Late reoperation for systemic atrioventricular valve regurgitation after repair of congenital heart defects

Since 1979, 17 infants and children have undergone reoperation for systemic atrioventricular (AV) valve regurgitation 6 weeks to 7 years after repair of congenital heart defects. Prior operations were repair of incomplete or complete AV canal (14 patients), Mustard repair of complex transposition of the great arteries including ventricular septal defect closure (2 patients), or first-stage operation for hypoplastic left heart (1 patient). Age ranged from 6 months to 11 years. In 12 of the 17 patients (10, AV canal; 1, transposition; 1, hypoplastic left heart), valve reconstruction was possible. Operative techniques included a combination of septal cleft approximation, leaflet resection, commissural annuloplasty, or ring annuloplasty. There were no operative deaths, and there were no reoperations in the repair group. The condition of these patients has improved. Follow-up ranges from 1 month to 9 years (mean follow-up, 4.1 years). Five of the 17 patients (4, AV canal; 1, transposition) underwent valve replacement. There were no operative deaths. Follow-up ranges from 3 to 8 years. Three patients later underwent re-replacement of the prosthetic valve; there was 1 late death. The condition of all 4 survivors is improved. Substantial AV valve regurgitation can occur months or years after repair of congenital heart defects. A combination of reconstructive techniques may be useful in preserving native valve function and avoiding systemic AV valve replacement.     

Mitral valve replacement in the first year of life

From 1973 through 1987 25 patients underwent mitral valve replacement in the first year of life for mitral stenosis and mitral regurgitation. The patients with mitral stenosis included two with mitral arcade, two with supravalvular mitral stenosis with hypoplastic mitral valve, and one with parachute mitral valve. Included in the group of patients with mitral regurgitation were 12 with atrioventricular canal defect, six with chordal and leaflet defects, one with Marfan's syndrome, and one with bacterial endocarditis. Prostheses included 12 Bj?rk-Shiley (17 mm), seven St. Jude Medical (19 mm in four, 21 mm in three), five stent-mounted dura mater valves (12 mm to 16 mm), and one porcine xenograft (19 mm). In four patients the valves were placed in the left atrium in a supraannular location. There were nine operative (atrioventricular canal defect seven, mitral regurgitation two) and five late (atrioventricular canal defect four, mitral stenosis one) deaths, giving actuarial 1- and 5-year survival rates of 52% and 43%, respectively. All 6 patients with tissue valves died; the four with supraannular mitral valve replacement survived. Since 1983 operative mortality has been reduced to 0% (70% confidence limits 0% to 24%). Nine patients required a second mitral valve replacement for prosthetic stenosis 5 to 69 (mean 30) months after the original mitral valve replacement (one operative death). Because of improvements in repair of atrioventricular canal defect in infancy, the need for mitral valve replacement at atrioventricular canal defect repair has decreased. Although valvuloplasty has been advocated for repair of congenital mitral valve disease and is applicable in some infants with mitral regurgitation, mitral valve replacement is frequently unavoidable for congenital mitral disease and can now be accomplished at a low operative risk, even when the prosthesis has to be positioned supraannularly.     

Neochordal repair of the posterior mitral leaflet

BACKGROUND: Myxomatous mitral valve insufficiency is traditionally repaired by posterior leaflet quadrangular resection and reconstruction. A simplified repair technique without leaflet resection is described, and our initial experience is reviewed. METHODS: Thirty-nine consecutive patients with significant mitral regurgitation underwent repair since January 2000 by placement of expanded polytetrafluoroethylene sutures between the leading (coapting) edge of the posterior leaflet and the corresponding papillary muscle. An annuloplasty ring was placed, and no leaflet tissue was resected. Patient medical records were obtained and retrospectively reviewed. RESULTS: Twenty-five men and 14 women (median age, 61 years; range, 40-88 years) had their mitral valve repaired by a variety of surgical approaches, including robotic (18 patients), right thoracotomy (6 patients), and sternal (15 patients). Three patients have required valve replacement: 1 at the initial operation, 1 because of dehiscence of the annuloplasty ring, and 1 after subsequent rupture of a previously normal native chorda. At follow-up (median, 12 months), 92% (33/36) of the remaining patients had an intact mitral repair with no to mild regurgitation, 8.3% (3/36) of patients had moderate regurgitation, and 92% of all patients (36/39) were in New York Heart Association class I. There were no deaths. CONCLUSIONS: Myxomatous mitral regurgitation due to posterior leaflet insufficiency can be repaired without leaflet resection by placement of neochordae. This repair technique is effective and is readily accomplished by traditional and minimally invasive surgical approaches.     

