室间隔缺损修补术后继发性主动脉瓣下狭窄的单中心回顾性研究

目的 总结室间隔缺损患者术后继发性主动脉瓣下狭窄（secondary subaortic stenosis,SSS）的临床经验，探究其潜在发生机制。方法 纳入2008—2019年在阜外医院行单纯开胸室间隔缺损修补手术，术前无左室流出道梗阻，但术后出现SSS并于阜外医院进行二次手术的0～18岁患者。回顾性收集患者的临床资料，分析室间隔缺损修补术后出现SSS的形态学特征、再次干预方式及随访结果。结果 共纳入6例患者，其中女2例、男4例。首次室间隔缺损修补手术中位年龄9个月（1个月～3岁）。首次手术后2.9年（1～137个月）患者被诊断为SSS,2例患者在确诊后立即行二次手术，其余4例等待1.2年（6～45个月）后行瓣下狭窄手术治疗。最常见的室间隔缺损外科术后SSS为隔膜型，新生隔膜位于左室流出道并呈环形，小部分沿室间隔补片生长。SSS术后中位随访时间8.1(7.3～8.9)年，所有患儿均未复发左室流出道梗阻。结论 无论是单纯室间隔缺损外科修补还是合并其他心脏畸形的先天性心脏病外科手术，术后长期随访复查、及时干预，都是阻止主动脉瓣反流或左室流出道梗阻进展的最佳方法。     

解剖性双心室流出道重建技术:双根部调转手术

目的 总结双根部调转术治疗合并室间隔缺损(VSD)和左心室流出道狭窄的心室大动脉连接异常的中期结果.方法 2007年12月至2013年9月,78例患者接受双根部调转手术.男55例,女23例;年龄0.3～22.0岁,中位年龄3.0岁.涤纶补片修补VSD,主动脉根部调转时进行冠状动脉再植,用带单瓣的牛颈静脉或同种血管片重建肺动脉.随访2～ 98个月,中位随访时间56个月,超声心动图评估双侧心室流出道功能.结果 院内死亡3例,占4.4％,死因分别为肾功能衰竭、低心排血量综合征和脓毒血症.随访期间无再次手术;2例死亡,原因分别是心功能衰竭和猝死.术后超声心动图提示患者重建后的双心室流出道血流动力学满意,心功能正常.左心室流出道压差接近正常,提示左心室流出道疏通满意;平均右心室流出道压差10.4 mmHg(1.38 kPa),多数患者的肺动脉仅少到中量反流.结论 采用带单片的补片和自体肺动脉重建的右心室流出道,保持通畅性和生长潜能.双根部调转手术中期随访结果良好,从心脏血流动力学的角度来看,此方法是对合并室间隔缺损和左心室流出道狭窄的大动脉转位和右心室双出口这一疾患的真正意义上的解剖学矫治.     

Konno-Rastan手术治疗儿童复杂性多水平左心室流出道梗阻

目的 总结Konno-Rastan手术治疗复杂多水平左心室流出道梗阻的疗效、并发症和常见失误及预防.方法 1996年1月至2012年8月,13例患儿行主动脉根部及左心室流出道扩大、人工机械瓣膜主动瓣置换(Konno-Rastan术).男8例,女5例;年龄5～13岁;体质量12～51 kg,中位值21 kg.诊断先天性主动脉瓣狭窄8例,先天性主动脉瓣狭窄合并主动脉瓣上狭窄3例,先天性主动脉瓣狭窄合并室间隔缺损、主动脉缩窄及右心室流出道狭窄1例,先天性主动脉瓣狭窄、主动脉瓣球囊扩张术后再狭窄1例.患儿均合并继发性室间隔增厚、左心室流出道梗阻.主动脉瓣环直径12.0 ～ 16.4 mm,术前跨主动脉瓣压差90～ 151 mm Hg(1 mm Hg =0.133 kPa).置入St.Jude AG19号机械瓣膜8例,AG17号5例.结果 无手术死亡.呼吸机辅助4～74 h,中位时间6h;ICU停留1～6天.1例术后第3天出现突发性晕厥,确诊为Ⅲ度房室传导阻滞,植入永久性心外膜起搏器,术后7天恢复窦性心律,传导功能正常,将起搏器设定为60次/min,VVI模式备用.2例术后3个月复查发现新生心室水平残余分流,其中1例因反复心功能衰竭再次手术,修补残余分流,术后心功能状态良好,无残余心内畸形;1例因无明显症状,门诊随访,术后3年患类风湿,感染性心内膜炎及肾病综合征,拟择期行残余分流修补术.其余患儿无不适症状.全部患儿随访1 ～78个月,终生服用华法林抗凝,控制国际标准比值(INR) 1.8 ～2.5.随访期内无死亡,未发现人工瓣膜相关性并发症发生.完全性房室传导阻滞发生率为7.7％;残余分流发生率为15.4％;术后早期感染性心内膜炎发生率为0,远期发生率为7.7％.结论 Konno-Rastan手术可以有效地治疗患儿复杂的多水平左心室流出道梗阻,但由于手术操作复杂,可导致较高比例的手术并发症;完善手术操作是提高手术成功率的关键因素之一.     

