Case Report: Retroaortic Innominate Vein With Supracardiac Total Anomalous Pulmonary Venous Connection.

A retroaortic innominate vein (RAIV) is a rare anomaly that passes posterior to the ascending aorta to join the superior vena cava and is associated with congenital heart disease (CHD). The RAIV and normal left innominate vein (LIV) rarely duplicate. The etiology of the RAIV and its relationship with CHD remains unknown. We report a case involving a 1-month-old baby girl with RAIV and supracardiac total anomalous pulmonary venous connection (TAPVC). Transthoracic echocardiogram demonstrated a pulmonary venous confluence (CPV) posterior to the left atrium, an abnormal vertical vein (VV) that originated from the CPV, and a normally positioned LIV. Three-dimensional cardiac computed tomography revealed the VV and RAIV to which it merged. This is the first reported case of a combination of RAIV and isolated TAPVC. We speculate that the VV is connected to the CPV during fetal life, thus leaving the RAIV behind. The RAIV may be detected in various forms with the development of new diagnostic imaging methods. Although a RAIV itself does not require treatment, establishing a correct diagnosis before invasive tests and procedures are performed can help prevent unexpected complications.

Ascending aortic extension to enlarge the retroaortic space in children after the Norwood procedure.

Left pulmonary artery stenosis is a well-recognized complication following the Norwood procedure. We herein report two cases in which ascending aortic extension was performed to enlarge the retroaortic space in children with left pulmonary artery stenosis after the Norwood procedure. We used graft interposition in the ascending aorta to increase the retroaortic apace and concomitantly performed extended left pulmonary artery reconstruction. This procedure obtains a more balanced distribution of the pulmonary blood flow, which is crucial to achieve good Fontan circulation.

Outcomes of systemic-to-pulmonary artery shunt for single ventricular heart with extracardiac total anomalous pulmonary venous connection.

OBJECTIVES: A few studies have described the outcomes of systemic-to-pulmonary artery shunt (SPS) for functional single ventricular heart with extracardiac total anomalous pulmonary venous connection (TAPVC). This study aimed to examine the outcomes of SPS with extracardiac TAPVC and identify the predictors of mortality before bidirectional Glenn operation (BDG). METHODS: Medical records of 41 children with single ventricular heart and extracardiac TAPVC who underwent SPS between 1998 and 2019 were reviewed retrospectively. The median age and body weight at SPS were 36 days and 3.4 kg, respectively. Surgical outcomes and predictors of mortality were investigated. RESULTS: Four operative deaths (10%) and 10 late deaths (27%) occurred before BDG. Of all the children, 19 underwent BDG at a median of 10 months since SPS and eight are waiting for BDG. In the multivariate analysis, preoperative pulmonary venous obstruction (p = 0.01) at initial surgery was most predictive of death before BDG. Patients who underwent simultaneous SPS and TAPVC repair were younger, had high preoperative rate of pulmonary venous obstruction, and more deaths before BDG. CONCLUSIONS: Survival outcomes of SPS for SVH with extracardiac TAPVC were improved as a whole due to the increase in knowledge and technique of management SPS. However, the patients who have preoperative pulmonary venous obstruction (PVO) and need SPS and TAPVC repair concomitantly in the early postnatal period have poor outcomes and still challenging. In such a case, staged TAPVC repair and SPS may be beneficial.

Expansion of a Huge Compressive Left Atrial Appendage Aneurysm in a 29-Day-Old Infant.

We report a case of symptomatic and progressive enlargement of a huge left atrial appendage aneurysm in a 29-day-old infant. The aneurysm was detected by fetal echocardiography and exertional dyspnea developed during the neonatal period. The aneurysm was successfully resected by decompressing the aneurysm using cardiopulmonary bypass without cardiac arrest. Our findings suggest that fetal echocardiography enables early diagnosis of the rare left atrial appendage aneurysm, and early surgical resection may protect affected patients from life-threatening symptoms.

Outcomes of mitral valve replacement with bileaflet mechanical prosthetic valve in children.

