Ross-Konno手术治疗儿童主动脉瓣及瓣下狭窄的临床疗效

目的评价Ross-Konno手术治疗儿童主动脉瓣及瓣下狭窄的早期临床疗效。方法 2018年12月至2019年12月,共12例患儿因主动脉瓣及瓣下狭窄在我科接受手术治疗,其中男8例、女4例,接受手术时中位年龄4岁(11个月~12岁)。Ross-Konno手术11例,单纯Ross手术1例。主动脉瓣重度狭窄8例,主动脉瓣关闭不全2例,狭窄合并关闭不全2例。合并主动脉二瓣化畸形6例,瓣下流出道显著狭窄4例,合并二尖瓣病变4例,合并弓部病变3例。手术采用自体肺动脉带瓣管道移植于主动脉瓣位,同期采用Konno法扩大左室流出道。采用牛颈静脉或带瓣Gore-tex人工血管重建右室流出道,并同期矫治心内合并畸形。所有患者均经多普勒彩色超声心动图定期进行随访评估。结果全组无手术死亡。术后中位随访时间5.5(1~12)个月,1例患儿残余左室流出道中度狭窄,其余患儿无显著左室流出道残余梗阻或复发梗阻,无新主动脉瓣显著反流。1例患儿肺动脉带瓣管道主干中度狭窄,余重建右室流出道血流通畅,无明显瓣膜反流及赘生物形成。结论对于不适合瓣膜成形或置换术的儿童主动脉瓣膜病变,Ross-Konno手术早期结果满意。     

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.     

保留瓣膜的主动脉根部置换术治疗法洛四联症术后主动脉根部扩张的临床疗效

目的 评价保留瓣膜的主动脉根部置换术（valve-sparing aortic root replacement,VSARR）治疗法洛四联症术后主动脉根部扩张的临床效果。方法 回顾性分析2016—2022年四川省人民医院采用VSARR治疗法洛四联症术后合并主动脉根部扩张患者的临床资料。结果 共纳入14例患者,其中男8例、女6例,中位年龄22(12～48)岁。主动脉瓣重度反流5例、中度反流4例、轻度及以下反流5例。主动脉窦部扩张6例,升主动脉明显扩张8例,同期合并室间隔缺损残余分流1例,合并重度肺动脉瓣反流9例。VSARR方式为David手术10例,Yacoub手术2例,Florida sleeve 2例。全组无手术死亡。术后患者中位随访时间2.9(0.4～6.0)年,1例患者出现轻度主动脉瓣反流,其余患者均为微量或无主动脉瓣反流,1例患者出现左室流出道轻度狭窄,余所有患者左室流出道未见明显狭窄。结论 VSARR在法洛四联症术后主动脉根部扩张患者中治疗结果满意,中期随访未见明显左室流出道狭窄,主动脉瓣反流发生率无明显上升。     

先天性心脏病合并肺动脉瓣感染性心内膜炎的外科治疗

目的探讨先天性心脏病合并肺动脉瓣感染性心内膜炎的外科治疗方法和效果。方法 2009年1月至2012年1月第二军医大学长海医院收治先天性心脏病合并肺动脉瓣感染性心内膜炎患者6例,男1例,女5例;年龄8～41(24.8±11.8)岁。动脉导管未闭(PDA)4例,PDA合并先天性主动脉瓣狭窄1例,室间隔缺损(VSD)合并三尖瓣反流1例。3例行PDA结扎+肺动脉内赘生物清除术,1例行PDA结扎+肺动脉瓣赘生物切除术,1例行PDA结扎+肺动脉瓣赘生物切除术+主动脉瓣置换术(AVR,置换19 mm CarboMetics环上机械主动脉瓣),1例肺动脉瓣膜破坏严重,行VSD修补+右心室流出道赘生物清除+右心室流出道拓宽+三尖瓣成形术+肺动脉生物瓣置换术(置换27 mm HancockⅡ型生物瓣)。结果 6例患者术后均恢复良好,无围术期死亡和心内膜炎复发。所有患者复查超声心动图提示,未见赘生物、残余漏、瓣周漏等并发症。术后随访6个月～3年中,患者临床症状完全消失,均无明显不适。术后心功能恢复至Ⅰ级5例,Ⅱ级1例。结论对于先天性心脏病合并肺动脉瓣感染性心内膜炎患者,正确把握手术时机,积极行外科手术治疗是最有效的治疗方法。     

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。     

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。     

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.     

