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.     

Tetralogy of Fallot with Congenital Aortic Valvar Stenosis: The Tetralogy–Truncus Interrelationship

Two rare patients are reported with tetralogy of Fallot and congenital aortic valvar stenosis. The anatomic and developmental interrelationship between tetralogy of Fallot and truncus arteriosus is summarized. A study of 100 randomly selected postmortem cases of tetralogy revealed aortic valve pathology in 8%, myxomatous aortic valve leaflets without stenosis in 4%, bicuspid aortic valves without stenosis in 3%, and congenital aortic valvar stenosis in 1%. The frequency of systemic semilunar valve pathology in truncus was much higher (66%): moderate to marked myxomatous change in 44%, mild myxomatous change in 22%, truncal valvar stenosis in 11%, and truncal valvar regurgitation in 15%. Being aware of the tetralogy–truncus interrelationship and knowing that myxomatous aortic valves are prone to premature calcific aortic stenosis and/or regurgitation, physicians should follow the aortic valves of surgically repaired patients with tetralogy of Fallot and truncus arteriosus long term with great care. Timely aortic valvuloplasty or replacement may well prove life-saving in such patients.     

The Abnormal Contralateral Atrioventricular Valve in Mitral and Tricuspid Atresia in Neonates: An Echocardiographic Study

Abnormalities of the mitral valve (MV) or the tricuspid valve (TV) morphology and/or function in patients with functional single ventricle may result in early morbidity and death. The purpose of this study was to determine the incidence of contralateral atrioventricular valve (AVV) pathologies in mitral valve atresia (MA) and tricuspid valve atresia (TA). We retrospectively reviewed the echocardiographic data of 50 neonates with MV and 20 with TA. Appearance of the papillary muscles, chordae tendinae, and valve leaflets was assessed. AVV regurgitation was semiquantitated by color-flow Doppler and the AVV annulus diameter was measured and indexed to body surface area. MV abnormalities were found in 9 of 20 (45%) of patients with TA. The MV was myxomatous in 9 patients, the leaflets were redundant in 5 patients, and prolapsing occurred in 4 patients. Mild regurgitation was found in 2 patients. In 18 of 20 (90%) patients MV annulus size was larger than 95% of predicted normal values. TV abnormalities were found in 12 of 50 (24%) patients with MA. The TV was myxomatous in 4 patients, prolapsing in 2, and redundant in 3, and moderate TV regurgitation was found in 3 patients. In 29 of 50 (58%) patients TV annulus size was larger than 95% of predicted normal values. Contralateral AVV abnormalities in tricuspid and mitral valve atresia are common and should be assessed carefully before surgical procedures.     

Morphologic Analysis of Common Atrioventricular Valves in Patients with Right Atrial Isomerism

The objective of the study was to examine the relation between the morphology of the common atrioventricular valve and regurgitation of the valve in patients with right atrial isomerism. We examined seven consecutive patients with documented right atrial isomerism who subsequently underwent postmortem examination during a 10-year period. The degree of regurgitation and the diameters of the common valve were evaluated via cineangiography, and the site of regurgitation was evaluated by echocardiography. The morphology of the common atrioventricular valve was assessed further at autopsy. Cineangiography revealed valve diameters ranging from 14.8 to 27.8 mm (mean 20.9 mm). Valvar regurgitation was revealed within 2 months of birth in all patients. Regurgitation abruptly worsened in three patients after placement of a Blalock–Taussig shunt or a central shunt and postintubation. Autopsies revealed that the common atrioventricular valve consisted of four leaflets in five patients, and three leaflets in two. The anterior leaflets were large and protruding in all patients, and the lateral leaflets were thickened in six. All patients had a mass consisting of the left lateral leaflets and chordae with direct attachment of the chordae to the ventricular muscle (the right lateral leaflet was attached to the ventricular muscle and immobile in one patient). The lateral leaflets clung to the ventricular wall and exhibited poor movement in six patients. Leaflets with poor mobility corresponded to the regurgitant valvar site as assessed by echocardiography in six patients; and the regurgitation in three patients with acute deterioration occurred at the valvar side with poor mobility. It is concluded that the common atrioventricular valve in patients with right atrial isomerism has morphologic characteristics that may be associated with valvar regurgitation and malignant potential for abrupt deterioration after replacement of systemic–pulmonary shunting.     

