Three-dimensional echocardiographic evaluation of an incidental quadricuspid aortic valve

Background: Quadricuspid aortic valve is one of the rare forms of congenitalcardiac valvular disease. Its diagnosis is often missed, evenwith the transthoracic echocardiogram. Many of these patientsprogress to aortic incompetence later in life requiring surgicalintervention. In addition, quadricuspid aortic valve can beassociated with other congenital cardiac deformities. Henceearly recognition and follow-up is critical in these patients. Case presentation: We report a patient with quadricuspid aortic valve identifiedon intraoperative transesophageal 3-D echocardiography. This66-year-old male presented with the features of congestive heartfailure. The preoperative transthoracic echocardiogram (TTE)disclosed, moderately severe aortic valve insufficiency alongwith severe mitral and tricuspid regurgitation, but failed toreveal the quadricuspid anomaly of the aortic valve. Interestingly,this patient had undergone transthoracic echocardiography ontwo previous occasions during the past seven years for the evaluationof his valvular heart disease, which all failed to documentthis anomaly. Intraoperatively, transesophageal echocardiography(TEE) displayed an aortic valve composed of three medium andone small cusps. Conclusion: Our patient's case demonstrates the usefulness of transesophagealechocardiography in detection of this uncommon congenital malformation.     

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。     

62例室间隔缺损合并主动脉瓣脱垂的外科治疗

目的 总结室间隔缺损（VSD）合并主动脉瓣脱垂的外科治疗经验.方法 2001年1月至2010年12月间,我院收治62例VSD合并主动脉瓣脱垂患者,行单纯VSD心内修复术31例、主动脉瓣置换术（AVR）29例、主动脉瓣成形术（AVP）11例.结果 术后早期死亡2例,1例VSD修补和AVR患者术后3 d因室性心律失常死亡;1例15岁患者,7年前在外院行VSD修复和AVP,复查超声心动图示主动脉瓣重度关闭不全、巨大左心室,再次行AVR,术后1周因心衰死亡.余60例均存活.术后门诊随访,随访时间6个月至10年,共55例,随访率91.7 %.心功能Ⅰ级50例、Ⅱ级5例,均能从事正常生活及学习.1例单纯VSD修复患者出现VSD残余漏,术后1年复查超声心动图示主动脉瓣中度关闭不全,行AVR,目前已治愈.11例AVP患者中,轻度关闭不全8例（2例为肺动脉瓣下VSD,6例为围膜部VSD）;轻-中度关闭不全2例（均为围膜部VSD）;中度关闭不全1例（围膜部VSD）,于术后5年行AVR后治愈.行全迷宫双极射频消融术治疗心房颤动1例,患者心律转为窦性心律.结论 根据主动脉瓣脱垂的程度,合理处理VSD及主动脉瓣病变,可取得满意的疗效.     

Echocardiographic diagnosis of quadricuspid aortic valve

Isolated quadricuspid aortic valve is very rare. The identificationof this malformation can be made by cross-sectional echocardiography.Two cases of quadricuspid aortic valve diagnosed by cross-sectionalechocardiography are described.     

Left ventricular systolic and diastolic function in subjects with a bicuspid aortic valve without significant valvular dysfunction

BACKGROUND:

The bicuspid aortic valve (BAV) represents the most common cardiac congenital malformation in adults. It is frequently associated with dilation, aneurysm and dissection of the ascending aorta.

OBJECTIVE:

To evaluate left ventricular systolic and diastolic function in subjects with BAVs.

METHODS:

Thirty-five subjects with BAV (mean [± SD] age 25.9±5.7 years [range 17 to 36 years]; 18 male, 17 female) with either no valvular impairment or mild valvular impairment were recruited along with 30 control subjects (24.5±4.4 years of age [range 15 to 35 years]; 15 male, 15 female) who were matched for age, sex and body surface area. Left ventricular systolic and diastolic function were evaluated using conventional and tissue Doppler echocardiography. Left ventricular systolic and diastolic parameters were compared between the two groups.

RESULTS:

In subjects with BAVs, the ratio of mitral early diastolic velocity to late diastolic velocity was lower (0.95±0.4 versus 1.27±0.9; P=0.001), the ratio of mitral early diastolic velocity to myocardial early diastolic velocity was higher (10.1±3.2 versus 6.5±2.4; P=0.001) and the myocardial early diastolic velocity was lower (8.4±2.1 versus 15.3±3.6; P<0.001) compared with control subjects. In addition, the myocardial performance index was higher in subjects with BAVs than in control subjects (P=0.03). The left ventricular ejection fraction was also lower (53±11% versus 64±13%; P<0.001). No other statistically significant differences were observed between the two groups with regard to left ventricular systolic and diastolic parameters. In addition, the number of mitral valve prolapses and atrial septal aneurysms was higher in subjects with BAVs.