OBJECTIVES: We examined the outcomes following mitral valve replacement with bileaflet mechanical prosthetic valve in children and identified the predictors for mortality and reoperation. METHODS: Medical records from 49 children who underwent mitral valve replacement between 1982 and 2015 were reviewed retrospectively. Median age and body weight at initial mitral valve replacement were 2.4 years and 9.7 kg, respectively. The median follow-up was 13 years. Surgical results and predictors for mortality and reoperation were investigated. RESULTS: There was no operative mortality; eight late deaths occurred. The actuarial survival rates were 89.5%, 84.2%, and 80.7% at 5, 10, and 15 years, respectively, after initial mitral valve replacement. The actuarial freedom rates from related complications were 89.5%, 78.3%, and 70.7% at 5, 10, and 15 years, respectively. Nineteen patients required 1st re-mitral valve replacement at a median of 5.9 years; six of these 19 required 2nd re-mitral valve replacement at a median of 8.9 years after 1st re-MVR. The actuarial freedom rates from re-mitral valve replacement were 86.0%, 56.8%, and 44.2% at 5, 10, and 15 years, respectively. No predictor for death was determined; however, the predictor for re-mitral valve replacement was initial valve diameter less than 19 mm. CONCLUSIONS: Survival outcomes among children after mitral valve replacement with bileaflet mechanical prosthetic valve in biventricular heart were satisfactory. However, complications, including re-mitral valve replacement, were frequent and the predictor was of a small prosthesis size.

Long-Term Growth of the Neoaortic Root After Arterial Switch Operation.

BACKGROUND: The growth of the neoaortic root after the arterial switch operation for the transposition of the great arteries remains unclear. This study aimed to investigate the growth of the neoaortic root and identify risk factors for neoaortic root dilatation. METHODS: Serial angiographic measurements of the neoaortic root for at least 10 years were evaluated in 145 patients. A total of 1,876 measurements of the sinuses of the Valsalva and the neoaortic annuli were obtained. A linear mixed effects model was used for z-score analysis, including evaluation of risk factors for neoaortic root dilatation. To assess changes in the time course of neoaortic root absolute diameters, a nonlinear mixed effects model with a growth curve model was used. RESULTS: The growth curve revealed progressive growth of the neoaortic root during somatic growth and stabilization in adulthood without normalization. The growth rates of the sinus and annulus were 0.0046 and 0.029 z-score per year, respectively. The sinus and annulus were estimated to grow up to 47 ± 1 mm and 31 ± 1 mm, respectively. Major risk factors for neoaortic root dilatation were double-outlet right ventricle (parameter estimate [PE] = 2.1, 95% confidence interval [CI] = 1.5 to 2.7, p < 0.0001 for sinus; PE = 1.2; 95% CI = 0.7 to 1.6, p < 0.0001 for annulus) and presence of neoaortic valve insufficiency (PE = 0.9; 95% CI = 0.4 to 1.5; p < 0.001 for sinus; PE = 1.6, 95% CI = 1.2 to 2.0, p < 0.0001 for annulus). CONCLUSIONS: The risk for neoaortic root dilatation was common. Long-term surveillance is mandatory, particularly in patients with double-outlet right ventricle and neoaortic valve insufficiency.

Surgical results of total anomalous pulmonary venous connection repair in 256 patients.

OBJECTIVES: This study was performed to analyse the surgical results of total anomalous pulmonary venous connection (TAPVC) repair at a single institution and to identify trends and variables associated with mortality and morbidity, particularly predictors of recurrent pulmonary venous obstruction (PVO). METHODS: Our surgical database contained 256 patients with biventricular anatomy who underwent surgical repair for TAPVC from 1981 to 2016. The anatomic TAPVC subtypes in this study were as follows: 114 supracardiac (44.5%), 56 cardiac (21.9%), 64 infracardiac (25.0%) and 22 mixed (8.6%) types. The follow-up for the entire study ranged from 1.6 months to 28.2 years (median 10.4 years). Preoperative PVO was present in 128 patients (50.0%). All patients with TAPVC with single-ventricle anatomy were excluded from the analysis. Data were retrospectively reviewed. RESULTS: Seven (2.7%) early deaths and 26 (10.1%) late deaths occurred. The actuarial survival rate at 20 years postoperatively was 85.3%. The preoperative predictors of operative mortality were younger age and the era of TAPVC repair (before 1998). In addition to these variables, associated cardiac anomalies were predictors of late mortality. Those for postoperative PVO were younger age, lower weight and being an emergency case. The actuarial survival rate at 20 years was 38.6% for patients with postoperative PVO and 92.2% for patients without postoperative PVO (P < 0.001). CONCLUSIONS: The long-term outcomes after TAPVC repair in patients with biventricular anatomy were satisfactory. Mortality was significantly associated with the rate of progression of postoperative PVO, and careful follow-up was required especially within 6 months after the primary operation.

