Systemic Atrioventricular Valve Research Articles

Double switch operation in a young infant

We performed a combined Senning and arterial switch operation on a 2-month-old patient with congenitally corrected transposition, Ebstein's malformation producing severe tricuspid regurgitation, ventricular septal defect, pulmonary hypertension, and congestive heart failure. The tricuspid regurgitation was improved. The double switch operation has the advantage of improving the function of the systemic atrioventricular valve, especially in newborns or young infants in whom the outcome of the valvar repair is poor.

Congenitally Corrected Transposition of the Great Arteries: Current Treatment Options.

Congenitally corrected transposition of the great arteries is a relatively rare cardiac malformation characterized by atrioventricular and ventriculoarterial discordance. This double discordance results in a physiologically corrected circulation with the morphologic right ventricle (RV) serving as the systemic pump. Associated anomalies are present in approximately 98% of cases and include most commonly ventricular septal defect, pulmonary stenosis, and anomalies of the systemic atrioventricular valve (SAVV). Conduction abnormalities are common as well, predisposing these patients to the development of complete heart block. The management of these patients is primarily determined by the presence and severity of the associated anomalies. Conventional repair, which leaves the morphologic RV as the systemic ventricle, has resulted in high incidence of tricuspid regurgitation (TR) and progressive dysfunction of the RV. In the majority of patients, congestive heart failure secondary to RV dysfunction occurs by the fifth or sixth decade. The cause and effect relationship between TR and RV dysfunction remains to be determined. The advent of the Double Switch operation, which restores the morphologic left ventricle (LV) as the systemic ventricle, has yielded favorable outcomes at early follow-up. This procedure should be applied in young patients to achieve optimal results. Retraining the LV by pulmonary artery banding is associated with high morbidity and mortality when attempted after infancy or early childhood. Long-term follow-up is needed to evaluate the potential superiority of this procedure and the incidence of rhythm, baffle, and conduit complications for this management option.

After repair, atrioventricular valve regurgitation during cardiac extracorporeal membrane oxygenation predicts survival

Background When echocardiography is used to follow up cardiac patients on extracorporeal membrane oxygenation (ECMO), ejection fraction as a measure of ventricular function has been used to determine likelihood of survival after decannulation. We hypothesized that systemic atrioventricular (AV) valve regurgitation while on ECMO may be a better predictor of survival. Methods From February 1995 to March 2001, 64 patients required ECMO postoperatively. Three were removed from ECMO owing to brain death, so 61 patients formed our study group. Retrospective chart review was performed with systemic AV valve regurgitation and ejection fraction while on ECMO recorded from echocardiography reports. Patients alive 1 month after decannulation were considered survivors. Results There were 29 survivors (47.5%). Ejection fraction in survivors (mean 41% ± 0.13%) was not significantly different from that of nonsurvivors (41% ± 0.18%; p ≤ 0.839) but severity of systemic AV valve regurgitation was significantly different. Only 2 survivors (6.8%) had at least moderate AV valve regurgitation compared with 17 of the 32 nonsurvivors (53%). Patients with moderate to severe regurgitation while on ECMO were less likely to survive for 1 month after decannulation, with an odds ratio of 16.63 (95% confidence interval 3.08, 89.70; p = 0.001). Also female sex increased the odds of death by a factor of 5.43 (95% confidence interval 1.45, 20.36; p = 0.012). Conclusions Severity of systemic AV valve regurgitation on ECMO is a more reliable predictor than ejection fraction for survival at 1 month after decannulation. Patients with less than moderate AV valve regurgitation have a significantly better chance of survival.

Outcome of the unoperated adult who presents with congenitally corrected transposition of the great arteries.

