Systemic Atrioventricular Valve Regurgitation Research Articles

Catheter Ablation of Atrial Fibrillation in Transposition of the Great Arteries Treated With Mustard Atrial Baffle

The survival of patients with D-transposition of the great arteries (D-TGA) has dramatically improved with corrective surgical procedures, including the atrial and arterial switch operations. In the current era, the majority of the adult patients typically have undergone atrial switch operations, either a Senning or a Mustard procedure. Late complications include baffle obstruction, systemic ventricular failure, systemic atrioventricular valve regurgitation, and rhythm disturbances. The most common atrial arrhythmia in this population is intra-atrial reentrant tachycardia (IART), which has been associated with development of heart failure and death.1–4 Proarrhythmic factors include atrial enlargement from tricuspid regurgitation and systemic right ventricular failure, as well as suture lines. As this population ages, chronic hemodynamic stress may result in atrial fibrillation as well. We present the first report of catheter ablation for treatment of atrial fibrillation in a patient with D-TGA treated with prior Mustard repair. ### Case Report A 41-year-old man with D-TGA status post Mustard repair presented with longstanding, recurrent atrial arrhythmias. He originally underwent balloon atrial septostomy 2 days after birth and a subsequent atrial switch procedure using the Mustard technique at 2 years of age.5 He did well until age 32, at which time he had recurrent symptomatic IART. EP study was performed. Cavotricuspid isthmus (CTI)-dependent flutter was induced and confirmed by entrainment mapping. Lines of ablation were drawn across the systemic venous baffle portion of the “medial” CTI and the pulmonary venous “lateral” CTI using a retrograde, aortic approach. Flutter terminated with ablation and could not be reinduced. Bidirectional block was demonstrated across the CTI. He remained arrhythmia-free for 2 years, then presented again at age 34 with exertional intolerance and fatigue due to partial obstruction of the pulmonary venous baffle. He underwent patch augmentation of the stenotic …

SYSTEMIC RIGHT VENTRICULAR DYSFUNCTION IN CONGENITALLY CORRECTED TRANSPOSITION OF THE GREAT ARTERIES: IS THE ATRIOVENTRICULAR VALVE ALWAYS THE CULPRIT?

Systemic right ventricular (SRV) dysfunction and systemic atrioventricular valve (SAVV) regurgitation are common in adult patients (pt) with congenitally corrected transposition of the great arteries (CCTGA). Our aim was to determine the prevalence and relationship between SAVV regurgitation and SRV

Congenitally Corrected Transposition of the Great Arteries: Ventricular Function at the Time of Systemic Atrioventricular Valve Replacement Predicts Long-Term Ventricular Function

The objective was to evaluate the systemic ventricular ejection fraction (SVEF) at the time of systemic atrioventricular valve (SAVV) replacement as a predictor of SVEF ≥1 year after surgery in patients with congenitally corrected transposition of the great arteries (CCTGA). Progressive SAVV regurgitation causes systemic ventricular failure in CCTGA patients, who are commonly referred late for intervention. Survival after surgery is poor when the pre-operative SVEF is <44%. We retrospectively reviewed 46 patients (pre-operative SVEF ≥ 40% in 27 patients and <40% in 19 patients) with 2 good-sized ventricles, a morphologically right systemic ventricle, and SAVV regurgitation requiring surgery. Median follow-up was not different in patients with a pre-operative SVEF ≥ 40% (8.8 years) or <40% (7.7 years, p = 0.36). Pre-operative SVEF was the only independent predictor of ≥ 1-year post-operative SVEF (p < 0.0001). The late SVEF was preserved (defined as ≥ 40%) in 63% of patients who underwent surgery with an SVEF ≥ 40% compared with 10.5% of patients who underwent surgery with an SVEF <40%. Pre-operative variables associated with late mortality were an SVEF ≤ 40%, a subpulmonary ventricular systolic pressure ≥ 50 mm Hg, atrial fibrillation, and New York Heart Association functional class III to IV. Post-operative systemic ventricular function after SAVV replacement can be predicted from the pre-operative SVEF. For best results, operation should be considered at an earlier stage, before the SVEF falls below 40% and the subpulmonary ventricular systolic pressure rises above 50 mm Hg.

