included 3 patients with LVAD installed). There were five hospital deaths (21.7%), ventricular fibrillation was occurred in one patient 48 hours postoperatively, return to sinus rhythm by cardiopulmonary resuscitation and LVAD was installed at operation room immediately, but died with multi-organ failure as a result of diffuse intravascular coagulation disorders. There were sudden death in 3 patients due to ventricular fibrillation 12 hours, 32 hours and 48 hours after operation respectively. Severity of mitral regurgitation was occurred and aggravated at the time of 72 hours after surgery in 1 patient, and died of related complications. 18 survival with a median follow-up of 3.5 years (range, 2 to 5 years). Reoperations was performed for one patient with the supravalvar pulmonary stenosis due to the Takeuchi procedure 4 years postoperatively. Echocardiographic demonstrated the improvement in left ventricular function in all patients. None of the patients showed any evidence of significant residual mitral insufficiency. Mitral regurgitation was mild in 7, mild to moderate in 9 and moderate in 2. EF value were 47%-65%. All patients were in good condition. Conclusions Although late results are satisfactory and left ventricular function always recovery, early mortality is higher even though the protective methods are carried out during the whole cardiopulmonary bypass procedure. In order to decrease the early mortality, early diagnosis and cardiac support technique such as LVAD should be used as an effective method to prevent heart failure in time.