The goal of this study was to determine the presentation and outcome of the unoperated adult with congenitally corrected transposition of the great arteries. The presentation of this disorder and the outcome in unoperated adults have not been well defined. All unoperated patients > or =18 years old were evaluated for spectrum of disease, hemodynamic severity, timeliness of diagnosis and referral, and outcome. Forty-four patients aged 20 to 79 years (mean, 44) were followed up to 144 months. In 29 (66%), the correct diagnosis was first made at age > or =18 years; the diagnosis was missed in seven of these patients in a prior cardiology consultation, despite cardiac imaging. Systemic atrioventricular valve (SAVV) regurgitation (grade > or =3/4) was noted in 26 patients (59%). Thirty (68%) had surgical intervention, including SAVV replacement in all, with no early mortality. Preoperatively, this subset had significant dysfunction of the systemic ventricle (SV) (ejection fraction [EF], 40 +/- 10%), and most had advanced symptoms (25 with ability index > or =2/4). In 16 (53%), SAVV regurgitation > or =3/4 and ventricular dysfunction had been documented for >6 months. The mean EF of the SV decreased significantly postoperatively (34 +/- 11%, p = 0.006). Four patients (13%) eventually required cardiac transplantation. Poor preoperative EF of the SV predicted eventual need for transplantation (p = 0.001). Patients with unoperated congenitally corrected transposition of the great arteries are often misdiagnosed in adulthood and are referred late despite symptomatic SAVV regurgitation and significant SV dysfunction. Although excellent early surgical results can be achieved, significant residual dysfunction of the SV is common.

Atrioventricular valve regurgitation in patients with single ventricle: impact of the bidirectional cavopulmonary anastomosis

Background. It is not known whether atrioventricular valve regurgitation (AVVR) can improve in some patients after the bidirectional cavopulmonary anastomosis (BCPA) without undertaking concomitant valvuloplasty. Methods. We retrospectively reviewed our experience with patients who underwent the BCPA between June 1989 and June 2000 with specific attention to those patients with moderate or severe AVVR. The degree of AVVR was determined by color Doppler echocardiography. During the study period there were 576 patients who underwent BCPA at a median age of 6.8 months (range, 1.3 months to 4.8 years). The majority of subjects (66%) had a tricuspid valve as the systemic atrioventricular valve. Significant (moderate or severe) AVVR before BCPA was noted in 36 of 576 patients (6%) and was significantly more prevalent (8 of 52 patients, 15%; p = 0.01) in patients with a common atrioventricular canal. Results. Of the 36 subjects with moderate or severe AVVR, 8 had interventions (6 valvuloplasty, 2 aortic arch dilation) at BCPA. There was 1 hospital death (2.8%). Of the remaining 27 survivors who did not undergo additional interventions at BCPA, improvement in AVVR (mild or none) was noted in 6 (22%) at intermediate follow-up (median, 2 years; range, 0.5 to 3.1 years). The presence of significant AVVR before BCPA was not significantly associated with hospital survival or intermediate-term freedom from death or transplantation. Conclusions. We conclude that AVVR improves in some patients after BCPA and that valvuloplasty is not justified in all patients with moderate preoperative AVVR.

Early and late results of operations for defects associated with corrected transposition and other anomalies with atrioventricular discordance in a pediatric population

Objectives: The purpose of this study was to determine the early and late results for children having operations for defects associated with corrected transposition of the great arteries and other anomalies with atrioventricular discordance. Methods: Data on 111 children operated on from July 1, 1971, through January 31, 1996, including clinic records, operative reports, and follow-up visits and questionnaires, were analyzed with particular reference to variables associated with early and late mortality, reoperations, ventricular function, and status of the atrioventricular valves. Results: Complex associated anomalies were common and included double-outlet right ventricle (n = 43) and situs abnormalities (n = 38). Overall early mortality was 16%; for the 29 patients operated on after 1986, early mortality was 3%. Early survival was adversely affected by patch repair of ventricular septal defect and early operative interval. Follow-up of the 93 early survivors extended to 26.5 years (mean 11.4 years). Overall survival was 77% (±4%) at 5 years and 67% (±5%) at 10 years. Late survival was adversely affected by prior operations, more severe preoperative functional class, and cardiac rhythm other than sinus. Reoperation was required for 38 (41%) patients, most commonly for conduit replacement (n = 22) or repair/replacement of the systemic ventricle atrioventricular valve (n = 13). Conclusions: These results can serve as a basis for comparison with newer surgical alternatives proposed for corrected transposition of the great arteries.J Thorac Cardiovasc Surg 2001;122:234-41

40 years after the first atrial switch procedure in patients with transposition of the great arteries: long-term results in Toronto and Zurich.