Valvuloplasty with glutaraldehyde-treated autologous pericardium in patients with complex mitral valve pathology

BACKGROUND: Severe mitral regurgitation associated with complex mitral valve disease often precludes successful surgical repair. The feasibility and the results of valvuloplasty with glutaraldehyde-treated autologous pericardium remain largely unknown. METHODS: The cases of 63 patients who underwent operation within an 11-year period were studied. A pretreated autologous pericardial patch was used for leaflet extension plasty, for paracommissural plasty, as a substitute for part of the leaflet, and for reimplantation of ruptured papillary muscles to eliminate severe mitral regurgitation. Patients with a severely calcified annulus after en bloc decalcification had straddling endoventricular pericardial patch annuloplasty for reconstruction of the affected atrioventricular groove. Chordal replacement with a strip of pericardium was chosen if no suitable chordae were available. Pericardium-reinforced suture annuloplasty was used in patients with acute endocarditis resistant to medical therapy. Associated valvuloplasty procedures with Carpentier techniques were also employed. RESULTS: There were no operative deaths in this series. At a mean follow-up of 61.1 months (range, 4 to 132 months), mitral regurgitation was absent or trivial in 92.1% of patients by echocardiography. Freedom from reoperation was 95.2% at 1 year and 5 years. Thromboembolic events have not been detected. Thirty percent of patients returned to sinus rhythm. Two patients required valve replacement. CONCLUSIONS: Our beneficial results indicate that glutaraldehyde-treated autologous pericardium is suitable for valvuloplasty. It provides durable and predictable repair of valves that might otherwise need to be replaced because of the complex mitral valve disease. The technique is reliable, allows further efficacious repair possibilities, and improves postoperative outcomes. Whether it can prevent late deterioration and calcification requires more investigation.     

Surgical treatment of double-orifice mitral valve in atrioventricular canal defects. Experience in 25 patients

Double-orifice mitral valve is an uncommon but surgically important condition. The experience in 25 cases of double-orifice mitral valve associated with atrioventricular canal defects was reviewed. This constituted 4.3% of the 581 cases of atrioventricular canal defects operated upon between 1961 and July, 1984. The combined mitral orifice area ranged from 85% to 91% of normal in those patients whose valves were sized intraoperatively. Ten associated cardiac defects were repaired in six patients. Of 23 patients having cleft mitral valve, 21 had partial closure of the cleft. There was one operative death (4.0%), which occurred early in the series in a patient in whom the tissue bridge was severed and massive mitral regurgitation resulted. In the remaining 24 patients the tissue bridge was left intact, and all survived operation. No patient had clinically significant mitral stenosis during a follow-up of 1 to 14 years (mean 4.9 years). Two patients (8%) developed progressive mitral regurgitation and required mitral valve replacement 3 and 11 years postoperatively. One of these patients died and a second death occurred suddenly 2 years following operation. All survivors are in Functional Class I or II. The noncleft orifice of a double-orifice mitral valve usually is competent and rarely requires closure. The cleft, because it constitutes a type of parachute (single papillary muscle) valve, should be closed partially so as to relieve valve incompetence without causing undue stenosis. The incidence of late development of mitral regurgitation is similar to that of atrioventricular canal without double-orifice mitral valve. Repair of atrioventricular canal associated with double-orifice mitral valve can be achieved with a low operative mortality and excellent late results.     

