中华儿科杂志
中華兒科雜誌
중화인과잡지
Chinese Journal of Pediatrics
2014年
10期
777-782
,共6页
张程%张智伟%丁以群%王树水%庞程程%李渝芬
張程%張智偉%丁以群%王樹水%龐程程%李渝芬
장정%장지위%정이군%왕수수%방정정%리투분
心脏缺损,先天性%婴儿%冠状动脉疾病%诊断
心髒缺損,先天性%嬰兒%冠狀動脈疾病%診斷
심장결손,선천성%영인%관상동맥질병%진단
Heart defect,congenital%Infant%Coronary artery disease%Diagnosis
目的 探讨左冠状动脉起源于肺动脉(ALCAPA)患儿的临床特征及治疗策略.方法 对2006年至2013年广东省心血管病研究所收治的25例诊断为ALCAPA并行手术治疗的＜1岁患儿的临床资料进行分析,术后随访超声心动图评估心功能.结果 患儿临床特征以气促、多汗、喂养困难、生长发育落后多见.心电图示异常Q波23例,ST-T改变16例.超声心动图检查示左心室扩大25例,心内膜增厚5例,右冠动脉扩张并存在左右冠状动脉交通血流11例.左心室射血分数(LVEF) (45.5±l3.9)％(25％～77％),左心室缩短率(LVFS) (22.0±7.3)％(12％ ～ 38％).21例患儿行高速多层心脏CT检查,提示左冠状动脉开口于肺动脉瓣左后侧窦9例,有后侧窦1例,开口于肺动脉主干二下段5例,开口于肺动脉近分叉处l例,开口位置不确定5例.本组25例患儿中17例在外院首诊后转入我院,除l例在外院行心血管导管检查确诊ALCAPA外,其余16例均被误诊;8例我院首诊的病例中,误诊2例,后经超声心动图复查及心脏CT检查后修正诊断.术前根据患儿的心功能情况予以不同程度的强心、利尿、扩血管治疗.2例结扎左冠状动脉口,23例采用左冠状动脉移植技术重建双冠状动脉系统.对于心功能Ⅳ级合并中度以上二尖瓣反流或心功能Ⅲ级合并重度以上二尖瓣反流者同期行二尖瓣成形术.对术后LVEF＜ 30％者,如果应用药物无法维持血压,乳酸呈进行性升高,则施行体外膜肺氧合(ECMO)辅助.2例患儿行左冠状动脉移植术后因顽固性室颤早期死亡;存活的23例患儿,呼吸机使用时间为7 ～500 h,其中11例＜60 h.平均住院天数(23.4±13.9)d(8～65 d).中位随访时间为28.5个月(1～91个月),失访1例,因感染性休克术后3个月院外死亡.随访的22例患儿心功能均较术前改善,14例左心室大小恢复至正常,20例LVEF恢复至正常.无行2次手术者.结论 结合病史、心电图、超声心动图及其他多种影像学诊断方法有利于正确诊断ALCAPA;对于危重患儿采取个体化治疗策略,并对术后低心排患儿积极进行ECMO辅助,可显著提高手术成功率.
目的 探討左冠狀動脈起源于肺動脈(ALCAPA)患兒的臨床特徵及治療策略.方法 對2006年至2013年廣東省心血管病研究所收治的25例診斷為ALCAPA併行手術治療的＜1歲患兒的臨床資料進行分析,術後隨訪超聲心動圖評估心功能.結果 患兒臨床特徵以氣促、多汗、餵養睏難、生長髮育落後多見.心電圖示異常Q波23例,ST-T改變16例.超聲心動圖檢查示左心室擴大25例,心內膜增厚5例,右冠動脈擴張併存在左右冠狀動脈交通血流11例.左心室射血分數(LVEF) (45.5±l3.9)％(25％～77％),左心室縮短率(LVFS) (22.0±7.3)％(12％ ～ 38％).21例患兒行高速多層心髒CT檢查,提示左冠狀動脈開口于肺動脈瓣左後側竇9例,有後側竇1例,開口于肺動脈主榦二下段5例,開口于肺動脈近分扠處l例,開口位置不確定5例.本組25例患兒中17例在外院首診後轉入我院,除l例在外院行心血管導管檢查確診ALCAPA外,其餘16例均被誤診;8例我院首診的病例中,誤診2例,後經超聲心動圖複查及心髒CT檢查後脩正診斷.術前根據患兒的心功能情況予以不同程度的彊心、利尿、擴血管治療.2例結扎左冠狀動脈口,23例採用左冠狀動脈移植技術重建雙冠狀動脈繫統.對于心功能Ⅳ級閤併中度以上二尖瓣反流或心功能Ⅲ級閤併重度以上二尖瓣反流者同期行二尖瓣成形術.對術後LVEF＜ 30％者,如果應用藥物無法維持血壓,乳痠呈進行性升高,則施行體外膜肺氧閤(ECMO)輔助.2例患兒行左冠狀動脈移植術後因頑固性室顫早期死亡;存活的23例患兒,呼吸機使用時間為7 ～500 h,其中11例＜60 h.平均住院天數(23.4±13.9)d(8～65 d).中位隨訪時間為28.5箇月(1～91箇月),失訪1例,因感染性休剋術後3箇月院外死亡.隨訪的22例患兒心功能均較術前改善,14例左心室大小恢複至正常,20例LVEF恢複至正常.無行2次手術者.結論 結閤病史、心電圖、超聲心動圖及其他多種影像學診斷方法有利于正確診斷ALCAPA;對于危重患兒採取箇體化治療策略,併對術後低心排患兒積極進行ECMO輔助,可顯著提高手術成功率.
