先天性心脏病术中心脏瓣膜的保护和矫治

目的 总结先天性心脏病术后因瓣膜功能不全再手术的经验。方法 回顾分析先天性心脏病术后再行瓣膜手术13例患者的临床资料,其中室间隔缺损修补术后8例,部分心内膜垫缺损修补术后3例,法洛四联症和房间隔缺损修补术后各1例。第1次手术时即存在二尖瓣轻～中度关闭不全6例,主动脉瓣关闭不全1例;新出现瓣膜功能异常6例,其中2例因补片漏致三尖瓣关闭不全,2例因前叶腱索断裂致三尖瓣关闭不全,1例因残留右心室流出道狭窄继发三尖瓣关闭不全,1例因伤及主动脉瓣并发二尖瓣和三尖瓣关闭不全。13例中,行二尖瓣置换6例,三尖瓣置换2例,主动脉瓣置换1例,行主动脉瓣置换并二尖瓣、三尖瓣成形1例,三尖瓣成形3例。同时修补残余漏,疏通右心室流出道。结果 术后发生低心排综合征3例。2例术后早期分别死于脑气栓和呼吸循环衰竭。11例术后痊愈出院,随访1～8年,心功能良好。结论 先天性心脏病矫治术中应注意心脏瓣膜的保护,合并的瓣膜功能异常应积极修补,及时地再手术可取得良好效果。     

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.     

Mechanical Aortic Valve Replacement in Children and Adolescents After Previous Repair of Congenital Heart Disease

Due to improved outcome after surgery for congenital heart defects, children, adolescents, and grown‐ups with congenital heart defects become an increasing population. In order to evaluate operative risk and early outcome after mechanical aortic valve replacement (AVR) in this population, we reviewed patients who underwent previous repair of congenital heart defects. Between July 2002 and November 2008, 15 (10 male and 5 female) consecutive patients (mean age 14.5 ± 10.5 years) underwent mechanical AVR. Hemodynamic indications for AVR were aortic stenosis in four (27%), aortic insufficiency in eight (53%), and mixed disease in three (20%) after previous repair of congenital heart defects. All patients had undergone one or more previous cardiovascular operations due to any congenital heart disease. Concomitant cardiac procedures were performed in all of them. In addition to AVR, in two patients, a mitral valve exchange was performed. One patient received a right ventricle‐pulmonary artery conduit replacement as concomitant procedure. The mean size of implanted valves was 23 mm (range 17–29 mm). There were neither early deaths nor late mortality until December 2008. Reoperations were necessary in five (33%) and included implantation of a permanent pacemaker due to complete atrioventricular block in two (15%), mitral valve replacement with a mechanical prosthesis due to moderate to severe mitral regurgitation in one (7%), aortocoronary bypass grafting due to stenosis of a coronary artery in one (7%), and in one (7%), a redo subaortic stenosis resection was performed because of a secondary subaortic stenosis. At the latest clinical evaluation, all patients were in good clinical condition without a pathological increased gradient across the aortic valve prosthesis or paravalvular leakage in echocardiography. Mechanical AVR has excellent results in patients after previous repair of congenital heart defects in childhood, even in combination with complex concomitant procedures. Previous operations do not significantly affect postoperative outcome.     

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.     

Management of Systemic Atrioventricular Valve Regurgitation in Infants and Children

A bstract Since September 1979, 53 patients have required operation for systemic atrioven-tricular valve regurgitation at Children's Hospital and Health Center of San Diego. (Primary repairs of atrioventricular canal defects are excluded from this report.) Diagnoses include single ventricle, cardiomyopathy, congenital mitral insufficiency, Marfart's disease, rheumatic heart disease, and a history of prior repair of atrioventricular canal defect. Ages ranged from 4 months to 19 years; median age is 5 years. In 31 patients, the atrioventricular valve could be repaired. In 24 patients, the valve was replaced (including two patients previously repaired). There were four operative deaths, all in the valve replacement group: three following valve replacement, and one following emergency thrombectomy. Two early failures in the repair group required valve replacement. Techniques for repair included leaflet resection, commissural annuloplasty, ring annuloplasty, and chordal shortening. Follow-up reveals good-to-excellent status in 38 patients. There were seven late deaths: six following valve replacement (one death valve related). Current surgical technique permits repair of the systemic atrioventricular valve in many infants and children requiring operation for regurgitation. The long-term results of valve repair are good to excellent. Repair avoids the morbidity and mortality of valve replacement, e.g., anticoagulation, fixed orifice size, and catastrophic mechanical valve malfunction. ( J Card Surg 1993; 8:612–621 )     