Evaluation of long-term results of bicuspidalization annuloplasty for functional tricuspid regurgitation. A seventeen-year experience with 133 consecutive patients

Between 1968 and 1985, 133 consecutive patients underwent bicuspidalization annuloplasty for moderate to severe functional tricuspid regurgitation associated with mitral or combined mitral and aortic valve disease. Over this period, the incidence of tricuspid valve replacement was only 2.3% (3/136 patients). There were 18 early deaths (13.5%) in the entire series--three (5.0%) of 60 patients in the last 5 years of the study--and 10 late deaths (8.7%). Actuarial survival rate for the entire series, excluding early deaths, was 91.0% +/- 3.0% at 10 and 17 years. There were seven reoperations (6.1%) on the tricuspid valve, needed because of residual or recurrent mitral valve lesions after the initial operation. Actuarial rates of freedom from reoperation on the tricuspid valve were 93.6% +/- 3.0% (10 years) and 69.7% +/- 16% (17 years) for the entire series: 78% +/- 10% (15 years) for the open mitral commissurotomy plus tricuspid annuloplasty group (44 patients); 90% +/- 9.0% (15 years) for the mitral plus tricuspid annuloplasty group (10); 75.2% +/- 22% (17 years) for the mitral replacement plus tricuspid annuloplasty group (58); and 92.6% +/- 7.0% (16 years) for the combined aortic and mitral valve surgery plus tricuspid annuloplasty group (21). Ninety-eight percent of the survivors were in New York Heart Association class I or II postoperatively. Of 21 randomly selected patients investigated by pulsed Doppler echocardiography, 14 (67%) had no regurgitation or grade 1/4 tricuspid regurgitation and the remaining seven (33%) had grade 2/4 regurgitation postoperatively. Our experiences suggest that bicuspidalization annuloplasty can be a reliable method in the vast majority of patients with functional tricuspid regurgitation.     

Early and late results of partial plication annuloplasty for congenital mitral insufficiency

BACKGROUND: Partial plication annuloplasty is the main technique for congenital mitral insufficiency because this technique allows the mitral anulus to grow, in contrast to ring annuloplasty. However, this technique is not satisfactory for mitral insufficiency with some anomalies of the mitral valve apparatus. METHODS: Forty-one patients underwent partial plication annuloplasty for mitral regurgitation from July 1979 to December 1998. Mitral regurgitation associated with an atrioventricular defect, an atrioventricular discordance, and a univentricular heart was excluded from this study. RESULTS: There were no early or late deaths. In early results, partial plication annuloplasty was more effective for mitral regurgitation with abnormality of the posterior leaflet (n = 14) or normal leaflet motion (n = 8) than with abnormality of the anterior leaflet and its apparatus (n = 14) or absence of chordae (n = 4). The mean follow-up period was 145.8 months. During the follow-up period, 2 patients underwent mitral valve replacement, and a third patient underwent mitral valve repair with partial plication annuloplasty after the first repair. The main cause of mitral regurgitation of 2 of the 3 patients was absence of chordae. The actuarial freedom from reoperation rate was 94.9% +/- 3.6%, 91.9% +/- 4.7%, and 91.9% +/- 4.7% at 5, 10, and 15 years after the operation, respectively. CONCLUSION: Early and long-term results of partial plication annuloplasty were acceptable for congenital mitral insufficiency with any type of malformation of the mitral valve, and results were excellent with abnormality of the posterior leaflet and its apparatus or normal leaflet motion. However, late results were suboptimal for mitral regurgitation with absence of chordae. Other techniques, such as artificial chorda replacement, should be adapted in these cases.     

Surgery for aortic regurgitation in the young: repair versus replacement.

