Mitral Cleft Research Articles

Presentation of mitral valve cleft with concurrent atrial septal defect and ventricular septal defect detected by three-dimensional transesophageal echocardiography: a case report

BackgroundCleft in the mitral valve leaflet is a primary cause of congenital mitral regurgitation, stemming from developmental anomalies in the mitral valve and frequently associated with other congenital heart defects. Concurrent presence of cleft in mitral valve leaflet with atrial septal defect and ventricular septal defect is relatively rare. Echocardiography, especially transesophageal echocardiography, is essential in diagnosing cleft mitral valve leaflet and related congenital heart defects, providing critical, detailed imagery for accurate assessment. This study presents a young female patient whose anterior mitral cleft, along with atrial septal defect and ventricular septal defect, was revealed through three-dimensional transesophageal echocardiography.Case presentationA 25-year-old Iranian female, experiencing progressive dyspnea and diminished physical capacity over 3 months, was referred to our hospital. Initial examination and transthoracic echocardiography indicated severe mitral regurgitation. Further evaluation with transesophageal echocardiography corroborated these findings and identified a cleft in the anterior mitral valve leaflet, coupled with mild left ventricular enlargement and significant left atrial enlargement. The complexity of the patient’s condition was heightened by the diagnosis of cleft mitral valve leaflet in conjunction with atrial septal defect and ventricular septal defect, showing the complex nature of congenital defects.ConclusionThis case emphasizes the critical role of transthoracic echocardiography in diagnosing cleft of mitral valve leaflet and associated cardiac anomalies, showcasing its superiority over transthoracic echocardiography for detailed visualization of cardiac structures. The identification of multiple congenital defects highlights the necessity for a comprehensive diagnostic approach to manage and treat patients with complex congenital heart diseases effectively. Future research should aim to refine diagnostic methodologies to enhance patient outcomes for cleft of mitral valve leaflets and related congenital conditions.

The significance of 3-D echocardiography in identifying mitral clefts

The significance of 3-D echocardiography in identifying mitral clefts

Percutaneous Mitral Cleft Repair with MitraClip: An Interesting Case of Owl Eye Appearance in the Mitral Valve

Percutaneous Mitral Cleft Repair with MitraClip: An Interesting Case of Owl Eye Appearance in the Mitral Valve

An Unusual Presentation of Partial Atrioventricular Canal Defect in an Elderly Woman

A partial atrioventricular canal defect (PAVCD) is a form of endocardial cushion defect. It’s a rare congenital cardiac malformation, few reported patients have survived into the seventh decade of life. A 71-year-old female with a history of diabetes and hypertension was admitted for heart failure (HF) symptoms. Transthoracic echocardiography revealed a left to right shunt in the basal portion of the interatrial septum, 19mm ostium primum atrial septal defect (ASD), a mitral valve cleft, and severe mitral valve regurgitation. PAVCD consists of an ostium primum ASD and the dividing of the common atrioventricular (AV) valve into 2 separate AV orifices, resulting in a so-called cleft in the mitral valve. The ventricular septum is intact. Echocardiography is the key tool for diagnosis and anatomic classification. Surgical repair is recommended in adults with primum ASDs causing impaired functional capacity, right atrial or right ventricular enlargement, and left-to-right shunt without cyanosis, as long as systolic pulmonary artery pressures are lower than 50% of systemic pressures. Reconstructive surgery includes, in addition to the closure of the interatrial septum, a suturing of the mitral cleft. The estimated mean age at death of the patients with ostium primum ASD is about 40 years. The objective of this clinical case is to highlight the good clinical tolerance of atrial shunts and the anatomical particularities in the PAVCD.

Off-label Use of ADO II® in the Closure of Various Congenital Heart Defects

Devices may be used for special purposes different than their production purpose. For instance, Amplatzer Ductal Occluder is actually designed for duct closure and its usage for closing defects other than ductus is named as off-label. The aim of this study is to emphasize off-label use of device: not only for PDA and VSD but also for other various defects. This study is designed retrospectively, performed by the evaluation catheterization records of patients in whom ADO II and ADO II-AS devices were used in Erciyes University Medical Faculty Children Hospital, Pediatric Cardiology Department between 2011 and 2018. Patients’ demographic criteria: age, weight at the time of procedure was gathered. The diagnosis, size of device, follow-up period and complications were also noted. From April 2011 to March 2018, a total of 121 patients underwent transcatheter closure by ADO II and 66 patients by ADO II AS. The number of PDA closure with ADO II was 48; with ADO II AS were 62. Rest of the procedures were all off-label. Types of off-label procedures performed were: VSD closure, residual mitral cleft closure, Aorta-Right atrium tunnel closure, pulmonary arteriovenous fistula occlusion, aorta-pulmonary window closure, and occlusion of the artery feeding accessory lobe in scimitar syndrome, Gerbode defect occlusion. Up to our knowledge; this study includes the largest pediatric case series with various different congenital heart defects which were closed with ADO-II. Also our ADO-II occluded VSD case series is one of the largest series in the literature with almost 6 years’ follow-up. We believe in that ADO-II device may be an alternative in percutaneous closure of various rare heart defects. It is used successfully for non-ductal defects with low complication and high compliance rates.

