Complete atrioventricular septal defect repair: Simplified single patch technique

Background: There has been a rekindling of interest in alternatives to conventional two patch technique for the repair of complete atrioventricular septal defect in infancy in the recent past. We applied the simplified single patch technique to 15 consecutive infants and herein report our intermediate term results. Methods: Between March 1998 and September 2001, fifteen patients underwent repair of complete atrioventricular septal defect with this technique (mean age 6 months, mean weight 5.4 kg). Downs syndrome was present in 11 patients. Repair was done in all patients by direct suturing of the common atrioventricular valve leaflets to the crest of the ventricular septum irrespective of the size of the ventricular septal component. The cleft in the anterior mitral leaflet was closed in all patients. The atrial septal component was closed by a pericardial patch. Results: There was no mortality. There were no pulmonary arterial hypertensive crises or heart block. The mean follow up was 13.2 months. One patient underwent mitral valve replacement after one year due to severe mitral regurgitation. The remaining fourteen patients had no significant mitral regurgitation, residual ventricular septal defect or left ventricular outflow tract obstruction on echocardiography. Conclusion: Simplified single patch technique is an easily reproducible method for surgical repair of complete atrioventricular septal defect. It is less time consuming and minimises ischaemic time. Atrioventricular valve function is preserved and there is no incidence of obstruction to left ventricular outflow tract. The intermediate term results are encouraging. Presented in the poster session of the 37^th Annual Meeting of Association for European Paediatric Cardiology (AEPC) at Porto, Portugal, May 2002     

Surgical anatomy of left ventricular outflow tract obstruction in complete atrioventricular septal defect. A concept for operative repair

An integral part of the heart with an atrioventricular septal defect and a Rastelli type A valve configuration is left ventricular outflow tract obstruction. Current surgical techniques do not cater to this particular anatomic facet, and left ventricular outflow tract obstruction has been reported as a postoperative problem. The present study has focused on the surgical anatomy of the mode of attachment of the left superior atrioventricular valve and its relationship to the left ventricular outflow tract. It appeared that the anchoring of the superior leaflet was a major factor in limiting the excursions of the superior leaflet, contributing also to the tightness of the subaortic left ventricular outflow channel. On that basis a surgical repair is proposed in which the greater part of the tightly bound superior leaflet is detached from the septal crest, so that the left ventricular outflow tract is widened.     

A novel technique for the repair of complete atrioventricular septal defects: simplified 2-patch technique

Today complete atrioventricular septal defects can be corrected with acceptable mortality and postoperative morbidity. Although opinions still differ regarding the most appropriate method of choice, some surgeons have proposed a new technique that involves the direct suturing of common atrioventricular valve leaflets to the crest of the ventricular septum. However, we believe that this new approach may be associated with left ventricular outflow tract obstruction and neomitral valve regurgitation. To avoid these potential complications, we decided to modify our repair technique in 2 patients. In this simplified 2-patch technique, the operation was done by direct suturing of postero-inferior common bridging leaflet to the ventricular crest while the space under the antero-superior bridging leaflet was closed with a small triangular shaped Dacron patch. No echocardiographic evidence of neomitral valve regurgitation and left ventricular outflow tract obstruction were detected in either early or late postoperative examinations of these patients.     

Simplified single patch technique for the repair of atrioventricular septal defect.

OBJECTIVE: Because of the complexity of traditional 1- and 2-patch techniques for the repair of complete atrioventricular septal defect, we modified our repair technique to avoid the use of any ventricular septal patch material. We report our prospective experience with this simplified 1-patch technique. METHOD: Forty-seven consecutive patients between May 1995 and August 1998 underwent repair with the use of this technique without modification. Repair was done in all patients by direct suturing of the common atrioventricular valve leaflets to the crest of the ventricular septum. No division of valve leaflets was necessary. A single pericardial patch was used to close the defect in the atrial septal component. Follow-up included electrocardiography and echocardiographic assessment of ventricular function, atrioventricular valve function, and adequacy of the left ventricular outflow tract. RESULTS: There were 2 deaths (4%), only 1 cardiac related, in the series. There were 17 male patients and 30 female patients. Mean age at repair was 5.6 months (median, 3.4 months). Associated lesions were repaired in 19 patients (40%). Mean follow-up was 1.85 years (median, 1.9 years). There was no heart block. There were no significant residual ventricular septal defects detected and no left ventricular outflow tract obstruction seen on echocardiography in any patient to date. Mitral valve status after operation was assessed as no incompetence in 13 patients (28%), minimal in 19 patients (40%), mild in 12 patients (26%), and moderate in 3 patients (6%). CONCLUSION: The repair of complete atrioventricular septal defect by direct suturing of the atrioventricular valve leaflets to the crest of the ventricular septum with a single-patch technique greatly simplifies the repair and does not lead to left ventricular outflow tract obstruction nor interfere with valve function.     