Aortic valve replacement in the young patient represents a serious problem in terms of anticoagulation and limited durability of the bioprosthesis. In order to determine the possible advantages of conservative surgery, consecutive patients with aortic regurgitation, aged below 35 and operated upon between July 1988 and July 1990 were analyzed. There were 149 patients (mean age 22 years). The aetiology was rheumatic disease in 122 (81.9%) and 83.2% of the patients were in NYHA class III-IV. Sixty-one valves were replaced (AVR) and 88 (59%) were repaired by a variety of techniques (annuloplasty, cusp unrolling, resuspension or extension). The mean ischaemic times for isolated aortic regurgitation were 73.6 mins for AVR and 80.5 mins for repair. Associated mitral surgery was performed in 94 patients (63%); 33% of these had a valve replacement and 67% a repair. In the AVR group there was 1 hospital death (2.2%), 2 late deaths (3.3%), 3 major embolic events (5%) and one reoperation for infection. In the repair group there were 2 hospital deaths (2.4%), but no late deaths or embolic events. Twelve patients required reoperation which was performed without mortality; this was due to aortic dysfunction in 4 cases (4.6%) and mitral dysfunction in 8. It is concluded that aortic valve conservation in a young, predominantly rheumatic, population can be performed in a significant number of patients with a low mortality and morbidity.     

Surgery for partial atrioventricular septal defect in the adult

BACKGROUND: We sought to determine the clinical profile, operative results, and long-term outcome for adult patients undergoing operations for partial atrioventricular septal defects. METHODS: Between 1976 and 1996, 50 adults (mean age, 36.6 +/- 13.2 years) underwent surgery for partial atrioventricular septal defects. Thirty-nine of them underwent primary repair for a substantial left-to-right shunt (> or =1.8), associated with symptoms in 29. The remaining 11 patients had previous atrioventricular septal defect repair in childhood, but required reoperation as adults for severe left atrioventricular valve regurgitation (6), subaortic (3) or mitral (1) stenosis, and a residual atrial shunt (1). RESULTS: No patients died in hospital. Of the 39 patients first repaired in adulthood, left atrioventricular valve repair was performed in 37, valve replacement in 1, and no repair in 1. In contrast, left atrioventricular valve replacement was necessary in 2 of the 6 adults undergoing reoperation for left atrioventricular valve regurgitation. At 7 years median follow-up, 8 patients have died (2 from noncardiac causes). Of 42 patients alive in 1997, 39 are New York Heart Association class I or II, and 3 were class III (class improved in 81%). Two patients required left atrioventricular valve replacement (1 week and 5 years after repair, respectively) for valvar failure. CONCLUSIONS: Low operative risk and excellent long-term results support repair of partial atrioventricular septal defect in adults.     

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.     

Long-term results of mitral valve repair for non-rheumatic mitral regurgitation

The durability of mitral valve repaired with reconstructive techniques is variable. If the durability continues to be good, mitral valve repair may be the procedure of choice in many patients with mitral regurgitation. Between December 1970 and June 1993, 54 patients had mitral valve repair for non-rheumatic mitral regurgitation. There were 38 men and 16 women with a mean age of 46.8 (range 19–68) years. The pathology which required surgical treatment was torn chordae in 38 patients, elongation of the chordae in five, valve prolapse without elongation or rupture of the chordae in six, infective endocarditis in three, and annular dilatation in two. Forty-four patients had triangular or quadrangular resection of the mitral leaflet, and seven had annuloplasty alone. Choral reconstruction was performed on three patients. There were no operative deaths. Five patients (9%) died late after operation. The actuarial survival rate and the valve-related death-free rate at 10 years were 83.9% and 90.0%, respectively. Seven patients (13%) required reoperation. Freedom from reoperation at 10 years was 84.5%. Improper evaluation of residual regurgitation during operation and suture dehiscence were the principal causes of reoperation. It was concluded that mitral valve repair for non-rheumatic mitral regurgitation showed low operative mortality and stable long-term results. It is suggested that intraoperative transoesophageal colour Doppler echocardiography provides accurate assessment of mitral valve competence and may be helpful in reducing the need for reoperation.     