The atrial switch procedure dramatically improved the prognosis of children with complete transposition of the great arteries (TGA). Overall actuarial survival was approximately 75% after 25 years and was better in patients with simple TGA than in those with complex TGA. Mortality by any cause (16%) and cardiovascular mortality (12% and 13%) were comparable in both centers. Progressive congestive heart failure and sudden death were the principal modes of death. Most of the survivors denied any symptoms or had mild limitations in their daily activities. However, long-term problems in this growing population of adults are challenging and include late arrhythmias (up to two thirds of the patients), systemic ventricular (SV) failure, systemic atrioventricular valve regurgitation and reoperations, such as baffle reconstruction, being the most frequent. Objective assessment of SV function obtained by echocardiography is difficult. It may include fractional area change and tricuspid annular motion. Survivors after an atrial switch procedure are unique and have a good quality of life. However, the definitive and true history of the RV supporting the systemic circulation is not as yet known.

Atrioventricular discordance: Results of repair in 127 patients

Objective: The conventional management of patients with atrioventricular discordance is directed at associated lesions, taking advantage of physiologic “correction”; however, the morphologic right ventricle and tricuspid valve support the systemic circulation. Questions surrounding survival using this approach led us to analyze our institutional results. Methods: All patients with atrioventricular discordance undergoing biventricular repair were analyzed (n = 127, 1959-1997), excluding those with functionally univentricular hearts. The ventriculoarterial connection associated with atrioventricular discordance varied and was most commonly discordant (87%), but occasionally concordant (6%), double-outlet right ventricle (6%), or double-outlet left ventricle (1%). At initial presentation, the most common lesions associated with atrioventricular discordance were ventricular septal defect (86%), pulmonary stenosis (64%), tricuspid regurgitation (28%), and atrioventricular block (12%). Nine patients underwent a double switch procedure to create ventriculoarterial concordance and the remainder were managed conventionally without correcting discordant connections. Results: Operative mortality was 6% and did not vary by associated lesion. Twenty years after repair, survival was 48%. Within 20 years, 56% of patients required reoperation, usually for atrioventricular valve incompetence (n = 16), pulmonary stenosis (n = 16), or both (n = 3). Pacemakers were required in 50 patients, 4 before repair, 40 within 2 months of repair, and 6 remotely after repair. In early follow-up, the double switch procedure (n = 9) had equivalent mortality and a high pacemaker requirement for atrioventricular block. Conclusions: Analysis of conventional management of atrioventricular discordance revealed cumulative increases in mortality, systemic atrioventricular valve (tricuspid) replacement, complete atrioventricular block, and incidence of reoperation. Alternative management should be examined. (J Thorac Cardiovasc Surg 1999;117:1190-203)

Pregnancy among women with congenitally corrected transposition of great arteries

OBJECTIVESThe outcome of pregnancy in congenitally corrected transposition of the great vessels was studied in 22 women.BACKGROUNDWomen with congenitally corrected transposition of the great vessels often reach childbearing age. Although reports on the outcome of pregnancy in these women are available, the number of patients is small.METHODSThe medical and surgical databases at the Mayo Clinic were reviewed, and 36 women >16 years old with congenitally corrected transposition of the great vessels were identified. All of them were contacted, and 22 who had pregnancies were identified and the outcome of pregnancy was evaluated.RESULTSTwenty-two women had 60 pregnancies resulting in 50 live births (83%). Forty-four deliveries (88%) were vaginal and 6 (12%) were by cesarean section. One delivery was premature at 29 weeks. There was one successful twin pregnancy. There were 11 unsuccessful pregnancies. One patient developed congestive heart failure late in pregnancy because of systemic atrioventricular valve regurgitation and required valve replacement in the early postpartum period. One patient had a total of 12 pregnancies, including 1 twin pregnancy and 2 unsuccessful pregnancies. She had multiple pregnancy-related complications, including toxemia, congestive heart failure, endocarditis and myocardial infarction (single coronary artery). No other serious pregnancy-related maternal complications and no pregnancy-related deaths occurred. The mean birth weight of the infants (n = 32) was 3.2 ± 0.4 kg. None of the 50 live offspring have been diagnosed with congenital heart disease.CONCLUSIONSSuccessful pregnancy can be achieved in most women with congenitally corrected transposition of the great arteries. The rate of fetal loss and maternal cardiovascular morbidity is increased. Because of the small number of births, the risk of congenital heart disease in offspring of women with congenitally corrected transposition of the great arteries is uncertain.