Morphology of Tricuspid Valve in Pulmonary Atresia with Intact Ventricular Septum

Pulmonary atresia with intact ventricular septum (PAIVS) is a rare congenital cardiac anomaly that has been classified into two types: one is a more frequent type having dysplasia of tricuspid valve (TV) with a small annulus, underdeveloped right ventricle (RV) with a hypoplastic cavity and a hypertrophic wall; the other type has severe dysplasia of TV and dilatation of RV, right atrium (RA), and right atrioventricular junction with thinning of the RV wall. We performed a morphologic study on 11 autopsied hearts with PAIVS, giving particular emphasis to the variation of morphology of the TV. We could classify these hearts into 3 groups according to the degree of right ventricular development. In the first group of 7 cases (type I), the RVs were underdeveloped. Thick leaflets, restricted valve apparatus with short chordae, and small annuli were characteristics of the TV. In the second group of 3 cases (type II), the RVs showed marked enlargement of the cavity and thinning of the wall. The TV showed redundant, dysplastic, sail-like anterior leaflets, and the downward displacement of septal leaflet and/or posterior leaflet, which are the findings frequently observed in Ebstein's malformation. The RVs were dilated and with partially unguarded tricuspid orifice. The septal leaflet of the TV was dysplastic and, in two cases, the septal leaflet showed chordal structure at the upper surface facing the RA, which is a peculiar finding that has not been described in the literature. The remaining case was a heart with a moderately developed RV (type III). The TV showed mildly dysplastic appearance and we classify this as a separate type, because we could expect the best surgical results in this type. This type had optimal size of RV and the mildest degree of dysplasia of TV. In PAIVS, the morphology of TV correlates well with the type of the right ventricular development.     

Aplasia of semilunar valve leaflets: Two case reports and developmental aspects

Summary Two fetuses of approximately 11 weeks development with aplasia of the pulmonary as well as the aortic valve leaflets are reported. Both cases showed additional cardiac malformations. Case 1, with all leaflets missing, also had double-outlet right ventricle, hypplastic left ventricle, large ventricular septal defect, straddling tricuspid valve, and atretic mitral valve. Case 2, with only one hypoplastic aortic valve leaflet, showed hypoplasia of the mitral valve and the left ventricle, and a subaortic ventricular septal defect.The observations made and data in the literature suggest that aplasia of semilunar valve leaflets reflects an underdevelopment of the endocardial cushion swellings at the ventriculoarterial junction, rather than resulting primarily from a malseptation of the cardiac outflow tract.     

A New Method for Evaluating Tricuspid Valve Displacement in Children With Ebstein’s Anomaly: Using the Annulus and Coronary Sinus as a Reference Point

This study aimed to investigate the reference point for the downward displacement of the posterior and anterior leaflets of the tricuspid valve using echocardiography in children with Ebstein’s anomaly. This study enrolled 25 patients with Ebstein’s anomaly. The extent of downward displacement of the posterior and anterior leaflets of the tricuspid valve was evaluated by echocardiography using the tricuspid annulus and the coronary sinus as reference points. These results were compared with the surgical findings. The findings showed displacement of the simple septal leaflet in 1 patient, displacement of both the septal and posterior leaflets in 22 patients, displacement of both the anterior and posterior leaflets in 1 patient, and displacement of all the leaflets in 1 patient. Because the septal and posterior leaflets were close to the apex or because the posterior leaflet was nearly absent, the displacement distance of the septal and posterior leaflets could not be measured accurately in two patients. The displacement distance of the septal and posterior leaflets in the remaining 22 patients were 2.08 ± 1.15 and 2.58 ± 1.06 cm, respectively. The displacement distances of the anterior leaflet in two patients were respectively 1.0 and 2.2 cm. These results were similar to those measured during surgery. The direction of the valvular regurgitation flow was anterolateral in the apical four-chamber and apical right heart two-chamber views in patients with the downward displacement of the anterior leaflet. The tricuspid valve annulus and the coronary sinus are ideal reference points for evaluating the downward displacement of the posterior and anterior leaflets of the tricuspid valve. It is critical to evaluate the downward displacement of the anterior leaflet that the direction of the tricuspid regurgitation flow is changed.     