66例部分性房室管畸形的外科治疗

目的总结部分性房室管畸形手术治疗的经验,以提高治疗效果。方法回顾性分析我院1984年1月－2007年12月经外科治疗部分性房室管畸形患者66例的临床资料,对二尖瓣大瓣裂的处理：单纯裂缺缝合52例,缝合加交界折叠缝缩8例,加小瓣成形1例,加缝置St．Jude软质人工成形环3例,人工机械瓣置换术2例;对原发孔型房间隔缺损的修补：采用涤纶补片修补12例,自体心包54例;采用Kirklin法将冠状静脉窦口隔入左心房5例,MeGoon法将冠状静脉窦口隔入右心房61例;同期处理其他合并畸形。结果术后早期死亡2例（3．03％）,1例死于心律失常,另1例死于呼吸功能衰竭。术后发生Ⅲ°房室传导阻滞2例,均安装永久性心脏起搏器。术后随访52例（81．3％）,随访时间5个月～22年,平均15年,心功能均有明显改善,尤其是术前心功能Ⅲ～Ⅳ级的19例患者,术后改善为Ⅰ～Ⅱ级。再次手术4例,其中1例经再次行二尖瓣置换术治愈;术后死亡3例,1例死于急性。肾功能衰竭,2例死于低心排血量综合征。结论早期手术治疗可以保全房室瓣结构、功能,避免发生肺动脉高压和降低死亡率。手术治疗的关键是消除二尖瓣关闭不全、修补原发孔型房间隔缺损和避免损伤传导组织,术后近、中期疗效良好;有残留中度以上二尖瓣反流者,远期效果不满意。     

Ebstein心脏畸形外科矫治39例

目的　总结Ebstein心脏畸形外科治疗经验。方法　回顾近 18年收治Ebstein畸形 39例 ,其中重型 8例 ,中间型 2 7例 ,轻型 4例。采用改良Danielson法矫正畸形 30例 ,其中 19例同时行瓣环成形 ,3例行瓣叶修补 ,6例行瓣交界成形 ;单纯三尖瓣成形 3例 ;Minale法修复 1例 ;双向上腔静脉—肺动脉连接和三尖瓣成形 1例 ;4例行三尖瓣置换。结果　 2例 (成形和换瓣各 1例 )术后早期死于右心衰竭(5 1% )。成形术后三尖瓣功能正常 2 7例 (77 1% ) ,轻至中度关闭不全 5例 (14 3 % ) ,中至重度关闭不全 3例 (8 6 % )。生存者随访 0 5～ 18年 ,平均 8 6年。 33例心功能I级 ,3例心功能II级 (成形 2例、瓣膜替换 1例 ) ,1例行人工瓣置换者术后顽固性右心衰竭 ,于 1年后失访。结论　Ebstein畸形应尽早外科治疗。修复成形多能矫正畸形 ,重症者可同时行双向腔—肺动脉连接术     

Corrective repair of complete atrioventricular canal defects and major associated cardiac anomalies

Twenty-nine patients with balanced forms of complete atrioventricular (AV) canal defects and tetralogy of Fallot in 12, double-outlet right ventricle (DORV) in 10, and additional anomalies associated with atrial isomerism in 7 underwent corrective repair. Age ranged from 39 days to 24 years (median, 3.7 years). Repair included reconstruction of the common AV valve in 25 patients and prosthetic valve replacement in 4. Construction of an intraventricular tunnel connecting the left ventricle with the aorta was accomplished in 23 of 25 patients with ventriculoarterial concordant or double-outlet connections. In 2 patients with situs inversus and associated DORV and in 4 patients in the isomerism group who had ventriculoarterial discordant connections, repair included intraatrial venous diversion by a modified Senning procedure in 2, a modified Mustard venous switch in 1, and a tailored atrial baffle in 3. A valved extracardiac conduit was required for repair in 4 patients. Partial anomalous systemic venous connection was repaired in 7 patients and total anomalous pulmonary venous connection, in 5. There was 1 hospital death (3%) and 3 late deaths (11%). There was 1 (3.6%) reoperation to replace an incompetent right (systemic) AV valve 17 months postoperatively, and each of the 25 surviving patients was in New York Heart Association Functional Class I (80%) or II (20%) at follow-up.     

