Corrected transposition of the great arteries with associated tricuspid valve incompetence--report of a case of successful tricuspid valve replacement]

A 36-year-old woman with corrected transposition (C-TGA) of great arteries with associated systemic atrioventricular valve (A-V valve) regurgitation was presented. Cineangiography revealed that the right sided ventricle was morphologic left ventricle, and left sided ventricle was morphologic right ventricle. In addition, severe systemic A-V valvular regurgitation (Sellers IV) was demonstrated. Under the diagnosis of C-TGA with associated A-V valvular regurgitation, left sided atrioventricular valve replacement was performed using 29 mm SJM valve. Left sided atrioventricular valve was tricuspid valve (TV). A part of the anterior tricuspid leaflet and two pieces of papillary muscle was resected. Postoperative course was uneventful. The pathological findings indicated that valvular tissue had myxomatous degeneration.     

A successful surgical repair of bilateral atrioventricular valve regurgitation in corrected transposition of the great arteries

The patient was a 52-year-old female with bilateral atrioventricular valve regurgitation, who had SLL-type corrected transposition of the great arteries (C-TGA) accompanied by atrial fibrillation and ventricular extrasystoles (Lown 4b). In performing C-TGA in a patient at such an age, the ability of the anatomical right ventricle to maintain the systemic circulation is reduced, and so it is most important to maintain ventricular function at operation. In this case, we preserved the tricuspid valve and transplanted a biological valve at the tricuspid annulus, while the mitral valve was repaired with Kay's method of annuloplasty. Her postoperative progress was favorable and the echocardiography confirmed that the preserved tricuspid valve did not disturb the motion of the leaflets of the biological prosthesis.     

Unbalanced atrioventricular septal defect with parachute valve

A 5-month-old male patient presented with right-dominant unbalanced atrioventricular septal defect and left-sided parachute valve, and underwent successful biventricular repair. Because of the presence of a small left ventricle, left atrium, and a single left papillary muscle, an additional orifice was created in the left-sided atrioventricular valve with artificial partitioning of the right-sided atrioventricular valve. There was no evidence of mitral stenosis or regurgitation on follow-up echocardiography.     

Surgical repair of a common atrium in an adult.

We report a rare successful surgical repair of a common atrium (CA) with mild tricuspid valve (TV) regurgitation due to valvular annulus enlargement in a 39-year-old man, who had a complete atrial septum defect (ASD) without the characteristic of an endocardial cushion defect. The left-to-right shunt ratio was 85 percent and the Qp/Qs was 6.7 due to the CA. Left ventriculogram revealed no evidence of typical goose-neck deformity and no mitral valve regurgitation. The operation consisted of making a new atrial septum with an autologous pericardial patch and tricuspid annuloplasty (DeVega) using extracorporeal circulation. There was no evidence of a cleft on the anterior leaflet of the mitral valve or the septal leaflet of the TV. The postoperative echocardiogram showed no residual shunt flow through a new atrial septum and no TV regurgitation, and atrioventricular (AV) dissociation did not occur. We consider this procedure to be widely applicable in consideration of the favorable results obtained after surgical treatment.     

Surgical Treatment for Epicardial Metastasis of Myxoid Liposarcoma Involving Atrioventricular Sulcus

Abstract   We report the case of a 52-year-old man who presented with a cardiac metastasis of myxoid liposarcoma invading the atrioventricular sulcus. The tumor was arising from the anterior wall of the right atrium and basal right ventricle. After complete resection of the tumor (14 × 7.5 × 6 cm, 279 g), the annulus had no strong supporting tissue because of invasion of the atrioventricular sulcus. The tricuspid valve was reconstructed with reinforcement of the annulus using an autologous pericardial strip. The defect of the right atrial wall was reconstructed with a pericardial patch. After the reconstruction, echocardiography showed grade 1 tricuspid regurgitation. Although the patient had a small metastasis to the lung after surgery, he is doing well after 31-month follow-up. We believe that it is justified to perform aggressive resection with annuloplasty, when it is feasible, for cardiac metastasis of myxoid liposarcoma.     

Post-operative left atrioventricular valve function after the staged repair of complete atrioventricular septal defect with tetralogy of Fallot

Objective

This study reviewed surgical outcomes of staged repair for complete atrioventricular septal defect with tetralogy of Fallot, especially with focusing on the post-operative left-sided atrioventricular valve function.

