The congenital bicuspid aortic valve with post-inflammatory disease--a neglected pathological diagnosis of clinical relevance

It is unusual to find surgically excised congenital bicuspidaortic valves with post-inflammatory disease. We have analysedretrospectively all surgically excised aortic valves over a6-year period for this particular diseased state in relationto relevant clinical data. There were 181 congenital bicuspid aortic valves, and the characteristicgross pathology of the tricuspid aortic valve with post-inflammatorydisease served as reference. Twelve of the 181 congenital bicuspidaortic valves were identified as post-inflammatory (6–6%);one with clinically established rheumatic disease. The meanage at operation of these 12 patients was significantly lowercompared to ‘other’ aortic valves. The study indicates that post-inflammatory involvement of acongenital bicuspid aortic valve can be identified, which isof clinical relevance since these patients have a significantlyyounger age at operation.     

经导管主动脉瓣置换术治疗二叶式主动脉瓣狭窄的术中投照角度预测值分析

目的在二叶式主动脉瓣(BAV)行经导管主动脉瓣置换术(TAVR)中,分析术前多排螺旋CT(MDCT)预测的最佳导丝跨瓣角度、球囊预扩张角度和瓣膜释放角度规律,总结三种投照角度预测值的规律。方法回顾性分析2019年7月至2020年6月在复旦大学附属中山医院因严重症状性重度主动脉瓣狭窄(AS)而行TAVR的BAV患者31例。收集基线资料、术前评估和手术情况。使用MDCT预测TAVR最佳导丝跨瓣角度、球囊预扩张角度和瓣膜释放角度,按照横裂式BAV和纵裂式BAV分组,比较两组之间的差异和规律。结果最佳导丝跨瓣角度,横裂式BAV为右前斜(RAO)8°(18°,3°)、足位(CAU)25°(29°,17°),纵裂式BAV为左前斜(LAO)26°(21°,34°)、头位(CRA)13°(6°,22°),两者差异均有统计学意义(均P<0.001);最佳球囊预扩张角度(显示左冠状动脉开口),横裂式BAV为LAO 11°(9°,26°)、CRA 8°(1°,19°),纵裂式BAV为LAO 36°(30°,39°)、CRA 22°(14°,25°),两者差异均有统计学意义(均P<0.05);最佳球囊预扩张角度(显示右冠状动脉开口),横裂式BAV为LAO 48°(43°,60°)、CRA 26°(3°,29°),纵裂式BAV为LAO 48°(39°,70°)、CRA 25°(22°,33°),两者差异均无统计学意义(P=0.320、P=0.560);最佳瓣膜释放角度,横裂式BAV为RAO 12°(16°,4°)、CAU 25°(28°,19°),纵裂式BAV为LAO 21°(17°,26°)、CRA 3°(-2°,12°),两者差异均有统计学意义(均P<0.001)。结论术前MDCT可预测BAV行TAVR的最佳导丝跨瓣、球囊预扩张和瓣膜释放投照角度,这些角度与BAV为横裂式还是纵裂式相关,存在明显规律。     

实时三维超声心动图对主动脉瓣二叶畸形的再探讨

【】 目的：探讨实时三维超声心动图( RT-3DE)诊断主动脉瓣二叶畸形(bicuspid aortic valve malformation,BAV)的应用价值。方法：采用Philips iE Elite彩色多普勒超声诊断仪, S5-1和X3-1探头,检测36例BAV患者,经胸二维超声心动图( 2DE) 和RT-3DE通过胸骨旁左心室长轴切面及大动脉短轴切面, 观察主动脉瓣瓣膜数目、形态、启闭情况及血流动力学改变。结果：2DE确诊BAV28例,漏诊2例,不能明确6例,上述均经 RT-3DE复查并被临床证实,其中横裂式20例、纵裂式11例、斜裂式5例。钙化28例,占78%;瓣膜狭窄22例,占61%,轻度狭窄18例,中度狭窄4例,无重度狭窄;瓣膜关闭不全14例,占39%,轻度关闭不全11例,轻-中度关闭不全3例,无重度关闭不全。结论：RT-3DE对诊断BAV有重要价值,明显提高诊断符合率。     

Aortic valve repair in patients with aortic regurgitation: Experience with the first 100 cases