In situ implantation of a prosthetic systemic atrioventricular valve in corrected transposition

J Thorac Cardiovasc Surg 1999;117:190-1

Fontan operation in five hundred consecutive patients: Factors influencing early and late outcome

Objective: The purpose of this study was to review a large, evolving, single-center experience with the Fontan operation and to determine risk factors influencing early and late outcome. Methods: The first 500 patients undergoing modifications of the Fontan operation at our institution were identified. Perioperative variables were recorded and a cross-sectional review of survivors was undertaken. Results: The incidence of early failure decreased from 27.1% in the first quartile of the experience to 7.5% in the last quartile. In a multivariate model, the following variables were associated with an increased probability of early failure: a mean preoperative pulmonary artery pressure of 19 mm Hg or more ( p < 0.001), younger age at operation ( p = 0.001), heterotaxy syndrome ( p = 0.03), a right-sided tricuspid valve as the only systemic atrioventricular valve ( p = 0.001), pulmonary artery distortion ( p = 0.04), an atriopulmonary connection originating at the right atrial body or appendage ( p = 0.001), the absence of a baffle fenestration ( p = 0.002), and longer cardiopulmonary bypass time ( p = 0.001). An increased probability of late failure was associated with the presence of a pacemaker before the Fontan operation ( p < 0.001). A morphologically left ventricle with normally related great arteries or a single right ventricle (excluding heterotaxy syndrome and hypoplastic left heart syndrome) were associated with a decreased probability of late failure ( p = 0.003). Conclusions: These analyses indicate that early failure has declined over the study period and that this decline is related in part to procedural modifications. A continuing late hazard phase is associated with few patient-related variables and does not appear related to procedural variables. (J Thorac Cardiovasc Surg 1997;114:376-91)

Flail Tricuspid Valve in an Adult Patient with Congenitally Corrected Transposition of the Great Arteries

We describe a case of a 50-year-old woman with congenitally corrected transposition of the great vessels, in whom severe left-sided tricuspid (systemic atrioventricular) valve insufficiency was the only associated anomaly. The tricuspid valve was dysplastic and abnormally oriented toward the interventricular septum, without the downward displacement of Ebstein's anomaly. The mechanism of atrioventricular regurgitation was unusual in that it consisted of the rupture of chordae tendineae of both the anterior and septal leaflets. The left-sided tricuspid valve was replaced with a St. Jude prosthesis and the postoperative course was uneventful.

Congenitally corrected transposition of the great arteries in the adult: Functional status and complications

We sought to assess the clinical outcome, functional status and complications of adult patients with congenitally corrected transposition of the great arteries. Congenitally corrected transposition is a rare form of congenital heart disease, although survival into adult life may be expected. Little information is available on the long-term prognosis of these patients once they have reached adulthood. This study focuses exclusively on patients >18 years old followed up at a single tertiary referral center. The charts of all patients with a diagnosis of congenitally corrected transposition of the great arteries from the Toronto Congenital Cardiac Centre for Adults since 1985 were reviewed. Data were available for 52 patients, 26 of whom had undergone radionuclide angiography. Mortality, clinical and functional status, surgical procedures and complications were reviewed. Thirteen patients (25%) died; age at death was 38.5 +/- 12.5 years (mean +/- SD). The current age of survivors is 32.7 years (range 18.2 to 54.3). Of the survivors, 17 had palliative procedures, and 25 had definitive repair, 11 of whom required reoperation. Left ventricle to pulmonary artery conduit replacement was necessary in seven patients. Eighteen patients have permanent pacemakers, nine of whom developed complete heart block perioperatively. Nine patients developed progressive atrioventricular (AV) block unrelated to operation. Supraventricular arrhythmias occurred in 15 patients. Progressive systemic AV valve regurgitation developed in 10 patients and endocarditis in 6. Congenitally corrected transposition in the adult patient is not a benign condition. Late complications are common and warrant careful, long-term follow-up.