49例儿童先天性主动脉瓣狭窄的外科治疗

目的 回顾性总结先天性主动脉瓣狭窄患儿手术治疗的效果及经验.方法 2006年2月至2011年11月,共收治49例先天性主动脉瓣狭窄患儿,男29例,女20例;年龄1个月～ 14岁;体质量3.2 ～47.0 kg.轻度狭窄2例,中度狭窄21例,重度狭窄26例.术前跨瓣压差45～123 mm Hg(1 mm Hg =0.133 kPa),平均(74.9±20.4)mm Hg.单纯主动脉瓣狭窄14例,伴有中—重度反流4例.伴其他心内畸形33例,包括室间隔缺损、房间隔缺损、动脉导管未闭、主动脉缩窄、左心室流出道狭窄、二尖瓣瓣上环等.行主动脉瓣交界切开术31例,主动脉瓣成形术9例,主动脉瓣置换术2例,Ross手术1例,Ross-Konno手术2例,Konno+主动脉瓣置换术4例.术后随访2～55个月,平均20个月.评估患儿心功能、主动脉瓣跨瓣压差及瓣膜反流程度.结果 死亡1例,生存患儿术后心功能良好,左心室射血分数0.69±0.10,短轴缩短分数0.38±0.09.术后跨瓣压差20 ～ 73 mm Hg,平均(38.6±15.8)mm Hg,较术前降低(36.2±18.3)mm Hg(P ＜0.001).40例患儿行主动脉瓣交界切开和成形,术后主动脉瓣反流程度为无或轻微8例,轻度25例,中度7例.1例主动脉瓣交界切开术后因切开处复粘连再次行交界切开术.结论 先天性主动脉瓣狭窄的患儿如无明显反流,可行主动脉瓣交界切开术或同时行主动脉瓣成形术,如果反流中度以上,则需根据患儿年龄等情况选择合适的手术方法.     

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。     

双心室矫治术治疗右心室双出口合并完全性房室间隔缺损

目的总结右心室双出口(DORV)合并完全性房室间隔缺损(AVSD)的双心室解剖矫治经验。方法回顾性分析1996年1月至2010年12月阜外心血管病医院14例DORV-AVSD患者施行双心室解剖矫治术的临床资料,其中男9例,女5例;年龄6个月～31岁。患者均行双心室解剖矫治术,经右心房和右心室切口,疏通右心室流出道,分隔并成形房室瓣,采用"逗号状"补片修补室间隔缺损,同时构建通畅的左心室流出道,自体心包闭合Ⅰ孔房间隔缺损,用心包或跨瓣环补片加宽右心室流出道。结果 1996年1月至2008年12月收治的10例患者中,住院死亡5例,其中术中不能脱离体外循环3例,不能脱离呼吸机2例;住院时间23～105 d,住ICU时间5～90 d,机械通气时间1～52 d。2009年1月至2010年12月收治的4例患者中,无住院死亡,术后未发生并发症;住院时间21～41 d,住ICU时间4～21 d,机械通气时间1～7 d。随访9例,随访时间6～26个月,随访期间无死亡,无流出道残余梗阻。结论 DORV-AVSD患者可一期行双心室矫治术,近年来手术效果明显提高。     

