Ross 手术治疗先天性主动脉瓣膜疾病

目的　总结 Ross手术治疗先天性主动脉瓣膜疾病的临床经验和手术结果。　方法　自 1998年 3月至2 0 0 2年 7月 ,16例主动脉瓣膜疾病患者 (平均年龄 14 .0± 9.9岁 )接受 Ross手术 ,即自体肺动脉瓣移植术。诊断为主动脉瓣二瓣化畸形 ,主动脉瓣狭窄 9例 ,主动脉瓣发育不良呈穹隆状狭窄 2例 ;主动脉瓣脱垂 5例 ,其中合并室间隔缺损和动脉导管未闭各 1例。　结果　无手术死亡 ,全部患者治愈出院。随访 1～ 4 8个月 ,平均 30± 13个月 ,无远期死亡 ,无瓣膜相关并发症。所有患者心功能 级。超声心动图提示主动脉瓣及同种肺动脉瓣功能良好 ,仅 1例患者主动脉瓣有极少量反流 ;所有患者主动脉瓣跨瓣压差 2 .1± 0 .8mm Hg(1k Pa=7.5 mm Hg) ,左心室流出道及主动脉瓣环随着年龄的生长而增长 ,平均瓣环直径较术后增加 4 .0± 2 .1mm。　结论　 Ross手术治疗主动脉瓣膜疾病安全 ,效果好 ,随机体发育而生长 ,可适于某些主动脉瓣瓣膜疾病 ,尤其适于小儿及年轻患者。     

11例主动脉根部细小病人的主动脉瓣置换手术

自 1995年 1月至 2 0 0 2年 1月 ,我们采用改良Manouguian方法对 11例病人施行细小主动脉根部拓宽和主动脉瓣置换术 ,手术效果满意。资料和方法　 11例中男 4例 ,女 7例 ;年龄 6～ 6 2岁 ,平均 ( 2 5 7± 11 6 )岁。小儿先天性主动脉瓣二叶瓣畸形合并主动脉瓣重度狭窄、主动脉瓣下管样狭窄 5例 ,其中 3例合并单支左冠状动脉 ,1例曾行球囊扩张术 ;儿童主动脉瓣感染性心内膜炎伴主动脉瓣重度关闭不全 4例 ,合并室间隔缺损 2例、动脉导管未闭 1例 ;老年性主动脉瓣钙化伴主动脉瓣重度狭窄 2例。心功能 (NYHA)III级 7例 ,II级 4例。心电图示…     

风湿性瓣膜病二尖瓣与主动脉瓣置换术1154例长期效果分析

目的　评价风湿性联合瓣膜病二尖瓣与主动脉瓣双瓣置换术的近期与远期疗效 ,分析影响手术疗效的因素。　方法　回顾性分析 1981年 5月～ 2 0 0 1年 5月 2 0年间 ,115 4例风湿性心脏病患者行双瓣膜置换术的临床资料和长期随访结果 ,其中二尖瓣与主动脉瓣均为狭窄病变者 2 5 3例 ,二尖瓣狭窄合并主动脉瓣关闭不全者 345例 ,二尖瓣关闭不全合并主动脉瓣狭窄者 119例 ,二尖瓣与主动脉瓣均为关闭不全者 437例 ;合并三尖瓣病变的占 5 4 0 0 %( 75 7例 ) ,其中器质性病变 7 2 7%( 84例 ) ,功能性关闭不全 5 8 31%( 6 73例 ) ;合并中度以上肺动脉高压者 339例 ;术前NYHA心功能分级Ⅲ级与Ⅳ级者分别为 873例和 186例。应用侧倾碟瓣或双叶机械瓣施行瓣膜置换术 ,合并三尖瓣功能或器质性病变者 ,同期行瓣膜成形手术。　结果　本组患者术后住院病死率为 6 5 0 %( 75 / 115 4)。早期死亡的主要原因为低心排出量综合征、顽固性心律失常、肾功能或呼吸功能衰竭 ,以及抗凝有关的出血等。长期生存 10 79例 ,随访时间为 8个月～ 2 0年 ,平均随访时间为 4 5 %病人·年。晚期死亡 6 6例 ( 0 39%病人·年 ) ;5、10与 15年累计生存率分别为 ( 89 46± 1 35 ) %、( 86 5 0± 1 91) %与 ( 6 7 86±6 16 ) %。生存的 92 9例患     