Methods

Between 1992 and 2013, 10 patients with complete atrioventricular septal defect with tetralogy of Fallot underwent total correction by the following surgical strategy. Systemic-to-pulmonary shunt was placed at first at the mean age of 1.5 ± 1.3 months. Then confirming sufficient development of the left heart structures, the total correction was performed at the mean age of 1.4 ± 0.6 years. Second shunt was required in 4 (40 %) patients to develop the left heart structures.

Results

The left ventricular end-diastolic volume before total correction was 127 ± 30 % of normal size. The two-patch repair was applied in 8 (80 %) patients. There was no mortality, and 1 reoperation case for left-sided atrioventricular valve regurgitation. The follow-up was completed on all patients and the mean follow-up period was 7.4 ± 7.0 years. The post-operative left-sided atrioventricular valve regurgitation kept less than moderate for 10-year follow-up in all patients except one patient who required the repair of left-sided atrioventricular valve 1 year after the total correction.

Conclusions

The post-operative left-sided atrioventricular valve function after the repair of complete atrioventricular septal defect with tetralogy of Fallot maintained with the application of the two-patch repair, early and repeated palliative systemic-to-pulmonary shunt, and the early definitive surgery.     

Prosthetic mitral valve replacement after atrioventricular septal defect repair: a technique for small children

An 11-month-old girl was transferred for consideration of cardiac transplantation. She had previously undergone repair of complete atrioventricular septal defect at another institution. Her postoperative course was notable for severe mitral regurgitation, pulmonary hypertension, and heart failure. At reoperation, the left atrioventricular valve was considered irreparable with a very small (11 mm) annulus. Using a technique to enlarge the mitral annulus, a 17-mm prosthetic valve was placed. Her postoperative course was unremarkable and she is doing very well at 3 years follow-up.     

New technique for enlargement of the pulmonary outflow tract in corrected transposition of the great arteries [SLL]--a report of 2 cases

New technique for enlargement of the pulmonary outflow tract was performed in two patients with corrected transposition of the great arteries [SLL] associated with atrial septal defect, ventricular septal defect, pulmonary stenosis and mitral regurgitation. The middle of the anterior leaflet of the mitral valve was incised to the valve annulus towards the mid-point of the mitral-pulmonary fibrous continuity. In this approach, anterior node and anterior atrioventricular conduction bundle were securely protected from the surgical incision. The pulmonary annulus was divided posterolaterally and the incision was further extended into the pulmonary trunk to the bifurcation. The pulmonary trunk was enlarged with a fusiform patch of the xenogenous pericardium bearing monocusp. In case 1, St. Jude Medical valve #31 was implanted in the mitral position. In case 2, mitral valvular annuloplasty with Carpentier ring #36 which was deformed to admit enlarged portion of the pulmonary trunk. The VSD was closed through the right atrium, placing the suture on the left side of the septum. However, complete A-V block ensured temporarily due to retraction at the operation in case 1. No conduction disturbance ensured in case 2. This technique can provide some advantage in avoidance of injuries to the anterior node and the anterior atrioventricular conduction bundle. Application of this technique to the corrected transposition of the great arteries without mitral regurgitation is to be further evaluated.     

Role of the septal leaflet in tricuspid valve closure. Consideration for treatment of complete atrioventricular canal.

A septal leaflet of the tricuspid valve is thought to work differently from other anterior and posterior leaflets. We studied its role in valve closure in dogs by means of a dynamic area meter. During the control state, the tricuspid valve orifice area increased twice in diastole coincidentally with either atrial systole or rapid ventricular filling. We observed several findings after the septal leaflet resection: (1) two peak area patterns of the tricuspid valve orifice in diastole, (2) no elevation of right atrial pressure on ventricular systole (there was no V wave), (3) no tricuspid valve regurgitation on right ventriculography. These findings suggest that a complete valve closure occurred without the septal leaflet in regular sinus rhythm. An elevation of the right ventricular pressure produced by pulmonary artery stenosis without septal leaflet, however, easily caused tricuspid valve regurgitation in contrast to the same pressure of the right ventricle with the normal tricuspid valve. The right ventricular pacing caused severe valve regurgitation without the septal leaflet. Results indicate that in the repair of the complete atrioventricular canal defect and other tricuspid valve lesions, the septal leaflet of the tricuspid valve rarely requires attention. An atrioventricular block should be avoided, however, because electrical cardiac pacing on the right ventricle causes severe valve regurgitation without the septal leaflet.     