Can Right Infra-Axillary Vertical Thoracotomy Make a Big Difference in Surgical Technique Preference?

Minimally invasive techniques in the surgical treatment of congenital heart diseases have gained popularity recently. Right infra-axillary vertical thoracotomy (RIAVT) is one of these techniques. The aim of this study is to investigate the postoperative morbidity and cosmetic results of the right infraaxillary thoracotomy technique and compare them with the results in the literature. All patients who had undergone cardiac surgery using RIAVT at the Ankara City Hospital Pediatric Cardiovascular Surgery Clinic between 2019 and 2021 were enrolled in the study. Preoperative echocardiographic diagnoses, intraoperative surgical data and postoperative morbidity data of the patients was obtained. In addition, surgical incision length was measured and recorded for the evaluation of cosmetic outcome. This data was then compared with data from similar studies in the literature. Between January 2019 and December 2021, 59 patients were operated on using RIAVT. Of the RIAVT patients, 45 (76%) were female and 14 (24%) were male. The mean weight of the patients was 22.38±12.48 kg. Although the youngest patient was a 5-month-old infant weighing 4 kg, the oldest one was 15-years-old weighing 57 kg. Isolated secundum atrial septal defect (ASD) repair (primary or patch) was performed in 47 patients. One (1) patient with Primum ASD and mitral cleft, 1 patient with secundum ASD and perimembranous ventricular septal defect (VSD) repair, nine patients with high venosum ASD and PAPVC, and one patient with a discrete subaortic membrane were operated on. The surgical incision length was between 4 to 5 cm. RIAVT is an important surgical alternative to median sternotomy in selected cases with certain pathologies. It can be performed safely in various congenital heart anomalies and provides excellent functional and cosmetic results in the long-term when compared to other surgical methods.

Mitral valve anomalies in transposition of the great arteries: Anatomical features, surgical management and outcomes

Mitral valve (MV) anomalies can be associated with transposition of the great arteries (TGA), but their influence on the choice of the surgical technique and the outcomes after anatomical repair, mainly mitral regurgitation (MR) and LVOT obstruction, have rarely been addressed. Our study objectives are to describe the MV anomalies found in patients with TGA, the different surgical procedures performed and the fate of the anomalous MV. From 1990 to 2020, 52 patients out of 1590 (3.3%) were identified. Main MV anomalies were a mitral cleft (88%) and anomalies of the subvalvular apparatus (60%) such as abnormal chordae attachments (16/52, 30.8%), straddling (6/52, 11.5%), accessory tissue (7/52, 13.5%) or an abnormal papillary muscle (4/52, 7.7%). The mitral cleft was described as anterior (ejection/outflow tract) in 40 and as AVSD-type in 6. A ventricular septal defect (VSD) was associated in 88.5% (58% outlet) ( Fig. 1 ). Main surgical procedure performed at repair was the arterial switch operation (ASO) ( n = 47, 90.4%). Two patients (3.8%) underwent a Bex-Nikaidoh procedure, 2 (3.8%) a half turned truncal rotation and 1 (1.9%) a “reparation à l’étage ventriculaire” (REV) procedure. Overall survival was 90% at 1 year and 88% at 10 and 20 years during a mean follow-up period of 9.5 years. Ten patients (19.2%) had a concomitant mitral procedure at repair (cleft closure in 7, procedure on the subvalvular apparatus in 6), of which 4 had a preoperative grade ≥ 2 MR. Five patients required reoperation for MR and/or for left ventricle outflow tract obstruction (LVOTO), none of them had previously undergone a mitral procedure at repair. All five underwent cleft closure, with LVOTO relief in 3. Freedom from MV reoperation was 96% at 1 year, 93% at 5 years, and 84.5% at 20 years. Main MV anomalies in TGA are the presence of an anterior cleft and abnormalities of the subvalvular apparatus, frequently associated with an outlet VSD. Mortality and reoperation rates are higher than in the other subtypes of TGA. Mitral repair at initial surgery should be considered only in cases of MR or LVOTO. Reoperation involves cleft closure and LVOTO relief. No MV replacement was required.