Older children at the time of the Norwood operation have ongoing mortality vulnerability that continues after cavopulmonary connection

Delayed first-stage palliation of children with hypoplastic left heart syndrome and related pathologies can be associated with poor outcomes because of development of progressive pulmonary vascular disease and volume load effects on the systemic ventricle and atrioventricular valve. We examine the current era's survival in this subgroup. Fifty-five infants older than 2 weeks underwent the Norwood operation (2003-2007). Separate competing risk analyses were performed to model outcomes (death and transition to the next stage) after the Norwood operation and after bidirectional cavopulmonary connection. Median age was 32 days (range, 15-118 days). Forty-seven percent had hypoplastic left heart syndrome, and 53% had other complex univentricular variants. Mean ascending aortic size was 4.4 ± 1.9 mm, 10% had impaired ventricular function, 11% had moderate atrioventricular valve regurgitation, and 32% had restrictive pulmonary venous return. Pulmonary blood flow was established through an aortopulmonary shunt (n = 30) or Sano shunt (n = 25). After the Norwood operation, patients required longer ventilation and more oxygen and nitric oxide and had higher inotropic scores compared with those undergoing the traditional management protocol. Competing risks analysis showed that 2 years after the Norwood operation, 39% had died, and 57% underwent bidirectional cavopulmonary connection. Four years after bidirectional cavopulmonary connection, 15% had died, and 85% underwent the Fontan operation. Overall 3-year survival after the Norwood operation was 53%. Factors associated with mortality were age, lower weight at the time of the Norwood operation, impaired ventricular function, longer circulatory arrest, and lower pre-bidirectional cavopulmonary connection saturation. Children older than 2 weeks undergoing the Norwood operation frequently require postoperative pulmonary vasodilatation and high inotropic support. A significant hazard of death persists through all steps of multistage palliation. Increased pulmonary vascular resistance and volume load effects, such as systemic ventricular impairment and atrioventricular valve regurgitation, are commonly evident in patients in whom treatment fails or who do not qualify to proceed to the next stage of palliation. Those patients should be closely monitored for timely referral for heart transplantation when indicated.

Significance of the residual aortic obstruction in multistage repair of hypoplastic left heart syndrome

The population of children who received right ventricle-to-pulmonary artery shunt (RV-PA) at Norwood procedure (NP) is growing, but limited data are available regarding the long-term outcome. The aim of this study was to present operative outcomes and mid-term hemodynamics and to assess the impact of the residual aortic obstruction on the results in patients undergoing three-staged surgery with RV-PA application at NP. Between June 2001 and June 2009, 229 children with hypoplastic left heart syndrome (HLHS) and variants underwent NP with RV-PA; 172 have proceeded to stage II and 95 to stage III. The medical records (clinical data, echocardiographic records, cardiac catheterization reports, electrocardiograms, and surgical notes) were retrospectively reviewed. The later era of NP was associated with significantly better outcome (2001-2004: 1- and 5-years' survival were 64.3% and 59.8%, respectively; 2005-2009: 1- and 4-years' survival were 93.1% and 86.9%, respectively) (p<0.001). There was no association between long-term survival and diagnosis (HLHS/HLHS variant) (p=0.39). The incidence of depressed ventricular function and moderate or severe systemic atrioventricular valve regurgitation among the children who required balloon aortoplasty (BA) before stage II surgery was significantly higher than in children without aortic arch obstruction (p=0.027, p=0.008, respectively). In midterm follow-up, BA had no significant influence on the actuarial survival (p=0.089). No ventricular arrhythmias were noticed at any stage. The RV-PA shunt is a safe technique that does not seem to impair systolic or electrical ventricular function; its outcomes continue to improve with growing experience. Combined cardiologic interventional and surgical procedures are required to optimize the outcomes.

Congenitally Corrected Transposition of the Great Arteries and Concomitant Coronary Artery and Valvular Disease in the Adult Patient

Congenitally corrected transposition of the great arteries (ccTGA) accounts for less that 1% of cardiac anomalies, and is defined as ventriculoarterial and atrioventricular (AV) discordance. The double discordant connection allows for survival with the right ventricle performing as the systemic ventricle, and the left ventricle as the pulmonary ventricle. We report a case of ccTGA in a 35-year-old male with situs inversus totalis status post repair of a ventricular septal defect (VSD) with a residual VSD, severe systemic AV valve regurgitation, and coronary artery disease who presented with chest pain. He subsequently underwent tricuspid valve replacement and VSD repair, followed by percutaneous coronary revascularization. This case highlights many important issues of adults with congenital cardiac disease, as well as the specific surgical management of anomalies associated with ccTGA. We review the literature and discuss the management of these complicated patients.

