Assessment of the severity of aortic stenosis: comparison of gradient and valve area

Hemodynamic data, including simultaneously measured left ventricular pressure, aortic pressure, and cardiac output, were obtained in 17 patients with isolated valvar aortic stenosis, before and during infusion of dobutamine, isoprenaline, dopamine, and pacing. Infusion of sympathomimetic drugs resulted in a significant increase of both mean and peak systolic aortic valve gradient, cardiac output, heart rate, and systolic aortic valve flow per second. On the other hand, the calculated aortic valve area did not change significantly in spite of variable alterations of all values used for calculating aortic valve area and different causes of hemodynamic changes induced. Aortic valve area is considered a better indicator of the severity of aortic stenosis than is the aortic valve gradient and thus a more suitable indicator for operation.     

自体心包修复儿童主动脉瓣狭窄9例病例系列报告

目的：总结并分析以自体心包修复儿童先天性主动脉瓣狭窄的手术方法和早期预后。方法：纳入2013年7月至2015年6月在复旦大学附属儿科医院行自体心包主动脉瓣成形术的先天性主动脉瓣狭窄患儿,收集患儿的一般资料,围手术期情况,并发症,术前、术中、术后和随访时的超声心动图资料。结果：符合本文纳入标准的9例患儿进入本文分析,男6例,女3例,年龄4月龄至9岁。术前超声提示,三叶式和二叶式主动脉瓣分别为3例和6例;重度狭窄6例,中度狭窄1例,重度狭窄伴中度返流2例。9例均以自体心包再造或扩大主动脉瓣瓣叶。术后即刻与术前超声心动图比较：主动脉瓣最大跨瓣压差［（31.6±9.4）mm Hg vs（73.0±22.2）mm Hg, P=0.000］和主动脉瓣平均跨瓣压差［（15.8±18.3）mmHg vs （35.8±18.3）mmHg, P= 0.004］均下降。术后随访24～48（32±8）月,无死亡和再干预病例,未见严重不良事件,未见主动脉瓣重度狭窄或重度反流、升主动脉狭窄或扩张、主动脉瓣瓣环狭窄与扩张、瓣膜脱垂或瓣膜赘生物病例;3例患儿分别在随访18、24和12个月时出现瓣叶活动僵硬,瓣叶开放不完全。术后左室后壁厚径均呈下降趋势;末次随访时,8例患儿左室后壁厚径Z值（Z-LVPWd）均下降至正常水平（＜2）。结论：以自体心包修复主动脉瓣可改善先天性主动脉瓣狭窄患儿的血流动力学,手术风险低,术后早期疗效可,避免或延缓儿童主动脉瓣置换术,减少施行ROSS手术机会。     

Autologous Ross Operation for Congenital Aortic Stenosis

Congenital aortic stenosis is a relatively common cardiac anomaly encountered in approximately 5% of all children with heart disease. The Ross procedure is increasingly used for replacement of the aortic valve in children. We report a 12-year-old boy who was born with congenital aortic stenosis secondary to a bicommissural aortic valve. The patient underwent open valvotomy in infancy and aortic valvuloplasty 2 years later. Residual/recurrent stenosis prompted referral for aortic valve replacement, and he underwent an autologous Ross procedure, in which the aortic root was replaced with a pulmonary autograft and the repaired aortic valve was used to restore right ventricular-to-pulmonary artery continuity. The postoperative course was unremarkable. Nitroprusside, esmolol, and labetolol were used to control postoperative hypertension. He was discharged 4 days after surgery on oral furosemide and aspirin, and he has had no cardiovascular symptoms during follow-up. Recent echocardiography demonstrated mild right ventricular outflow tract obstruction with a peak velocity of 3.6 m/sec, with a gradient of 42 mmHg and moderate pulmonary insufficiency. There was no left ventricular outlet tract obstruction or aortic insufficiency.     