Anomalous semilunar valve relationships in transposition of the great arteries

Summary We compared semilunar valve relationships of 26 normal hearts with 66 specimens with transposition of the great arteries (TGA). The TGA hearts exhibited a spectrum of dextroposition of the aorta, which brought the point of fibrous continuity between aortic and pulmonary valves 15 to 120° closer to the tricuspid annulus than in the normal specimen. Aortic dextroposition was associated with malalignment of the posterior semilunar (pulmonary) valve in relation to the aortic valve, the tricuspid and mitral valves, and to the membranous ventricular septum. Supported by HL 20132 from the National Institutes of Health.     

Defining biomechanical endpoints for tissue engineered heart valve leaflets from native leaflet properties

The design and development of functional engineered tissues is dependent on multiple considerations, with biomechanics paramount for load-bearing constructs such as tissue engineered heart valves. As the cryopreserved allograft is the current standard for valve replacement in pediatric patients, identifying and quantifying essential structural–mechanical properties of the native valve leaflet is a crucial step in the engineered valve leaflet design process. Native valve leaflet properties provide an intuitive basis for assessing engineered valvular tissue performance, and can potentially be used as biomechanical endpoints for qualifying engineered leaflets prior to clinical applications. In this short review, we present three analysis techniques that have been used by our lab and others for characterizing heart valve leaflet biomechanical response and discuss the relevance of these properties as candidate endpoints for engineered leaflet tissues. The studies presented herein focused primarily on the aortic valve, which most frequently warrants repair or replacement in the general population and has been useful in our understanding of bioprosthetic heart valve mechanics. However, these analysis techniques are directly applicable for pulmonary and most engineered valve leaflets. Where data is available, initial studies applying these techniques for in vitro assessment of scaffolds and engineered valve leaflets are presented. The development of a tissue engineered heart valve for the pediatric population is conceptually appealing, since few options currently exist due to the lack of growth potential in non-viable prosthetics and size limitations. While significant challenges remain, we believe that a derivative of the current tissue engineered heart valve paradigm will ultimately yield a design suitable for clinical evaluation. The role of biomechanics in this process will be to identify and quantify the structural–mechanical endpoints essential for appropriate heart valve leaflet function, while guiding investigators prior to and during clinical evaluation.     

肺动脉闭锁合并室间隔缺损104例诊断分析

目的　分析肺动脉闭锁合并室间隔缺损 (PA/VSD)的解剖类型及血流动力学改变 ,探讨其适宜的手术方式。方法　对 1992年 6月至 2 0 0 2年 5月在广东省心血管病研究所儿科住院的PA/VSD患儿共 10 4例 ,采用超声心动图结合心血管造影术 ,确定闭锁的部位、肺动脉的发育情况及血供来源。结果　右室流出道及瓣膜闭锁、有肺动脉总干 31例 ;肺动脉总干闭锁、左右肺动脉有汇合 5 0例 ;左肺动脉闭锁 10例 ,右肺动脉闭锁 8例 ;左右肺动脉均闭锁 5例。肺动脉的血供来源 :大的主肺动脉侧支血管 5 1例 ,动脉导管未闭 2 7例 ,多支小的侧支血管 2 6例。合并畸形有卵圆孔未闭、房间隔缺损、大动脉转位、完全性房室间隔缺损、右室双出口、镜面右位心、右旋心及左旋心。结论　合并室间隔缺损的肺动脉闭锁可发生在不同部位 ,肺动脉的血供来源多样化     

