先天性主动脉瓣上狭窄的治疗体会

作者对10例先天性主动脉瓣上狭窄治疗进行分析，以探讨主动脉瓣上狭窄的病理生理，病理解剖特点，为其进一步治疗提供依据。     

局限性主动脉瓣上狭窄的外科治疗

目的探讨局限性主动瓣上狭窄的外科治疗要点。方法1990年4月～2000年6月收治局限性主动脉瓣上狭窄的手术治疗患者7例,均具有典型的小精灵面容。超声心电图及左室造影发现起自主动脉嵴长约12～20mm膜样狭窄环,主动脉瓣环无缩窄,其中1例并发重度主动脉瓣关闭不全,2例主动脉弓降部轻度狭窄。1例右上肺动脉起始部狭窄,有3例冠状动脉扩张增粗。中度低温体外循环下进行手术,斜形切开升主动脉前壁,斜向无冠状窦,剪除狭窄环,取泪滴状补片以5-0prolene线或6-0prolene线连续缝合加宽主动脉壁,加宽材料1例为自体心包,3例为同种主动脉片,3例以Groe-Tex血管片,并发主动脉瓣重度关闭不全者不同期以ST.Jude23#双叶机械瓣置换,1例右肺动脉起始部狭窄以自体心包片加宽。结果无低心血输出量综合征及切口出血,术后跨环压差0～3mmHg,随访3～5年无假性动脉瘤形成。结论局限性主动脉瓣上狭窄的手术治疗,术前应详细检查了解并发畸形情况,手术重点是彻底解除狭窄,加宽主动脉,重视并发畸形的处理。     

先天性主动脉瓣上狭窄的外科治疗

先天性主动脉瓣上狭窄是一种较为少见的左心室流出道梗阻疾病,为染色体部分缺失导致动脉弹性蛋白异常从而表现升主动脉局限型或者弥漫型的狭窄,引起血流动力障碍,往往合并伴有其他的解剖畸形,右心室流出道狭窄、主动脉瓣二瓣化、主动脉缩窄、冠状动脉狭窄等。该种疾病一类特殊类型为Williams-Beuren综合征。本文从病因、分类、临床表现、外科治疗方法以及术后结果对该类疾病进行综述,对多种术式的早期以及晚期随访结果进行比较,探讨手术危险因素。针对患者疾病的具体情况选择合适的手术方式才能获得满意的疗效。     

先天性主动脉瓣上狭窄的外科治疗

目的总结6例先天性主动脉辫上狭窄（SVAS）术前诊断和外科治疗中的经验、教训和疗效。方法2003年1月至2008年6月收治SVAS患者6例，男性3例，女性3例，年龄1．9-19．0（5．73±6．36）岁，局限性狭窄5例，弥漫性狭窄1例。Williams综合征3例，3例并发其他心脏畸形，其中1例同时合并动脉导管未闭、主动脉瓣下狭窄、主动脉瓣两叶畸形、二尖瓣狭窄关闭不全和细菌性心内膜炎，1例合并动脉导管未闭，还有1例合并肺动脉狭窄。术前超声心动图检查均有初步诊断，有2例患者手术前做了左心导管和造影，3例行64排螺旋CT扫描，对诊断和手术方案的确定很有意义。手术前检查发现跨狭窄环的压差为45-121mmHg。手术方法：单片法加宽3例，倒“Y”法3例，同期行主动脉瓣置换、二尖瓣置换、左室流出道疏通和动脉导管结扎1例，行肺动脉加宽1例，行动脉导管缝闭1例。结果本组手术无死亡，除合并细菌性心内膜炎及多种畸形的患者术后出现低心排和呼吸功能不全和感染，其余患者恢复顺利，所有患者痊愈出院。手术后有1例虽然主动脉瓣上狭窄解除理想，但是发现主动脉弓有弥漫性轻度狭窄。结论SVAS经术前超声心动图检查基本可以诊断，对Williams综合征患者术前应进行更为详细的检查，如果有条件应该考虑行无创伤的64排螺旋CT检查，可以发现合并的畸形如主动脉弓的病变，肺动脉狭窄等；一旦发现主动脉辫上狭窄应该积极手术治疗，否则可能引起心律失常和猝死。SVAS的手术治疗效果良好，目前不主张应用单片法进行矫治。     