Perforation of the Right Aortic Valve Cusp: Complication of Ventricular Septal Defect Closure with a Modified Rashkind Umbrella

An 18-month-old boy with a perimembranous ventricular septal defect (VSD) had undergone transcatheter closure of the defect with a modified 17 mm Rashkind umbrella device at age 4 months (weight 3.8 kg). The clinical signs of a VSD persisted, and he developed aortic incompetence, first detected 5 months after the procedure, which progressed from mild to moderate. A three-dimensional echocardiographic study demonstrated that one of the four arms holding the umbrella was protruding into the aortic valve and had perforated the right aortic valve cusp. This diagnosis was confirmed at subsequent surgery. Surgical repair of the perforated right aortic valve leaflet was necessary. The umbrella was adherent to the tricuspid valve and could not be removed. Instead it was left in situ, but three of the stainless steel arms were cut off. When umbrella closure of a perimembranous VSD is undertaken, the close proximity of part of the distal umbrella to the aortic valve can lead to aortic regurgitation.     

Aortic valve replacement surgery for a case of infantile Takayasu arteritis

Takayasu arteritis is a chronic inflammatory disease of unknown etiology primarily affecting the aorta and its major branches and usually occurring in the second or third decade of life. Here, we report a case of Takayasu arteritis in a 10-month-old patient. The infant presented with signs of congestive heart failure and severe aortic regurgitation. Echocardiography and computed tomography angiography showed an abnormally dilated thoracic and abdominal aorta. The infant was initially treated with prednisolone, followed by commissuroplasty of the aortic valve but neither approach ameliorated the heart failure. The patient was eventually treated with a mechanical aortic valve replacement surgery at the age of 12 months, and her condition stabilized. Although unusual, this case indicates that the diagnosis of Takayasu arteritis should be considered in children with unexplained systemic symptoms, aortic valve regurgitation, and heart failure. Because severe aortic regurgitation may be a fatal complication of Takayasu arteritis, early aortic valve replacement surgery should be considered, even in very young children.     

Surgical Strategy for Severe Aortic Hypoplasia and Aortic Stenosis With Ventricular Septal Defect and Normal Left Ventricle

At our institution, the strategy for patients with bicuspid aortic valve, aortic valve stenosis (<5 mm), and aortic hypoplasia [hypoplastic aortic arch, coarctation of the aorta (CoA), or interrupted aortic arch (IAA)] with ventricular septal defects (VSDs) as well as normal left ventricular (LV) volume and mitral valve size consists of two parts. The Norwood operation is applied as the first palliation for this group of patients. Second, the decision whether the patients are to undergo the Rastelli operation or a univentricular repair is made depending on the size of the right ventricle after the Norwood operation. This study aimed to examine whether the aforementioned surgical strategy for this group of patients is adequate or not. Seven patients undergoing the Norwood operation as the first palliation for bicuspid aortic valve, aortic valve stenosis (<5 mm), and aortic hypoplasia with VSDs as well as normal LV volume and mitral valve size between February 2005 and March 2010 at Kitasato University Hospital and the Gunma Children’s Medical Center were reviewed. Postoperative serum B-type natriuretic peptide (BNP) and central venous pressure (CVP) were measured in the patients undergoing the staged Norwood–Rastelli operation to assess whether the authors’ right ventricular end-diastolic volume index (RVEDVI) cutoff (80 % of normal) is adequate. At this writing, all seven patients are alive after a mean follow-up period of 58.8 ± 17.8 months. They all had aortic valve stenosis of <5 mm and a bicuspid aortic valve. Four patients had a diagnosis of CoA with VSD, and three patients had IAA with VSD. Six patients underwent biventricular repair, and one patient had univentricular repair due to the small RVEDVI (74 % of normal). The patients with 80–90 % of normal RVEDVI had higher BNP and higher CVP than those with more than 90 % of normal RVEDVI after the Rastelli operation, whereas the patient undergoing the Fontan operation had a low BNP level. In conclusion, the described strategy for patients with severe aortic hypoplasia and aortic stenosis with VSD as well as normal LV and mitral valve size is reasonable.     