Complete Echocardiographic Assessment of the Postoperative Fontan Patient

Postoperative echocardiographic assessment of the Fontan patient should include both anatomical and physiological parameters. Anatomical features to be defined with two‐dimensional (2‐D) imaging should include a detailed demonstration of the atriopulmonary connection, the pulmonary arterial tree, and the pulmonary venous return. Color flow imaging should be included to provide visual clues for abnormal flow patterns to suggest obstruction or residual shunts. Intracardiac thrombi, pericardial, and pleural effusions should be excluded. Inadequate transthoracic images should mandate the use of transesophageal imaging to adequately detail the extracardiac anatomy and connections. Physiological parameters should include M‐mode and 2‐D studies to assess ventricular systolic function and the degrees of ventricular hypertrophy. Additionally, extensive pulsed Doppler examination should be performed to determine the systemic venous, pulmonary arterial, pulmonary venous, and atrioventricular (AV) valve inflow patterns. Significant information detailing abnormalities of pulmonary artery flow and ventricular diastolic function parameters can be obtained. Doppler color flow imaging should be obtained to assess competency of AV and semilunar valves. Continuous‐wave Doppler should be utilized to determine the presence and severity of aortic outflow obstruction. Complete echocardiographic evaluation should include all facets of echocardiographic study as detailed previously.

Intermediate results from the period of the congenital heart surgeons transposition study: 1985 to 1989

The period of the Congenital Heart Surgeons Society (CHSS) study (1985 to 1989) provided a transition in the treatment of d-transposition of the great arteries. During this unusual time frame neonatal arterial switch (AS) and neonatal or late atrial baffle repair (Senning) were used in near equal proportion at one reporting institution. All the procedures were performed at this single institution, avoiding the variability intrinsic to a multicenter study. Intermediate follow-up of the results is presented. During the period of the CHSS study, January 1985 to March 1989, 46 patients were enrolled in the CHSS study at one institution. Forty-four underwent either neonatal arterial (n = 14, 32%) or neonatal atrial (n = 19, 43%) or late atrial (n = 11, 25%) repair of d-transposition of the great arteries. Ages ranged from 4 to 80 days. Overall survival for the entire series was 91% (40/44). The survival of the AS group operate on in the neonatal period was 93% (13/14). The survival of the Senning group was 90% (27/30); late Sennings, 91% (10/11); and neonatal Sennings, 92% (12/13). Six neonatal Sennings were crossovers from the AS group with an 83% survival (5/6). Intermediate follow-up of 5.2 to 9.2 years revealed no late deaths. In the AS group there was no ventricular failure, no arrhythmias, and one reoperation for supravalvar pulmonic stenosis. In the Senning group, there was no ventricular failure, but significant complications developed in 10 patients: cardiac arrhythmias in 7, tachyarrhythmias requiring pharmacologic therapy in 4, and bradyarrhythmias in 3, 2 requiring permanent pacemaker insertion. Left ventricular outflow tract (subpulmonic) stenosis developed in 3 patients, 1 requiring a left ventricular to pulmonary artery conduit and permanent pacemaker. Systemic atrioventricular valve insufficiency has developed in 3 patients. Results at one reporting institution from the CHSS study during this period of transition from late atrial repair (Senning) to neonatal atrial or arterial repair show comparable early mortality in all groups. However, the intermediate results at a mean of 6.7 years reveal fewer arrhythmic and functional complications in the AS group. The possibility of neonatal repair combined with low early and intermediate morbidity and mortality confirm AS as the treatment of choice for d-transposition of the great arteries.

The modified fontan operation: An analysis of risk factors for early postoperative death or takedown in 702 consecutive patients from one institution

To better understand risk factors associated with early postoperative death or failure, we reviewed our entire experience with 702 consecutive patients who had the modified Fontan operation at the Mayo Clinic between October 1973 and December 1989. The event rate for takedown of repair or death during the initial hospitalization or within 30 days of the operation was 14.8% (successful takedown of the repair, n = 6; death, n = 98). To identify variables associated with early death or Fontan takedown, we analyzed 33 clinical and hemodynamic variables in a univariate and multivariate manner. On the basis of a stepwise logistic discriminant analysis, patients who were younger and operated on before 1980 with a higher preoperative pulmonary artery mean pressure, asplenia, higher intraoperative (after Fontan operation) right atrial pressure, longer aortic crossclamp time, and pulmonary artery ligation were more likely to have the outcome event of interest ( p values < 0.05). A new variable, corrected pulmonary artery pressure (that is, mean preoperative pulmonary artery pressure divided by the ratio of pulmonary to systemic flow if the ratio of pulmonary to systemic flow is greater than 1.0), was significantly associated with the outcome event univariately ( p = 0.002), but was no more predictive than the preoperative pulmonary artery mean pressure. Variables less predictive of the outcome event in this analysis included multiple prior operations, polysplenia syndrome, complex anatomy other than asplenia syndrome, and systemic atrioventricular valve regurgitation. These results represent the largest single-institution review of the Fontan operation and suggest that some anatomic and hemodynamic variables previously predictive of poor early outcome have been nullified by current operative methods. (J T HORAC C ARDIOVASC S URG 1995;109:1237-43)