Modified Konno procedure for a child with left ventricular outflow tract obstruction after repair of atrioventricular septal defect: a transaortic transpulmonary approach

A one-year-old infant underwent repair of atrioventricular septal defect with common orifice. About 2 years later, echocardiography revealed a left ventricular outflow tract obstruction for the first time. Because of progression of the obstructive lesion, a modified Konno procedure through a transaortic transpulmonary approach was later performed at 8 years old, and the postoperative course was uneventful. This is a useful procedure for left ventricular outflow tract obstruction with normal aortic valve and aortic annulus, because it can both preserve a native aortic valve and dose not necessitate right ventriculotomy in resection of hypertrophied muscle and patch enlargement of interventricular septum.     

Left ventricular outflow obstruction after Fontan procedure involving a patient with complete atrioventricular septal defects complicated by a small left ventricle

We report a 1-year-old girl who developed ventricular outflow tract obstruction early after a Fontan operation, necessitating surgical relief using the Damus-Kaye-Stansel procedure. The patient had a complete atrioventricular septal defect complicated by a muscular ventricular septal defect (VSD) and a small left ventricle, a morphology that has not previously been reported in cases of systemic outflow tract obstruction after the Fontan procedure. Postoperative systemic outflow obstruction must be considered as a possible sequela following Fontan surgery in patients with an atrioventricular septal defect and a small left ventricle.     

Secondary left atrioventricular valve replacement in atrioventricular septal (AV canal) defect: a method to avoid left ventricular outflow tract obstruction

Patients with atrioventricular septal defects are morphologically predisposed to subaortic obstruction. Some individuals require secondary left atrioventricular valve replacement for severe incompetence persisting after repair, and they are especially susceptible to left ventricular outflow tract obstruction. A surgical technique is described for replacement of the atrioventricular valve which seems to avoid the complication of left ventricular outflow tract obstruction by the prosthesis.     

Repair of complete atrioventricular septal defect with tetralogy of Fallot

Repair of complete atrioventricular canal with tetralogy of Fallot was performed in 9 patients. Ventricular septal defect was closed through the right atrium using a single polytetrafluoroethylene patch with ample anterior extension to avoid subaortic obstruction. The atrial septal defect was closed with a separate patch. Undivided atrioventricular valve leaflets were sandwiched between the two patches. Right ventricular outflow tract stenosis was relieved by pulmonary valvotomy and an infundibular patch in 7, a supravalvar patch (none transannular) in 6, and right ventricle-to-pulmonary artery conduit in 2. There was one hospital death (1/9, 11%) in a patient with persistent clinically significant postoperative pulmonary stenosis and low cardiac output requiring reoperation and right ventricle-to-pulmonary artery conduit insertion. There was no late mortality. All patients are asymptomatic 0.3 to 5.6 years after operation. Follow-up right ventricular outflow tract gradient ranged from 11 to 43 mm Hg and was 70 mm Hg in 1 patient who later had successful relief of obstruction. Three patients had mitral valve insufficiency; 1 needed reoperation. Aggressive relief of right ventricular outflow tract stenosis with maintenance of pulmonary valve competence and use of two separate patches for closure of the septal defects contribute to optimum immediate and long-term results after repair of this lesion.     

完全性房室间隔缺损合并法乐四联症的手术矫正

目的　报告完全性房室间隔缺损合并法乐四联症的外科治疗。　方法　 6例患者 ,年龄 3～ 9岁 ,均施行手术根治 ,经右房 右室切口修补VSD ,房室间隔缺损采用两块补片技术修复 ,左房室瓣裂隙的修复均采用间断缝合 ,右室流出道均用带单瓣的补片跨瓣加宽。　结果　全组 6例术后出现低心排综合征、多器官衰竭、灌注肺并发症各 1例 ,早期死亡 1例为多器官衰竭者 ,5例长期存活。术后随访 6个月～ 5 5年 ,1例有轻度左房室瓣关闭不全。心功能Ⅰ级 4例 ,Ⅱ级 1例。　结论　完全性房室间隔缺损合并法乐四联症可应用 2块补片法行手术根治 ,左房室瓣瓣裂的常规修补可降低其关闭不全的发生率 ,大部分患者术后心功能可获得良好的改善。     