Anterolateral muscle bundle of the left ventricle in atrioventricular septal defect: Left ventricular outflow tract and subaortic stenosis

Summary The anatomy of the left ventricular outflow tract (LVOT) in 77 hearts with atrioventricular septal defect (AVSD), 36 with a separate AV orifice and 41 with a common AV-orifice, were investigated.In all specimens, an anterolateral muscle bundle of the left ventricle was identified between the superior bridging leaflet and the left coronary aortic cusp. It displaced the attachment of the superior bridging leaflet, resulting in its clockwise rotation. The muscle bundle frequently bulged into the LVOT, but was never prominent enough to have caused significant subaortic stenosis.Measurement of the LVOT aortic ratio was possible in 54 hearts and ranged from 36–100%. In 23 cases (43%), there was mild to moderate subaortic narrowing with a ratio ranging from 53–88%. In six cases (11%), unequivocal subaortic stenosis was present, mainly in AVSD with separate AV orifices (five of six) and iatrogenic in one case with surgically corrected complete defect.A decreased ratio was mainly due to decreased anteroposterior width of the septum in the subaortic area, with anterior displacement of the superior bridging leaflet in cases with dense septal attachment of the superior bridging leaflet (i.e., in AVSD with separate AV orifices, type A complete defect with small ventricular septal defect, or surgically corrected complete defect).Significant subaortic stenosis was caused by hypertrophy of the ventricular septum in the subaortic area with anteroseptal twist in four cases, by anomalous chordal insertion of the superior bridging leaflet in one case, and iatrogenic in one case after surgical correction with left AV valve replacement in a type C complete defect. Other additional obstructive forces were an anomalous papillary muscle, a small left ventricle, and an aneurysm of the membranous septum.     

Congenitally unguarded tricuspid orifice: Its differentiation from Ebstein's malformation in association with pulmonary atresia and intact ventricular septum

Summary Dysplasia of the leaflets is a well-known integral part of Ebstein's malformation. It is less well-recognized that occasionally the septal leaflet may be completely absent and not simply displaced from its usual annular attachment. This may make it difficult to differentiate by echocardiography between Ebstein's malformation and the rare lesion in which the tricuspid orifice is completely devoid of leaflet tissue (congenitally unguarded orifice). A neonatal case is described in which a partially unguarded orifice was present in association with pulmonary atresia and intact ventricular septum but misdiagnosed as Ebstein's malformation. In addition we reviewed the morphologic features of all 46 cases of pulmonary atresia and intact septum in the Cardiopathological Collection of the Children's Hospital of Pittsburgh: 17 also had Ebstein's malformation, while three had a congenitally unguarded tricuspid orifice.We found that the differential diagnosis occurred when the right ventricle was dilated rather than being a hypoplastic cavity, as was a feature of all the cases with unguarded orifice and five of those with Ebstein's malformation. The difference between the lesions is best demonstrated by examining the mural leaflet of the valve, which is absent when the orifice is unguarded but displaced in association with Ebstein's malformation. This feature should be recognizable by cross-sectional echocardiography. We have confirmed previous studies that dilatation of the chambers of the right heart indicates a very poor prognosis in cases with pulmonary atresia and an intact ventricular septum.The work was done at the Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania, USA, when RHA was on study leave from the National Heart and Lung Institute, London, supported by the Joseph Levy and British Heart Foundations, as well as the Patrick Dick Memorial Fund.     

Postoperative mitral leaflet rupture in an infant with Loeys–Dietz syndrome

This report describes an infantile case of Loeys–Dietz syndrome (LDS) with spontaneous mitral leaflet rupture. The patient was diagnosed with a type B interruption of the aortic arch. Bilateral pulmonary artery banding was performed 5 days after birth. On the 53rd day, intra‐cardiac repair was performed without valvuloplasty. Although the operation was successful, mitral regurgitation deteriorated at 4 weeks after operation. On the 88th day, a mitral valvuloplasty was performed and a severely ruptured anterior leaflet was observed. Seven days after valvuloplasty, the mitral valve insufficiency again worsened and a fourth operation was performed. Two tears were observed in the anterior and posterior mitral valve leaflets, and a mitral valve replacement was required. Subsequently, the patient was diagnosed with LDS according to gene mutational status. LDS is known to have a poor prognosis with cardiovascular complications, but valve rupture has not been previously reported in other cases.     