Septal aneurysm with associated anomalies

A 15-year-old boy had a history of a functional systolic murmur with systolic and diastolic murmurs and radiological evidence of left ventricular hypertrophy. The angiocardiogram demonstrated an aneurysm of the membranous septum, bicuspid aortic valve, dilatation of the left subclavian artery and left common carotid artery. Echocardiography showed a holosystolic prolapse of the mitral valve with mitral insufficiency and a bicuspid aortic valve with insignificant incompetence of the aortic valve. Classical TM mode echocardiography did not show an aneurysm of the membranous septum.     

儿童先天性主动脉瓣畸形32例病理组织学特征

目的 探讨儿童先天性主动脉瓣畸形的病理组织学特征，为诊断提供依据。方法 对2003年1月至2005年12月因先天性主动脉瓣畸形、主动脉瓣功能障碍行外科手术切除的32例儿童主动脉瓣标本进行大体观察和组织学检查，复习病史并摘录相关资料，分析其病理组织学特征。诊断依据临床表现、术前超声心动图、术中所见及术后病理组织学检查，除外风湿性或退行性主动脉瓣病变、感染性心内膜炎及原发性结缔组织病变如Marfan综合征。结果 32例儿童先天性主动脉瓣畸形中，男性27例，女性5例，男：女=5.4：1，年龄6~18岁，平均年龄14.9岁。二叶型占37.5％（12例），三叶型59.4％（19例），四叶型3.1％（1例）。主动脉瓣狭窄（aortic stenosis，AS）5例（15.62%），主动脉瓣关闭不全（aortic insufficiency，AI）25例（78.13%），AS-AI 2例（6.25%），均不伴其他心脏瓣膜病变。20例伴其他先天性心脏病：室间隔缺损19例，动脉导管未闭2例，右室双腔心1例，主动脉右冠窦瘤3例。病理组织学改变为瓣叶增厚、大小不等、不规则（卷曲或脱垂）及坚度增加，部分伴钙化，光镜下示纤维组织增生，粘液样变性，胶原纤维溶解、断裂，弹性纤维破坏，偶见胶原纤维玻璃样变性、灶性钙化，无小血管增生及炎性细胞浸润。少数可见心内膜面局部破溃，内皮下毛细血管增生，纤维素性渗出，炎性细胞浸润，继发性钙质、脂质沉着和纤维化。结论 儿童先天性主动脉瓣畸形以男性为多见，二叶型、三叶型主动脉瓣常见，常伴有其他先天性心脏病，瓣膜功能障碍以AI为主，病理组织学改变主要为为瓣叶增厚、大小不等、不规则（卷曲或脱垂），光镜下示纤维组织增生，粘液样变性，胶原纤维溶解、断裂，弹性纤维破坏，无小血管增生及炎性细胞浸润，而纤维化、钙化少见。     

Tetralogy of Fallot with Aortic Stenosis: Repair Without Aortic Valve Intervention

A 14-day-old boy with tetralogy of Fallot (ToF), ductus-dependent pulmonary circulation, and significant aortic valve stenosis (AVS) underwent primary repair of ToF without aortic valve intervention. The pressure gradient through the aortic valve decreased spontaneously after the operation. This may have been due to the reduction of blood flow through the aortic valve postoperatively. For patients with ToF, AVS, and ductus-dependent circulation, ToF repair alone may alleviate the severity of the AVS.     