IntroductionThe aim of this study was to analyze short- and mid-term results of aortic valve repair.Material and methodsOne hundred consecutive patients (24 females; mean age 50.3 years, range 23–77 years) with aortic regurgitation underwent aortic valve repair between November 2007 and October 2012. Sixty patients had bicuspid aortic valve, and 82 patients demonstrated aortic regurgitation greater than mild (> grade 2). The ascending aorta/aortic root was replaced in 67 patients. Aortic cusp repair was necessary in 74 patients and additional aortic annulus stabilization was required in 48 cases. Follow-up ranged from 1 to 59 months (cumulative of 220 patient-years, median 25 months) and was complete in 100%.ResultsThere was no 30-day mortality and two patients died in the follow-up. The overall 4-year survival was 98% and freedom from cardiac death was 99% at 4 years. During the follow-up eight patients underwent aortic valve-related reoperation due to progression of aortic regurgitation and another six patients showed aortic regurgitation more than mild (> grade 2). In both aspects there was no statistically significant difference between patients without and with aortic root replacement (p=0.402 and p=0.650). There were no significant bleeding or thromboembolic events during the follow-up.ConclusionsShort- and mid-term data analysis revealed an excellent survival and acceptable results of aortic repair, comparable with other larger published studies. We think, therefore, aortic valve repair should be a part of contemporary cardio-surgical armamentarium, especially in younger patients with an appropriate indication.     

Aortic elasticity and size in bicuspid aortic valve syndrome.

AIMS: To investigate the relation between aortic elastic properties and size in bicuspid aortic valves (BAVs). METHODS AND RESULTS: 127 BAV outpatients (121 males; age 23 +/- 10 years) with no or mild valvular impairment, were recruited with 114 control subjects comparable for age, gender, and body size. Aortic distensibility (DIS) and stiffness index (SI) were derived by M-mode evaluation of the aortic root together with blood pressure measured by cuff sphygmomanometer. BAVs vs. controls had increased aortic diameter (P < 0.0001), higher systolic (P = 0.02) and pulse (P = 0.04) pressures. DIS was lower in BAVs than in controls (4.71 +/- 3.67 vs. 7.44 +/- 3.94 10(-6) cm(2)dyne(-1), respectively; P < 0.0001) and SI was greater in BAVs (7.21 +/- 4.93 vs. 3.57 +/- 1.88, respectively; P < 0.0001). Definite impairment in aortic elasticity was present in 53 (42%) BAVs. Both DIS and SI were related (P < 0.0001) to aortic size in BAVs and controls. After adjusting for aortic size and blood pressure, the regression relations between SI and aortic diameter of BAVs were significantly different from controls (P = 0.0052). CONCLUSION: Abnormal aortic elasticity is a common finding in BAVs with no or mild aortic valve impairment. However, impaired aortic stiffness is not due to aortic dilation. Simple assessment of aortic size may thus fail to identify early abnormal load bearing characteristics of the aortic wall in BAVs.     

Rates and determinants of progressive aortic valve dysfunction in aortic coarctation

Purpose

Aortic valve dysfunction is common in coarctation patients(CoA). Bicuspid aortic valve (BAV) in CoA is associated with aortic valve stenosis (AS), aortic valve regurgitation (AR), and ascending aortic dilatation. The aim of this study was to evaluate the progression of and predictors for aortic valve dysfunction in CoA.

Methods

96 CoA patients prospectively underwent echocardiography twice between 2001 and 2010. AS was defined as an aortic valve gradient ≥ 20 mm Hg, AR as none/minor, or moderate/severe. Aortic dilatation as an ascending aortic diameter ≥ 37 mm.

Results

All patients (median age 28.0 years, range 17–61 years; male 57%) were followed with a median follow-up of 7.0 years. Sixty patients (63%) had BAV. At baseline 10 patients had AS (10%, 9 BAV), 6 patients AR (6%, 3 BAV) and 11 patients aortic dilatation (11%, 11 BAV). At follow-up 15 patients had AS (15%, 13 BAV) and 12 patients AR. (13%, 8 BAV).Median AS progression was 1.1 mm Hg/5 years (range — 13–28). Determinants for AS at follow-up were age (ß = 0.20, P = 0.01), aortic dilatation (ß = 4.6, P = 0.03), and baseline aortic valve gradient (ß = 0.93, P < 0.001). BAV was predictive for AR. (ß = 0.91, P = 0.049).

Conclusion

Progression of AS in adult CoA patients is mild in this young population. Older age, aortic dilatation and the baseline aortic valve gradient are determinants for AS at follow-up. BAV is predictive for AR. These findings point towards a common embryological pathway of both valvular and aortic disease in CoA.     