Ross手术的临床应用

目的　总结主动脉瓣疾病患者行 Ross手术的临床应用经验。　方法　自 2 0 0 2年 1月至 2 0 0 2年 8月 ,对12例主动脉瓣疾病患者行 Ross手术 ,其中先天性心脏病、主动脉瓣病变 11例 ,老年退行性主动脉瓣狭窄 1例。术前所有患者均经超声心动图 (UCG)检查示主动脉瓣狭窄和 /或关闭不全 (中重度 ) ,均在全身麻醉中度低温体外循环下行 Ross手术。结果　全组患者无手术死亡 ,无并发症 ;术后主动脉瓣跨瓣压差在正常范围 ,左心室舒张期末内径(L VEDD)明显缩小 (P<0 .0 0 1) ,左心室射血分数 (L VEF) 0 .5 5± 0 .14 ,心功能 (NYHA) 级。所有患者均接受随访 ,随访 7天～ 8个月 ,心功能 ～ 级 ,主动脉瓣、肺动脉瓣功能良好。　结论　Ross手术是一种临床疗效较好的治疗主动脉瓣病变的手术方法。     

左冠状动脉起源于肺动脉的外科治疗

目的　探讨左冠状动脉起源于肺动脉的术前诊断、手术治疗的术式和疗效。　方法　1991年 8月～ 1999年 3月 ,8例患者进行了手术治疗 ,其中 7例采用肺动脉内隧道成形术 ,1例采用冠状动脉旁路移植术 ,1例患者合并二尖瓣关闭不全 ,同时进行了二尖瓣成形术。术前进行了心电图、心脏超声和心导管、心脏造影等全面检查。术后随访 7例 ,随访时间平均 (4 6 8± 10 7)个月。　结果　 8例患者无住院死亡和严重并发症 ,7例随访无晚期死亡 ,1例患者因肺动脉内隧道缝线撕脱 ,导致主动脉 主肺动脉瘘 ,二次手术修补成功。随访患者NYHA心功能Ⅰ级 ,LVED平均 (4 6 4± 3 7)mm ,较术前平均 (5 4 4± 2 8)mm明显减少 (P <0 0 1) ,EF值 (6 9 4± 2 3) %较术前 (5 9 6± 2 8) %明显提高(P <0 0 5 )。　结论　左冠状动脉起源于肺动脉是一种较为罕见的先天性心脏病 ,治疗的主要原则为重建 2支冠状动脉系统并处理好合并病变。肺动脉内隧道矫治方法 ,简便易行 ,疗效满意     

活动期感染性自然心内膜炎的外科治疗

目的　总结活动期感染性自然心内膜炎外科治疗的经验。方法　自 1996年 10月 1日至 2003年 12月 31日,阜外心血管病医院外科共手术治疗活动期感染性自然心内膜炎 54例。有明确感染诱因的 21例,先天性心内结构畸形 23例,风湿性瓣膜病 1例。术前心功能NYHA分级:Ⅰ级6例,Ⅱ级 12例,Ⅲ级 7例,Ⅳ级 29例。术前左心室舒张末径 ( 63±11 )mm。发病至手术间隔 8 ~629d(中位数 125d)。行主动脉瓣置换 25例,主动脉瓣及二尖瓣置换 15例,二尖瓣置换 6例,二尖瓣成形 3例,肺动脉瓣置换 1例,单纯心内分流修补 4例。术后应用足量敏感抗生素 6 ~8周。结果手术死亡 5例,死因均为感染,术后即失访 4例,手术死亡率 17% (9 /54)。14例 ( 26% )发生手术并发症。45例随访 6~67个月,平均(31±19)个月。术后心功能NYHA分级Ⅰ级 41例,Ⅱ级 3例,Ⅲ级 1例,左心室舒张末径 (52±8)mm。2例病人接受再次手术,术后康复;有再次手术指征但未手术者 3例。术后晚期意外死亡 1例,抗凝过量致颅内出血 1例。结论　活动期感染性自然心内膜炎经积极的外科治疗能够取得较好的治疗效果。     