继发于先天性心脏病术后的主动脉瓣下狭窄手术矫治分析

目的 总结少见病例——继发于先天性心脏病（congenital heart disease,CHD）手术后主动脉瓣下狭窄（secondary subaortic stenosis,SSS）的手术疗效,并分析导致SSS发生的原因。 方法 回顾性分析2008年6月1日至2012年12月31日期间广东省人民医院对SSS进行手术矫治19例患者的临床资料,其中男10例,女9例。CHD类型主要包括右心室双出口、室间隔缺损、部分性房室间隔缺损等。患者矫治CHD时的年龄：4个月（15 d～5岁11个月）;行主动脉瓣下狭窄矫治时的年龄：5岁3个月（1岁～15岁3个月）。患者行SSS矫治与CHD矫治的相隔时间：4年10个月（8个月～13年11个月）。 结果 所有患者均行主动脉瓣下狭窄矫治术,无术中死亡;体外循环时间79 （39～172） min,主动脉阻断时间42 （22～124） min。术后住院时间7 （5～9） d。1例术后需安装永久性心脏起搏器。所有患者均随访。随访时间1年10个月（5个月～4年4个月）。所有SSS患者术后无临床症状,心功能正常,随访过程中无死亡。1例患者随访过程中左心室流出道狭窄逐渐加重,再次行主动脉瓣下狭窄矫治术。 结论继发先天性心脏病术后出现主动脉瓣下狭窄极少见,其发生原因考虑CHD矫治后左心室流出道血流动力学紊乱造成,其发生时间与继发先心病手术类型无法预测,主动脉瓣下狭窄矫治术对SSS矫治简单、有效,但术后左心室流出道仍有可能再次狭窄并加重。     

Ross手术治疗主动脉瓣病变

目的　探讨Ross手术主动脉根部置换技术 ,总结临床经验。方法　 1994年 10月至 2 0 0 2年5月为 7例主动脉瓣病变者行Ross手术。右室流出道重建均采用同种异体肺动脉。其中男 4例 ,女 3例 ;年龄 11～ 44岁 ,平均 ( 2 8± 10 )岁。 7例均有重度主动脉瓣关闭不全 ,伴有中度狭窄 2例 ,二瓣畸形和房、室间隔缺损各 1例 ;7例肺动脉瓣均正常。结果　全组无手术死亡。术后随访 3～ 78个月 ,1例 3年后因感染性心内膜炎二次手术死亡 ;其余 6例超声心动图显示主动脉瓣无反流 ,肺动脉瓣功能正常 ,左心室舒张末径明显缩小 ,心功能正常。结论　尽管Ross手术技术有一定的难度 ,但其恢复瓣膜性能和远期疗效良好。     

先天性心脏病外科治疗中国专家共识（二）：小儿先天性主动脉瓣狭窄

正主动脉瓣狭窄(aortic valve stenosis,AS)是一种左室流出道梗阻性病变,发生率为0.03～0.34/1 000存活新生儿,占所有先天性心脏病的3%～5%~([1])。AS约占左室流出道狭窄的75%,男性多见,男性发生率要高于女性3～5倍,常合并室间隔缺损、动脉导管未闭、左心室发育不良综合征、主动     