Repair of intracardiac defects associated with congenitally corrected transposition of the great arteries

From October 1976 to June 1986, 12 patients with corrected transposition of the great arteries (c-TGA) underwent repair of associated intracardiac defects. Ventricular septal defect (VSD) was closed by the method of de Leval in 7 patients, pulmonary outflow tract obstruction was relieved by valvotomy in 2, and bypassed by an external valved conduit in 6. The systemic atrioventricular (A-V) valve was replaced in 2. There was one operative death, giving a mortality rate of 8%. None of the patients developed complete heart block. The ejection fraction of the systemic ventricle was impaired (29-38%), particularly after ventriculotomy of this chamber. Surgical repair of intracardiac defects associated with c-TGA currently can be performed with acceptable risk, and de Leval's method for VSD closure is recommended; but the possibility of the postoperative development of systemic ventricular dysfunction and A-V valve regurgitation necessitates careful follow-up.     

Successful Repair of the Right Atrial Isomerism, Double Outlet Right Ventricle, Cornmon Atrioventricular Canal, Pulmonary Stenosis, and Total Anomalous Pulmonary Venous Connection

A 6-year-old boy was successfully operated on for double outlet ventricle, common atrioventricular canal with severe valvular regurgitation, right atrial Isomerism, L-loop ventricles, total anomalous pulmonary venous connection, and pulmonary stenosis with hypoplastic left pulmonary artery. The Interventricular rerouting from the left ventricle to the ascending aorta was performed with a spiral patch, the lnteratrlal switching was performed by a Mustard patch, the common atrioventricular orifice was partitioned and valve repair was performed, and an 18-mm valved conduit was inserted between the right ventricle and the pulmonary artery. Although the patient had a small residual ventricular septal defect and pulmonary stenosls, the patient Is alive and well.     

Aortic and tricuspid valve replacement in a case of corrected transposition of the great arteries

It is well known that corrected transposition of the great arteries (C-TGA) is accompanied with tricuspid valve regurgitation, but very few reports have described complication of this anomaly with aortic valve regurgitation (AR). We have performed aortic valve replacement (AVR) in a case of C-TGA associated with AR which had been evaluated the grade of 3/4 according to Sellers' classification, simultaneously done the valve replacement for the dysplastic tricuspid valve and annuloplasty for the right-side atrioventricular valve (mitral valve). The patient has a favorable postoperative course.     

房室间隔缺损矫治术后左侧房室瓣反流的外科治疗

目的为提高房室间隔缺损修补术后左侧房室瓣反流的外科治疗效果,探讨其瓣膜的病理改变、手术方法的选择,总结围手术期处理经验。方法回顾分析上海交通大学医学院附属新华医院1995年1月至2009年12月收治29例房室间隔缺损矫治术后左侧房室瓣反流患者的临床资料,其中男16例,女13例;年龄4~62岁,平均年龄26.5岁;术前心功能分级（NYHA）Ⅱ级10例,Ⅲ级17例,Ⅳ级2例;曾行部分性房室间隔缺损矫治术18例,完全性房室间隔缺损矫治术11例;再次手术行心瓣膜修复术17例,心瓣膜置换术12例。结果手术后早期因多器官功能衰竭死亡1例;1例患者行心瓣膜置换术后发生永久性Ⅲ°房室传导阻滞而安装永久心脏起搏器,1例4岁患者行心瓣膜置换术后不能撤离体外循环而行左心辅助56 h后痊愈出院。术后随访25例,失访3例,均为行瓣膜修复患者;术后随访6个月~14年,平均8.2年;随访14例心瓣膜修复患者,左侧房室瓣前向血流均无明显加快,10例左侧房室瓣轻微或轻度反流,1例中度反流,3例分别于再次手术后10 d、3年和6年再发左侧房室瓣重度反流,最终行心瓣膜置换术;25例患者术后心功能明显改善,Ⅰ级17例、Ⅱ级6例、Ⅲ级2例;胸部X线片示：心影较术前明显缩小,心胸比率0.53~0.67（0.60±0.11）;未发现远期死亡。结论对房室间隔缺损矫治术后再发生的左侧房室瓣反流,进行及时的手术治疗,选择恰当的手术方式,能取得满意的近期和远期疗效。     

Prosthetic valve implantation without removal of tricuspid apparatus for an old case of corrected transposition of the great arteries

Prosthetic valve implantation was performed for the systemic atrioventricular regurgitation in an old patient with corrected transposition of great arteries. SJM27M prosthesis was implanted without removing posterior and septal leaflet, chordae and papillary muscles with respect to preserve the function of the morphological right ventricle. It is important to preserve the function of the morphological right ventricle because it must continuously play a role of systemic ventricle even after surgical procedure.     