Surgical treatment of infective valve endocarditis in children with congenital heart disease

Abstract Objective: This study assesses surgical procedures, operative outcome, and early and intermediate‐term results of infective valve endocarditis in children with congenital heart disease. Methods: Seven consecutive children (five females, two males; mean age, 10.8 years) who underwent surgery for infective valve endocarditis between 2006 and 2010 were included in the study. The aortic and mitral valves were affected in two and tricuspid in five patients. Indications for operation included cardiac failure due to atrioventricular septal rupture, severe tricuspid valve insufficiency, and septic embolization in one, moderate valvular dysfunction with vegetations in three (two tricuspid, one mitral), and severe valvular dysfunction with vegetations in the other three patients (two tricuspid, one mitral). The pathological microorganism was identified in five patients. Tricuspid valve repair was performed with ventricular septal defect (VSD) closure in five patients. Two patients required mitral valve repair including one with additional aortic valve replacement. Results: There were no operative deaths. Actuarial freedom from recurrent infection at one and three years was 100%. Early echocardiographic follow‐up showed four patients to have mild atrioventricular valve regurgitation (three tricuspid and one mitral) and three had no valvular regurgitation. No leakage from the VSD closure or any valvular stenosis was detected postoperatively. Conclusions: Mitral and tricuspid valve repairs can be performed with low morbidity/mortality rates and satisfactory intermediate‐term results in children with infective valve endocarditis . (J Card Surg 2012;27:93‐98)     

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.     

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.     

Mitral valve replacement in the first 5 years of life

Between 1976 and 1986, 19 children aged 1 month to 5 years underwent replacement of the mitral (systemic atrioventricular) valve. Indications for valve replacement included isolated congenital mitral stenosis (n = 2), valve dysfunction associated with a more complex procedure (n = 15), and failed valvuloplasty (n = 2). Seven different valve types were used; nine were mechanical valves and ten were bioprosthetic valves. There were 6 hospital deaths (32%; 70% confidence limits, 20% to 47%). Among the 13 survivors there were 3 late deaths at a mean of 14 months after operation. The late deaths were unrelated to valve malfunction. Thromboembolic events occurred in 2 patients, both with mechanical valves. One minor bleeding complication occurred among 10 patients on a regimen of Coumadin (crystalline warfarin sodium). Five patients, all with bioprostheses, required a second valve replacement. Indications for reoperation included prosthetic valve regurgitation (n = 1) and calcific stenosis (n = 4). No early or late deaths occurred after second valve replacement. Survival was 51% +/- 12% (standard error) at 112 months after valve replacement. Analysis failed to identify age, weight, sex, previous operation, underlying cardiac lesion, or prosthesis size and type as significant risk factors for mortality. Mechanical valves had a lower reoperation rate compared with bioprostheses. These data suggest that although mitral valve replacement within the first 5 years of life is associated with a high operative and late mortality, satisfactory long-term palliation for many patients can be achieved. Mechanical valves are superior to bioprosthetic valves, and offer the best long-term results.     

Diagnosis and management of the double orifice mitral valve: three case reports

Double orifice mitral valve (DOMV) is an uncommon congenital heart defect. The isolated occurrence of this anomaly is very rare and, more often, is associated with another congenital malformation, dominated by atrioventricular canal defects (AVCD). Mitral insufficiency and/or stenosis may complicate this malformation. Treatment may be summarized as abstention, surgical valve repair, or valve replacement. In here, we report three cases with DOMV. The first patient was an 18-month-old boy who was operated for severe mitral valve stenosis and mild insufficiency (in 1980s), the second was a 47-year-old female, who was operated recently for mitral valve replacement (MVR) under cardiopulmonary bypass due to severe mitral valve insufficiency. This patient had been operated due to the secundum-type atrial septal defect in our institution previously. The last one, a 3-year-old boy, with DOMV and three papillary muscles, was on clinical follow-up because he had no symptoms.     

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.     