获得性主动脉瓣瓣膜病变的整形手术

目的为了恢复主动脉瓣的功能,总结４０例获得性主动脉瓣整形术的经验。方法对４０例获得性主动脉瓣病变进行整形手术,包括主动脉瓣边缘楔形削除、交界切开、钙化灶清除、穿孔修补及自体心包加宽瓣叶等整形手术。结果手术死亡率５％。单纯主动脉瓣关闭不全组术后反流面积明显减少;单纯主动脉瓣狭窄组或狭窄合并关闭不全组术后跨瓣压差明显下降,而后者反流加重。结论在获得性主动脉瓣病变中,瓣膜条件尚好的单纯关闭不全或狭窄可行整形矫正,而狭窄合并关闭不全者行整形难以恢复正常瓣膜功能     

保留主动脉瓣主动脉根部置换术治疗主动脉根部瘤的疗效分析

目的评价使用保留主动脉瓣主动脉根部置换术治疗主动脉根部瘤的临床应用疗效。方法 2001年2月至2010年9月阜外心血管病医院对60例主动脉根部瘤患者行保留主动脉瓣主动脉根部置换术,其中男44例,女16例;年龄9～64(37.2±13.0)岁。主动脉夹层15例,升主动脉瘤10例,马方综合征25例,主动脉瓣二瓣化畸形2例。行主动脉根部重建术53例,主动脉瓣瓣叶再植术7例。比较术前、术后心功能及主动脉瓣反流程度等指标。结果全组中无手术死亡和严重并发症发生,呼吸机使用时间中位数为13(2～1 110)h,住ICU时间1～18(2.7±2.5)d。术后复查超声心动图提示:主动脉瓣反流程度均明显减轻,仅3例为中大量反流,其余无反流或微少量反流。术后随访2～122(61.5±35.9)个月,随访56例,失访4例,随访期间死亡9例,生存率83.9%(47/56)。2例因主动脉瓣反流分别于术后13个月和14个月再次入院行主动脉瓣置换术。47例患者心功能较术前明显改善,心功能Ⅰ级35例(74.4%),Ⅱ级8例(17.0%)。免于主动脉瓣中重度反流40例(85.1%)。结论保留瓣叶的主动脉根部置换术治疗主动脉根部瘤的远期疗效满意,瓣膜相关并发症发生率低。     

Chronic dissection of the ascending aorta: surgical results during a 20-year period (previous surgery excluded)

Objective: We study here the surgical results of chronic dissection involving the ascending aorta over the last 20 years. Patients with previous cardiac surgery, or proximal aortic repair, were excluded. The patients survived an acute dissection, undiagnosed as pauci- or asymptomatic. The aorta was normal or pathological (atheromatous aneurysm in 15 cases, Marfan's disease in 12 cases, and annuloectasic disease in 18 cases). Two patients had a bicuspid aortic valve. Methods: Between January 1981 and December 2001, 77 patients (mean age 48 ± 15) underwent surgery for chronic dissection of the ascending aorta; 60 patients had severe aortic regurgitation, 12 had Marfan syndrome, and 18 had annuloaortic ectasia. Only the ascending aorta was dissected in 37 patients, the ascending aorta and arch in 26, and the whole aorta in 14. Coronary artery disease occurred in five patients. Statistical analysis was performed using SAS software. Different surgical procedures were used. The aortic arch was repaired in 40 cases; selective antegrade cerebral perfusion and partial circulatory arrest were used. Total aortic replacement was performed on four patients. Results: In-hospital mortality was 10%. The only risk factor was the extent of the dissection. The rate of neurologic stroke was 2.5%. Late survival rate was 42 ± 7.5% at 12 years for all the patients; it was 71 ± 10% when only the ascending aorta was dissected, 44 ± 11% when the ascending aorta and arch were dissected, and 33 ± 15% when the whole aorta was dissected (p = 0.0329). The extent of the dissection was the only risk factor for late mortality. Reoperation was required for one proximal and five distal problems. Conclusion: In chronic aortic dissection, in-hospital and late mortality were related to the extent of the dissection; in-hospital mortality remained unchanged during the operative period.     