先天性心脏病术中心脏瓣膜的保护和矫治

目的 总结先天性心脏病术后因瓣膜功能不全再手术的经验。方法 回顾分析先天性心脏病术后再行瓣膜手术13例患者的临床资料,其中室间隔缺损修补术后8例,部分心内膜垫缺损修补术后3例,法洛四联症和房间隔缺损修补术后各1例。第1次手术时即存在二尖瓣轻～中度关闭不全6例,主动脉瓣关闭不全1例;新出现瓣膜功能异常6例,其中2例因补片漏致三尖瓣关闭不全,2例因前叶腱索断裂致三尖瓣关闭不全,1例因残留右心室流出道狭窄继发三尖瓣关闭不全,1例因伤及主动脉瓣并发二尖瓣和三尖瓣关闭不全。13例中,行二尖瓣置换6例,三尖瓣置换2例,主动脉瓣置换1例,行主动脉瓣置换并二尖瓣、三尖瓣成形1例,三尖瓣成形3例。同时修补残余漏,疏通右心室流出道。结果 术后发生低心排综合征3例。2例术后早期分别死于脑气栓和呼吸循环衰竭。11例术后痊愈出院,随访1～8年,心功能良好。结论 先天性心脏病矫治术中应注意心脏瓣膜的保护,合并的瓣膜功能异常应积极修补,及时地再手术可取得良好效果。     

β-受体阻滞剂对主动脉瓣置换术后心室内压力阶差的影响

目的探讨β-受体阻滞剂对主动脉瓣重度狭窄行主动脉瓣置换术患者心室内压力阶差及其左心室重塑的影响。方法选取郑州大学第一附属医院2008年1月至2010年1月因单纯主动脉瓣重度狭窄行主动脉瓣置换术患者56例,将术后得到随访的32例患者在相同基础临床特征下分为两组,试验组：12例,常规口服β-受体阻滞剂（倍他乐克6.25-25.00 mg/次,2次/天）;对照组：20例,未服用β-受体阻滞剂。在术后早期（1周内）、晚期（6-24个月）复查超声心动图,对两组患者随访的超声心动图指标进行比较。结果两组患者术后晚期随访无死亡,胸闷、气促等症状均明显改善。两组患者术后晚期左心室收缩期末内径（LVESD）、左心室流出道内径（LVOTD）较术后早期均有增加,仅试验组LVOTD与术后早期比较差异有统计学意义（t=-47.937,P=0.001）;两组患者术后晚期室间隔厚度（IVS）、左心室后壁厚度（LVPWT）、左心室流出道射流速度（V）、心室压力阶差（G）、左心室心肌重量指数（LVMI）均较术后早期有所减小,以上指标试验组术后晚期与术后早期结果比较差异均有统计学意义（t=7.781,P=0.001;t=5.749,P=0.001;t=2.637,P=0.023;t=7.167,P=0.001;t=100.061,P=0.001）,而对照组仅V、G和LVMI差异有统计学意义（t=4.051,P=0.001;t=4.759,P=0.001;t=-0.166,P=0.001）;试验组EF值术后晚期与术后早期比较差异有统计学意义（t=-6.621,P=0.001）。组间比较：两组术后晚期EF值差异无统计学意义（t=-0.354,P=0.726）,但术后晚期左心室舒张期末内径（LVEDD）、IVS、G和LVMI两组间比较差异均有统计学意义（t=-2.494,P=0.018;t=-3.434,P=0.002;t=-2.171,P=0.038;t=-2.316,P=0.028）。结论重度主动脉瓣狭窄患者行主动脉瓣置换术后常规应用β-受体阻滞剂是安全、可靠的,可显著降低其残存的心室内压力阶差,并可加速改善左心室的重塑。     

心包卷重建右心室流出道矫治肺动脉闭锁合并室间隔缺损——单中心回顾性随访研究

目的明确肺动脉闭锁合并室间隔缺损行心包卷重建右心室流出道后肺动脉发育、再次狭窄及瓣膜反流情况。方法回顾性分析2002年11月至2013年9月我院41例肺动脉闭锁合并室间隔缺损患者的临床资料,其中男25例、女16例,手术年龄4.00个月至22.70岁(56.60±63.92)个月,均采用心包卷重建右心室流出道。随访评估心包卷及肺动脉发育情况、肺动脉与三尖瓣反流及其与随访时间的相关关系。结果术后早期死亡5例(12.19%)。随访4.00个月至10.75年(3.00±2.35)年。随访期间失访3例(7.31%),二期手术后死亡1例。术后早期无吻合口狭窄,随访期间心包卷及左肺动脉无明显增长;10例出现心包卷重度狭窄,5例肺动脉分支中-重度狭窄;肺动脉及三尖瓣反流均较术后早期明显增加,反流增长量与随访时间长短无相关关系。结论心包卷重建右心室流出道矫治肺动脉闭锁合并室间隔缺损早期效果良好,心包卷无远期生长能力,再次狭窄发生率较高,术后需严密随访。     