Long-term results after surgical repair of incomplete endocardial cushion defects

Since 1964, 56 children (mean age, 6.7 years) underwent repair of incomplete endocardial cushion defects. Forty patients had isolated ostium primum defects. Additional congenital defects were present in 17 patients (30%). All patients underwent patch closure of the ostium primum defect and 47 of 56 patients (84%) underwent mitral valvuloplasty. Hospital mortality was 1.8% (one death). Arrhythmias developed in 7 other patients in the early postoperative period, of which six were transient and resolved completely. One patient required early pacemaker placement for complete heart block. Cumulative follow-up was 378 patient-years. There were three late deaths (5.7%), and additional operations were required in 12 patients (22.6%). Seven of these 12 patients required mitral valve replacement for severe mitral regurgitation. The mean interval between initial repair and mitral valve replacement was 4.2 years, with only three valves needing replacement within 12 months. There was a significant correlation between the severity of mitral regurgitation before initial repair and subsequent need for mitral valve replacement. Late onset atrial arrhythmias have developed in 6 patients. Current functional status has been evaluated in 50 of 52 surviving patients and 88.5% are in NYHA class I, with the remainder in class II.     

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.     

"缘对缘"瓣膜成形术在先天性心脏病合并重度三尖瓣关闭不全中的应用

目的 总结运用"缘对缘"成形技术治疗先天性心脏病病人的重度三尖瓣关闭不全的效果.方法 2001年4月至2010年3月,对14例先大性心脏病合并重度三尖瓣关闭不全病人采用常规三尖瓣瓣环成形和"缘对缘"技术行三尖瓣成形.年龄7～62岁,平均(31.2±16.1)岁.先大性心脏畸形包括继发孔房间隔缺损6例,房室管畸形5例,继发孔房间隔缺损合并二尖瓣关闭不全2例,三房心1例.结果 14例出院时均无不适,无住院死亡及术后并发症.术后超声心动图检查示三尖瓣关闭不全无或微量11例,轻度3例.随访3～97个月,平均(51.6±26.8)个月.随访时超声心动图检查示均无三尖瓣狭窄,三尖瓣关闭不全无或微量5例,轻度8例,中度1例.结论 "缘对缘"成形技术纠治先天性心脏病合并重度三尖瓣关闭不全简单、有效.     

Repair of congenitally malformed mitral valve in children

We report our experience with 30 children under 15 years of age treated surgically for congenital mitral valve disease from March 1972 to July 1986. Valve reconstruction was possible in 26 patients (87%), whereas in four the valve was replaced with a mechanical prosthesis. Five patients died in the hospital (16.6%), four after conservative operations and one after mitral valve replacement. There was only one late death in a child in chronic congestive heart failure. Three patients, treated conservatively, required valve replacement 2, 22, and 24 months, respectively, after the operation. Follow-up data reveal that 22 of 24 patients are asymptomatic 5 months to 15 years after operation. Two-dimensional echocardiographic studies were performed in 19 patients treated conservatively, 17 of whom are asymptomatic. Eleven of them have no signs of mitral regurgitation or stenosis, six show only mild mitral incompetence, and two have moderate mitral regurgitation or stenosis. Peak pulmonary artery pressure is within normal limits in all. Our results indicate that mitral valve reconstructive procedures for congenital valve dysplasia may be effective and reliable in children despite the frequent severity of valve malformation. Although no major prosthesis-related complications were observed in the present series, we believe that mitral valve repair should always be attempted in the pediatric population to avoid the drawbacks of the currently available prostheses.     

Fate of left-sided cardiac bioprosthesis valves in children

To avoid anticoagulation and minimize thromboembolic phenomena, between 1975 and 1980 we used 18 porcine bioprosthetic valves (BPVs) to replace 11 aortic and seven mitral valves in 17 children ranging from 7 to 18 years of age (mean, 8.2 years). Ten BPVs (91%) in the aortic position had to be replaced one to six years (mean, 4.2 years) after insertion. Nine of these valves developed severe calcification with leaflet immobility and severe stenosis. The tenth valve became insufficient with a disrupted cusp. Six (86%) of seven BPVs inserted in the mitral position required replacement two to four years (mean, 3.1 years) after insertion. Massive mitral regurgitation developed in three, while in the other three mitral stenosis was prominent. All explanted BPVs exhibited calcification with disruption and loss of mobility of the leaflets. Hemodynamic deterioration often occurred catastrophically, with nine patients requiring emergency valve replacement. Elective valve replacement carried no hospital mortality, whereas emergency valve replacement carried a 33% mortality. The BPV failure rate of 94% within six years leads us to recommend against the use of biologic valves in the pediatric age group in the aortic or mitral position. Bioprosthetic valve failure may occur catastrophically and replacement should be carried out early to avoid the higher operative mortality associated with emergency surgery.     

