室间隔缺损合并右室流出道狭窄的手术治疗

室间隔缺损合并右室流出道狭窄的手术治疗王占明赵希武郑有仁高峰１９８８年１１月至１９９５年５月我们手术治疗室间隔缺损（ＶＳＤ）合并右室流出道狭窄（ＲＶＯＴＳ）２４例，占同期先心病手术总数的２．３％。现报告如下：临床资料２４例中男１６例，女８例。术前诊断...     

三尖瓣下移畸形锥形重建术后左心室流出道梗阻一例

左心室流出道梗阻是三尖瓣下移畸形少见并发症之一,手术相关的左心室流出道梗阻更加少见.1例46岁女性患者术前诊断三尖瓣下移畸形,行锥形重建术后于重症监护室出现左心室流出道梗阻并最终心脏骤停.     

伴左心室流出道梗阻的完全性大动脉转位行动脉调转术后左心室流出道及主动脉瓣功能随访

目的探讨伴左心室流出道梗阻的完全性大动脉转位患者行动脉调转术后,左心室流出道梗阻的改善情况及主动脉瓣功能情况。方法 2002~2013年共549例患儿于阜外心血管病医院行动脉调转术,其中42例患者合并左心室流出道梗阻,其中男31例、女11例,中位月龄12个月(7 d至96个月);中位体重6.5(3.5~26.0)kg,外周经皮血氧饱和度52%~85%;左心室流出道病变类型包括肺动脉瓣异常,瓣下隔膜,隧道样狭窄,肌性狭窄,附属瓣膜组织及复合病变。术中根据病变类型采取不同方法:瓣交界粘连行交界切开,瓣下隔膜予以切除,单纯肌性狭窄则切除肥厚肌束或部分室间隔,环形或隧道样狭窄则切除纤维组织和肥厚肌肉,副瓣样组织或无功能腱索,予以切除,通过室间隔缺损跨越至左心室的腱索,切下重植。结果平均体外循环时间147~344(193.5±73.1)min,主动脉阻断时间139(109~305)min,呼吸机使用时间36(3~960)h,住ICU时间5(1~48)d。体外膜式氧合(ECMO)辅助3例,均成功撤除。早期死亡2例,1例为多器官功能衰竭,1例为严重感染。随访期间死亡1例,原因不明,失访3例,接受随访患者36例,随访时间24(3~116)个月;再发左心室流出道梗阻1例,为瓣下局限增厚纤维组织所致,新主动脉瓣轻度狭窄1例,新主动脉瓣少量反流11例,中量反流2例;随访时中位左心室-主动脉压差4(2~49)mm Hg,较术前[37.2(12.1~70.6)mm Hg]有明显改善(Z=-5.153)。1年时心脏事件免除率为91%±5%,5年时为78%±8%。结论对于合并左心室流出道梗阻的完全性大动脉转位,需结合解剖情况与压差评估梗阻严重程度,指征把握恰当,行动脉调转术可获得满意的中远期效果。     

Konno术在小儿左心室流出道梗阻中的临床应用

目的 总结Konno手术治疗小儿左心室流出道梗阻(LVOTO)的近、中期效果.方法 2006年5月至2012年5月共收治16例LVOTO患儿,年龄1.9～13.6岁,平均(5.1±0.7)岁;体质量12 ～39 kg,平均(18.3±6.3) kg.其中13例(81.3％)有两个以上部位的LVOTO.超声评估LVOTO压差56 ～ 185mmHg(1mmHg =0.133 kPa),平均(96±31) mmHg.手术在中低温(28C ～ 30℃)体外循环下进行,体外循环70 ～182min,平均(98±21) min;主动脉阻断34 ～148min,平均(51±11) min.单纯Konno手术3例;Konno± Ross手术4例;Konno-Rastan(主动脉瓣置换)手术9例.结果 患儿术后无死亡.呼吸机辅助3～9天,平均(4.0±1.5)天.住ICU5～16天,平均(8.0±2.9)天.术后随访6个月～3年.心脏超声检查无明显残余梗阻,LVOTO流速1.10 ～2.42 m/s;左心室射血分数0.58 ～0.72,平均0.66±0.03.主动脉瓣反流轻到中度2例,中-重度1例,余均为轻度;1例出现肺动脉瓣中度反流,三尖瓣轻到中度反流.结论 Konno手术治疗小儿LVOTO安全、有效,利于患儿左心功能恢复,远期并发症情况尚需进一步随访.     