Unusual mitral valve abnormalities complicating surgical repair of endocardial cushion defects

A review of 155 cases of surgically repaired endocardial cushion defects revealed 16 patients (10%) with additional unusual mitral valve abnormalities that complicated the surgical procedure. Eight patients had accessory mitral valve tissue that connected the anterior and posterior leaflets to form a double-orifice valve (Group I). In four (50%), the lesion was associated with intermediate atrioventricular canal and small left ventricle; all four died following repair. In the other four, it was associated with ostium primum defect; all survived and are well. A single papillary muscle in the left ventricle was present in six patients (Group II). Two had intermediate atrioventricular canal and both died postoperatively. The other four had complete endocardial cushion defect and three are well following the operation. Perforation of the valve leaflets was present in two patients with ostium primum (Group III). Both patients are well postoperatively. Modification of the surgical technique is required to effect satisfactory repair. The bridge connecting the posterior and anterior leaflets of the mitral valve should be left undisturbed. Otherwise, severe regurgitation may result. In patients with single papillary muscle and complete atrioventricular canal, repair may be accomplished by borrowing from the tricuspid portion of the anterior leaflet, rotating that part posteriorly, and partially closing the cleft. Small perforations of the mitral leaflet do not require closure and do not result in regurgitation. Echocardiographic and angiographic delineation of these abnormalities and thorough intraoperative exploration are important in avoiding pitfalls at the time of repair.     

Early experience with mitral valve reconstruction for mitral insufficiency

Mitral valve repair for mitral regurgitation has been reported to have more favorable early and late results than mitral valve replacement. From July 1985 through July 1990, 63 patients have undergone valve repair at Good Samaritan Hospital. Twenty-two men and 41 women whose ages ranged from 34 to 81 years (mean 67.9 years) were treated. Twenty-eight patients were in New York Heart Association functional class III or IV. Twelve (19%) had undergone prior cardiac surgery. Isolated valve repair was performed in 18 patients. Valve repair was combined with coronary artery bypass grafting, other valve procedures, or aneurysm resection in the remainder (71%). Two patients (3%) died while in the hospital, and four deaths (one valve-related) occurred after discharge. Leaflet resection for ruptured chordae was done in 24 patients (38%), chordal shortening in 5 patients (8%), and leaflet transposition in 2 patients. Rigid ring annuloplasty (Carpentier) was performed in 62 patients. Eight patients required mitral valve replacement at the same operation because of unsatisfactory valve repair. Results of valve repair evaluated by echocardiography at discharge show that 48 patients (88%) are free of significant regurgitation. Follow-up to date reveals that all surviving patients who underwent valve repair have clinically improved and are stable. Four of five patients with moderate mitral regurgitation are currently asymptomatic. There have been two valve-related late failures requiring reoperation. Based on this early experience, we conclude that valve repair compared with mitral valve replacement has a low operative mortality with good early results. Continued efforts to preserve native mitral valve function in the presence of mitral regurgitation appear justified.     

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.     

Management of the left atrioventricular valve in the repair of complete atrioventricular septal defects.