Modification of expanded polytetrafluoroethylene valved conduit using the thin-type leaflets.

OBJECTIVES: The expanded polytetrafluoroethylene conduit with fan-shaped leaflets and bulging sinuses for right ventricular outflow tract reconstruction was modified with a newly developed thin-type expanded polytetrafluoroethylene leaflet. The purpose of this study was to evaluate the clinical outcomes and hemodynamic performance of the modified conduit. METHODS: From January 2010 to December 2013, 149 patients underwent definitive right ventricular outflow tract reconstruction using the expanded polytetrafluoroethylene conduit; the 55 patients receiving a conventional conduit (group N) were compared with the 94 patients receiving a modified conduit (group T). RESULTS: There were no conduit-related deaths, operative deaths, or reimplantations for conduit failure. The overall survival and freedom from reintervention for conduit-related reasons at 3 years were 98.2% versus 95.6% (P = .438) and 94.7% versus 97.9% (P = .954) for groups N and T, respectively. The mean peak pressure gradients were 22.6 ± 15.6 mm Hg versus 18.2 ± 11.5 mm Hg (P = .161), and in the subanalysis within small-sized conduits, they were 30.2 ± 16.5 mm Hg versus 20.4 ± 10.7 mm Hg (P = .034). Regarding conduit insufficiency, the modified conduit showed a significantly worse grade of insufficiency (P = .014) only in the subanalysis within large-sized conduits. CONCLUSIONS: Although the clinical outcomes did not differ within midterm observation, the thin-type expanded polytetrafluoroethylene leaflet was considered to be suitable for the small-sized conduits, but not for large-sized conduits, based on the comparison of the hemodynamic performance. Long-term follow-up is necessary to address the appropriate sheet type for middle-sized conduits and to estimate the durability of the thin-type leaflet.

Prolonged PR Interval at Birth Predicting the High Occurrence of Fatal Atrioventricular Block in Hypoplastic Left Heart Syndrome.

Infants with hypoplastic left heart syndrome (HLHS) are at high mortality especially when they are associated with bradyarrhythmias. However, the risk factor of developing high-grade atrioventricular block (HAVB) is still unclear. Seventy-three patients with HLHS in our institutions from 2002 to 2011 were enrolled. The survival rate was assessed by the anatomical types, treatments, occurrence of HAVB, severe tricuspid regurgitation (TR), and restrictive atrial septal defect (ASD) along with electrocardiogram findings at birth. There were 23 (32%) cardiogenic and 7 (10%) non-cardiogenic deaths. The occurrence rate of HAVB but not severe TR or restrictive ASD was higher in 30 deceased patients than in 43 survived patients [7 (23%) vs. 1 (2.3%), p = 0.0038]. The overall mortality rate was higher in patients with HAVB than in those without it (p = 0.0002). Of 7 deceased patients with HAVB, 6 HAVB occurred within 10 days post-surgery, and 3 HAVB led to the early death. The mortality rate of patients with prolonged PR (≥ 0.15 s) but not wide QRS (> 0.08 s) or prolonged QTc (> 0.43 s) at birth was higher than each without it (p = 0.0106). Multivariate analysis indicated that prolonged PR but no other variables was independently associated with the mortality (hazard ratio: 2.948, p = 0.0104). Prolonged PR at birth in HLHS infants predicts the development of fatal HAVB.

Long-Term Results of Bilateral Pulmonary Artery Banding Versus Primary Norwood Procedure.

Bilateral pulmonary artery banding (PAB) has emerged to be an attractive option as an initial procedure for the treatment of hypoplastic left heart syndrome (HLHS), and some centers report excellent survival. However, its usage is variable among institutions and its true efficacy is unknown due to reporting biases. We aimed to describe the results of bilateral PAB use, preoperative risk factors, and long-term outcomes compared with primary Norwood procedure, using a national database. Infants who underwent bilateral PAB or Norwood procedure as an initial palliation for HLHS between January 2008 and December 2012 listed in the Japan Congenital Cardiovascular Surgery Database (JCCVSD) were included. The total number of patients diagnosed with HLHS was 334. Bilateral PABs were performed for 256 patients and primary Norwood procedures for 78 patients, as an initial procedure. Actuarial 5-year survival was 59.0%. The primary Norwood procedure group had better 5-year survival [75.5%; 95% confidence interval (CI) 63.2-84.1] than the bilateral PAB group (75.5 vs. 54.0%, log-rank p < 0.001). However, the bilateral PAB group had more significant risk factors. When the risk-adjusted outcomes were evaluated, there was no significant difference between the primary Norwood group and the bilateral PAB group (76.4 vs. 78.1%, log-rank p = 0.87) in higher volume institutions. The primary Norwood group had better 5-year survival than the bilateral PAB group, but preoperative risk was higher in the bilateral PAB group. Because outcomes are comparable when performed at higher HLHS volume institutions, proper patient selection is important in achieving good long-term result.