主动脉瓣二瓣畸形所致主动脉瓣狭窄的外科治疗

目的 总结主动脉瓣二瓣畸形所致的单纯主动脉瓣狭窄外科手术治疗经验.方法 1993年5月至2007年12月,共收治主动脉瓣二瓣畸形所致的单纯主动脉瓣狭窄病人103例,其中男66例,女37例.年龄15～75岁,平均(52.9±13.2)岁.心功能Ⅱ级58级,Ⅲ级33例,Ⅳ级12例.所有病人均接受主动脉瓣置换术,同期行左室流出道疏通2例、房颤射频消融2例、升主动脉替换7例,冠状动脉旁路移植11例.结果 手术死亡1例(死亡病0.93%),Ⅲ度房室传导阻滞1例,余者均治愈出院.随访6个月～14年,平均(86.3±26.8)个月.失访14例,随访率84.2%.3例出现脑出血或栓塞并发症,1例猝死,1例非心脏原因死亡.最后随访心功能Ⅰ级67例,Ⅱ级17例,Ⅲ级2例.结论 主动脉瓣置换治疗主动脉瓣二瓣畸形所致的单纯主动脉瓣狭窄有较好的疗效,合并升主动脉扩张应积极处理.     

左室流出道憩室1例

病人　男 ,32岁。发热、偏瘫、心悸、气促 11个月。查体 :心尖部和胸骨左缘第 3、4肋间均可闻及 3～ 4 VI级双期杂音 ,水冲脉 (+)。X线胸片和心电图均示左房室长大。超声心动图示二尖瓣增厚狭窄伴反流 ,主动脉瓣口径 11mm ,关闭裂隙3mm(图 1) ;心内未探及血栓或赘生物 ;左室射血分数 0 5 1。术前诊为慢性风湿性心脏瓣膜病 ,感染性心内膜炎。　　 2 0 0 0年 12月手术中食管超声检查见 ,主动脉瓣环下有异常的囊状结构开口于左室流出道(LVOT) ,随心动开闭 ,少量血流经此开口进出。该囊向主动脉根之侧后方延伸 ,但不与主动脉交通 ,末端呈…     

儿童心脏瓣膜置换手术及其疗效

目的探讨儿童心脏瓣膜置换手术及治疗效果。方法1990年1月至2002年12月，45例14岁以下儿童施行了心脏瓣膜置换手术。其中男26例，女19例。年龄3—14岁，平均10．8岁；≤10岁15例，10～14岁30例。包括先天性心脏瓣膜病32例，风湿性瓣膜病6例，心内膜炎3例，部分型房室管畸形修补术后二尖瓣关闭不全3例，室间隔缺损修补术后主动脉关闭不全1例。行二尖瓣置换23例，其中2例为矫正型大动脉转位行解剖位三尖瓣置换，主动脉瓣置换9例；二尖瓣与主动脉瓣双瓣置换4例；三尖瓣置换9例。5例使用生物瓣膜或同种主动脉瓣，余40例均采用机械瓣膜，包括进口双叶瓣34枚，进口单叶瓣3枚，国产单叶瓣7枚。结果本组手术死亡(术后30d内)2例，死亡率4．4％，均死于手术当日，1例因顽固性心律失常，1例术后低心输出量综合征。生存者随访8个月-12年，平均4．9年。4例晚期死亡，晚期死亡率9．3％。置入机械瓣膜者均采用华法林抗凝治疗，未发生血栓栓塞及抗凝相关并发症，病儿术后心功能均Ⅰ-Ⅱ级。结论儿童瓣膜病病人，大多数可以置入合适的成人型号人工瓣膜，保证其术后生长发育，减少二次手术。采用华法林进行抗凝治疗，经过平均4．5年随访，无血栓栓塞或抗凝有关的出血并发症发生，治疗效果较为满意。     