목적 탐토좌관상동맥기원우폐동맥(ALCAPA)환인적림상특정급치료책략.방법 대2006년지2013년광동성심혈관병연구소수치적25례진단위ALCAPA병행수술치료적＜1세환인적림상자료진행분석,술후수방초성심동도평고심공능.결과 환인림상특정이기촉、다한、위양곤난、생장발육락후다견.심전도시이상Q파23례,ST-T개변16례.초성심동도검사시좌심실확대25례,심내막증후5례,우관동맥확장병존재좌우관상동맥교통혈류11례.좌심실사혈분수(LVEF) (45.5±l3.9)％(25％～77％),좌심실축단솔(LVFS) (22.0±7.3)％(12％ ～ 38％).21례환인행고속다층심장CT검사,제시좌관상동맥개구우폐동맥판좌후측두9례,유후측두1례,개구우폐동맥주간이하단5례,개구우폐동맥근분차처l례,개구위치불학정5례.본조25례환인중17례재외원수진후전입아원,제l례재외원행심혈관도관검사학진ALCAPA외,기여16례균피오진;8례아원수진적병례중,오진2례,후경초성심동도복사급심장CT검사후수정진단.술전근거환인적심공능정황여이불동정도적강심、이뇨、확혈관치료.2례결찰좌관상동맥구,23례채용좌관상동맥이식기술중건쌍관상동맥계통.대우심공능Ⅳ급합병중도이상이첨판반류혹심공능Ⅲ급합병중도이상이첨판반류자동기행이첨판성형술.대술후LVEF＜ 30％자,여과응용약물무법유지혈압,유산정진행성승고,칙시행체외막폐양합(ECMO)보조.2례환인행좌관상동맥이식술후인완고성실전조기사망;존활적23례환인,호흡궤사용시간위7 ～500 h,기중11례＜60 h.평균주원천수(23.4±13.9)d(8～65 d).중위수방시간위28.5개월(1～91개월),실방1례,인감염성휴극술후3개월원외사망.수방적22례환인심공능균교술전개선,14례좌심실대소회복지정상,20례LVEF회복지정상.무행2차수술자.결론 결합병사、심전도、초성심동도급기타다충영상학진단방법유리우정학진단ALCAPA;대우위중환인채취개체화치료책략,병대술후저심배환인적겁진행ECMO보조,가현저제고수술성공솔.
Objective To investigate the clinical features and individualized treatment strategies for infants with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA).Method Data of 25 less than 1-year-old infants with ALCAPA who presented at Guangdong Cardiovascular Institute between 2006 and 2013 were retrospectively reviewed.The patients' cardiac function was evaluated with echocardiography during follow-up.Result Most patients presented with symptoms of heart failure,such as tachypnea,diaphoresis,poor feeding,failure to thrive etc.Electrocardiogram showed abnormal q wave in 23 patients and ST-T segment change in 16 patients.Echocardiography showed dilated left ventricle in 25 patients,endocardial hyperplasia in 5 patients,dilated right coronary artery and extensive collateralization between the right and left coronary artery systems in 11 patients.The left ventricular ejection fraction (LVEF) was (45.5 ± 13.9) ％ (25％-77％).The left ventricular fractional shortening (LVFS) was (22.0 ± 7.3)％ (12％-38％).Twenty one patients underwent cardiovascular CT scan.Left coronary artery originated from left posterior sinus in 9 patients,from right posterior sinus in 1 patient,from lower main pulmonary artery in 5 patients,from the bifurcation of main pulmonary artery in 1 patient.Five patients showed ambiguous left coronary artery origination.Sixteen patients were misdiagnosed in other primary or secondary hospitals in 17 patients who were transferred to our tertiary hospital,only 1 case who underwent angiography was diagnosed correctly.Two patients were misdiagnosed in 8 patients first-presented in our hospital.Their diagnoses were corrected after reexamining with echocardiography and cardiovascular CT scan.The preoperative therapies included using inotropic agents,diuretics and vasodilators according to cardiac function.Two patients underwent left coronary artery orifice ligation.Twenty three patients underwent reimplantation of left coronary artery to reconstruct dual coronary system.Patients of NYHA Ⅳ with moderate mitral regurgitation (MR) and NYHA Ⅲ with severe MR underwent mitral annuloplasty.If LVEF was less than 30％ after weaning from cardiopulmonary bypass,blood pressure could not be maintained with medication,or lactates increased progTessively,extracorporeal membrane oxygenation (ECMO) was demanded.Two patients showed low cardiac output syndrome immediately after surgical procedures,died from refractory ventricular fibrillation even with ECMO.In survived 23 patients during the early stage after surgeries,duration of ventilation was 7-500 hours,11 of them were supported with ventilator for less than 60 hours.The mean length of hospital stay was(23.4 ± 13.9) d (8-65 d).The follow-up duration ranged from 1-91 months (median 28.5 months).One case was lost to follow up.The patient died from infection 3 months after discharge.The cardiac functions of the remaining 22 patients were improved.The size of left ventricle of 14 patients recovered to normal.LVEF increased to the normal level in 20 cases.No patient underwent redo procedure.Conclusion The accurate diagnosis can be made based on history,electrocardiogram,echocardiography and other imaging diagnostic tools.Individualized treatment strategy is helpful for seriously sick infants.Aggressive ECMO support can increase surviving rate for patients with postoperative low cardiac output syndrome.