Morphological Nature of the Atrioventricular Valves in Hearts with Double Inlet Left Ventricle

The rudimentary right ventricle in hearts with double inlet to a dominant left ventricle can exist either to the right or the left side of the ventricular mass. These variants have been interpreted to imply differences in ventricular topology. If correct, they also imply that differences should exist in the morphology of the atrioventricular valves, previously thought to be indistinguishable one from the other. To test this possibility, we examined 15 hearts with double inlet to a morphologically left ventricle and 10 normal hearts. The distinguishing features of normal valves were evaluated for their consistency, and the most reliable were employed for interpreting the valves in the abnormal hearts. As shown by previous echocardiographic studies, insertion of valvar tension apparatus to the interventricular septum was unique to the morphologically tricuspid valve. Ten of the abnormal hearts had one valve showing this feature, it being absent in the other valve. The attachment, consistently on the same side as the rudimentary right ventricle, could be of diagnostic value in determining ventricular topology. In our small study, nonetheless, this approach still leaves one third of the hearts in which topology cannot be ascertained with certainty and in which it is not possible to distinguish the morphological nature of the atrioventricular valves.     

Outflow Tract Abnormalities in Atrioventricular Canal Malformations

Atrioventricular canal malformations are commonly regarded as being arrests of development of normal cardiac septation since their morphology strongly resembles the normal heart of Carnegie stages 14 through 18. Similarly, the spectrum of outflow tract abnormalities, which includes truncus arteriosus, transposition of the great arteries, double outlet right ventricle, and tetralogy of Fallot, has been interpreted as a developmental arrest because these malformations resemble the normal embryonic outflow tract in Carnegie stages 14 through 18, respectively. The causes of the developmental arrests in these two classes of malformations are unknown. Observation of an unusual autopsy case with features of both atrioventricular canal defect and tetralogy of Fallot prompted us to review the outflow tract morphology in 38 hearts with an atrioventricular canal malformation and two semilunar valves to determine if there was evidence to support the idea that both conditions may have the same pathogenesis. In all 38 hearts there was fibrous continuity between an anterior mitral leaflet and an aortic valve, which by anatomic definition means that no case had truncus arteriosus, transposition of the great arteries, or double outlet right ventricle. There were three cases with coarctation of the aorta, a lesion which arises when blood flow in the pulmonary trunk exceeds aortic flow during early development. There were four cases with pulmonary to aortic valve caliber ratios similar to those found in tetralogy of Fallot but the aortic-to-pulmonary valve angle relative to the heart base ranged over the full spectrum of angles seen in normal hearts and those with tetralogy of Fallot. Furthermore, this angle showed the same range of values for partial and complete canal defects, and did not correlate with patient age or ratio of pulmonary to aortic valve caliber. We conclude that the pathogenesis of the spectrum of outflow tract developmental arrests is different from that of atrioventricular canal malformations.     

Morphological nature of the atrioventricular valves in hearts with double inlet left ventricle

The rudimentary right ventricle in hearts with double inlet to a dominant left ventricle can exist either to the right or the left side of the ventricular mass. These variants have been interpreted to imply differences in ventricular topology. If correct, they also imply that differences should exist in the morphology of the atrioventricular valves, previously thought to be indistinguishable one from the other. To test this possibility, we examined 15 hearts with double inlet to a morphologically left ventricle and 10 normal hearts. The distinguishing features of normal valves were evaluated for their consistency, and the most reliable were employed for interpreting the valves in the abnormal hearts. As shown by previous echocardiographic studies, insertion of valvar tension apparatus to the interventricular septum was unique to the morphologically tricuspid valve. Ten of the abnormal hearts had one valve showing this feature, it being absent in the other valve. The attachment, consistently on the same side as the rudimentary right ventricle, could be of diagnostic value in determining ventricular topology. In our small study, nonetheless, this approach still leaves one third of the hearts in which topology cannot be ascertained with certainty and in which it is not possible to distinguish the morphological nature of the atrioventricular valves.     