CONCLUSION:

BAVs may be associated with left ventricular systolic and diastolic dysfunction.     

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.     

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。     

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.     

Quadricuspid aortic valve: a rare etiology of aortic regurgitation.

Quadricuspid aortic valve is a rare cause of aortic insufficiency. We report two unusual cases of this valvular pathology associated with a dilatation of the aortic root. The mechanism leading to this valve incompetence is incompletely understood and is discussed in regard to these cases.     

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。     

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.     

Quadricuspid pulmonary valve associated with atrial septal defects and pulmonary stenosis

A 1-month-old Hispanic female was referred to the cardiology service. During physical examination, a systolic ejection murmur at the pulmonic area was auscultated. The echocardiography evaluation of the pulmonary valve demonstrated a quadricuspid pulmonary valve (QPV) with slightly thickened leaflets, associated atrial septal defects and mild pulmonary stenosis. QPV is a rare congenital anomaly variant that can remain asymptomatic with few or non-hemodynamic alterations. Associations with structural or functional alterations have been reported. This is the first case of QPV that was diagnosed in a Hispanic newborn.     

主动脉瓣四叶瓣畸形合并主动脉瓣病变的外科处理

目的 探讨主动脉瓣四叶瓣畸形对主动脉瓣功能的影响及其外科治疗.方法 2000年1月至2013年1月,我院通过经胸超声心动图和术中病理诊断主动脉瓣四叶瓣畸形13例.其中4例主动脉瓣的功能基本正常,9例合并主动脉瓣病变：重度关闭不全7例,重度关闭不全伴狭窄2例,合并二尖瓣中度关闭不全2例,三尖瓣中重度关闭不全1例.结果 13例患者中4例主动脉瓣功能正常者,继续随访中.9例合并主动脉瓣病变者,均行主动脉瓣置换术,同期行二尖瓣成形术2例、三尖瓣成形术1例.围术期无严重并发症及早期死亡,均康复出院.术后平均随访（6.34±5.17）年,心彩超提示主动脉瓣功能良好,无远期死亡.结论 主动脉瓣四叶瓣畸形是一种少见的先天性畸形,超声心动图有助于早期诊断.主动脉瓣功能正常时,可以随访;当合并主动脉瓣功能障碍时,应及时行主动脉瓣置换或修复,手术后可获得良好的远期效果.     

Isolated quadricuspid aortic valve referred with diagnosis of rheumatic carditis

Quadricuspid aortic valve is a rare congenital malformation of the aortic valve. It appears often isolated or sometimes associated with other truncal anomalies, and approximately 50% of patients have aortic regurgitation. In this article we reported an eight-years-old boy referred with diagnose of rheumatic carditis from the outer medical center.     

A quadricuspid aortic valve with atrial septal defect

A quadricuspid aortic valve (QAV) is a rare congenital heart defect, previously described as an incidental finding at the time of surgery or postmortem, which is now being increasingly detected by 2D transthoracic or transesophageal echocardiogram. With advances in echocardiography, secondary cardiac anomalies are also being described in association with QAV. Herein we describe a patient with QAV with a secundum atrial septal defect.     

Unicuspid aortic valve mimicking quadricuspid valve

Unicuspid aortic valve is a rare congenital cardiac abnormality, leading to aortic stenosis or regurgitation. We report the case of a 55-year-old man with severe aortic regurgitation caused by a unicuspid valve mimicking quadricuspid valve.     

A case of 75-year-old survivor of unrepaired tetralogy of Fallot and quadricuspid aortic valve

In tetralogy of Fallot (TOF), the most common form of cyanoticcongenital heart disease, only a few patients reach adulthoodwithout surgical correction. We present the case of a man withTOF who survived until the age of 75 years without surgicalintervention and had a very unusual combination of TOF and quadricuspidaortic valve (QAV). QAV, complicated by aortic regurgitation,is an uncommon finding in TOF patients. The hemodynamic consequencesfor both the right and left ventricles are significant. Thiscase provides a rare insight into the late outcome of an elderlypatient with uncorrected TOF.     

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。