儿童主动脉瓣病变的外科治疗策略

目的 探讨儿童主动脉瓣病变的外科治疗策略.方法 回顾性分析2010年1月至2016年1月间收治的13例主动脉瓣病变患儿的临床资料.其中,男9例,女4例;年龄1～12岁,年龄分布1～3岁6例,3～6岁3例,6～12岁4例;体重12～36 kg,平均24 kg.术前经心脏超声及心脏CT明确诊断,先天性主动脉瓣病变8例,后天获得性主动脉瓣病变5例.手术在全身麻醉、低温、体外循环下进行,结合超声、CT及术中情况决定手术方式.结果 全组13例中,行改良Ross手术4例,主动脉瓣成形术3例,主动脉瓣机械瓣置换手术3例,Bentall术1例,Ross手术2例.全组无手术死亡,术中采用冷血停跳液或冷晶体停跳液灌注,合并主动脉关闭不全时,切开直接灌注,术中均应用超滤.术后有效随访患儿11例,2例患儿未规律随访,随访时间6个月～5年,全部存活,正常生活不受影响.机械瓣置换的长期使用华法林抗凝,目前无并发症出现.2例随诊过程中发现瓣膜压差大于40 mmHg,一般状况可,随访中未发现明显瓣膜严重反流.结论 儿童主动脉瓣病变的外科治疗方法有多样性的特点,本文旨在为心外科医师在儿童主动脉瓣病变的治疗策略方面提供更多的临床经验,儿童主动脉瓣疾病治疗的临床效果还有待进一步探讨.     

Transluminal dilatation of critical aortic stenosis and coarctation of a newborn infant aorta

We present a 20-day-old male infant with severe congestive heart failure. Echocardiography showed left ventricular hypertrophy secondary to critical aortic valve stenosis and severe coarctation of the aorta. The size of aortic annulus valve was 5.2 mm. At catheterization, the gradient of aortic valve stenosis was 104 mmHg and the gradient through aortic coarctation, measuring 14 mm in diameter, was 58 mmHg. Transverse and isthmic aorta showed moderate hypoplasia. After two dilatation procedures performed at an interval of 45 days, the pressure valve gradient decreased to 32 mmHg, that of the coarctation of the aorta decreased to 14 mmHg and the diameter increased to 5.4 mm.     

Small aortic valve annulus in children with fixed subaortic stenosis

Summary Twenty-one hearts with fixed subaortic stenosis (FSAS) were examined pathologically. Thirty children with no hemodynamically significant heart disease, 31 children with valvar aortic stenosis, and 25 children with FSAS were studied by echo- and angiocardiography. The following conclusions were drawn: (1) Patients with FSAS often have abnormal aortic valve leaflets as well as small aortic valve annulus. (2) A small aortic annulus/descending aorta ratio is probably present at birth, and may decrease with increasing age. (3) In some patients with FSAS the aortic valve annulus is too small for simple resection of the fibroelastic tissue. A Konno operation is needed for these patients. (4) M-mode echocardiography has not been useful in identifying abnormally small aortic valve annulus in FSAS patients.     

Transcarotid Balloon Valvuloplasty with Continuous Transesophageal Echocardiographic Guidance for Neonatal Critical Aortic Valve Stenosis: An Alternative to Surgical Palliation

Neonatal critical aortic valve stenosis is a life-threatening malformation if untreated. Before the late 1980s, the preferred treatment was surgical valvotomy; however, operative mortality was high. Early reports of transcatheter balloon dilation were encouraging, although femoral artery damage and aortic valve insufficiency were procedural limitations. With new balloon catheter technology, transumbilical, transvenous, and transcarotid approaches have been advocated, although a comparison with recent surgical results has not been performed. We compared all neonates who presented to our institution since 1985 with the diagnosis of critical aortic stenosis. Ten patients underwent surgical transventricular valvotomy and 13 patients underwent balloon valvuloplasty via a right carotid cutdown with continuous transesophageal echocardiographic guidance. Prior to intervention, all patients had either left ventricular dysfunction, an aortic valve gradient >100 mmHg, significant mitral valve insufficiency, and/or ductal dependent systemic blood flow. All patients had successful relief of aortic valve obstruction with normalization of left ventricular function and successful discontinuation of prostaglandin E₁. Use of continuous transesophageal echocardiographic guidance resulted in fluoroscopic exposure of only 12 ± 8 minutes. At the latest follow-up, a similar proportion of patients has required additional aortic valve procedures (38% vs 25%) and overall mortality (20% vs 15%) is similar. In the transcarotid group, 9 of 13 patients (69%) have a normal appearing right carotid artery by Duplex imaging, and no neurologic events have been reported. Balloon aortic valvuloplasty via a right transcarotid approach is safe, simplifies crossing the valve, and is effective for the initial palliation of neonatal critical aortic stenosis. The use of transesophageal echocardiographic guidance reduces fluoroscopy exposure, enables accurate assessment of hemodynamics without catheter manipulation or angiography, and avoids femoral artery injury.     