矫正型大动脉转位合并心内畸形的手术方法和效果

目的 探讨双调转手术和传统修复术治疗矫正型大动脉转位合并心内畸形的效果.方法 2002年4月至2006年12月,19例矫正型大动脉转位合并心内畸形患者接受手术治疗,男性14例,女性5例;年龄2～22岁,平均8.6岁.合并的心内畸形包括:室间隔缺损18例,右心室双出口1例,肺动脉狭窄17例,房间隔缺损4例,肺动脉高压2例.手术方式包括双调转手术解剖矫正修复15例,传统修复术治疗心内畸形4例.结果 双调转术后死亡1例,发生严重低心排血量综合征2例,一过性房室传导阻滞1例,反复胸腔积液2例.传统心内修复术后无死亡病例,发生严重低心排血量综合征1例.双调转手术存活者随访6个月～4年,均为窦性心律,心功能NYHA分级Ⅰ～Ⅱ级,射血分数51%～68%.传统心内修复术存活者随访1年,3例心功能达Ⅰ～Ⅱ级,射血分数52%～61%;1例心功能Ⅲ级,射血分数40%.结论 双调转手术解剖修复矫正型大动脉转位合并心内畸形早中期效果良好,严格选择手术适应证和手术时机是提高手术效果的关键.对于右心室功能较好,无三尖瓣畸形的患者,如果存在影响双调转效果的因素,仍应选择传统心内修复手术.     

三尖瓣置换术及近远期疗效

1994年 1月～ 2 0 0 1年 4月 ,我们对 19例风湿性心瓣膜病患者施行了三尖瓣置换术 (TVR) ,占同期心瓣膜置换术的3.4 1% ,并就 TVR的近远期疗效进行了观察。1　临床资料与方法本组共 19例 ,男 4例 ,女 15例 ;平均年龄 4 2 .3± 10 .2岁。风湿性二尖瓣狭窄 10例 ,风湿性二尖瓣、主动脉瓣双瓣膜病变 9例。术前均有肝肿大 ,12例出现心源性恶病质。胸部 X线片示 :心胸比率 0 .6 9± 0 .12 ;超声心动图示 :三尖瓣重度反流 ,合并二尖瓣和 /或主动脉瓣病变 ;心电图示 :心房颤动 18例。术前心功能 级 5例 , 级 14例。行 TVR+二尖瓣置换术 (MVR…     

二尖瓣狭窄伴主动脉瓣关闭不全30例

我院于1994年8月～1999年10月共收治30例风湿性二尖瓣狭窄伴轻度主动脉瓣关闭不全患者,行单纯二尖瓣置换术5例,双瓣膜置换术25例,现报告如下。1　临床资料与方法本组30例,男12例,女18例。年龄25～57岁,平均年龄36岁。病程2～25年。心功能:级6例,级20例,级4例。心胸比率0.50～0.70,平均0.55。肺动脉压均高于7.5kPa(56mmHg),其中>10kPa(75mmHg)20例,>12kPa(90mmHg)5例。肺功能重度减退8例。心电图示心房颤动24例,窦性心律6例;伴频发性、多源性或短暂阵发性室性心动过速9例。心脏超声心动图示轻度主动脉瓣关闭不全24例,轻至中度关闭不全6例;…     

升主动脉瘤合并主动脉瓣关闭不全的外科治疗

目的总结21例升主动脉瘤合并主动脉瓣关闭不全的外科治疗经验。方法19例行Bentall手术,2例行Cabrol手术;5例同时置换二尖瓣。术前心功能IV级15例,III级6例。动脉瘤直径6～11 cm,平均(8.5±2.6) cm;17例主动脉内膜有撕裂,其中5例升主动脉远端仍有夹层。主动脉瓣环直径2.7～5.4 cm,平均(3.2±1.8）cm。超声心动图检查均示主动脉瓣严重关闭不全。结果手术后无早、晚期死亡,术后超声心动图和造影检查示主动脉瓣关闭良好,移植的冠状动脉通畅、无扭曲,效果满意。结论动脉瘤直径大于6 cm时应及时随访和做预防性手术,一旦发现夹层动脉瘤应立即手术,Bentall手术治疗升主动脉瘤效果良好。     