主动脉瓣置换术后室间隔厚度对主动脉瓣跨瓣压差的影响

目的 探讨主动脉瓣置换术后室间隔厚度对主动脉瓣跨瓣压差的影响.方法 2005年1月至2010年12月,接受主动脉瓣置换术患者273例,全部为单纯主动脉瓣狭窄.根据手术时使用的主动脉瓣不同品牌,将患者分为3组:Regent组、On-x组、其他品牌组.定义术后跨瓣压差大小为:轻度(0～30 mm Hg,1 mm Hg=0.133 kPa)、中度(30 ～60 mm Hg)及重度(大于60 mm Hg).分别比较术前、术后测量的左心室收缩期末径、左心室舒张期末径、左心室射血分数(EF值)和室间隔厚度.结果 3组患者术前的左心室收缩期末径、左心室舒张期末径及EF值差异无统计学意义(P＞0.05).Regent组患者,术后跨瓣压差为重度的患者术前室间隔厚度明显多于轻、中度(P＜0.05).使用On-x组患者,术后跨瓣压差为重度与中度的患者术前室间隔厚度者明显多于轻度组(P＜0.05).其他品牌组患者,术后跨瓣压差为重度的患者术前室间隔厚度明显多于中、轻度组(P＜0.05).结论 行主动脉瓣置换术后,术前室间隔厚度大于13.6 mm的患者其主动脉瓣跨瓣压差较厚度小于13.6 mm的患者显著增高.室间隔厚度大于15.3 mm的患者,应同期行室间隔部分切除或替换无支架瓣膜以减少主动脉瓣替换后的跨瓣压差.     

Left ventricular outflow tract obstruction after partial atrioventricular septal defect repair

BACKGROUND: Narrowing of the left ventricular outflow tract has been associated with partial atrioventricular septal defect (PAVSD) in about 3% of patients. Because of the predisposing anatomy, hemodynamically significant obstruction in the subaortic area may appear after repair of ostium primum atrial septal defects. METHODS: From 1984 to 1998, 40 patients underwent surgical correction of PAVSD by patch closure. The mean age at the initial repair was 5.8 years (range 3 months to 22 years). RESULTS: Nine patients had 12 subsequent operations for hemodynamically significant subaortic obstruction. The mean age at PAVSD repair was 17 months (3 to 42 months) (p < 0.001 compared with others). Follow-up work-up was obtained due to symptoms in 5 patients and an abnormal echocardiogram in 4 asymptomatic patients. Subaortic stenosis developed at a mean of 5 years (range 4 months to 10 years), and 6 or more years in 4 patients. The mean age at subaortic stenosis repair was 6 years (range 2 to 12 years). Nine patients underwent subaortic fibromuscular resection. Of these, 4 developed recurrent stenosis and 2 have undergone additional operations. CONCLUSIONS: Left ventricular outflow tract obstruction after PAVSD repair may be more frequent than reported. Because of the progressive nature of the process, echocardiography should be utilized liberally on patients to uncover subclinical stenosis. Long-term follow-up is essential for diagnosis due to delayed appearance and lack of reliable clinical signs.     

Secondary subaortic stenosis in heart defects without any initial subaortic obstruction: a multifactorial postoperative event.