Mini right axillary thoracotomy for congenital heart defect repair can become a safe surgical routine.

Owing to its obvious cosmetic appeal, minimal invasive repair of congenital heart defects (CHDs) through the mini right axillary thoracotomy is becoming routine in many centres. Besides cosmesis, and before becoming a new norm, it is important to establish its outcomes as safe compared to repairs through traditional median sternotomy. Between 2013 and 2021, 116 consecutive patients underwent defect repairs through mini right axillary thoracotomy. Patient, operative data, and hospital outcomes were compared to contemporary mini right axillary thoracotomy and sternotomy series. There was no mortality or need for approach conversion (mean age 4.3 years, range 0.17-17, mean weight 18.6 kg, range 4.8-74.4) in 118 repairs for atrial septal defect, ventricular septal defect, partial anomalous pulmonary venous return, partial atrioventricular canal with mitral cleft, scimitar syndrome, double-chambered right ventricle, cor triatriatum, and tricuspid valve repair. Protocol included on-table extubation, achieved in 97 children, with 23 outliers leading to 0.7 average hours of mechanical ventilation (range 0-66 hours), indwelling chest drain time of 2.6 days (range 1-9 days), intensive care stay of 1.8 days (range 1-10 days), and hospital stay of 3.9 days (range 2-18 days). Late revisions were required in one patient after scimitar repair for scimitar vein stenosis at 2 weeks, and in another for repair of superior caval vein stenosis after a Warden operation at 2 months; reoperations (5/116 = 4.3%) were successfully performed through the same mini right axillary incision. While providing obvious cosmetic advantages, the minimally invasive right axillary thoracotomy approach for the surgical repair of common CHDs yields excellent results and is safe compared to the benchmark median sternotomy approach.

3D assessment of the residual cleft of the left atrioventricular valve after atrioventricular septal defect repair

Assessment of the residual cleft of left atrioventricular valve after the surgical correction of atrioventricular septal defect (AVSD) with 3D transthoracic echocardiography (TTE) and its correlation with mitral valve regurgitation (MVR) severity. We included 20 patients who underwent a 3D TTE after surgical repair of AVSD. MVR severity was evaluated on TTE by two different operators. The length of the residual mitral cleft was assessed compared to the total anterior leaflet depth using a multiplanar reformatted (MPR) mode. The median age at surgery was 1.5 years old (range: 1 month–8 years). Seven patients had ventricular septal defect, including 2 Down syndrome. All patients except one had surgical complete closure of the cleft. Eleven patients had an associated cardiac procedure and commissuroplasty was performed in 4 patients. The median follow-up period was 6.1 years (range: 0.3–12.5 years). The mean MVR severity was mild (1.1; range: 0–3) without significant stenosis. 3D analysis was feasible in 18 patients whom 14 had residual cleft. Residual cleft length was 16.2 mm (range: 0 mm–46 mm). Percentage of residual cleft related to the total anterior leaflet depth was 9.5% (range: 0%–41.6%). Correlation between the residual cleft and MVR severity was very good (R = 0.82; P < 0.0001). One patient had mild regurgitation due to dysplastic leaflet and no residual cleft (Fig. 1). Residual cleft assessed by 3D TTE is common after AVSD and correlated to MVR severity. Even after a complete repair, the AVSD may be uncompletely corrected, due to either a partially sutured cleft or a dehiscence of the leaflet sutures. The long-term prognosis of the left atrioventricular regurgitation has to be assessed.

Isolated Cleft of the Posterior Mitral Leaflet in 8 Year Old Patient

Cleft of the mitral valve is a rare cause of the congenital mitral regurgitation. Isolated cleft is uncommon finding and is more often found associated with some form of endocardial cushion defect. Cleft of the septal leaflet is found much more often than the defect in the posterior leaflet. Mitral clefts can extend to the mitral annulus or they can be less ''deep'' and show only minor regurgitation. Clinical importance of the isolated cleft of the mitral valve (ICMV) is that it can be successfully corrected by surgery and that it can have good long-term results.