Surgical management of congenital obstruction of the left main coronary artery with supravalvular aortic stenosis

OBJECTIVE: Stenosis of the left main coronary artery is a recognized complicating feature of supravalvular aortic stenosis. We have retrospectively identified three anatomic subtypes of left main coronary obstruction in patients with supravalvular aortic stenosis, each necessitating a distinct surgical approach. METHODS: From 1991 to 1998, 9 patients underwent surgical repair of supravalvular aortic stenosis and left main coronary stenosis. Five patients (group 1) had obstruction from near-circumferential thickening of the left main ostium, 2 patients (group 2) had restricted coronary flow due to fusion of an aortic valve leaflet to the supravalvular ridge, and 2 patients (group 3) had diffuse narrowing of the left main coronary artery. Group 1 patients were treated with patch aortoplasty encompassing the left main ostium and supravalvular aortic stenosis. Group 2 patients were treated with excision of the fused leaflet from the aortic wall and patch aortoplasty. Group 3 patients were treated with bypass grafting and aortoplasty. RESULTS: Surgical strategy was determined by coronary angiography and intraoperative assessment of coronary anatomy. There was 1 early death. All surviving patients underwent echocardiography with or without postoperative catheterization. The mean postoperative supravalvular gradient for 7 patients was 8 mm Hg (range 2-15 mm Hg). One patient required reoperation for a residual aortic gradient as a result of aortic arch involvement. No evidence of left main coronary artery stenosis was seen in groups 1 and 2; bypass grafts were patent in group 3 patients at a mean follow-up of 54.8 months. CONCLUSION: Three subtypes of left main coronary stenosis with supravalvular aortic stenosis are described. Each anatomic type mandates an individual surgical approach. Favorable surgical outcomes are achievable with each category.     

Anaesthetic management of a patient with Williams syndrome undergoing aortoplasty for supravalvular aortic stenosis

PURPOSE: A case of a patient associated with Williams syndrome undergoing aortoplasty for supravalvular aortic stenosis is presented. CLINICAL FEATURES: Williams syndrome is a rare disease associated with a characteristic facies, supravalvular aortic stenosis, and mental retardation. A 15-yr-old girl with Williams syndrome underwent aortoplasty for supravalvular aortic stenosis. Anaesthesia was induced with fentanyl and thiamylal, and maintained with nitrous oxide, oxygen, sevoflurane, and continuous intravenous infusion of fentanyl. Supravalvular aortic stenosis was evaluated using a multiplane transesophageal echocardiography (TEE) probe before and after repair. CONCLUSION: Multiplane TEE was found to be very useful for anaesthetic management in a patient with Williams syndrome undergoing aortoplasty for supravalvular aortic stenosis.     

Extended aortic and left main coronary angioplasty with a single pericardial patch in a patient with Williams syndrome.

In a 9-year-old girl with supravalvular aortic stenosis combined with severe left main coronary artery trunk obstruction (Williams syndrome), the repair for both lesions was performed successfully by simultaneous extended aortoplasty and direct coronary angioplasty using a single double-flanged pericardial patch.     

A case report of patch aortoplasty for supravalvular aortic stenosis associated with Williams syndrome]

A 15-year-old girl underwent patch aortoplasty for supravalvular aortic stenosis in association with Williams syndrome. Pressure gradient of before and after stenosis of the aorta decreased from 104 mmHg to 16 mmHg at the postoperative catheterization. Angiography after the operation showed no stenosis. Postoperative course was excellent and discharged on 14th postoperative day. The problems of the operation of supravalvular aortic stenosis were discussed.     

Long-term outcome of large artificial patch aortic repair for diffuse stenosis in Williams syndrome

There have been only a few reports concerning the long-term results of a surgical procedure using a large artificial patch for patients with Williams syndrome. Twelve years have passed since a patient with William’s syndrome underwent a surgery with a patch angioplasty for the diffuse supravalvular aortic stenosis and deformities of the neck branch arteries. The patient had a well-balanced aortic growth without stenotic or aneurysmal changes, which was confirmed during the time of the second surgery when replacing the mitral valve. This technique of using a large patch has proven to be safe for Williams syndrome patients with diffuse supravalvular aortic stenosis in the long term.     