主动脉瓣狭窄与关闭不全

问 :主动脉瓣狭窄的典型“临床三征” ?答 :呼吸困难、昏厥、心绞痛。问 :主动脉瓣狭窄 (主窄 )时 ,有什么体征 ?答 :典型的主窄杂音是菱形 (升高→下降型 )收缩期杂音 ,在胸骨右侧第二肋间 ,放射至颈部 ,粗糙。典型的主窄杂音有Ⅲ Ⅳ级 ,严重病例并左心衰时 ,杂音有所减弱。颈动脉搏动上升缓慢 ,持续 ,波辐缩小。老年病人 ,动脉弹性差 ,搏动可近乎正常。　心尖搏动 :左室肥厚的表现 ,心尖搏动抬举感 ,可触及心房收缩的杂音S4 ,如先天性二瓣型主动脉瓣狭窄时 ,可有排血期喀喇音。　严重病例 ,心脏收缩功能障碍 ,有心衰时可见 :心尖搏动向…     

主动脉瓣上狭窄的外科治疗(附21例报告)

目的 总结21例主动脉瓣上狭窄(SVAS)的外科治疗经验.方法 21例主动脉瓣上狭窄的患者接受了外科治疗.局限型15例、弥漫型6例、Willianms综合征3例;合并其他心脏畸形包括主动脉瓣狭窄6例、主动脉瓣关闭不全2例、冠状动脉畸形4例、动脉导管未闭3例、肺动脉瓣上隔膜1例、头臂动脉畸形5例、二尖瓣脱垂1例.均在全麻低温体外循环下应用补片直接行主动脉成形术.其中,同时行主动脉瓣成形4例,应用同种升主动脉左心尖与胸主动脉搭桥1例,升主动脉与腹主动脉架桥和主动脉瓣膜置换术1例,二尖瓣成形1例.结果 手术早期死亡2例,二次开胸2例.随访1～97(平均42.7)个月,所有患者狭窄解除满意,晚期死亡1例,存活患者的心功能为Ⅰ～Ⅱ级.结论 SVAS的外科治疗可以取得很好的治疗效果,补片扩大成形主动脉仍然是治疗局限型SVAS的重要方法 .     

不同手术方法治疗先天性主动脉瓣上狭窄的临床研究

目的：探讨先天性主动脉瓣上狭窄外科纠治的手术适应证选择、不同手术方法的近、中期疗效。方法：回顾性分析西京医院2005年2月至2020年2月先天性主动脉瓣上狭窄外科治疗病例27例,年龄1.4～13.5y（4.1±3.2）,合并染色体异常（Williams综合征）19例（70.4%）。术前主动脉瓣上峰值压差45～98(72.3±13.4)mmHg,其中。其中局限型24例(88.9%),弥漫型3例(11.1%)。主动脉瓣不同程度增厚24例,瓣窦发育不均衡9例,合并少量或微量主动脉瓣反流5例,无主动脉瓣狭窄。合并左、右肺动脉分支狭窄9例。手术均在中低温体外循环下实施,采用单片（McGoon）法7例(25.9%),双片(Doty)法11例(40.7%),三片法9例(33.3%),其中Brom法4例,Myers法5例。肺动脉狭窄同期补片加宽纠治。结果：全组无住院死亡。术后主动脉瓣上峰值压差14～54(21.6±8.4)mmHg,其中单片组20～54(30.6±11.9)mmHg,双片组14～23(19.3±2.9)mmHg,三片组14～22(17.6±2.5)mmHg,单片组与其他两组差异明显（p?0.01）,双片组与三片组无明显差异（p?0.05）。主动脉瓣少量反流2例,微量6例。随访21例,失访6例,随访3月～11年（平均3.7年）,主动脉瓣上峰值压差17～46(24.1±7.4)mmHg,其中单片组26～46(33.4±10.5)mmHg,双片组19～31(22.3±2.7)mmHg,三片组17～26(19.1±2.1)mmHg,单片组与其他两组差异明显（p?0.01）,双片组与三片组无明显差异（p?0.05）。无远期死亡和再次手术干预。主动脉瓣少量反流4例,微量7例。结论：手术纠治先天性主动脉瓣上狭窄是安全的,近、远期疗效满意。双片和三片法手术疗效无明显差异,但均优于单片法,应作为首选手术方法。     