主动脉根部瘤外科治疗危险因素和疗效评估

目的 评估主动脉根部瘤外科治疗危险因素和疗效.方法 1996年8月至2009年11月,连续92例主动脉根部瘤手术中男56例,女36例;年龄14～77岁,平均(44.8±1.4)岁.合并主动脉夹层动脉瘤45例,中度以上主动脉瓣关闭不全72例,马方综合征47例.行经典Bentall术59例,纽扣法Bentall术13例,主动脉瓣悬吊术14例,用带主动脉窦人工血管作David Ⅰ术6例.结果 住院死亡8.7%,并发症率31.7%.随访期死亡18例,远期并发症12例.单变量分析术后并发症危险因素为男性、非马方综合征、同期手术、深低温停循环、主动脉阻断≥120 min和术后第1天输血＞1500 ml;住院死亡危险因素为紧急手术、同期手术、主动脉阻断≥120 min、主动脉夹层和术后第1天输血＞1500 ml.Logistic多因素回归分析认为同期手术和术后第1天输血＞1500 ml是并发症和住院死亡危险因素.所有病人1年、5年和10年生存率分别为(97.1±2.0)%、(88.1±4.7)%和(54.0±9.2)%,平均生存(9.9±0.6)年,95%可信区间8.7～11.0.结论 各种主动脉根部瘤手术安全、有效,早、中、长期结果较满意,提倡用纽扣法Bentall术,谨慎选择合适病人作主动脉根部重建术.

Abstract：

Objective Evaluate the outcome of aortic root reconstruction on the analysis of the risk factors influencing surgical results. Methods Between August 1996 and November 2009, 92 patients(56 men, 36 women) aged from 14 to 77years [mean (44.8 ±1.4) years] with aortic root aneurysm underwent aortic root reconstruction. 72 patients had over moderate aortic valve insufficiency. 47 patients suffered from Marfan syndrome. The aortic pathology was aortic dissection in 45. Bentall technique was used in 59 patients, the button technique in 13, the David I with the Valsalva graft in 6 patients and the aortic valve resuspension in 14 patients. Results The hospital mortality rate was 8.7%. The major complications 31. 7%. 18patients died during the period of follow-up. Late complications among 55 survivors were 12. Univariate predictors of the morbidity were the presence of male, non-Marfan, concomitant procedure, deep hypothermia cardiac arrest, aortic cross clamp time and blood infusion. Risk facts for mortality were emergent or urgent operation, aortic dissection, concomitant procedure, aortic cross clamp time and blood infusion. Multivariate analysis revealed risk factors of concomitant procedure and blood infusion were responsible for both morbidity and mortality. The overall long-term survival rate is (97.1 ±2.0)% at 1-year, (88.1 ±4.7)% at 5-year, (54.0 ±9.2)% at 10-year. The mean for survival time is (9.9 ±0.59) years, 95% confidence interval 8.70 -11.01. Conclusion The aortic root restitution procedures are safe and effective in general. The short and long-term outcome is satisfactory. The button technique is the first choice for reimplantation coronary patch. Valve-sparring aortic root reconstructions show promise in safety and applicability.     

Therapeutic decisions for patients with symptomatic severe aortic stenosis: room for improvement?

Objective: Symptomatic severe aortic stenosis is an indication for aortic valve replacement. Some patients are denied intervention. This study provides insight into the proportion of conservatively treated patients and into the reasons why conservative treatment is chosen. Methods: Of a patient cohort presenting with severe aortic stenosis between 2004 and 2007, medical records were retrospectively analyzed. Only symptomatic patients (n = 179) were included. We studied their characteristics, treatment decisions, and survival. Results: Mean age was 71 years, 50% were male. During follow-up (mean 17 months, 99% complete) 76 (42%) patients were scheduled for surgical treatment (63 conventional valve replacement, 10 transcatheter, 1 heart transplantation, 2 waiting list) versus 101 (56%) who received medical treatment. Reasons for medical treatment were: perceived high operative risk (34%), symptoms regarded mild (19%), stenosis perceived non-severe (14%), and patient preference (9%). In 5% the decision was pending at the time of the analysis and in 20% the reason was other/unclear. Mean age of the surgical group was 68 years versus 73 years for medically treated patients (p = 0.004). Predicted mortality (EuroSCORE) was 7.8% versus 11.3% (p = 0.006). During follow-up 12 patients died in the surgical group (no 30-day operative mortality), versus 28 in the medical group. Two-year survival was 90% versus 69%. Conclusions: A large proportion (56%) of symptomatic patients does not undergo aortic valve replacement. Often operative risk is estimated (too) high or hemodynamic severity and symptomatic status are misclassified. Interdisciplinary team discussions between cardiologists and surgeons should be encouraged to optimize patient selection for surgery.     