二叶主动脉瓣的超声分析

目的 探讨二叶主动脉瓣的超声心动图特征。方法 采用回顾性方法分析了26例先天性二叶主动脉瓣,其中主动脉瓣狭窄17例（有或无主动脉瓣关闭不全）和单纯主动脉瓣关闭不全9例。结果 在主动脉瓣狭窄组、轻度狭窄14例（82％）;二叶主动脉瓣感染性心内膜炎的发生率为42％;主动脉瓣狭窄组与单纯主动脉瓣反流组相比,主动脉瓣增厚钙化有极显性差异（P＜0．00005）,结果表明,二叶主动脉瓣狭窄的发生与主动脉瓣的增厚钙化有关,且主动脉瓣狭窄多为轻度狭窄。结论：二叶主动脉瓣狭窄的发生与主动脉瓣的增厚钙化有关,且主动脉瓣狭窄多为轻度狭窄。二叶主动脉瓣感染性心内膜炎的发生率较高。彩色多普勒二维超声心动图对二叶主动脉瓣的诊断具有重要价值。     

Hereditary patterns of bicuspid aortic valve in a hundred families

Background

To study the following characteristics of bicuspid aortic valves (BAVs): 1) the recurrence rate in our population, 2) patterns of hereditary transmission in different BAV morphologies and 3) the aortic dimensions of BAVs in first-degree relatives (FDRs).

Methods

A cross-sectional, prospective study of 100 consecutive families of BAV patients attending a university hospital. The following aortic valve morphologies were analysed and categorised: fusion of the right and left coronary cusps (BAV type A), right and noncoronary cusps (type B) and of the left and noncoronary cusps (type C).

Results

There were 553 subjects studied, 100 cases with a BAV (46.8 ± 15 years, 66% male, type 67% A, 32% B and 1% C; 42% with aortic dilatation), 348 FDRs (44.8% male), and 105 healthy control subjects (50% male). We detected 16 BAVs among 348 FDRs. The recurrence rates were 15% for families, 4.6% for FDRs, 7.05% in men and 2.60% in women. The morphologic concordance in family members was 68.8%. The aortic dimensions in 270 adult FDRs with a tricuspid aortic valve were significantly smaller compared with BAV patients (sinus index diameter 1.60 ± 0.19 cm/m² vs. 1.82 ± 0.29 cm/m², p < 0.001; tubular index diameter 1.51 ± 0.23 cm/m² vs. 2.00 ± 0.45 cm/m², p < 0.001) and similar to 103 control subjects(sinus index diameter 1.60 ± 0.19 cm/m² vs. 1.59 ± 0.17 cm/m², p = 0.600 and tubular index diameter 1.51 ± 0.23 cm/m² vs. 1.53 ± 0.18 cm/m², p = 0.519).

Conclusions

In our population, the BAV recurrence rate in FDRs was low (4.6%). The hereditary transmission of morphologic BAV types seems by chance, and the aortic dimensions in tricuspid FDRs are normal.     

Transfemoral implantation of CoreValve Evolut R in patient with functionally bicuspid aortic valve

Catheter based aortic valve replacement became largely adopted technique to treat patients with severe aortic stenosis in the setting of prohibitive risk and in high risk operation patients. Based on the positive clinical data from the Nordic Aortic Valve Intervention (NOTION) Trial [1] and from a subset analysis from the CoreValve U.S. High Risk Pivotal Trial Medtronic plc. announced CE mark for the self-expanding CoreValve™ Evolut™ R to obtain an expanded indication to treat aortic stenosis in patients who are at intermediate risk for open-heart surgery. Bicuspid aortic valve was deemed contraindicated for CoreValve implantation and nowadays is considered to be “off label” use even if multiple publications showed successful CoreValve implantation in patients with stenotic bicuspid aortic valve [2], [3]. Current case report tackles preprocedural evaluation of stenotic aortic valve anatomy and together with the relevant pictures illustrates recapurability and repositionability of abovementioned TAVI system in patient with funcionally bicuspid severely stenotic aortic valve.     