Yasui Conversion for Repair After Left Ventricular Outflow Tract Obstruction.

We herein report two cases of progressive left ventricular outflow obstruction after primary repair of arch obstruction and ventricular septal defect that was successfully resolved with Yasui conversion. Patients who require surgical reintervention for progressive left ventricular outflow tract (LVOT) obstruction after primary biventricular repair of interruption of the aortic arch or coarctation of the aorta complex are occasionally experienced. The modified Konno procedure and Ross operation are well recognized as useful for these cases. However, in some patients, these procedures are difficult to perform because of anatomic restrictions or previous procedures. Although the indications are limited, the Yasui conversion is a safe, simple, and useful option for LVOT obstruction after primary biventricular repair.

Left Ventricular Outflow Tract Obstruction in Aortic Arch Anomalies With Ventricular Septal Defect.

BACKGROUND: The predictors of left ventricular outflow tract obstruction (LVOTO) after the repair of coarctation of the aorta or interruption of the aortic arch (CoA/IAA) with ventricular septal defect have been investigated. However, the predictors remain controversial. METHODS: We performed primary repair of CoA/IAA with ventricular septal defect for 75 patients from 1996 to 2005. Four of the 75 patients died within 5 years after primary repair without relation to LVOTO. The morphology of the aortic valve of 71 survivors was bicuspid in 23 patients and tricuspid in 48 patients. The mean follow-up was 9.2 ± 2.6 years after primary repair. RESULTS: There were 12 patients who showed LVOTO of 3.0 m/s or greater after primary repair. All of the 6 bicuspid patients demonstrated valvular aortic stenosis, and all of the 6 tricuspid patients showed discrete subvalvular LVOTO. In 5 of the 6 tricuspid patients, the aortic annular z-score before primary repair was -3.0 or less. A bicuspid aortic valve (p = 0.016) and the aortic annular z-score of -3.0 or less (p = 0.019) were significant risk factors for LVOTO after primary repair. At 10 years after primary repair, 82.6% and 95.6% of the bicuspid and tricuspid patients, respectively, were free from reoperation (p = 0.015). CONCLUSIONS: The presence of a bicuspid aortic valve and an aortic valve annular z-score of -3.0 or less before primary repair are risk factors for LVOTO, and stenotic bicuspid valves and discrete subvalvular LVOTO are the main causes of LVOTO after primary repair of CoA/IAA with ventricular septal defect. The bicuspid patients more frequently required reoperation than the tricuspid patients.

Results of extracardiac conduit total cavopulmonary connection in 500 patients.

OBJECTIVES: This single-institution study aimed to evaluate the early to mid-term outcomes of extracardiac conduit total cavopulmonary connection (EC-TCPC). METHODS: Between March 1994 and March 2014, 500 patients (median age, 3.4 years) underwent EC-TCPC at our hospital. One hundred and twenty-three patients (24.6%) showed heterotaxy, and fenestration was created in 6 patients (1.2%). The standard institutional treatment policy included postoperative anticoagulation and individualized cardiovascular medication. The mortality and morbidity rates, haemodynamic status, cardiopulmonary exercise capacity and liver examination results during the follow-up period (median, 6.7 years) were retrospectively reviewed. RESULTS: There were 2 early and 17 late deaths. The Kaplan-Meier estimated survival rate was 96.2% at 10 years and 92.8% at 15 years. Bradyarrhythmia and tachyarrhythmia occurred in 19 and 13 patients, respectively. Other late-occurring morbidities included protein-losing enteropathy in 8, thromboembolism in 5, bleeding complications in 6 and liver cirrhosis in 1 patient. The rate of freedom from late-occurring morbidities was 82.1% at 15 years. In the multivariate analysis, heterotaxy was found to be a predictor for mortality (P = 0.02), whereas age at operation was a predictor for new-onset arrhythmias (P = 0.048). In the cardiopulmonary exercise test (n = 312), the peak VO2 was 84.9 ± 17.3% of the predicted value, which tended to decrease with age (R(2) = 0.32) and elapsed time since operation (R(2) = 0.21). Postoperative cardiac catheterization (n = 468; time from surgery, 3.6 ± 4.3 years) showed central venous pressure of 9.9 ± 2.4 mmHg, ventricular end-diastolic pressure of 5.2 ± 3.3 mmHg, cardiac index of 3.4 ± 0.8 l/min/m(2) and arterial oxygen saturation of 94.2 ± 4.8%. In 101 patients who were followed up for ≥10 years, amino-terminal type III procollagen peptide and collagen type IV levels exceeded the normal ranges in 52.9 and 75.2% of patients, respectively, and liver ultrasonography revealed hyper-echoic spots in 43.3% of patients. CONCLUSIONS: The early to mid-term outcomes of post-EC-TCPC patients managed with individualized pharmacotherapy were excellent, with low mortality and morbidity rates; however, development of late-occurring morbidities specific to Fontan physiology, including exercise intolerance and liver disease, must be carefully monitored during the long-term follow-up.