Predictors of pregnancy complications in women with congenital heart disease

Data regarding pregnancy outcome in women with congenital heart disease (CHD) are limited. In 1802 women with CHD, 1302 completed pregnancies were observed. Independent predictors of cardiac, obstetric, and neonatal complications were calculated using logistic regression. The most prevalent cardiac complications during pregnancy were arrhythmias (4.7%) and heart failure (1.6%). Factors independently associated with maternal cardiac complications were the presence of cyanotic heart disease (corrected/uncorrected) (P < 0.0001), the use of cardiac medication before pregnancy (P < 0.0001), and left heart obstruction (P < 0.0001). New characteristics were mechanical valve replacement (P = 0.0014), and systemic (P = 0.04) or pulmonary atrioventricular valve regurgitation related with the underlying (moderately) complex CHD (P = 0.03). A new risk score for cardiac complications is proposed. The most prevalent obstetric complications were hypertensive complications (12.2%). No correlation of maternal characteristics with adverse obstetric outcome was found. The most prevalent neonatal complications were premature birth (12%), small for gestational age (14%), and mortality (4%). Cyanotic heart disease (corrected/uncorrected) (P = 0.0003), mechanical valve replacement (P = 0.03), maternal smoking (P = 0.007), multiple gestation (P = 0.0014), and the use of cardiac medication (P = 0.0009) correlated with adverse neonatal outcome. In our tertiary CHD cohort, cardiac, obstetric, and neonatal complications were frequently encountered, and (new) correlations of maternal baseline data with adverse outcome are reported. A new risk score for adverse cardiac complications is proposed, although prospective validation remains necessary.

Evaluation and management of the adult patient with transposition of the great arteries following atrial-level (Senning or Mustard) repair

Atrial-level repair for transposition of the great arteries is one of the greatest achievements of modern cardiovascular medicine, transforming a once fatal congenital heart condition into one with excellent long-term survival. Although atrial-level repair has been supplanted by the arterial switch, there remains a population of patients who underwent atrial-level repair as children and now require ongoing care as adults. Survival slowly continues to decline in this population mainly owing to systemic right ventricular dysfunction and sudden death. Other problems include sinus node dysfunction, atrial arrhythmias, systemic atrioventricular valve regurgitation, baffle problems, and pulmonary hypertension. Evaluation and management of these late complications is addressed.

Reduction of Atrioventricular Valve Regurgitation by Atrioventricular Sequential Pacing for an Adult with Corrected Transposition of the Great Arteries

The case of a 64-year-old woman who underwent corrected transposition of the great arteries with significant systemic atrioventricular valve regurgitation and complete atrioventricular block is described. The patient underwent implantation of a DDD-type pacemaker and experienced relief of symptoms after discharge. Follow-up evaluation 30 months after the pacemaker implantation demonstrated a decrease in the cardiothoracic ratio on chest X-ray, the b-type natriuretic peptide level, and regurgitation at the systemic atrioventricular valve.

Intention-to-Treat Analysis of Pulmonary Artery Banding in Conditions With a Morphological Right Ventricle in the Systemic Circulation With a View to Anatomic Biventricular Repair

Some patients with a morphological right ventricle (mRV) in the systemic circulation require early intervention because of progressive systemic ventricular dysfunction or atrioventricular valve regurgitation. They may be eligible for anatomic repair (correction of atrioventricular and ventriculoarterial discordance) but require prior training of the morphological left ventricle (mLV). Forty-one patients with congenitally corrected transposition of the great arteries or a previous atrial switch procedure embarked on a protocol of pulmonary artery (PA) banding with a view to anatomic repair. All had an mRV in the systemic circulation and a subpulmonary mLV that was not conditioned by either volume or pressure load. Two patients were not banded, and 39 were followed up for a median of 4.3 years (range, 25 days to 12.6 years). Sixteen patients achieved anatomic repair, with 3 in the early stages of the training protocol. After 2 years, 12 patients were not suitable for anatomic repair and persisted with palliative banding; 8 were functionally improved; and 4 died, underwent transplantation, or required debanding. PA banding improved functional class but did not improve tricuspid regurgitation in the long term for patients not achieving anatomic repair. mLV function was a critical determinant of survival with a PA band as well as survival after anatomic repair. Patients >16 years were unlikely to achieve anatomic repair. PA banding is a safe and effective method of training the mLV before anatomic repair. It is also an effective palliative procedure for those who do not attain this goal.

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.

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.

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.

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.

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)

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.

Congenitally corrected transposition and Ebstein's anomaly of the systemic atrioventricular valve: Association with aortic arch obstruction

Aortic arch abnormalities are uncommon in patients with congenitally corrected transposition of the great arteries. Over a 20-year period, 10 patients with congenitally corrected transposition and Ebstein's anomaly of the systemic atrioventricular (AV) valve were identified. Five neonates had severe systemic AV valve regurgitation with severe coarctation of the aorta (n = 4) or aortic atresia (n = 1) and presented with heart failure. Four died in the neonatal period and one who had coarctation repair died 7 months postoperatively. The remaining five patients with congenitally corrected transposition and Ebstein's anomaly had mild left AV valve regurgitation; none of these had aortic arch obstruction.In neonates who have coexisting Ebstein's anomaly of the systemic AV valve and congenitally corrected transposition, obstruction to aortic arch flow is common. Severe systemic AV valve regurgitation with a morphologic systemic right ventricle may have contributed to low anterograde flow in the ascending aorta prenatally and thereby to the aortic arch abnormality.

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 annuloplasly. There were no operative deaths, and there were no reopsrations 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.