用自制生物瓣复合管道行Bentall手术

目的介绍自制生物瓣复合管道成功完成Bental手术的经验。方法2005年12月至2006年6月,5例年龄47～67岁,平均(55.8±9.4)岁,诊断为主动脉瓣中至重度关闭不全,合并升主动脉窦部、窦管结合部位以及升主动脉瘤样扩张的病人完成生物瓣Bentall手术,主动脉弓部无明显扩张。根据主动脉瓣环选择大小合适的Carpentier-EdwardsPerimount牛心包瓣,再选择比瓣膜直径大3～5mm的人工血管,套入生物瓣,用带垫片双头针在主动脉瓣环位置间断褥式缝合,将生物瓣和人工血管一并缝合,垫片置于主动脉瓣环心室面。打结后将左、右冠状动脉“纽扣”移植于人工血管上。2例同期用大隐静脉与前降支吻合,近端与人工血管吻合。结果5例均无并发症,顺利康复,治愈出院。出院前复查心脏超声,主动脉瓣峰值压力阶差为(18.3±7.6)mmHg(1mmHg=0.133kPa),左心室前后径从术前(58.8±6.3)mm缩小至(43.7±3.1)mm(P<0.05),左室射血分数手术前后无显著变化(0.62±0.08对0.56±0.55)。术后随访2～8个月,瓣膜功能良好,心功能明显改善。结论用自制生物瓣复合管道行Bentall手术安全有效,为老年病人的主动脉根部手术提供更为合理的瓣膜材料,生物瓣的远期功能状态需要进一步随访。     

Manouguian法瓣环增宽加主动脉瓣置换术治疗成人小主动脉瓣环

目的总结成人主动脉瓣狭窄伴小主动脉瓣环行瓣环增宽并替换主动脉瓣的经验。方法 12例病人行主动脉瓣环增宽并替换主动脉瓣。手术径路均采用Manouguian法：将主动脉斜切口下延伸,经无冠瓣与左冠瓣交界处向下,垂直切开瓣环至二尖瓣基部。采用绦纶片内衬自体心包片作为增宽材料,替换23 mm或25 mm主动脉瓣;同期行二尖瓣替换4例。结果 1例同期行二尖瓣置换者因严重低心排死于手术当晚;1例术后第7 d因心包填塞经剑突下引流后痊愈;肺部感染并发呼吸功能不全1例,经气管切开及呼吸机支持1周后痊愈;1例主动脉根部明胶海绵物填充后感染于术后12 d再次开胸清除腐烂明胶海绵,并留管冲洗引流而愈。本组术后住院13~20 d,平均（16.2±1.5）d;11例患者均获随访,时间5~84个月,平均（36.7±21.2）个月,心功能恢复至Ⅰ级5例,Ⅱ级6例,无远期死亡。结论对大多数小主动脉瓣环,采用Manouguian术式简单易行,不需切开二尖瓣基部,已能满足替换23 mm或25 mm瓣膜,增宽材料易得,效果可靠。     

女性膀胱流出道梗阻诊断研究进展

女性膀胱流出道梗阻（female bladder outlet obstruction，FBOO）是指各种原因引起的，排尿期膀胱流出道排泄（引流）不畅所造成的，在病理生理过程、临床表现和自然病程等方面有诸多相似之处的一组疾病的总称，简称为女性膀胱出口梗阻。也有人曾称之为膀胱下梗阻。根据国际尿控学会（ICS）的分类，FBOO属于排尿期尿道功能异常中的梗阻性疾患。     

Accessory mitral valve causing left ventricular outflow tract obstruction in an adult

Accessory mitral valve (AMV) is a rare congenital abnormality that, rarely, causes left ventricular outflow tract (LVOT) obstruction in adults. We report the case of a 47-year-old man with deteriorating exertional dizziness. Evaluations revealed that the left ventricular outflow tract obstruction was caused by the accessory mitral valve. The patient underwent a successful operation for removal of the accessory mitral valve.     