Intraoperative evaluation of atrioventricular septal defect repair by color flow mapping echocardiography

Despite improving survival rates after repair of atrioventricular septal defect, many patients require reoperation because of postoperative heart failure. We used intraoperative color flow mapping echocardiography to assess the results of surgical repair of atrioventricular septal defect in 19 consecutive patients and compared those findings with results three to five days and 3 to 11 months after repair. There was close correlation between intraoperative and postoperative color flow mapping echocardiography in estimating the presence and severity of left atrioventricular valve regurgitation. All patients survived surgical repair, but in 4 (21%), postoperative congestive heart failure due to left atrioventricular valve regurgitation developed. The need for reoperation was significantly correlated with the severity of left atrioventricular valve regurgitation (r = 0.68) as estimated by intraoperative echocardiography and preoperative aberrancies in the atrioventricular valve (r = 0.68). Age, weight, additional congenital heart disease, preoperative pulmonary vascular resistance, preoperative atrioventricular valve regurgitation, and postoperative mean pulmonary arterial or left atrial pressure were not significantly correlated with the need for reoperation. Intraoperative color flow mapping echocardiography can accurately predict the development of early postoperative heart failure and subsequent reoperation after surgical repair of atrioventricular septal defect.     

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.     

A novel repair for patients with atrioventricular septal defect requiring reoperation for left atrioventricular valve regurgitation.

OBJECTIVE: Left atrioventricular valve regurgitation (LAVVR) is the most frequent indication for reoperation following atrioventricular septal defect (AVSD) repair. We estimate from our experience that within 10 years of initial repair, 14% of patients undergoing repair of atrioventricular septal defect (AVSD) require reoperation for this complication. We have developed a novel leaflet augmentation technique for LAVVR which may avoid failure of conventional repair and/or the need for valve replacement. METHOD: The novel technique consists of insertion of a glutaraldehyde-treated autologous pericardial patch to augment the bridging leaflets of the atrioventricular valve. We describe the outcome of eight patients in whom this technique was used and compared them to 68 other patients with AVSD undergoing reoperation for LAVVR by either conventional repair (n=54) or valve replacement (n=14). RESULTS: There were no early deaths or major complications following patch repair. The mean follow-up is 2.3 years (range 1-8.5 years) during which there were no late deaths. Two patients underwent reintervention at 3.5 and 5 years after patch repair for LAVVR and were successfully rerepaired. Mild residual LAVVR was seen at last echocardiography in six patients and mild to moderate in two. These results compare favorably with the 68 patients who underwent conventional surgery. The 3-year freedom from reoperation was 86% for both repair groups. Dysplastic valve tissue appears to be a major risk factor for failure of conventional repair or for valve replacement. Failure of conventional valve repair led to valve replacement in six of seven patients. CONCLUSIONS: For patients with late LAVVR after AVSD repair, pericardial leaflet augmentation is durable and may avoid failure of conventional repair or valve replacement in patients with dysplastic valves.     

Transatrial relief of diffuse subaortic stenosis after ventricular septal defect closure

Transatrial enlargement of the left ventricular outflow tract for serious obstruction was performed in 3 patients with previous ventricular septal defect closure. Two patients had recurrent subaortic stenosis as resection had already been performed at initial operation. In all patients, the obstruction was located below the ventricular septal defect patch. Patch enlargement of the left ventricular outflow tract was carried out by opening the ventricular septal defect patch through the tricuspid valve and extending the incision downward through the area of obstruction and the left ventricular body. All patients had uneventful postoperative course and effective relief of left ventricular outflow tract obstruction. We feel that the approach is simple and effective; it avoids a right ventriculotomy and provides a viable option in certain patients with left ventricular outflow tract obstruction.     

Direct closure of ostium primum defect in the repair of atrioventricular septal defect

BACKGROUND: Patch closure is generally performed for atrial septation of an atrioventricular septal defect. We recently developed a new surgical technique for repairing atrioventricular septal defects that avoids the use of any patch material for closing the atrial septal defect. We report our experience with this procedure. METHODS: Seven patients (complete type: 5, partial type: 2) underwent this new operation. The diameters of the atrial septal defects were measured by transesophageal echocardiography. The preoperative electrocardiograms were compared with those taken after the operations. RESULTS: Diameters of the atrial defects ranged from 3 to 10 mm. Electrocardiograms before and after the operations did not change. No significant atrioventricular valve regurgitation and no residual shunts were detected by postoperative echocardiography. CONCLUSIONS: This method simplifies the repair of atrioventricular septal defects. In the short-term results, no arrhythmia and no valve regurgitation was seen.     

