中华小儿外科杂志
中華小兒外科雜誌
중화소인외과잡지
CHINESE JOURNAL OF PEDIATRIC SURGERY
2011年
5期
333-338
,共6页
祝忠群%徐志伟%张海波%刘锦纷%郑景浩
祝忠群%徐誌偉%張海波%劉錦紛%鄭景浩
축충군%서지위%장해파%류금분%정경호
完全性肺静脉异位引流%肺高压危象%肺静脉狭窄
完全性肺靜脈異位引流%肺高壓危象%肺靜脈狹窄
완전성폐정맥이위인류%폐고압위상%폐정맥협착
Total anomalous pulmonary venous connection%Pulmonary hypertension crisis%pulmonary vein stenosis
目的 描绘完全性肺静脉异位引流(TAPVC)患儿肺静脉走行"路线图"和形态"变异图",阐明肺静脉病理谱的变化规律,促进有效个体化手术.方法 2006年4月至2009年9月,139例TAPVC患儿进行手术,心上型61例,心内型55例,心下型6例和混合型17例.病理诊断依据超声心动图、核磁共振、计算机断层扫描或心导管和心血管造影检查以及术中解剖,根据患儿TAPVC类型和解剖特点选择个体化手术方法.结果 1.病理谱:①肺静脉走行连接"路线图":心上型按照垂直静脉(VV)走行方式分为4种,左行(47例)、右行(9例)、后行(2例)和双行(3例);心内型按照肺静脉回流部位分为3种,汇入冠状静脉窦(49例)、汇入右心房(5例)和同时汇人CS和右心房(1例),再依据肺静脉开口数目分为4个开口、2个开口和1个开口三种亚型;心下型按照VV汇入体静脉方式分为4种:汇入门静脉(1例)、肝静脉(3例)、同时汇入门静脉和肝静脉(1例)以及汇入下腔静脉(1例);混合型按照肺静脉回流双侧是否对称分为双侧对称连接的"2+2"型(5例)、双侧不对称连接的"3+1"型(10例)和"怪异型"(2例).②肺静脉形态"变异图":肺静脉入口狭窄、发育不良或多分支(11例)、共汇肺静脉发育不良或伴内膜增生(4例)、VV扭曲、短小和狭窄(9例).2.手术结果:本组早期死亡6例(4.3%),其中低心排1例,肺静脉梗阻5例.中期随访因肺静脉梗阻再手术6例,术后轻微梗阻2例.结论 TAPVC患儿肺静脉病理谱广,个体差异大;按照肺静脉走行"路线图"和形态"变异图"有利术中肺静脉解剖的探查和个体化手术设计.
目的 描繪完全性肺靜脈異位引流(TAPVC)患兒肺靜脈走行"路線圖"和形態"變異圖",闡明肺靜脈病理譜的變化規律,促進有效箇體化手術.方法 2006年4月至2009年9月,139例TAPVC患兒進行手術,心上型61例,心內型55例,心下型6例和混閤型17例.病理診斷依據超聲心動圖、覈磁共振、計算機斷層掃描或心導管和心血管造影檢查以及術中解剖,根據患兒TAPVC類型和解剖特點選擇箇體化手術方法.結果 1.病理譜:①肺靜脈走行連接"路線圖":心上型按照垂直靜脈(VV)走行方式分為4種,左行(47例)、右行(9例)、後行(2例)和雙行(3例);心內型按照肺靜脈迴流部位分為3種,彙入冠狀靜脈竇(49例)、彙入右心房(5例)和同時彙人CS和右心房(1例),再依據肺靜脈開口數目分為4箇開口、2箇開口和1箇開口三種亞型;心下型按照VV彙入體靜脈方式分為4種:彙入門靜脈(1例)、肝靜脈(3例)、同時彙入門靜脈和肝靜脈(1例)以及彙入下腔靜脈(1例);混閤型按照肺靜脈迴流雙側是否對稱分為雙側對稱連接的"2+2"型(5例)、雙側不對稱連接的"3+1"型(10例)和"怪異型"(2例).②肺靜脈形態"變異圖":肺靜脈入口狹窄、髮育不良或多分支(11例)、共彙肺靜脈髮育不良或伴內膜增生(4例)、VV扭麯、短小和狹窄(9例).2.手術結果:本組早期死亡6例(4.3%),其中低心排1例,肺靜脈梗阻5例.中期隨訪因肺靜脈梗阻再手術6例,術後輕微梗阻2例.結論 TAPVC患兒肺靜脈病理譜廣,箇體差異大;按照肺靜脈走行"路線圖"和形態"變異圖"有利術中肺靜脈解剖的探查和箇體化手術設計.