Incidence and management of the left ventricular outflow obstruction in patients with atrioventricular septal defects

Open in a separate window OBJECTIVESLeft ventricular outflow tract obstruction (LVOTO) is a recognized complication after complete repair of atrioventricular septal defect (AVSD). This study reviewed the incidence and management of LVOTO following AVSD repair at a single institution.METHODSFrom 1975 to 2019, 24 patients (3.3%, 24/730) underwent reoperation due to LVOTO following partial AVSD (pAVSD) and complete AVSD (cAVSD) repair. The data were retrospectively reviewed.RESULTSThe incidence of LVOTO following pAVSD and cAVSD repair was 4.4% (12/275) and 2.6% (12/455). Freedom from LVOTO reoperation following pAVSD and cAVSD repair at 25 years was 94.3% [95% confidence interval (CI); 89.7–96.7] and 95% (95% CI; 91.1–97.3). The median time from complete repair of pAVSD and cAVSD to LVOTO reoperation was 4.4 years [interquartile range (IQR): 3.4–6.7] and 2.6 years (IQR: 2.2–4.7). Freedom from second LVOTO reoperation at 5, 10 and 15 years was 83.7% (95% CI; 57.2–98.2), 59.2% (95% CI; 28.7, 80.3) and 39.5% (95% CI; 13.2–65.3). The median time between the first and the second LVOTO reoperation in the groups of pAVSD and cAVSD was 6.1 years (IQR: 3.4–8.9) and 8.6 years (IQR: 5.7–9.8). There was no significant difference regarding the first (P = 0.7406) and subsequent LVOTO (P = 0.7153) following complete repair of pAVSD and cAVSD. Combined access to the left ventricular outflow tract was not protective regarding LVOTO reoccurrence. Survival for both groups after LVOTO reoperation at 15 years was 95.6% (95% CI 99.4–72.9).CONCLUSIONSIncidence of LVOTO after AVSD repair is low but the reoccurrence rate is high. Standard subaortic resection does not always provide definitive LVOTO relief. The survival after LVOTO reoperation is excellent.     

Repair of complex left ventricular outflow tract obstruction in children

Background Complex obstruction of the left ventricular outflow tract (LVOTO) in children may be treated with surgical procedures like the Ross–Konno or Koncz–Konno procedures, or modifications thereof. We present our results from the last 10 years.

Material and method During the period from December 1991 to April 2002, 17 patients/children with complex LVOTO were operated on. Patients treated with balloon valvuloplasty, open commisurotomy or simple valve replacement are excluded. The procedures performed were: Koncz–Konno (KK) or modified KK (n=10), Ross–Konno (RK) (n=2) and Ross (RO) (n=5). Concomitant procedures included mitral valve replacement (n=1), aortic arc plasty (n=1), subvalvular myotomi (n=1), resection of subvalvular membrane (n=1) and aorto-coronary bypass (n=1). Patients’ ages ranged from 1 to 178 months, median 71 months. Body weights ranged from 3.8 to 50.0 kg, median 19.4 kg. Eight cases were redo procedures.

Results Three early deaths occurred. One patient in the KK group, one in the modified KK group and one in the RK group. One patient died 8 years postoperatively of unknown reasons; no autopsy was performed. The rest of our patients who survived the initial major surgical treatment show normal physical performance and their quality of life seems to be good.

Conclusion Patients with complex LVOTO can be operated with biventricular repair in selected cases. Early and late mortality are acceptable.     

Bend relief fenestration might prevent outflow graft obstruction in patients with left ventricular assist device