经皮导管双球囊成形术治疗主动脉瓣上狭窄

目的:报告1例主动脉瓣上狭窄经皮导管双球囊成形术治疗成功,获得良好近期疗效,并做简要讨论.方法:女性患者7.5岁,具部分Williams综合征表现.超声显示于主动脉瓣上约1.0 cm处可见一隔膜样结构,中央开口约0.7 cm,主动脉瓣环内径为1.6 cm,主动脉瓣开口约为1.2 cm.造影诊断为主动脉瓣上狭窄.心导管检查测得狭窄的压力阶差为70 mmHg(1 mmHg=0.133 kPa).经双侧股动脉穿刺分别送入12 mm×20 mm及10 mm×40 mm的球囊进行扩张.结果:术后造影显示主动脉瓣上狭窄基本消失,其压力阶差降至12 mmHg.超声心动图复查示主动脉瓣上隔膜由原固定不动变得随主动脉舒缩而摆动并于舒张期明显张开.结论:作为一种少创有效的介入技术经皮导管双球囊成形术治疗主动脉瓣上狭窄1例获得良好疗效.双球囊径稍大于主动脉瓣环径(≤1.3)亦安全可行,远期疗效需进一步随访观察.     

经导管主动脉瓣置换术治疗二叶式主动脉瓣狭窄与三叶式主动脉瓣狭窄疗效比较的meta分析

目的:系统评价经导管主动脉瓣置换术治疗二叶式主动脉瓣狭窄与三叶式主动脉瓣狭窄的疗效。方法:检索建库至2020年2月PubMed、Embase、Cochrane图书馆、万方数据库和中国知网的随机对照及队列研究试验,同时采用Revman 5.3、Stata 15.1软件对纳入试验进行荟萃分析。结果:纳入17项研究,共分析132333例患者。荟萃分析结果显示:BAV组与TAV组在30 d死亡率(OR=1.13,95%CI:0.89～1.44,P=0.32)、1年死亡率(OR=0.91,95%CI:0.77～1.06,P=0.22)、脑卒中(OR=1.26,95%CI:0.94～1.68,P=0.12)、瓣周漏(OR=1.17,95%CI:0.95～1.44,P=0.15)、大出血事件(OR=0.84,95%CI:0.66～1.06,P=0.13)、血管并发症(OR=0.77,95%CI:0.59～1.00,P=0.051)、冠脉阻塞(OR=1.83,95%CI:0.93～3.60,P=0.08)、术后心梗(OR=1.14,95%CI:0.63～2.06,P=0.66)、急性肾损伤(OR=1....     