Echocardiographic Study on the Origin of the Innocent Flow Murmurs

To investigate the origin of the pulmonary systolic ejection innocent flow murmur (IFM), echocardiographic examinations were undertaken in 30 children with IFM and in a control group consisting of 28 healthy children without murmur. Compared to the controls, the diameters of the left ventricular outflow tract (LVOT) and aortic valve annulus and aortic valve area tended to be smaller, whereas stroke volume (SV) and cardiac output were slightly greater in children with IFM, but they were not statistically significant. Mean fractional shortening was significantly higher in children with IFM. Peak flow velocity of LVOT, right ventricular outflow tract, aorta, and pulmonary artery, mean velocity and velocity time integral of the aortic and pulmonary flow, and maximal acceleration of the aortic flow were all significantly higher in the IFM group compared to the controls. The variables of left-sided flow velocities in the same individuals with IFM were significantly higher compared to those derived from the right heart. The ratios of the SV to the LVOT diameter and to the aortic valve area were found to be significantly greater. It was concluded that IFM originates from higher blood flow velocities in the region of LVOT and aortic valve annulus, and that the increased flow velocity results from the larger SV passing through the relatively narrow LVOT and aortic valve in children with IFM.     

左胸肋间入路微创封堵手术治疗干下型室间隔缺损

目的总结左胸肋间入路微创封堵手术治疗干下型室间隔缺损(VSD)的初步经验。方法2014年2月至2 015年9月我们对19例干下型VSD患儿施行外科微创封堵手术,其中男性10例,女性9例;年龄0.5~17岁,平均(3.2±4.3)岁;体重5.5~50.0 kg,平均(14.3±10.9)kg;缺损直径3.5~9.0 mm,平均(5.4±1.8)mm;合并主动脉瓣右冠瓣轻度脱垂6例,主动脉瓣局限~轻微反流3例,肺动脉瓣轻中度狭窄1例,轻度狭窄1例。均采用左胸肋间入路,在经食管超声心动图(TEE)监视下建立VSD输送轨道并置入封堵器,观察有无残余分流及主动脉瓣反流;于出院前、手术后定期复查经胸超声心动图和心电图。结果 18例封堵手术获成功,因新发主动脉瓣中度反流及残余分流而中转开胸手术1例。术中新发主动脉瓣轻微反流2例,术前合并主动脉瓣反流患者中有2例反流加重。手术时间35~85 min,平均手术时间(55.7±15.9)min。术中出血量3~200 mL,平均(22.7±43.3)mL。围手术期输血1例。术后住院时间3~13 d,平均(4.2±2.3)d。术后出现残余分流、上呼吸道感染、伤口感染各1例。随访6~25个月,平均(14.7±7.0)个月,随访期间1例新发主动脉瓣反流,1例术中新发主动脉瓣反流消失,2例术后仍有主动脉瓣反流患者反流无明显变化,1例残余分流愈合,无传导阻滞发生。结论左胸肋间封堵手术治疗干下型VSD手术安全,操作简单,创伤小,恢复快,近期效果满意,远期效果需进一步随访;对合并主动脉瓣脱垂尤其主动脉瓣反流的干下型VSD需慎重施行外科徽创封堵手术。     

Retrograde transfemoral catheterization of the left ventricle in children with aortic valve stenosis

Summary In 19 consecutive children with aortic valve stenosis, the left ventricle was entered retrogradely with a Gensini catheter guided by a tip-deflector guidewire which could produce any desirable degree of curvature at its tip. In all patients, the aortic valve was retrogradely traversed for measurement of the pressure gradient (mean gradient=53 mmHg, mean valve area=0.36 cm²), and left ventriculography. No complications were encountered. This technique is safe, simple, and effective in retrograde catheterization of the left ventricle in children with aortic stenosis.     