Open in a separate window OBJECTIVESPatients with left ventricular assist devices may experience external obstruction of the outflow graft through a gelatinous substance within the bend relief (BR; a stiff tube graft guiding the outflow graft). Preventative strategies have been missing. Having faced this problem, we decided to fenestrate the BR to avoid outflow graft obstruction (OGO).METHODSSince December 2010, 167 patients underwent left ventricular assist device implantation using HeartMate II or 3. BR fenestration was introduced on July 2018 (108 patients before, 59 after the introduction of BR fenestration). Follow-up computed tomography scans were obtained from all patients and were screened for OGO by 3 independent investigators. Results were correlated with log file history, echocardiographic and clinical outcomes.RESULTSDemographic data were comparable between groups, with mostly male patients. Patients with BR fenestration were older [63 (standard deviation (SD):10.6) vs 58 (SD: 10.7) years] and had shorter support duration [494 (SD: 383) vs 951 (SD: 875) days]. OGO was observed in 5 patients and occurred only in patients without fenestration. Importantly, it occurred late on postoperative Days 412, 462, 1043, 1184 and 1506. Three patients are still asymptomatic. Surgical revision was required in the other 2 patients for pump thrombosis or continuous low flow. One of them died 36 days after revision due to right heart failure.CONCLUSIONSOur results suggest that fenestration of the BR may be a preventative strategy to avoid external OGO. OGO occurred late, which suggests a careful long-term follow-up.     

Reconstruction of right ventricular outflow tract with stentless xenografts in Ross procedure

To investigate whether the use of a stentless porcine aortic xenograft can be an alternative for right ventricular outflow tract (RVOT) reconstruction during the Ross procedure, 9 patients underwent the Ross procedure and RVOT reconstruction with a stentless xenograft since January 2000. After the aortic valve was replaced with a pulmonary autograft, a stentless xenograft with a xeno- pericardial roll was implanted in the RVOT. One patient required subsequent aortic valve replacement because of severe regurgitation of the pulmonary autograft. All patients recovered well from the operation. The right ventricle-pulmonary arterial pressure gradient was 18 +/- 7 mm Hg at discharge and was not significantly increased during the 2-year follow-up period. Although 1 patient died of ventricular arrhythmia 5 months after, his cardiac function was normal, and transpulmonary valve pressure was 19 mm Hg in the follow-up. The other 7 patients are currently in New York Heart Association functional Class I. Although long-term follow-up is required to explain the durability, the stentless xenograft with a pericardial roll is considered to be an alternative for reconstruction of the RVOT within 2 years after the Ross procedure.     

Management of difficult airway in pediatric patients with right ventricular outflow tract obstruction

We present two cases of difficult airway management for patients with Pierre Robin syndrome and right ventricular outflow tract obstruction in infants. To prevent the exacerbation of right ventricular outflow tract obstruction, adequate oxygenation and ventilation are mandatory in this population. This rule needs to be followed even while dealing with a difficult airway. Depending on the prediction of mask ventilation capability, we took two different approaches to difficult airway. In the first case, we fiberoptically intubated the patient while allowing him to breathe spontaneously with the aid of a nasopharyngeal airway under deep sedation. In the second case, we fiberoptically intubated the patient through a laryngeal mask airway while controlling ventilation. Through both cases, we highlight options of difficult airway management in the pediatric population. Although we can approach a difficult airway with or without spontaneous breathing, the important point is how we will prepare the methods to oxygenate and ventilate patients throughout the procedure. Patients with difficult airway and right ventricular outflow tract obstruction are good examples to make us realize this point.     

Modified Damus–Kaye–Stansel procedure using aortic flap technique for systemic ventricular outflow tract obstruction in functionally univentricular heart

Damus–Kaye–Stansel procedure is a useful method to relieve the systemic ventricular outflow tract obstruction in functionally univentricular heart. Regurgitation of pulmonary valve and recurrence of systemic ventricular outflow obstruction are the major concerns at the late phase of this procedure. Modification of original Damus–Kaye–Stansel procedure that can prevent the use of prosthetic materials is evaluated. The modified Damus–Kaye–Stansel procedure using aortic flap technique was performed in eight patients with functionally univentricular heart. Patients’ ages ranged from 3 to 28 months (mean 14 months). Follow-up period was 37 months as a mean (9–71 months), and the follow-up was complete. There was no operative mortality and no late death. In addition, there was no recurrence of systemic ventricular outflow tract obstruction throughout the follow-up period. Regurgitation of the pulmonary valve estimated by echocardiography at the latest follow-up was none to trivial in seven patients and mild in one. The modified Damus–Kaye–Stansel procedure using aortic flap technique is a safe, useful and reproducible technique to solve systemic ventricular outflow tract obstruction in functionally univentricular heart, and it can be an alternative for original technique or the so-called double-barrel modification.