主动脉瓣环扩大联合环上型生物瓣膜替换治疗老年小瓣环主动脉瓣狭窄的近中期结果

目的:总结主动脉瓣环扩大联合环上型生物瓣膜替换策略,治疗老年小瓣环主动脉瓣狭窄的近中期结果,探讨老年小瓣环主动脉瓣狭窄的治疗策略。方法:2010年1月至2017年1月,28例老年小瓣环主动脉瓣狭窄患者应用主动脉瓣环扩大联合环上型生物瓣替换策略进行治疗,主动脉瓣关闭不全或多个瓣膜病变等排除在本研究之外。男性8例,女性20例,年龄65～77岁,平均(70. 5±3. 48)岁,体表面积1. 55～1. 72 m~2,平均(1. 63±0. 05) m~2。按病因分类,退行性主动脉瓣狭窄17例,先天性二叶畸形6例,风湿性改变4例,其他病变1例。患者术前心功能分级(NYHA分级):Ⅱ级6例,Ⅲ级20例,Ⅳ级2例。同时合并疾病包括冠心病5例,2型糖尿病8例,高血压病7例,慢性肾功能不全5例,慢性阻塞性肺疾病3例,心房颤动7例,既往卒中史6例。手术前超声心动图测主动脉瓣环径(17. 5±1. 02) mm,平均跨瓣压差(59. 8±10. 2) mm Hg (1 mm Hg=0. 133 k Pa)。结果:体外循环时间(105. 0±18. 8) min,心肌血运阻断时间(71. 0±17. 1) min。实测主动脉瓣环径(18. 1±0. 87) mm,应用Nick法扩环25例,Nuez法3例,扩环后主动脉瓣环径(22. 2±0. 93) mm,平均增加(4. 13±0. 78) mm,平均替换瓣膜尺寸(21. 8±0. 99) mm,预期有效瓣口面积指数(1. 10±0. 07) cm~2/m~2。同期行冠状动脉旁路移植术5例,心房颤动射频消融术7例,左心耳切除术7例。围手术期死亡1例(3. 6%),死因为低心排血量综合征。开胸止血术1例,急性肾衰竭1例,肺部感染2例,切口并发症1例,临时起搏治疗6例。出院时平均跨瓣压差(13. 14±2. 14) mm Hg,超声实测有效瓣口面积指数(1. 12±0. 07) cm~2/m~2,仅1例发生轻度患者-人造瓣膜不匹配。手术后2年,常规超声心动图检查及临床评估。24例患者心功能Ⅰ级,3例Ⅱ级。患者平均跨瓣压差及左心室质量指数较术前明显改善[(59. 8±10. 18) vs.(13. 8±1. 93) mm Hg,P 0. 01;(151. 3±9. 95) vs.(110. 6±6. 95) g/m~2,P0. 01]。5例患者主动脉瓣听诊区可闻及2/6级收缩期杂音,19例患者左心室肥厚得到逆转,8例患者仍存在轻度左心室肥厚。随访时平均有效瓣口面积指数(1. 09±0. 09) cm~2/m~2,1例发生轻度患者-人造瓣膜不匹配。结论:主动脉瓣环扩大联合环上型生物瓣替换策略治疗老年小瓣环主动脉瓣狭窄近中期疗效良好,既有效避免患者-人造瓣膜不匹配现象,又避免了长期抗凝所致的血栓栓塞及出血风险。     

Diagnosis of Supravalvular Aortic Stenosis by Transesophageal Echocardiography

The role of transesophageal echocardiography (TEE) in diagnosis of disorders of the thoracic aorta is well established. In this report the TEE findings in an adult patient with supravalvular aortic stenosis are presented. This showed narrowing of the ascending aorta just above the sinuses, due to fibromuscular thickening, causing an hour-glass shaped deformity. The excellent image quality obtained by TEE is far superior to transthoracic echocardiography. Coronary artery ostial obstruction a known association of supravalvular aortic stenosis can be caused by different mechanisms including adherence of the aortic valve leaflet to the ridge of obstructive muscle or premature atherosclerosis. TEE can define the mechanism of coronary artery ostial obstruction associated with supravalvular aortic stenosis.     

Aortic Stenosis

Noninvasive assessment of aortic valve area by echocardiography has become the standard of practice over the past few years. The advent of transesophageal echocardiography (TEE) has provided a new method for the assessment of aortic valve area (AVA) using planimetry by two-dimensional imaging. Clear visualization of the anatomy of the valve, as well as accuracy of AVA assessment, makes TEE an invaluable tool for the evaluation of aortic valve stenosis. TEE is especially helpful in clinical settings when there is a discrepancy between the AVA obtained by transthoracic echocardiography and cardiac catheterization. TEE is particularly helpful in the assessment of the aortic valve during intraoperative echocardiography. This review discusses the techniques, imaging planes, and details for assessing AVA by TEE. The role of TEE in AVA assessment is described, with specific clinical case examples cited.     

Subvalvular Aortic Membrane Masquerading as Valvular Aortic Stenosis

Discrete subvalvular aortic membrane is an unusual cause of left ventricular outflow tract obstruction. While commonly presenting during childhood, one quarter of the individuals are asymptomatic in childhood but remain vulnerable to progressive outflow tract obstruction as well as bacterial endocarditis in adulthood. Unfortunately, because the condition is so uncommon in adults, the diagnosis of subvalvular membrane is often missed or delayed. We recently had the opportunity to care for an adult patient who finally had subvalvular membrane identified as the cause of a left ventricular outflow obstruction, only after a protracted series of diagnostic investigations. Pitfalls in the noninvasive and invasive diagnosis of this condition are reviewed.     