목적 묘회완전성폐정맥이위인류(TAPVC)환인폐정맥주행"로선도"화형태"변이도",천명폐정맥병리보적변화규률,촉진유효개체화수술.방법 2006년4월지2009년9월,139례TAPVC환인진행수술,심상형61례,심내형55례,심하형6례화혼합형17례.병리진단의거초성심동도、핵자공진、계산궤단층소묘혹심도관화심혈관조영검사이급술중해부,근거환인TAPVC류형화해부특점선택개체화수술방법.결과 1.병리보:①폐정맥주행련접"로선도":심상형안조수직정맥(VV)주행방식분위4충,좌행(47례)、우행(9례)、후행(2례)화쌍행(3례);심내형안조폐정맥회류부위분위3충,회입관상정맥두(49례)、회입우심방(5례)화동시회인CS화우심방(1례),재의거폐정맥개구수목분위4개개구、2개개구화1개개구삼충아형;심하형안조VV회입체정맥방식분위4충:회입문정맥(1례)、간정맥(3례)、동시회입문정맥화간정맥(1례)이급회입하강정맥(1례);혼합형안조폐정맥회류쌍측시부대칭분위쌍측대칭련접적"2+2"형(5례)、쌍측불대칭련접적"3+1"형(10례)화"괴이형"(2례).②폐정맥형태"변이도":폐정맥입구협착、발육불량혹다분지(11례)、공회폐정맥발육불량혹반내막증생(4례)、VV뉴곡、단소화협착(9례).2.수술결과:본조조기사망6례(4.3%),기중저심배1례,폐정맥경조5례.중기수방인폐정맥경조재수술6례,술후경미경조2례.결론 TAPVC환인폐정맥병리보엄,개체차이대;안조폐정맥주행"로선도"화형태"변이도"유리술중폐정맥해부적탐사화개체화수술설계.
Objective To delineate the morphological spectrum of total anomalous pulmonary venous connection (TAPVC) by building the "road map" and the " variation chart" of pulmonary veins,and to advance individualized surgical treatment Methods Between April,2006 to June,2009, 139 consecutive patients with TAPVC underwent operations. There were 61 supracardiac,55 intracardiac,6 infracardiac and 17 mixed types. Pathological diagnosis was made by echocardiogram,magnetic resonance imaging, computerized tomography,or the cardiac catheterization and operative findings during operation. The option of procedure was determined by findings. Results (1) Pathological spectrum:①" the road map"of the pulmonary veins: the patients with supracardiac type were divided into 4 subtypes according to the course of vertical veins:left course (47 cases),right course (9 cases),posterior course(2 cases) and double courses (3 cases). The patients with intracardiac type were divided into 3 subtypes according to the draining site of pulmonary veins: to coronary sinus (49 cases) ,to right atrium (5 cases) or to coronary sinus and right atrium (1 case),and the openings of pulmonary veins may be 4,2 or 1 in each subtype. The patients with infracardiac type were divided into 4 subtypes according to the draining site of vertical vein:to portal vein (1 case),to hepatic vein(3 cases),to portal vein and hepatic vein (1 case) and to inferior vena cava (1 cases). The patients with mixed type were divided into 3 subtypes: bilateral and symmetrical connections "2 + 2" pulmonary venous drainage pattern; bilateral and asymmetrical connections"3 +1" pulmonary venous drainage pattern and bizarre anatomic variants. ② The morphological "variation chart" of pulmonary veins individual pulmonary vein stenosis or excessive tributary veins (11 cases) showed: hypoplastic confluence veins (4 cases) and vertical veins distortion or elongation or forming hemodynamic vise or common pulmonary vein that drained to coronary sinus or right atrium through a narrowed short vertical vein or a small window(9cases).(2)Surgical results：early death was encountered in 6 cases(4.3％).The causes included 10W cardiac output syndrome in 1 case,and pulmonary veins stenosis and associated complications in the other 5 patients.Six patients with pulmonary restenosis underwent reoperation.All survived.Two patients had mild residual obstruction during Intermediate-term follow-up.Conclusions The patients with TAPVC had a wide spectrum of pulmonary veins with high inter-individual variation.It was useful to delineate the anatomy of pulmonary veins and to plan personalized procedures during operations according to the"road map"and"variation chart"of pulmonary veins.