Effectiveness of Angiotensin-Converting Enzyme Inhibitors in Pediatric Patients with Mid to Severe Aortic Valve Regurgitation

The long-term benefit of angiotensin-converting enzyme inhibitors in pediatric patients with aortic valve regurgitation is under consideration. Eighteen patients with mid to severe aortic valve regurgitation were retrospectively evaluated. Echocardiographic parameters (left ventricular end-diastolic diameter, shortening fraction, left ventricular posterior wall thickness, and grade of aortic valve regurgitation) were analyzed before and during therapy with angiotensin-converting enzyme inhibitors. Data are given as standard deviation scores (Z-scores) derived from body surface-adjusted normal values. Median (interquartile range) age at start of therapy was 8.4 (5.4 to 10.0) years, and total follow-up 2.3 (0.9 to 5.4) years. Left ventricular end-diastolic diameter increased from 3.6 (2.3 to 4.5) to 3.7 (2.4 to 4.8), and left ventricular posterior wall diameter decreased from 1.9 (1.1 to 3.0) to 1.1 (0.5 to 2.3). Grade of aortic valve regurgitation increased from 3.5 (2.3 to 4.0) to 4.0 (2.0 to 4.0), and shortening fraction decreased from 39% (34% to 43%) to 37% (34% to 42%). No significant effect of angiotensin-converting enzyme inhibitors on left ventricular dimensions or function was found in our population of patients with mid to severe aortic valve regurgitation. Angiotensin-converting enzyme inhibitors may not alter left ventricular overload in pediatric patients with aortic valve regurgitation.     

Mitral Valve Injury During Balloon Valvuloplasty for an Infant with Severe Aortic Stenosis: Spatial Evaluation Using Three-Dimensional Echocardiography

We report on a 4-month-old infant with mitral valve injury that was induced by percutaneous balloon valvuloplasty for severe aortic valve stenosis. Three-dimensional echocardiography revealed a laceration at the anterior leaflet of the mitral valve. The mitral valve injury was successfully repaired by surgical operation 1 year after the valvuloplasty. This unexpected complication is associated with anatomical disorders of congenital aortic stenosis, including hypoplastic left ventricle, short chordae tendae, and large papillary muscles.     

Aortic valvuloplasty in the fetus: technical characteristics of successful balloon dilation

OBJECTIVES: To describe technical aspects of successful fetal aortic valvuloplasty, with particular attention to balloon size. STUDY DESIGN: We retrospectively reviewed all procedural records and echocardiograms pertaining to 26 attempts at fetal aortic valve dilation performed at a single center over a period of 4 years. We assessed the effect of valvuloplasty as determined by echocardiographic appearance at the time of intervention and in follow-up. RESULTS: In 20 of 26 fetuses who had technically successful aortic valve dilation (median balloon:annulus ratio=1.1), all had improved antegrade flow and 12 had at least mild regurgitation after dilation. Use of an oversized balloon was associated with the onset of moderate or severe aortic regurgitation, seen in 5 fetuses. This aortic regurgitation was well tolerated and improved through the remainder of gestation. CONCLUSIONS: These data imply that fetal aortic valves can be dilated safely with larger balloons than are commonly used for postnatal dilation. The observation of spontaneous improvement in postdilation aortic regurgitation further suggests that fetal valve tissue behaves uniquely.     

Absent Aortic and Dysplastic Pulmonary Valves Associated with Ventricular Septal Defect in Fetal Hydrops

We describe worsening cardiac failure in a hydropic male fetus with a large ventricular septal defect associated with severe pulmonary and mild aortic regurgitation detected prenatally by echocardiography. The hemodynamic transition after emergency cesarean section at 31 weeks of gestation resulted in amelioration of the pulmonary incompetence but exacerbation of aortic insufficiency causing coronary steal. Death followed withdrawal of support 4 hours after delivery. Autopsy revealed absent aortic valve cusps, dysplastic pulmonary valve, and a large ventricular septal defect.