[Surgical strategy and the results for hypoplastic left heart syndrome].

OBJECTIVE: We retrospectively reviewed our surgical results in patients with hypoplastic left heart syndrome( HLHS) to investigate the influence of surgical strategy on outcome. METHODS: Seventy-seven patients with classic HLHS were involved in this study. For the initial palliation, 23 patients underwent Norwood operation with modified Blalock-Taussig (BT) shunt, 23 patients underwent Norwood operation with right ventricle to pulmonary artery( RV-PA) shunt and 31 patients underwent bilateral pulmonary artery banding (BPAB). Surgical results, freedom from Fontan operation, hemodynamic data and incidence of complication after Fontan operation were compared between the 3 groups. RESULTS: BPAB group had more preoperative risk factors than Norwood group. Total actuarial survival was 62.4% at 1 year and 58.2% at 3 and 5 years, and there was no difference between the groups. Freedom from Fontan completion was 59.4% at 3 years and 48.7% at 5 years without difference between the groups. The incidence of intervention for pulmonary artery stenosis was higher in Norwood with RV-PA shunt group(52.9%). Hemodynamic data obtained by cardiac catheterization were similar in the 3 groups, however, end-systolic elastance, which represents ventricular contractility, was lower in Norwood with RV-PA group. CONCLUSIONS: Surgical results of Norwood with BT shunt and Norwood with RV-PA shunt were comparably satisfactory, however, there was possible concern of reduced ventricular contractility in RV-PA shunt group. BPAB was a effective and useful initial palliation in high risk cases.

The Yasui operation for patients with adequate-sized ventricles and ventricular septal defect associated with obstructions of the aortic arch and left ventricular outflow tract.

OBJECTIVE: To review the surgical outcome of the Yasui operation in patients with adequate-sized ventricles and ventricular septal defect (VSD) associated with obstructions of the aortic arch and left ventricular outflow tract (LVOT). METHODS: Since 1985, 17 patients have undergone the Yasui operation at our institution. Interrupted aortic arch was present in 11 patients and coarctation of the aorta/hypoplastic arch was present in 6. Twelve patients had aortic stenosis, and 5 patients had aortic atresia. The minimum diameter of the LVOT and the z-score in patients with aortic stenosis were 3.7 ± 0.4 mm and -9.2 ± 1.2, respectively. Primary repair was performed in 6 patients, and 11 patients were staged, with bilateral pulmonary artery banding (PAB) in 8, arch repair with PAB in 2 and Norwood operation in 1. The mean age and body weight at the time of the Yasui operation was 4.7 ± 5.3 months and 4.5 ± 1.8 kg, respectively. The ascending aorta and aortic arch were reconstructed by Damus-Kaye-Stansel (DKS) anastomosis with graft interposition in 2, DKS with direct anastomosis in 6 and Norwood-type reconstruction in 9. VSD was enlarged in 6 patients. Right ventricle to pulmonary artery continuity was established with a valved conduit in 14 patients, the Lecompte manoeuvre in 2 patients and another method in 1 patient. The mean duration of the follow-up was 7.6 ± 9.2 years. RESULTS: There was 1 early death due to myocardial infarction and 1 late death due to non-cardiac cause. The actuarial survival at 10 years was 87.8%. Six patients underwent reoperation, including 5 conduit exchanges, 2 LVOT repairs and 2 aortic arch repairs. The freedom from reoperation for all causes at 5 and 10 years were 71.3 and 28.5%, respectively. In the last echo study, LVOT flow velocity was 1.2 ± 0.8 m/s, and neoaortic valve regurgitation was mild in 1 patient and trivial or absent in the remaining patients. CONCLUSIONS: The results of the Yasui operation were excellent, showing low mortality and good mid-term left ventricular function without outflow tract stenosis or neoaortic valve insufficiency. Bilateral PAB as initial palliation is a useful option in symptomatic neonates.