3D assessment of the residual cleft of the left atrioventricular valve after atrioventricular septal defect repair

Residual mitral valve regurgitation is frequent after surgical repair of atrioventricular septal defect (AVSD). We aimed to assess residual mitral valve regurgitation (MVR) severity after surgical repair of AVSD and to correlate it with residual cleft assessed by 3D transthoracic echocardiography (TTE). We included 20 patients who underwent a 3D TTE after surgical repair of AVSD. MVR severity was evaluated on TTE by two different operators. The length of the residual mitral cleft was assessed compared to the total anterior leaflet depth using a multiplanar reformatted (MPR) mode from 3D TTE dataset. The median age at surgery was 1.5 years old (range, 1 month–8 years). Seven patients had ventricular septal defect, including 2 Down syndrome. All patients except one had surgical complete closure of the cleft. Eleven patients had an associated cardiac procedure and commissuroplasty was performed in 4 patients. The median follow-up period was 6.1 years (range, 0.3–20years). The mean MVR severity was mild (1.1; range, 0-3) without significant stenosis. 3D analysis was feasible in 18 patients whom 14 had residual cleft. Residual cleft length was 16.2 mm (range, 0mm–46 mm). Percentage of residual cleft related to the total anterior leaflet depth was 9.5% (range, 0%-41.6%). Correlation between the residual cleft and MVR severity was very good ( R = 0.82; P < 0.0001). One patient had mild regurgitation due to dysplastic leaflet and no residual cleft ( Fig. 1 ). Residual cleft assessed by 3D TTE is common after AVSD and correlated to MVR severity. Even after a complete repair, the AVSD may be incompletely corrected, due to either a partially sutured cleft or a dehiscence of the leaflet sutures. The long-term prognosis of the left atrioventricular regurgitation has to be assessed.

3D assessment of the residual cleft of the left atrioventricular valve after atrioventricular septal defect repair

Assessment of the residual cleft of left atrioventricular valve after the surgical correction of atrioventricular septal defect (AVSD) with 3D transthoracic echocardiography (TTE) and its correlation with mitral valve regurgitation (MVR) severity. We included 20 patients who underwent a 3D TTE after surgical repair of AVSD. MVR severity was evaluated on TTE by two different operators. The length of the residual mitral cleft was assessed compared to the total anterior leaflet depth using a multiplanar reformatted (MPR) mode ( Fig. 1 ). The median age at surgery was 1.5 years old (range, 1 month–8 years). Seven patients had ventricular septal defect, including 2 Down syndrome. All patients except one had surgical complete closure of the cleft. Eleven patients had an associated cardiac procedure and commissuroplasty was performed in 4 patients. The median follow-up period was 6.1 years (range, 0.3–12.5 years). The mean MVR severity was mild (1.1; range, 0–3) without significant stenosis. 3D analysis was feasible in 18 patients whom 14 had residual cleft. Residual cleft length was 16.2 mm (range, 0 mm–46 mm). Percentage of residual cleft related to the total anterior leaflet depth was 9.5% (range, 0%–41.6%). Correlation between the residual cleft and MVR severity was very good (R = 0.82; P < 0.0001). One patient had mild regurgitation due to dysplastic leaflet and no residual cleft. Residual cleft assessed by 3D TTE is common after AVSD and correlated to MVR severity. Even after a complete repair, the AVSD may be incompletely corrected, due to either a partially sutured cleft or a dehiscence of the leaflet sutures. The long-term prognosis of the left atrioventricular regurgitation has to be assessed.

MitraClip Implantation in a Patient With Post-Surgical Repair of Primum Atrial Septal Defect and Residual Mitral Cleft

This paper presents the case of a 67-year-old female with primum atrial septal defect and congenital mitral cleft status-post surgical repair 40 years previously who was recently found to have severe mitral regurgitation. Percutaneous mitral valve repair was successfully performed using implantation of 2 MitraClips with mild residual mitral regurgitation. (Level of Difficulty: Advanced.)

Additional cleft mitral valve diagnosed by a combination of 2-D and 3-D echocardiography using transesophageal echocardiography during mitral valve prolapse: a case report

BackgroundA mitral cleft may be an important etiological factor for significant mitral regurgitation. We diagnose an additional cleft mitral valve by a combination of 2-dimensional (2-D) and 3-dimensional (3-D) echocardiography.Case presentationWe describe the case of a severe mitral regurgitation due to posterior leaflet prolapse (P2). In the 2-D view, which is obtained after turning the probe clockwise from the mid-esophageal long-axis view, TEE showed a moderate central regurgitation jet. In the 3-D en face view, a cleft between P2 and P3 was identified, and we found that the cause of mitral regurgitation was not only P2 prolapse but also a cleft between P2 and P3.ConclusionA complex mitral valve lesion was detected by a combination of 2-D and 3-D TEE. The presence of a cleft could affect the surgical procedure because of the possibility that an enlarged cleft would increase with leaflet resection.