Mitral valve replacement in children

Rheumatic fever leading to advanced valvular heart disease, in adults and children, is still frequently seen in developing countries. In the period 1981-87, 1137 patients underwent open heart surgery for either repair (489 patients), or replacement (639 patients) of defective cardiac valves. The experience with 75 children who underwent mitral valve replacement among this group is reviewed. The aetiology of mitral valve disease was rheumatic in 71, and infective endocarditis in four; 85% of the children were in NYHA functional class III, and 15% in class IV. Seven children had intra-operative findings of rheumatic activity. Pure mitral regurgitation was seen in 41, while mixed mitral valve disease was observed in 34 children. Twenty-seven children underwent mitral valve replacement with Ionescu-Shiley bovine pericardial valves, and 48 with mechanical Bi-leaflet valves. The operative mortality was 9.3%, and the actuarial survival rate, calculated by the Cutler and Ederers method, was 87% at 5 years.     

Mitral valve repair and replacement for rheumatic disease

OBJECTIVES: Mitral valve repair may be technically feasible in patients with suitable anatomy, but the appropriateness of repair for rheumatic disease remains controversial. We evaluated our late outcomes after mitral repair and replacement for rheumatic disease. METHODS: Five hundred seventy-three patients underwent mitral valve surgery for rheumatic disease at our institution from 1978-1995. Follow-up was 98% complete (mean, 68 +/- 46 months). Survival and morbidity were evaluated by Kaplan-Meier analysis and Cox regression, including propensity score analysis. RESULTS: Mean age was 54 +/- 14 years, 55% of patients had congestive heart failure, 22% were undergoing redo mitral valve surgery, and 9% also underwent coronary bypass. Mitral stenosis was present in 53%, regurgitation in 15%, and both in 32%. Valve repair was performed in 25%, bioprosthetic replacement was performed in 28%, and a mechanical valve was placed in 47%. Patients undergoing repair were younger and less likely to be undergoing reoperation or to have atrial fibrillation than those undergoing replacement (P =.001). The operative mortality rate was 4. 2%. Better late cardiac survival was independently predicted by valve repair rather than replacement (P =.04) after adjustment for baseline differences between patients. Freedom from reoperation was greatest (P =.005) but that from thromboembolic complications was worst (P <.0001) after mechanical valve replacement. Twenty-three patients underwent reoperation after initial repair, with no operative deaths. CONCLUSIONS: Mechanical valves minimize reoperation but limit survival and increase thromboembolic complications. Patients undergoing valve repair had improved late cardiac survival independent of their preoperative characteristics. Rheumatic mitral valves should be repaired when technically feasible, accepting a risk of reoperation, to maximize survival and reduce morbidity.     

Two-patch repair of complete atrioventricular septal defect in the first year of life. Results and sequential assessment of atrioventricular valve function

Before January 1987, 62 infants underwent two-patch repair of complete (51) or intermediate (11) atrioventricular septal defect at the Royal Children's Hospital, Melbourne. Median age at repair was 4.3 months and median weight was 4.4 kg. Early deaths (3%) were confined to two infants with preoperative respiratory tract infections; a further two patients died during follow-up (late mortality rate 3%). Reoperation for severe postoperative mitral regurgitation was necessary in 10 infants (16%), two of whom subsequently required mitral valve replacement with a prosthesis. Preoperative atrioventricular valve regurgitation was assessed retrospectively in 49 patients from angiography or Doppler echocardiography and was found to be absent or mild in 33 (68%), moderate in 9 (18%), and severe in 7 (14%). At the time of latest review (at a mean of 2.4 years after repair), judged from a combination of clinical and echocardiographic criteria, mitral regurgitation was absent or mild in 49 (84%) of the 58 survivors; none of them had symptomatic regurgitation or were requiring continuing medical treatment. Analysis of sequential atrioventricular valve function in 46 of the 49 patients in whom objective preoperative data were available showed no relationship between the degree of preoperative and postoperative atrioventricular valve regurgitation. Infants without Down's syndrome, however, had a significantly higher reoperation rate for severe postoperative mitral valve regurgitation (50%) than those with Down's syndrome (10%) (p = 0.007). Complete atrioventricular septal defect can be repaired in early infancy with a low mortality rate and good intermediate term results.     

One-Stage Repair of Truncus Arteriosus, CAVC, and TAPVC

An infant with truncus arteriosus, complete atrioventricular canal, and total anomalous pulmonary venous connection successfully underwent one-stage complete repair. Residual mitral valve regurgitation required reoperation after 12 days. The patient is doing well at 6 months' follow-up. Echocardiography demonstrates no residual defects, competent atrioventricular valves, and normal pulmonary pressure. This case illustrates the potential for successful one-stage repair even of associated complex heart defects involving venous, intracardiac, and arterial pathways.