Surgical repair of double-orifice of the mitral valve in cases with an atrioventricular canal defects

OBJECTIVE: A Double-orifice in the mitral valve is an uncommon congenital cardiac lesion which occurs as an isolated anomaly or in association with other cardiac malformation. This report deals with our surgical experience of a double-orifice of the mitral valve in cases with an atrioventricular canal defect. PATIENTS AND METHODS: From 1991 through 1999, ten patients were diagnosed to have a double-orifice of the mitral valve at Shizuoka Children's Hospital. Each patient had associated major cardiac malformations, among which atrioventricular canal defect underwent surgical management, with five of these undergoing complete correction with or without previous pulmonary artery banding. Of these 10, the five cases were enrolled in this study. Two of these had a complete type, and the other three had a partial type. The cleft in the left-sided atrioventricular valve was closed partially in four and left untouched in one. Bridging tissue, when present, was left intact. There was no regurgitation from any accessory orifice and no repair for an accessory orifice was needed. RESULT: There was no late death and no replacement of the valve with prosthesis. During follow-up ranging from 1 to 4 years, none of the patients developed severe stenosis or progressive regurgitation in the left-sided atrioventricular valve. CONCLUSION: Meticulous surgical management of a double-orifice in the mitral valve in association with atrioventricular canal defect an achieve an acceptable midterm result without developing severe dysfunction in the left-sided atrioventricular valve.     

Double-orifice repair for left atrioventricular valve regurgitation in atrioventricular septal defect: report of two cases

We describe two patients who successfully underwent a surgically created double-orifice repair using the edge-to-edge repair for residual left atrioventricular valve (LAVV) regurgitation in an atrioventricular septal defects (AVSD). Both patients had previously received patch closure of the AVSD and partial closure of a cleft of the LAVV. Preoperatively, echocardiography showed a wide open cleft and remarkable dilatation of the LAVV annulus. Doppler study revealed severe regurgitation through the cleft and the central portion of the LAVV orifice and no intracardiac shunt. Postoperative echocardiography showed a remarkable decrease of the AV valve regurgitation to none or trivial levels without stenosis of the LAVV in both patients. Among several valve-sparing techniques, our experience suggests that the surgically created double-orifice repair is one of the most effective reparative procedures for LAVV regurgitation in AVSD.     

Surgically created double-orifice left atrioventricular valve: a valve-sparing repair in selected atrioventricular septal defects

OBJECTIVES: Some features of the left atrioventricular valve (large mural leaflet, dystrophic tissue) represent a challenge for repair of atrioventricular septal defects without postoperative regurgitation. A retrospective study was conducted to evaluate the results of surgically creating a double-orifice left atrioventricular valve in such circumstances. Clinical results were analyzed according to valvular and subvalvular left atrioventricular valve measurements in pathologic specimens with atrioventricular septal defects. METHODS: Among 157 patients operated on for atrioventricular septal defect since October 1989, 10 patients underwent primary repair (n = 8) or reoperation (n = 2) by this procedure. Median age at repair was 3.3 years (0.1-33 years). Anatomic types were complete (n = 3), intermediate (n = 5), and partial (n = 2). Preoperative moderate to severe left atrioventricular valve regurgitation was present in 6 patients. After the repair (two-patch technique in complete atrioventricular septal defect, cleft closed in each case), these 10 patients were found to have moderate to severe residual regurgitation not amenable to repair by annuloplasty. The top edge of the mural leaflet was anchored to the facing free edge of the cleft. RESULTS: No hospital death or morbidity was observed. Left atrioventricular valve regurgitation was absent or trivial (8 patients) and mild (2 patients). Color-coded echocardiography did not show significant left atrioventricular valve stenosis. The mean diastolic pressure gradient across the left atrioventricular valve was 3.2 +/- 1.1 mm Hg (1.4-4.5 mm Hg). At a median follow-up of 72 months (6-91 months), there was 1 late death, unrelated to left atrioventricular valve malfunction, due to pulmonary vascular obstructive disease. Left atrioventricular valve regurgitation did not increase over time, except in 1 patient in whom regurgitation recently progressed from mild to moderate. At rest, the mean diastolic pressure gradient across the left atrioventricular valve was 3.8 +/- 2.9 mm Hg (1.5-11.2 mm Hg). One child had an early moderate stenosis without pulmonary hypertension. Studies on pathologic specimens (n = 34) indicated that long chordal lengths and large mural leaflet size are essential independent anatomic features to assess its feasibility. CONCLUSIONS: Surgical creation of a double-orifice left atrioventricular valve is an effective additional procedure for repair of atypical cases of atrioventricular septal defect. The operation may decrease the need for reoperation or left atrioventricular valve replacement.     