Mitral cleft endocarditis presenting with confusion.

Mitral cleft endocarditis presenting with confusion.

Long-term outcomes following partial atrioventricular septal defect (AVSD) repair in Ireland.

We describe the long-term results of partial atrioventricular septal defect (AVSD) repair in a single centre encompassing a 22-year period. Described are rates of survival, reoperation and complications. We performed a retrospective review of 556 patients undergoing AVSD repair to identify the 51 patients who underwent partial AVSD repair in Our Lady's Children's Hospital, Crumlin, Ireland, between 1993 and 2015 with long-term follow-up where available. A total of 29 (56.8%) of patients were male and mean age at operation was 3.32years. Mean weight was 13.2kg. Trisomy 21 was present in 29 (56.8%). Five patients (9.6%) had undergone prior surgery. Mean cardiopulmonary bypass time was 89 ± 36min and mean aortic cross-clamp time was 57 ± 28min. One patient underwent partial AVSD repair and concomitant tracheal resection and extracorporeal membrane oxygenation decannulation. One patient was managed with suture atrial septal defect (ASD) closure, the remainder with patch repair of ASD and mitral cleft closure. The length of hospital stay was 9 ± 5days. Median follow-up was 6.06years (IQR, 1.65-10.2years). There were no early mortalities. One patient died 1year following surgery (1.9%). One patient required reoperation at an interval of 2years for severe mitral regurgitation (1.9%). Short- and long-term survival following partial AVSD repair in Ireland revealed excellent results compared with other published series. Reoperation incidence also compared excellently with other reports published in the literature.

Invited Commentary

Invited Commentary

Post-Cardiac Surgery Localized Alveolar Hemorrhage: A Diagnostic Challenge

SESSION TITLE: Fellow Case Report Poster - Cardiac and Transplant Surgery SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Alveolar hemorrhage is associated with syndromes causing capillaritis, diffuse alveolar damage or bland pulmonary hemorrhage. We present a case of localized alveolar hemorrhage following cardiac surgery. CASE PRESENTATION: A 23-year old woman with a history of primum type atrial septal defect repair and mitral cleft closure presents as an adult with left bronchial stenosis associated with a vascular ring and RVOT obstruction. She underwent ductus arteriosus ligation, RVOT augmentation and stenting of left bronchus. She returned with severe mitral regurgitation associated with hemoptysis and chest pain. She underwent mitral valve repair and uproofing of the coronary sinus which had been previously baffled to the left atrium during repair of her primum ASD. Post operatively, she had persistent hypoxemia and hemoptysis. Chest X-ray and non-contrast computerized CT scan revealed infiltrates worse on the right. Bronchoscopy with serial lavages showed bloody fluid consistent with alveolar hemorrhage. A trans esophageal echocardiogram was technically difficult but appeared to show patent right sided pulmonary veins. However, subsequent CT scan with contrast demonstrated no flow in the right-sided pulmonary veins. Urgent repeat surgery revealed a single, right-sided pulmonary vein orifice with possible narrowing near the left atriotomy. We removed extensive clot within the pulmonary venous system and reconstructed the junction between the pulmonary vein and left atrium. During this time she developed thrombocytopenia suggesting the possibility of heparin induced thrombocytopenia and thrombosis. However the serotonin release assay was negative. DISCUSSION: Alveolar hemorrhage is an infrequently reported complication following cardiac surgery. It is usually diffuse and considered to be the result of inflammation or drug reaction. This patient presented with localized alveolar hemorrhage secondary to unilateral pulmonary venous thrombosis. The etiology of her clot development was likely mechanical from low flow through a narrowed orifice. The process improved following surgical clot removal and post-operative anticoagulation. CONCLUSIONS: The differential diagnosis of alveolar hemorrhage in patients who have recently undergone cardiac surgery should include pulmonary venous thrombosis, particularly when hemorrhage is localized Reference #1: A case of diffuse alveolar hemorrhage developing after open heart surgery. Nakamura Y, Shimokawa S, Watanabe S, Sakasegawa K, Sakata R, Higashi M, Miyahara K. Kyobu Geka. 2001;54:892-4 DISCLOSURE: The following authors have nothing to disclose: Yashaswini Yeragunta, Luke Wiggins, Kriti Lonial, David Kelley, Craig Baker No Product/Research Disclosure Information