Effect of Bisphosphonates on the Progression of Degenerative Aortic Stenosis

Bisphosphonates appear to regulate mineralization in both bone and vasculature. Degenerative aortic stenosis (AS) is thought to be due to vascular calcification. We studied the effect of bisphosphonates on the progression of degenerative AS. A retrospective study was performed on patients >70 years, who had transthoracic echocardiograms (TTE) >1 year apart and an initial aortic valve area (AVA) of 0.6–2.0 cm². Patients were excluded if they had an ejection fraction <40%, other significant valvular or congenital heart disease, end‐stage renal disease or heart transplant. The cohort was divided depending on the use of bisphosphonates. Data were obtained by review of the TTE reports. AVA, peak and mean aortic valve gradient (AVG), and the change between the studies were calculated. Of 4,270 patients screened for AS, 76 patients fit study criteria with 8 in the bisphosphonate group and 68 in the nonbisphosphonate group. The period between the TTEs was 23 ± 5 months in both the groups. AVA in the nonbisphosphonate group worsened by 0.2 cm² and in the bisphosphonate group it improved by 0.1 cm² (P = 0.001 vs. nonbisphosphonate). The changes in peak and mean AVG between groups and compared to baseline were not significant. Bisphosphonates show promise for slowing the progression of degenerative AS. (Echocardiography 2011;28:1‐7)     

Aortic Stenosis: Pathophysiology,Diagnosis, and Therapy

The incidence of aortic stenosis increases with age, affecting up to 10% of the population by the eighth decade. Once symptoms develop, aortic stenosis is rapidly fatal. Proper management requires an understanding of the physiology and criteria used to define disease severity. There is no effective pharmacologic treatment. Surgical aortic valve replacement has been the gold standard treatment for decades. However, over the last 10 years transcatheter aortic valve replacement has emerged as an attractive, less-invasive option for appropriately selected patients. Refinements in valve design and delivery systems have led to widespread use of this breakthrough technology in selected patients. We review the pathophysiology, criteria for valve replacement, and the results of the trials comparing transcatheter aortic valve replacement with surgical aortic valve replacement.     

Assessment of Subendocardial Contractile Function in Aortic Stenosis: A Study Using Speckle Tracking Echocardiography

Background: Angina and an electrocardiographic strain pattern are potential manifestations of subendocardial ischemia in aortic stenosis (AS). Left ventricular (LV) twist is known to increase proportionally to the severity of AS, which may be a result of loss of the inhibiting effect of the subendocardial fibers due to subendocardial dysfunction. It has also been shown that the ratio of LV twist to circumferential shortening of the endocardium (twist‐to‐shortening ratio [TSR]) is a reliable parameter of subendocardial dysfunction. The aim of this study was to investigate whether these markers are increased in AS patients with angina and/or electrocardiographic strain. Methods: The study comprised 60 AS patients with an aortic valve area <2.0 cm² and LV ejection fraction >50%, and 30 healthy—for age and gender matched—control subjects. LV rotation parameters were determined by speckle tracking echocardiography. Results: Comparison of patients without angina and strain (n = 22), with either angina or strain (n = 28), and with both angina and strain (n = 8), showed highest peak systolic LV apical rotation, peak systolic LV twist, and TSR, in patients with more signs of subendocardial ischemia. In a multivariate linear regression model, only severity of AS and the presence of angina and/or strain could be identified as independent predictors of peak systolic LV twist and TSR. Conclusions: Peak systolic LV twist and TSR are increased in AS patients and related to the severity of AS and symptoms (angina) or electrocardiographic signs (strain) compatible with subendocardial ischemia.     

A Case of Aortic Stenosis and Hypertrophic Cardiomyopathy

A patient with severe aortic valvular stenosis and coexisting obstructive hypertrophic cardiomyopathy, in whom diagnostic difficulties and management dilemmas arose, is presented. The complex issues involved in such a combination are discussed.