Performance of the Pulmonary Autograft in Four Infants After the Ross Procedure

Pulmonary valve autografts have proven to be valid aortic valve substitutes in children. The objective of this study is to evaluate the performance of the pulmonary autograft (PAG) in systemic position and its adjustment to growth in four infants who underwent a Ross operation. Between April 1998 and January 2000, four infants whose aortic valve anatomy and function were judged unsuitable for a valve-sparing operation underwent successful Ross operation. All patients were followed postoperatively with echocardiograms to evaluate the function and the dimensional changes of the PAG within the systemic circulation. The PAG adjustments to growth were compared to the growth of the pulmonary root of healthy patients matched for body surface area, with the purpose to evaluate the effects of the systemic pressure on the pulmonary vascular wall, as a passive dilatation process versus a true and active structural adaptation. Median follow-up was 53 months (range, 48–65). There were no early deaths or reoperations. All patients were asymptomatic and in stable hemodynamic conditions. The PAG annulus grew according to body surface area without dilatation. PAG valve regurgitation was present but mild in three patients. Trans-PAG peak gradients were not significant. The PAG diameter at the level of the sinuses of Valsalva and sinotubular junction increased rapidly and steadily in all patients during follow-up compared to controls. The Ross operation in infants provides acceptable immediate surgical and hemodynamic results. However, significant midterm increases in PAG sinuses and sinotubular junction diameters require close and continued observation.     

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。     

Persistent truncus arteriosus: Pathologic anatomy in 54 cases

Summary Fifty-four specimens of heart with persistent truncus arteriosus (PTA) were reviewed anatomically. According to the Collett-Edwards classification [11] there were 28 examples of type I and 26 type II. The sex distribution was equal. The number of the truncal cusps ranged from one to four (42% tricuspid, 30% bicuspid, 24% quadricuspid, and 4% unicommissural). A unicommissural truncal valve has not been previously reported. In 72% of cases, the truncal valve leaflets were thickened or dysplastic. Two valves were stenotic. The truncus arteriosus originated from both ventricles equally in 42% of the cases, predominantly from the right ventricle in 42%, and predominantly from the left ventricle in 16% of the cases. In unoperated cases of PTA originating predominantly from the right ventricle, it appeared to us that usual operative correction might result in left ventricular outflow obstruction. Variations in coronary arterial origins and patterns were present in nearly half of the cases. A single coronary artery was observed in ten cases (18.5%). Stenosis of the ostium of one coronary artery was seen in each of four cases (7%). High posterior origin of the left coronary artery was observed in ten cases (18.5%). Among the associated cardiovascular anomalies, the most common were right aortic arch (36%) and interruption of the aortic arch (11%). Three cases with the latter condition exhibited crossed pulmonary arteries. Isolated cases with tricuspid atresia, vascular sling (left pulmonary artery arising from right pulmonary artery), and persistent common atrioventricular canal were encountered.     

Libman-Sacks Endocarditis in Pediatric Patient With Systemic Lupus Erythematosus

A 16 year old female patient with systemic lupus erythematosus presented to rheumatology clinic with a new I?CII/VI honking-quality mitral regurgitation murmur. The patient was initially evaluated by transthoracic echocardiogram that revealed mitral valve regurgitation and a large band of tissue under the mitral valve leaflets. Blood cultures were obtained and were negative. Transesophageal echocardiogram provided better visualization of the lesion and showed the band of tissue involving most of the chordae of the posterior mitral leaflet. A diagnosis of Libman-Sacks endocarditis was made given the aseptic nature of the lesions and the patient??s underlying lupus. Aggressive management of the lupus showed reduction of the mitral regurgitation and the size of the lesion. Libman-Sacks endocarditis is best evaluated by transesophageal echocardiogram.