Extended aortoplasty to relieve supravalvular aortic stenosis

An extended aortoplasty was used to relieve severe supravalvular aortic stenosis in 5 patients whose preoperative left ventricular-aortic gradient ranged from 85 to 140 mm Hg (median, 120 mm Hg). The stenotic ring above the commissures was divided in two places by an inverted U incision extending into the right and noncoronary sinuses. A distal vertical incision in the ascending aorta converted this into an inverted Y. The ridge above the left coronary sinus was excised. The aortic incision was repaired with an inverted Y-shaped Dacron gusset. The postoperative gradient ranged from 0 to 30 mm Hg (median, 15 mm Hg). The extended aortoplasty provides excellent relief of supravalvular aortic stenosis and, in addition, restores the aortic root geometry to a much more anatomical configuration than is achieved with the simple patch technique.     

Brom's three-patch technique for repair of supravalvular aortic stenosis.

OBJECTIVE: Case histories of all patients (n = 29) operated on for supravalvular aortic stenosis from 1962 to the present were reviewed to study different techniques and outcomes. The technique of symmetric aortoplasty with 3 patches (1 in each sinus) is described and compared with other methods. METHODS: Case reports were reviewed and follow-up was completed by contacting the patient's (pediatric) cardiologist. We aimed for a last follow-up visit, including Doppler echocardiographic studies, in a period no more than 12 months earlier than December 1997. Supravalvular aortic stenosis was discrete in 25 and diffuse with involvement of the aortic arch and arch vessels in 4 patients. Additional anomalies were bicuspid aortic valve (n = 5), coarctation (n = 3), ascending aortic aneurysm (n = 1), mitral valve insufficiency (n = 2), pulmonary valvular stenosis (n = 1), and peripheral pulmonary artery stenosis (n = 2). Eleven patients had Williams syndrome and 1 patient had Noonan syndrome. Symmetric aortoplasty with 3 patches (1 in each sinus) was used in 13 patients, whereas other nonsymmetric methods (1, 2, or Y-shaped patches) were used in 16 patients. Mean follow-up was 10.5 years (range: 4 months-36 years). RESULTS: All techniques adequately decreased the pressure gradient. Progression of preoperative aortic valve insufficiency or de novo regurgitation was not observed except in 1 patient in whom the patches inserted were too large. CONCLUSIONS: No difference could be demonstrated in outcome for any surgical technique; however, reconstruction of the aortic root with autologous pericardial patches in each sinus after transection of the aorta has the advantage of symmetry while restoring the normal aortic root anatomy.     

自体肺动脉补片主动脉弓成形治疗婴儿主动脉缩窄合并弓发育不良

目的 探讨缩窄段切除加自体肺动脉补片主动脉弓成形治疗婴儿主动脉缩窄合并主动脉弓发育不良的方法和效果.方法 2007年5月至2009年12月,14例主动脉缩窄合并主动脉弓发育不良病婴行主动脉缩窄段切除加自体肺动脉补片主动脉弓成形手术,其中男9例,女5例;年龄23天至17个月,中位值4.33月龄;平均体重(6.14±2.36)kg.所有病婴均诊断为合并室间隔缺损的主动脉缩窄,同时存在主动脉弓发育不良.手术在深低温体外循环下完成,其中8例采用选择性脑灌注技术,6例停循环.主动脉成形采用新鲜的自体肺动脉补片.同期修补合并的室间隔缺损.结果 围术期死于循环衰竭1例.13例生存,其中1例合并低心排血量综合征,经相应治疗恢复.术后超声心动图检查主动脉弓无残余梗阻.随访4个月至3年.超声心动图示主动脉弓压力阶差均<16 mm Hg(1 mm Hg=0.133 kPa),随访期间主动脉弓降部血流速度与出院时无明显变化.CT扫描显示主动脉弓几何构形正常;术后半年原左主支气管受压者症状明显改善或完全消失.无主动脉夹层动脉瘤发生.结论 缩窄段切除加自体肺动脉补片主动脉弓成形是治疗婴儿主动脉缩窄合并主动脉弓发育不良理想的手术方法.