Impact of anatomic characteristics and initial biventricular surgical strategy on outcomes in various forms of double-outlet right ventricle

ObjectivesSurgical management of various forms of double-outlet right ventricle uses a variety of approaches depending on the underlying anatomic form. In this study, we sought to determine the risk factors of mortality and reoperation in those with double-outlet right ventricle undergoing biventricular repair, according to anatomic characteristics and initial surgical strategy. MethodsBetween 1992 and 2013, 433 patients were included in the study. Double-outlet right ventricle was classified as double-outlet right ventricle with subaortic ventricular septal defect associated with subpulmonary obstruction in 33% of patients (n = 141), with subaortic ventricular septal defect without subpulmonary obstruction in 30% of patients (n = 130), with subpulmonary ventricular septal defect in 32% of patients (n = 139), and with noncommitted ventricular septal defect in 5% of patients (n = 23). Three types of repairs were performed: (1) intraventricular baffle repair, n = 149 (34%); (2) intraventricular baffle repair with right ventricular outflow tract reconstruction, n = 163 (38%); and (3) intraventricular baffle repair with arterial switch operation, n = 121 (28%). ResultsThirty-day overall mortality was 7.4%. Early reoperation was needed in 6% of the cases. Early mortality was higher in the intraventricular baffle repair with arterial switch operation group (P = .01). Survival at 10 years was 86.2%, and freedom from reoperation at 10 years was 61.4%. At last follow-up (median, 5.7 years; 95% confidence interval, 4.5-6.6), mortality and reoperation rates were similar in the different surgical strategy groups. Late reoperation and late mortality were significantly higher in the double-outlet right ventricle with noncommitted ventricular septal defect group (P < .01). In multivariate analyses, risk factors for reoperation were concomitant surgical procedures (P = .03) and duration of cardiopulmonary bypass (P < .01). Risk factors for mortality were restrictive ventricular septal defect (P = .01), mitral cleft (P < .01), and associated coronary artery anomalies (P = .01). ConclusionsThose with the anatomic type of double-outlet right ventricle with noncommitted ventricular septal defect were at higher risk for reoperation and mortality. Intraventricular baffle repair with arterial switch operation was the surgical strategy in patients at higher risk of early death. Initial surgical strategy did not influence the late outcomes.

Double-Outlet Right Ventricle With Noncommitted Ventricular Septal Defect and 2 Adequate Ventricles: Is Anatomical Repair Advantageous?

The management of double-outlet right ventricle associated with anatomically noncommitted ventricular septal defect constitutes a surgical challenge. The limits for, and the specific outcomes after anatomical vs univentricular repair still remain to be established. Between 1993 and 2011, 36 consecutive patients presenting with double-outlet right ventricle or noncommitted ventricular septal defect (21 inlet, 10 muscular, and 5 central perimembranous) and 2 adequately sized ventricles underwent surgical repair at 2 centers. Right ventricular outflow tract obstruction was present in 18 of 36 patients (50%). A total of 21 patients had undergone previous palliative procedures. Anatomical repair (group I) by means of intraventricular baffle construction was performed in 24 (associated right ventricular outflow tract reconstruction in 12 and arterial switch in 5) at a median age of 10.5 months. Ventricular septal defect was surgically enlarged in 12 (50%) patients. The remaining 12 patients underwent univentricular repair (group II). There were 4 hospital deaths (11%), all in group I (P = 0.30 vs group II). A total of 8 of 20 patients in group I survivors underwent 13 reoperations after a median delay of 24 months: subaortic stenosis was the main cause for reoperation in 6 of 8 patients. There was 1 late death in group I and 2 late deaths in group II. The median follow-up was 5.6 years (95% CI: 0.2-9.8). The 10- year actuarial survival rate and freedom from reoperation were 74.7 ± 5% and 58 ± 5% in group I and 71 ± 7% and 70 ± 7% in group II, respectively. At the last visit, all survivors were in New York Heart Association class I-II. Univariate analysis showed that atrioventricular septal defect and isolated mitral cleft were associated with death (P = 0.04) and need for reoperation (P = 0.038). In conclusion, anatomical repair, associated with substantial rates of mortality and need for reoperation, should be considered with caution. Associated atrioventricular septal defect and isolated mitral cleft were the only risk factors for mortality and reoperation.