Late results of systemic atrioventricular valve replacement in corrected transposition

From December 1964 to October 1993, 40 patients (aged 5 months to 70 years, mean 21.8 years, median 13.6 years) with corrected transposition and systemic atrioventricular valve insufficiency underwent replacement ( n = 39) or repair ( n = 1) of the systemic atrioventricular valve. Thirty-nine patients had situs solitus and 1 had situs inversus. Associated anomalies included Ebstein's malformation of the systemic atrioventricular valve ( n = 22), ventricular septal defect ( n = 19), and pulmonary stenosis ( n = 14). Preoperatively, 16 patients (40.0%) had complete heart block and 27 patients (67.5%) were in New York Heart Association functional classes III and IV. The early mortality was 10.0% ( n = 4) and 8 patients died subsequently. The principal cause of death in all 12 patients was systemic ventricular failure. Overall survival including early mortality was 78.0% at 5 years and 60.7% at 10 years; survival excluding early mortality was 86.7% at 5 years and 67.5% at 10 years. Survivorship correlated with preoperative systemic ventricular ejection fraction of 44% or more ( p < 0.001) and later interval of operation (9 deaths in 15 patients before 1981 versus 3 deaths in 25 patients subsequently) ( p = 0.06). There were no cases of surgically induced complete heart block. Two patients underwent late reoperations related to the systemic atrioventricular valve prosthesis. Follow-up extended to 26.0 years (median 4.7 years). At last follow-up, 18 of the 28 survivors were in New York Heart Association functional class I, 9 were in class II, and 1 was in class III. We conclude that the results of systemic atrioventricular valve replacement in corrected transposition have improved significantly during the past decade. To preserve systemic ventricular function, we suggest operation be considered at the earliest sign of progressive ventricular dysfunction as assessed by serial clinical evaluation and echocardiography. (J T HORAC C ARDIOVASC S URG 1995;109:642-53)

Tricuspid valve abnormalities in patients with atrioventricular discordance: Surgical implications

From 1975 to 1990,90 patients (age, 6 months to 30 years; mean, 9.1 years) underwent operation for defects associated with atrioventricular discordance. Twenty-one had an anatomically abnormal tricuspid (systemic) atrioventricular valve (SAVV) of the following types: Ebstein, 11; straddling, 6; and dysplastic, 4. Sixteen valves were regurgitant: régurgitation was trivial to mild in nine and moderate to severe in seven. Two patients underwent a successful Fontan-type operation. None of the 5 patients with a competent SAVV underwent valve repair or replacement; 1 of these patients died. A ventricular septal defect was closed in 14 and an extracardiac valved conduit was placed in 7. Sixteen had a regurgitant valve: it was replaced in 10 and repaired in 2 (early mortality. 25%). All 4 patients who did not undergo repair or replacement of their regurgitant SAVV died. Two patients died late after repeat replacement. Four other reoperations (closure of a residual ventricular septal defect, SAVV replacement, left ventricle-to-pulmonary artery conduit replacement, and a redo Fontan procedure) were successful. Two patients are lost to follow-up, and 9 have been followed up for from 27 to 156 months (median, 117 months). All were well when last seen. We suggest that in abnormal régurgitant SAVV should be replaced. Alternatively, a “double-switch” procedure that leaves the tricuspid valve in the pulmonary circulation may be used.

Management of systemic atrioventricular valve regurgitation in infants and children.

Since September 1979, 53 patients have required operation for systemic atrioventricular 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 , Marfan'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.

Systemic atrioventricular valve replacement in an infant with corrected transposition of the great arteries

A 3-month-old infant with corrected transposition of the great arteries and severe systemic atrioventricular valve regurgitation due to “Ebstein-like anomaly” is reported. Through a right thoracotomy and longitudinal left atrial incision, a 19-mm St. Jude Medical valve was implanted into the annulus without removing the native valve. He is doing well 7 months after operation.