Effects of tricuspid valve surgery on tricuspid regurgitation in patients with hypoplastic left heart syndrome: a non-randomized series comparing surgical and non-surgical cases.

OBJECTIVES: Tricuspid regurgitation (TR) remains a significant risk factor affecting the survival of patients with hypoplastic left heart syndrome (HLHS). We performed this study to investigate differences in the clinical course based on the timing of the development of TR and the effects of tricuspid valve surgery (TVS). METHODS: One hundred and five patients of classic HLHS underwent staged operations from May 1991 to July 2010. Forty-four patients (41.9%) exhibited moderate or greater TR during the follow-up. We defined the early TR group (30 patients, around the first palliative surgery) and the late TR group (14 patients, the later period) based on the timing of the appearance of moderate or greater TR. We performed TVS when moderate or greater TR was detected in 28 patients. The follow-up period was 5.5 ± 5.1 (plus/minus values are means ± SD) years (range: 0.01-14.6 years) after the first palliative surgery and 4.9 ± 4.4 years (range: 0.01-13.3 years) after TVS. RESULTS: The early TR group exhibited poorer survival than the late TR group (42.9 vs 92.9% at 5 years, P = 0.003). However, in the early TR group, the TVS significantly improved survival compared with that observed in the non-TVS cases (52.1 vs 23.3% at 5 years, P = 0.046). The right ventricular ejection fraction (RVEF) significantly decreased (62.7 ± 11.4 → 57.2 ± 12.6% (plus/minus values are means ± SD), P = 0.040) and the right ventricular end-diastolic diameter (RVDd) became significantly enlarged (27.7 ± 7.6 → 36.7 ± 3.4 mm, P < 0.001) in association with deterioration of the TR degree. TVS significantly improved the degree of TR (2.5 ± 0.5 → 1.5 ± 0.9°, P < 0.001) and RVDd (37.7 ± 7.4 → 30.4 ± 5.0 mm, P = 0.007); however, the RVEF was not improved 1 month after surgery (54.4 ± 12.1 → 54.3 ± 12.4%, P = 0.931) or at the latest follow-up (53.7 ± 14.9%, P = 0.836). CONCLUSIONS: The survival of HLHS patients who develop moderate or greater TR around the time of the first palliative surgery is worse than that of HLHS patients who develop moderate or greater TR at a later time. In this study, TVS for early TR improved survival and decreased right ventricular dimensions during the 4.9-year follow-up period.

A 17-year experience with mitral valve repair with artificial chordae in infants and children.

OBJECTIVES: We sought to examine our long-term results of mitral valve (MV) repair with expanded polytetrafluoroethylene (ePTFE) sutures and to determine the predictors for the outcome of this procedure. METHODS: Between 1995 and 2011, MV repair with chordal reconstruction by artificial chordae was achieved in 78 patients (34 males and 44 females). Median age at repair was 1.5 years (range 3.6 months-13.4) and weight was 9.1 kg (2.5-31.4). The mean follow-up was 8.3 years. A Cox proportional hazards model was used to analyse the risk factors for a composite outcome of death, conversion to other MV repair techniques or MV replacement, reoperation on MV and recurrent mitral regurgitation (MR). RESULTS: According to Carpentier classification, 65 (83.3%) patients were Type 2 and 13 (16.7%) were Type 3. Mitral annuloplasty was performed in all cases, except 2. During MV repair, 8 (10.3%) patients were ineffective with artificial chordae and converted to other techniques. Six (7.7%) patients underwent MV reoperation (three repairs and three replacements). Freedom from MV reoperation was 92.5 and 90.4% at 5 and 10 years, respectively. There was 1 in-hospital death. At the latest follow-up, moderate or more MR was observed in 3 (3.8%) patients. Risks for the composite outcome were low body weight at operation and Carpentier classification Type 3. CONCLUSIONS: MV repair with artificial chordae in infants and children is safe and effective and associated with a low reoperation rate. Further investigation into the long-term durability and biological adaptation of ePTFE sutures after patient growth is mandatory.