Aortic valve leaflet sparing and salvage surgery: evolution of techniques for aortic root reconstruction

Over the past 20 years, a series of procedures have been designed to reconstruct the aortic root of patients with aortic insufficiency, in whom the pathology and hence the surgery spares the valve leaflets. Such techniques have various names. Usually ‘valve sparing’ is used in context with chronic aortic dissection or aortic root aneurysm as in patients with Marfan's syndrome. ‘Aortic valve salvage’ tends to be the term of choice for similar surgical reconstruction in the setting of aortic dissection. ‘Aortic valve repair’ is often chosen when direct surgical procedures are performed on the leaflets themselves. All of the techniques have evolved based upon an increased understanding of the functional anatomy of the aortic root complex. The different technical approaches, their applications and results need to be understood by the cardiology community. The failure modes for such techniques are specific and different from prosthetic valve failure modes, but are adequately followed with echocardiography. Over two-thirds of patients remain free of re-development of significant aortic insufficiency at 8–10 years following surgery. The overall patient survival is more dependent upon the underlying cardiovascular status of the patient than the surgical technique itself. Perioperative mortalities vary between 0 and 6% and are comparable to composite valve+graft techniques and isolated aortic valve replacement, in which the operative mortality approximates 3.3–4%. Long-term results are good to excellent and spare the patient anticoagulation and prosthetic valve disease.     

Aortic valve translocation for treatment of a deteriorated stentless valve

A 54-year-old woman was admitted to our hospital with recurrent chest pain for 1 month. She had a history of aortic root replacement with a stentless valve following aortic valve replacements done twice 12 years ago, and coronary artery bypass grafting 6 years ago. The stentless valve was implanted with the full-root technique. After admission, she was diagnosed with a saphenous vein graft aneurysm in the proximal anastomotic site and severe aortic regurgitation due to stentless valve deterioration. These lesions were successfully treated using aortic valve translocation. The advantage of this procedure is that it avoids dissection and removal of the stentless valve implanted using the full-root technique. Aortic valve translocation can be one of useful alternatives for stentless valve reoperation.     

Pulmonary trunk perforation during transapical minimal invasive aortic valve replacement

In this case report we illustrate our experience with a perforation of the pulmonary trunk during a transapical aortic valve implantation in a single case. The patient suffered from an aortic valve stenosis and was accepted for a minimally invasive procedure because of multiple comorbidities. After unproblematic transapical placement of the aortic valve, a venous bleeding from the anterolateral wound was observed. Median sternotomy showed a bleeding out of the pulmonary trunk that could be stopped with purse-string sutures. The further course of the patient was uneventful.     

Rare case of severe non-calcific aortic stenosis in an achondroplastic dwarf: surgical consideration

A 41-year-old patient with achondroplasia who had undergone surgery for congenital aortic stenosis >20 years ago presented with dyspnoea due to severe aortic stenosis. Computed tomography confirmed a small (16-mm) aortic annulus, thickened aortic valve leaflets without calcification and subaortic fibrous tissues. Intraoperatively, 3 non-calcific aortic leaflets were observed. Enlargement of the aortic root using a modified Manouguian technique for good exposure enabled the resection of subaortic tissues and replacement of the valve with a mechanical valve. The aortic root can be extremely small in patients with aortic stenosis and achondroplasia. The anatomy of the aortic root should be carefully assessed to enable appropriate surgical planning.