BACKGROUND/OBJECTIVE: Secondary subaortic stenosis (SSS) can occur after surgery for various congenital heart defects with or without initial left ventricular outflow tract obstruction (LVOTO). The objective of this study was to highlight the anatomical lesions and surgical procedures associated with the development of SSS after surgery on defects without initial LVOTO. METHODS: A retrospective study of 4710 patients was performed (1984-2005). The criterion for inclusion was a fixed subaortic obstruction requiring surgery, after an open- or closed-heart operation. The criterion for exclusion was an LVOTO at the time of the first operation. RESULTS: Twenty-eight patients were studied. The mean age at initial surgery was 32 months (4 days-47 years; median: 2 months). SSS occurred after three main types of surgery: repair of coarctation of the aorta, repair of AVSD and LV-aorta rerouting for double outlet right ventricle or transposition of great arteries. The mean delay of occurrence was 4.4 years (2 months-19 years). Frequently associated initial anatomical conditions were coarctation of the aorta (40%), lesions of the mitral valve (32%), bicuspid aortic valve (21%) and left superior vena cava (LSVC) (14%). Preoperative anatomical lesions of the LVOT were present in 93% of the cases. After the initial operation, only one patient had a mean echo-Doppler pressure gradient across the LVOT>20 mmHg. SSS was most frequently a subaortic membrane (n=23). The mean pressure gradient across SSS at the time of reoperation was 47+/-29 mmHg. Five patients developed a second SSS after 7.4 years (mean). One patient developed a third SSS. No patient died. When compared with patients without SSS, significant risk factors for SSS were low age at surgery (32 vs 74.9 months, p<10(-4)), pre-existing coarctation of the aorta (40 vs 10%, p<10(-4)), bicuspid aortic valve (21 vs 6%, p=0.002) and LSVC (14 vs 4%, p=0.02). CONCLUSIONS: SSS development is multifactorial, depending on initial anatomical lesions and initial surgery. Low age at initial surgery, coarctation of the aorta, bicuspid aortic valve and LSVC significantly increase the risk of SSS. These elements warrant long-term follow-up for early detection of SSS.     

Myectomy versus myotomy as an adjunct to membranectomy in the surgical repair of discrete and tunnel subaortic stenosis

The results of membranectomy and deep myectomy in the left ventricular outflow tract were compared to those of membranectomy and myotomy in 42 patients who underwent surgical repair of discrete and tunnel subaortic stenosis. Fifteen consecutive patients (Group A) underwent membranectomy and myotomy, and 27 consecutive patients (Group B) underwent membranectomy and myectomy. Two patients of Group A and nine of Group B had tunnel subaortic stenosis. The preoperative mean (+/- standard deviation) peak systolic gradients across the left ventricular outflow tract in patients with discrete subaortic stenosis types I and II were 64 +/- 29 mm Hg in Group A and 52 +/- 3 mm Hg in Group B (p = not significant). In the patients with tunnel subaortic stenosis the preoperative mean gradients were 97 +/- 74 mm Hg in Group A and 73 +/- 26 mm Hg in Group B (p = not significant). In patients with discrete subaortic stenosis types I and II, postoperative catheterization at a mean follow-up of 21 months revealed residual mean gradients of 29 +/- 24 mm Hg in Group A and 10 +/- 13 mm Hg in Group B (p less than 0.01). In the patients with tunnel subaortic stenosis, the postoperative mean gradients were 25 +/- 7 and 30 +/- 30 mm Hg in Groups A and B, respectively (p = not significant). We conclude that in the surgical management of discrete subaortic stenosis types I and II, deep myectomy (in addition to membranectomy) produces better relief of the left ventricular outflow obstruction than do membranectomy and myotomy. In patients with tunnel subaortic stenosis myectomy is less effective than in the non-tunnel type but still produces acceptable results and may delay radical procedures to a later age.     

Aortoventriculoplasty for different types of left ventricular outflow tract obstructions

Since the first clinical application of aortoventriculoplasty for tunnel subaortic stenosis in 1974 the indication for this method was extended to other types of left ventricular outflow tract stenoses (LVOTO). The operative technique consists of enlarging both the left and right ventricular outflow tracts and inserting an aortic prosthetic valve. 47 operations have been performed in patients with various types of LVOTO: 8 narrow annulus, 23 diffuse subaortic stenosis (multiple level stenosis), 9 complex forms of tunnel subaortic stenosis (Shone complex) 3 outgrown prosthesis, 4 obstructive idiopathic hypertrophic subaortic stenosis. Patients ages ranged from 4 to 35 years. Overall mortality was 13%, there were no late deaths, in the last 34 patients there was no death. In 25 patients there had been 1, and in 13 patients 2 previous procedures. As a result of the operation 9 patients developed complete right bundle branch block or left anterior hemi-block; 2 patients developed total a-v block with the need of a permanent pacemaker 25 patients had catheterization postoperatively. The mean gradient across the left ventricular outflow tract was significantly reduced from 91.5 +/- 21 mm Hg to 13.1 +/- 15 mm Hg. According to our experience aortoventriculoplasty can be used routinely in all forms of diffuse subaortic stenosis, narrow aortic annulus, reoperation in HOCM, multiple level stenosis and outgrown aortic prosthesis.     