The role of aortic translocation in the management of complex transposition of the great arteries

The surgical management of transposition of the great arteries with a ventricular septal defect and left ventricular outflow tract obstruction still remains a significant challenge despite major advances in pediatric cardiovascular surgery. Almost four decades after its inception, the Rastelli procedure continues to be the standard surgical technique used in the management of these patients, despite less than optimal long-term results. Aortic translocation with biventricular outflow tract reconstruction is an alternative surgical technique that creates anatomically sound connections between the ventricles and their respective great arteries, which could result in better long-term outcomes. This technique is especially useful in patients with transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction who have an inlet or restrictive ventricular septal defect, a small right ventricle, a straddling atrioventricular valve, or an aberrant coronary artery crossing the right ventricular outflow tract.     

Complete atrioventricular canal and tetralogy of Fallot: surgical management

Between 1982 and 1989, nine patients with complete atrioventricular (AV) canal and tetralogy of Fallot underwent successful repair of both anomalies. Seven patients had Down's syndrome. One or more previous palliative shunts had been used in six patients. Associated cardiovascular lesions included persistent left superior vena cava (two patients), patent ductus arteriosus with marked stenosis of the left pulmonary artery (one patient). The diagnosis was confirmed by cardiac catheterization and cineangiography. Closure of the ventricular septal defect was performed through a combined right atrial and ventricular approach in each patient. The right ventricular outflow obstruction was relieved with the use of a transannular patch (4), right ventricular infundibular patch (4) or a valved external conduit (1). There were no hospital deaths. One patient died as a result of meningitis 2 years after repair. Progressive left AV valve incompetence developed in one patient requiring valve replacement. A single atrial approach may not provide the best exposure of the subaortic end of the defect. In this regard, we recommend an additional right ventriculotomy in order to ensure safe closure of the septal defect. Although an adequately shaped isolated patch could be sufficient to close the anterior VSD, we favour a separate patch to minimize the possibility of producing subaortic obstruction. A further method to avoid this subaortic stenosis consists of dividing the common anterior leaflet to the right towards the lateral extent of the infundibular septum.     

Systolic anterior motion of the mitral valve despite the sliding leaflet technique for repair of the mitral valve

We report a systolic anterior motion of the anterior mitral leaflet despite employing the sliding leaflet technique for repair of mitral valve regurgitation. A 65-year-old man with chronic, symptomatic mitral regurgitation due to ruptured chordae tendineae underwent mitral valve repair by quadrangular resection of the posterior leaflet and sliding leaflet technique with ring annuloplasty. After weaning from cardiopulmonary bypass, left ventricular outflow obstruction developed and transesophageal echocardiography demonstrated systolic anterior motion of the mitral valve and severe mitral regurgitation. Non-operative treatment resolved the outflow tract obstruction, systolic anterior motion and mitral regurgitation. We conclude that post-repair systolic anterior motion can still occur after the sliding plasty procedure and that medical treatment can successfully resolve systolic anterior motion and outflow tract obstruction in most patients.     

Simplified technique for patch augmentation and chorda reconstruction of left atrioventricular valve in complete atrioventricular septal defect

Management of the left AV valve is the most crucial component of the repair of complete atrioventricular septal defect (cAVSD). A scarcity or deficiency of leaflet tissue may compromise satisfactory repair in a small number of patients with cAVSD, especially in patients with a normal karyotype. We describe the case of a 44-day-old baby who had cAVSD with severe left atrioventricular valve regurgitation due to dysplastic bridging leaflets. The repair was successfully performed by augmenting leaflet tissue and reconstructing the chorda using single patch. This technique could be one of the options in the repair of valves in order to avoid valve replacement in a small infant, although material of patch and reoperation need to be considered.     

Nonobstructing Accessory Mitral Valve Tissue and Ventricular Septal Defect

A 4-month-old boy with ventricular septal defect was found to have accessory mitral valve tissue attached to the anterior leaflet of the mitral valve. Operation was successfully performed to excise the accessory mitral tissue in the left ventricular outflow tract and close the ventricular septal defect. Most previously reported cases with accessory mitral valve tissue were associated with left ventricular outflow tract obstruction. This boy had no pressure gradient across the left ventricular outflow tract. The indications for prophylactic excision of nonobstructing accessory mitral valve tissue in a patient with other forms of congenital cardiac disease are discussed.     

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.