Abstract：

Objective To discuss the operative techniques and results of coarctation resection plus aortoplasty with pulmonary autograft patch for coarctation of the aorta combined with hypoplastic aortic arch in infant.Methods Between May 2007 and Dec 2009,14 cases including 9 males and 5 females with caorctation of the aorta and hypoplastic aortic arch underwent coarctation resection plus aortoplasty with pulmonary autograft patch in our hospital.The age ranged from 23 days to 17 months,with a median of 4.33 months.The mean body weight was (6.14 ±2.36) kg.All patients were diagnosed as aortic coarctation combined with VSD and hypoplastic aortic arch.The surgery was performed under deep hypothermia cardiopulmonary bypass with selective cerebral perfusion in 8 cases and circulation arrest in 6 cases.Fresh pulmonary autograft patch harvested from the main pulmonary artery was used for aortoplasty.The associated VSD was repaired in the same stage.Results All patients survived except one died from circulatory failure during the perioperative period.Low cardiac output syndrome occurred in another case who was cured afterwards by correspondent treatments.No residual obstruction was detected by echocar-diography after the operation.Follow-up was carried out in 13 cases from 4 months to 3 years.Echocardiographic examination showed that the pressure gradient across the aortic arch was less than 16 mm Hg in all cases.The blood velocity at the descending aortic arch was not significantly changed during the follow-up period as compared with that of the immediate after operation.Computed tomography showed that the morphology of aortic arch was normal.The left bronchus compression was relieved obviously or totally disappeared in patients who suffered from left bronchus stenosis before operation,and no aortic aneurysm were detected in these patients.Conclusion Conclusion Coarctation resection plus aortoplasty with pulmonary autograft patch is the optimal surgical method for treating coarctation of the aorta combined with hypoplastic aortic arch in infant.     

Staged operation for supravalvular aortic stenosis syndrome associated with severe multiple peripheral pulmonary stenoses: a case report and surgical methods

Successful surgical correction of supravalvular aortic stenosis (SAS) syndrome associated with severe peripheral pulmonary stenosis by a staged approach in a 6-year-old boy is reported. Right ventriculogram demonstrated bilateral severe peripheral pulmonary stenoses and cardiac catheterization revealed severe pressure gradient of 94 mmHg between the trunk of pulmonary artery and right and left peripheral pulmonary arteries, respectively. Bilateral severe peripheral pulmonary stenoses were relieved successfully by staged operations. Initially, a stenotic portion of the right pulmonary artery and its bifurcation was removed and a DeBakey Knitted Dacron graft was interposed between the right pulmonary artery and the peripheral pulmonary artery which was enlarged by arterioplasty. Nine days later, stenosis of the left peripheral pulmonary artery was relieved by arterioplasty using a Rygg patch. Finally, 8 months after the initial operation, the patient underwent definitive repair of SAS by extended aortoplasty. The postoperative course of the patient was uneventful. Our experience indicates that in the surgical treatment of the patient with SAS and associated severe multiple peripheral pulmonary stenoses, a staged approach, that is initial staged repair of the bilateral pulmonary stenoses by arterioplasty through thoracotomy followed by definitive intracardiac repair of SAS, is the procedure of choice.     

Surgical treatment of supravalvular aortic stenosis

Five surgically treated cases of supravalvular aortic stenosis were reported. Preoperative cardiac catheterization revealed marked left ventricular-aortic pressure gradient ranged from 20 to 180 mmHg (median, 80.2 mmHg) and retrograde aortogram showed localized (hourglass type) supravalvular aortic stenosis in all cases. A traditional diamond shaped patch aortoplasty was used to relieve supravalvular aortic stenosis in 1 case and an extended aortoplasty was used in last 4 cases. Pressure gradients were almost disappeared postoperatively with good surgical result. The extended aortoplasty is considered to be more reasonable than the traditional aortoplasty in relieving constriction symmetrically and restoring normal relationship of the aortic valve cusps and coronary ostia. The operative indication and selection of the operative procedures for supravalvular aortic stenosis were briefly discussed.