二叶式与三叶式主动脉瓣狭窄患者的临床对照研究

目的 通过对二叶式与三叶式主动脉瓣狭窄患者进行临床特征及围术期指标的回顾性对比分析,以期指导二叶式主动脉瓣狭窄患者的术前管理、手术方案制定及术后治疗。方法 选取我院心外科自 2011 年 01 月至 2017 年 12 月期间收治的所有因主动脉瓣狭窄而单纯行主动脉瓣置换术的患者90例（术中处理主动脉、马凡综合征、主动脉夹层及临床资料不全等患者未纳入本研究）,根据瓣叶特点分为二叶式主动脉瓣组（BAV组,n=45）和三叶式主动脉瓣组（TAV组,n=45）。分别收集两组患者围术期临床资料,从术前、术中、术后三个方面对比两组差异,所有资料均应用SPSS20.0进行相关统计分析。结果 BAV组患者中男性29名、女性16名,约2:1。在术前指标对比方面,相比较TAV组,BAV组发病年龄明显较小（47.76±10.80 vs.54.58±9.29 岁,P=0.002）,合并升主动脉扩张的比例高（66.7％ vs.37.8％,P=0.006）,且升主动脉直径更大（39.55±6.77 vs.35.55±4.78 mm,P=0.002）;BAV组合并三尖瓣反流的比例明显高于TAV组（62.2％ vs.40.0％,P=0.035）;对比两组左室内径、主动脉瓣口流速及跨瓣压差,差异无统计学意义（P ＞0.05）。在术中指标对比方面,BAV组主动脉阻断时间明显长于TAV组(72.07±22.05 vs.55.80±16.77 min,P=0.000)。在术后指标对比方面,两组患者在ICU时间、ICU呼吸机时间、住院时间及复查超声上差异无统计学意义（P ＞0.05）。结论 二叶式主动瓣狭窄患者容易较早发病,并且具有男性优势,所以应提高心脏疾病筛查能力,以便于早诊断、早治疗。同时,二叶式主动脉瓣狭窄患者易合并升主动脉扩张和三尖瓣反流,且升主动脉直径和主动脉阻断时间明显大于三叶式主动脉瓣狭窄患者,因此依据指南并结合手术经验术中积极处理扩张主动脉、修复三尖瓣是非常必要的,此外还应加强围手术期心肌保护、缩短阻断时间来减少术中损害。     

先天性二叶式主动脉瓣畸形所致主动脉瓣狭窄的外科治疗体会

目的总结先天性主动脉瓣二叶式畸形所致的主动脉瓣狭窄手术治疗的经验。方法1995年9月至2010年12月福建医科大学附属协和医院心外科共为46例二叶式主动脉瓣畸形所致主动脉瓣狭窄患者实施了瓣膜置换术。对这些患者的手术效果及其影响因素进行回顾性总结。结果术后随访3个月至12年,死亡1例（为猝死）,1例出现与抗凝有关的脑部并发症;心功能Ⅰ级36例,心功能Ⅱ级9例。术后超声心动图测得主动脉瓣跨瓣压差17～51（29．2±11．5）mmHg。结论主动脉瓣置换术是治疗先天性主动脉瓣二叶式畸形所致主动脉瓣的有效疗法,应尽可能选择有效瓣口面积较大的人造瓣膜,这样可以有效降低术后主动脉跨瓣压差,提高手术安全性和远期疗效。     

Acute traumatic disruption of a bicuspid aortic valve.

Cardiac involvement is frequent in patients with blunt chest trauma and multiple injuries. Different cardiac structures can be involved, but isolated aortic valve rupture is rare. We report the case of a man admitted to our hospital with multiple injuries as a result of a car crash. Transthoracic, followed by transesophageal echocardiography, revealed disruption of a large anterior bi-coronary cusp in a bicuspid valve with severe aortic regurgitation, without lesions or abnormalities of thoracic aorta. Aggressive pharmacological management consented to delay valve replacement to obtain resolution of concomitant pulmonary injuries.     

超声心动图对主动脉瓣二叶畸形的探讨

目的 探讨超声心动图评估主动脉瓣二叶畸形(BAV)的应用价值.方法 选取我院2012年3月至2014年6月经超声诊断并被临床证实的BAV患者70例(横裂组44例、纵裂组21例、斜裂组5例),并与正常组30例进行比较.测最主动脉窦部及升部内径、室间隔及左室后壁厚度、左心功能及左房横径,观察患者瓣膜有无钙化、狭窄、关闭不全及脱垂.结果 BAV患者部分伴有瓣膜回声增强、增厚、钙化和脱垂,可造成瓣膜狭窄或关闭不全.主动脉升部增宽,纵裂组(39.840±6.361)mm,横裂组(37.480±5.793)mm,高于正常组的(30.270±2.348)mm;室间隔增厚,纵裂组(11.180±1.968)mm,横裂组(11.430±1.912)mm,高于正常组的(9.900±0.403)mm;左房横径增大,纵裂组(37.090±8.203)mm,横裂组(37.950±9.058)mm,高于正常组的(30.330±2.820)mm.结论 超声心动图对诊断BAV有重要价值.BAV可合并主动脉升部增宽、室间隔增厚及左房横径增大,而瓣膜舒张期关闭线形态分类横裂式、纵裂式对心脏结构和功能影响不大.