Subvalvar aortic stenosis: timing of operation

Subvalvar aortic stenosis can be associated with progressive left ventricular outflow tract obstruction, aortic insufficiency, and infective endocarditis. We reviewed the records of 36 surgical patients who underwent 39 operations for subaortic stenosis. Seventeen patients had associated congenital cardiac anomalies. One perioperative death occurred in a patient with tetralogy of Fallot. The mean preoperative left ventricular outflow tract systolic pressure gradient was 64 +/- 5 mm Hg (+/- standard error of the mean) and decreased to 9 +/- 2 mm Hg postoperatively (p less than 0.001). Reliable preoperative and postoperative information regarding aortic valve function was available for 27 patients. Aortic insufficiency was found in 17 (63%) of those patients preoperatively. Postoperatively, insufficiency increased in 3 patients and decreased in 4; none of these changes was major. Severity of preoperative aortic insufficiency increased significantly with age (p less than 0.05), but did not correlate with left ventricular outflow tract gradient. The information from this study and previous studies suggests that resection of subaortic stenosis is safe and effective, and operation at the time of diagnosis, regardless of left ventricular outflow tract gradient or symptomatic status, is a reasonable therapeutic alternative.     

Diagnosis, transport, and outcome in fetuses with left ventricular outflow tract obstruction

Between March 1986 and April 1990, 22 consecutive fetuses (at gestational ages of 21 to 38 weeks) with a suspected diagnosis of critical (ductus-dependent) left ventricular outflow tract obstruction on fetal echocardiogram were referred to our center for delivery and surgical treatment. Diagnoses were hypoplastic left heart syndrome (n = 16), valvular aortic stenosis (n = 2), common atrioventricular canal with subaortic stenosis (n = 3), and single ventricle with subaortic stenosis (n = 1). Postnatal echocardiography revealed that fetal echocardiography was correct in predicting left ventricular outflow tract obstruction to be critical in all but one patient, for a positive predictive value of 96%. Of the 21 patients with true, critical left ventricular outflow tract obstruction, 17 patients underwent cardiac surgery as neonates (birth to 6 days of age, median 2 days); 13 (or 77%) survived and were discharged from the hospital. In addition, one patient underwent successful balloon aortic valvotomy for critical valvular aortic stenosis but later died of sepsis. Lethal chromosomal and congenital abnormalities should be sought and are contraindications for this approach. In utero transport of fetuses with suspected critical left ventricular outflow tract obstruction to a neonatal cardiac surgical center can result in improved neonatal condition and may improve overall survival.     

Surgical management of diffuse subaortic stenosis: an integrated approach

An integrated approach to the surgical management of diffuse subaortic stenosis has been designed to provide adequate relief of left ventricular outflow tract obstruction whatever the anatomical features encountered at operation. This approach was used in 22 patients with tunnel subaortic stenosis (19 patients) or diffuse hypertrophic obstructive cardiomyopathy (3 patients). The obstructive tissue was resected through an aortoseptal approach. In 18 patients, associated hypoplasia of the aortic orifice necessitated aortic valve replacement using the Konno procedure; in 4 patients with a normal-sized aortic orifice, the native aortic valve was preserved. There were two early deaths and one late death (all after a Konno operation). Long-term adequate relief of left ventricular outflow tract obstruction was achieved in all survivors. Operation for diffuse subaortic stenosis should be performed with two main goals: (1) to obtain complete relief of the left ventricular outflow tract obstruction by the appropriate procedure and (2) to preserve the native aortic valve whenever possible, particularly in young patients.     