Biventricular repair in children with atrioventricular septal defects and a small right ventricle: anatomic and surgical considerations

OBJECTIVE: We sought to characterize morphology and to evaluate a strategy of using biventricular repair in patients with a small right ventricle and an unbalanced atrioventricular septal defect. METHODS: Thirty-eight children with a small right ventricle and an atrioventricular septal defect underwent operative therapy. Thirty-two had biventricular repair, and 6 had single-ventricle palliation. A small right ventricle was defined on the basis of echocardiographic measurements expressed as the atrioventricular valve index (ie, right atrioventricular valve/left atrioventricular valve area) and as the ratio of right ventricular to left ventricular length. Validation of measurements in patients with a small right ventricle and an atrioventricular septal defect was achieved by comparison with balanced case-matched control subjects with atrioventricular septal defects. RESULTS: Children with a small right ventricle and an atrioventricular septal defect had a lower atrioventricular valve index (mean, 0.41 +/- 0.1 vs 0.52 +/- 0.1 [control], P < .0001) and lower right ventricular/left ventricular length ratio (0.78 +/- 0.1 vs 0.99 +/- 0.17 [control], P < .0001). Children with a small right ventricle undergoing single-ventricle palliation had the smallest right ventricular dimensions. There were 4 operative deaths. Three patients with a small right ventricle and an atrioventricular septal defect who underwent biventricular repair and had an atrioventricular valve index of less than 0.50 required early reoperation for right ventricular inadequacy. A restrictive atrial fenestration was created in 11 infants. Survival at 10 years was 87% for patients undergoing biventricular repair versus 100% for control subjects (P = .042). CONCLUSIONS: Extending the use of biventricular repair in properly selected patients with a small right ventricle and an atrioventricular septal defect is feasible. An atrial fenestration improves the physiologic tolerance to repair in selected patients. The use of biventricular repair in children with atrioventricular valve indexes of less than 0.50 requires caution.     

完全型房室间隔缺损人工瓣膜置换一例-站在“单心室循环共瓣成形或换瓣”分岔路口的思考

1例12岁女性患者因“左上腔静脉-肺动脉连接术后,完全型房室间隔缺损,右室双出口,肺动脉瓣狭窄,左异构,单心房,下腔静脉-半奇静脉引流”入院。术前超声显示共同房室瓣增厚、卷曲,对合不良,中大量反流,以左侧房室瓣为主;主肺动脉发育差。手术保留房室瓣,取31#二尖瓣机械瓣,以2/0 Prolene线连续缝合于二尖瓣瓣环。横断主肺动脉,5/0 Prolene线连续缝合近端,远端与20#Gore-tex人工血管端端吻合。将肝静脉自右房壁切下,与人工血管另一端吻合。术后血氧饱和度95%~100%之间。出院前复查超声、CT示人工瓣膜功能良好、心功能正常。虽然,共同房室瓣成形是瓣膜处理的首选,但当瓣膜功能障碍过于严重、成形很可能失败的情况下,勉强进行瓣膜成形术将使手术的风险骤然提升。此时,人工瓣膜的置换为最佳选择。     

Surgical repair of coronary sinus orifice atresia with persistent left superior vena cava in heterotaxia

A 6-month-old boy was diagnosed with coronary sinus orifice atresia, double-outlet right ventricle, complete atrioventricular septal defect, pulmonary stenosis, and moderate common atrioventricular valve regurgitation associated with heterotaxy syndrome. Cardiac venous flow drained through a persistent left superior vena cava. We decided to perform coronary sinus orifice unroofing through the right atrium under a guide using a bougie. The persistent left superior vena cava was divided. Bidirectional Glenn anastomosis and edge-to-edge common atrioventricular valve repair were concomitantly performed. After a 1-year follow-up period, the patient is alive and well without any ischemic event.