Left atrioventricular valve regurgitation in atrioventricular canal defects is usually due to malalignment of the edges of the cleft or to annular dilatation. Intraoperative assessment and correction of left atrioventricular valve incompetence is critical for successful outcome in the surgical management of complete atrioventricular canal defects. Although some have elected not to suture the cleft in the setting of minimal incompetence, we have found that this often results in significant left atrioventricular valve insufficiency, necessitating reoperation. From January 1982 through December 1990, 105 patients with complete atrioventricular canal underwent definitive repair. Repair was performed with a single pericardial patch technique in 86 patients (82%). Intraoperative assessment of left atrioventricular valve competence was performed in all cases. Ninety-six patients (91%) required suturing of the cleft and 63 (60%) required annuloplasty to establish satisfactory competence of the left atrioventricular valve. The overall early mortality rate was 10.5% (11/105 patients). From 1986 to 1990, the early mortality rate decreased to 7.7% (6/78 patients). In a mean follow-up of 39 months (range 1 to 106 months), late survival was 96% (90/94 operative or early survivors). Reoperation was performed on eleven (11.5%) patients; six (6.3%) for failure of the atrioventricular valve repair, three for patch dehiscence, and two for residual ventricular septal defects. These data demonstrate that routine approximation of the cleft and aggressive use of left atrioventricular valve annuloplasty is safe and results in an excellent outcome with a low incidence of reoperation for failure of left atrioventricular valve repair.     

Experience with Repair of Complete Atrioventricular Canal

Twenty-nine patients at our institution have undergone repair of complete atrioventricular canal since 1969. There were 3 operative and 5 late deaths, 4 of which were of infectious etiology. Age at operation ranged from 2 months to 12 years (mean, 50 months). Weight ranged from 3.6 kg to 30 kg (mean, 12 kg). Before repair, catheterization studies revealed pulmonary hypertension in all patients with unobstructed pulmonary arteries. Pulmonary to systemic flow ratio ranged from 1.5 to 10.6 (mean, 3.5). Pulmonary vascular resistance (PVR) ranged from 0.7 to 21.7 (mean, 5.3) Wood units/m². At repair, 14 patients had Rastelli type A anatomy, 14 had type C, and 1 patient had a variant with crossing chordae and double-outlet right ventricle (DORV).Postoperative catheterization has been done in 16 patients 2 months to 8 years (mean, 30 months) after repair. One patient had residual ventricular shunting and later underwent successful repair. A 2-year-old patient had severe mitral regurgitation and died following mitral valve replacement. One patient required a permanent pacemaker. The 21 surviving patients have been followed from 7 months to 10 years 7 months and have excellent hemodynamic status. Long-term studies are needed to assess the ultimate effect on patients with high PVR.     

Surgical repair of complete atrioventricular septal defect.

BACKGROUND: The objective of this study was to assess the outcome of complete atrioventricular septal defect repair from 1981 to 2000. METHODS: One hundred seventy-two consecutive patients with atrioventricular septal defect were operated on by a single surgeon using a consistent operative technique (single patch; "cleft" closure). The patients' age range was from 5 weeks to 9 years (mean, 10.8 +/- 1.2 months). RESULTS: Overall operative mortality was 15 of 172 (8.7%) and this decreased significantly from 12 of 73 (16.4%) in the first decade to 3 of 99 (3.0%) in the second decade (p = 0.0021) with no operative deaths in the last 51 patients. Operative mortality was related to decade of operation (p = 0.0021) and to use of crystalloid cardioplegia (p = 0.0047) by univariate analysis, and to decade of operation (p = 0.0016) and postoperative time on ventilator (p = 0.0023) by multivariate analysis. Actuarial long-term survival including operative deaths was 79.0% +/- 3.8% at 15 years. Ten of 157 (6.4%) operative survivors have undergone reoperation for late mitral regurgitation (9 mitral valve repair, 1 mitral valve replacement) with one death. Four of 8 patients surviving late mitral valve replacement have subsequently required mitral valve repair. Freedom from late reoperation for severe mitral regurgitation was 89.9% +/- 3.1% at 15 years. Freedom from late reoperation for mitral regurgitation did not decrease in the second decade (84.2% +/- 6.6% at 10 years) versus the first decade (94.5% +/- 3.1%) (p = 0.0679). CONCLUSIONS: Although operative mortality for repair of atrioventricular septal defect has decreased dramatically during the past decade, the incidence of late reoperation for mitral regurgitation has not improved, and better techniques to eliminate late mitral regurgitation are needed.