Apico-aortic conduit repair for the subaortic stenosis in the long-term period after correction of Taussig-Bing malformation]

Apico-abdominal-aortic bypass operation was successfully performed for the left ventricular outflow tract obstruction 17 years after correction of Tausig-Bing malformation. At the age of 14 months old, a 18-year-old patient underwent total correction of Taussig-Bing malformation according to Kawashima's intraventricular re-routing. The right ventricular hemodynamics were normal throughout, however, systolic ejection murmur due to aortic stenosis appeared 3 years after repair. Seventeen years postoperatively, marked aortic valve and subaortic obstruction was detected corresponding with the internal conduit made from the duplicated autopericardial baffle. A composite graft containing 23A-SJM valve was interposed between the left ventricular apex and infrarenal abdominal aorta. After the bypass operation, thickness of the posterior wall of the left ventricle and interventricular septum was significantly reduced and symptoms were remarkably improved. The present paper is concerned with the subaortic stenosis after repair of Taussig-Bing malformation and technique of apico-aortic bypass operation.     

Results of aortoventriculoplasty in 21 consecutive patients with left ventricular outflow tract obstruction

Results of aortoventriculoplasty (AVP) are reported in 21 patients with various types of left ventricular outflow tract obstruction (LVOTO). The concept of AVP is based on creating a surgical aortoseptal defect which is patched to provide the largest possible outflow tract to the left ventricle. Lesions consisted of isolated diffuse fibromuscular subaortic stenosis in six patients, diffuse subaortic stenosis and associated other cardiovascular anomalies in five, hypoplastic aortic anulus in two, idiopathic hypertrophic subaortic stenosis (IHSS) in two, and stenosis of a previously implanted aortic valvular prosthesis in three patients. Ten patients had had at least one unsuccessful previous surgical attempt to relieve the LVOTO. The coexisting mitral incompetence in IHSS disappeared after AVP alone. Immediate postoperative hemodynamic results were excellent in all cases. Postoperative death in five patients was due to advance myocardial failure in two, brain damage in one, transection of a dominant septal artery in one, and severe acidosis with renal failure in the last case. However, in the last 16 patients (17 operations) the only death (5.8 percent) was that caused by uncontrollable acidosis. Follow-up results indicate that 16 patients are clinically doing well, and hemodynamic studies in 14 patients are rated as excellent or good from 1 to 25 months postoperatively. It is concluded that AVP is an effective operation for managing all types of LVOTO and can be used routinely with an acceptably low mortality rate.     

Surgical treatment of subaortic stenosis: a seventeen-year experience

OBJECTIVE: The aim of the study was to analyze the long-term results of subaortic stenosis relief and the risk factors associated with recurrence and reoperation. METHODS: One hundred sixty patients with subaortic stenosis underwent biventricular repair. Before the operation the mean left ventricle-aorta gradient was 80 +/- 35 mm Hg, 57 patients had aortic regurgitation, and 34 were in New York Heart Association functional class III or IV. Median age at repair was 10 years. For discrete subaortic stenosis (n = 120), 39 patients underwent isolated membranectomy, 67 underwent membranectomy with associated septal myotomy, and 14 underwent septal myectomy. Tunnel subaortic stenosis (n = 34) was treated by myotomy in 10 cases, myectomy in 12, septoplasty in 7, Konno procedure in 3, and apical conduit in 2. Aortic valve replacement was performed in 6 cases, mitral valve replacement in 2 cases, and mitral valvuloplasty in 4 cases. RESULTS: There were 5 early (3.1%) and 4 late (4.4%) deaths. Within 3.6 +/- 3.3 years a recurrent gradient greater than 30 mm Hg was found in 42 patients (27%), 20 of whom had 26 reoperations. According to multivariable Cox regression analysis survival was influenced by hypoplastic aortic anulus (P =.01) and mitral stenosis (P =.048); recurrence and reoperation were influenced by coarctation and immediate postoperative left ventricular outflow tract gradients. At a median follow-up of 13.3 years, mean left ventricle-aorta gradient was 20 +/- 13 mm Hg. Relief of the subaortic stenosis improved the degree of aortic regurgitation in 86% of patients with preoperative aortic regurgitation. Actuarial survival and freedom from reoperation rates at 15 years were 94% +/- 1.3% and 85% +/- 6%, respectively. CONCLUSION: Although surgical treatment provides good results, recurrence and reoperation are significantly influenced by previous coarctation repair and by the quality of initial relief of subaortic stenosis.