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OBJECTIVES A common atrioventricular valve (CAVV) is considered to be a risk factor for early and late deaths in patients with functional single ventricle (FSV). CAVV surgery in patients with FSV is challenging and there is limited knowledge of the outcomes of CAVV repair with univentricular physiology. METHODS We reviewed all CAVV surgical procedures in patients with FSV who underwent univentricular palliation. End points of the study were survival after CAVV surgery and cumulative incidence of reoperation. RESULTS Between 1984 and 2019, 66 children with CAVV and FSV underwent single-ventricle palliation, of whom 45.5% (30/66) required CAVV surgery. Indication for surgery was moderate CAVV regurgitation in 40% (12/30) and severe CAVV regurgitation in 60% (18/30). CAVV repair was performed in 93.3% (28/30) and CAVV replacement in 6.7% (2/30). The median age and weight at surgery were 0.9 years (interquartile range 0.3–1.8) and 6.5 kg (interquartile range 3.9–8.7), respectively. Operative and late mortality were 23.3% and 8.7%, respectively. Survival and cumulative incidence of reoperation at 4 years after CAVV surgery were 68.9% [standard deviation (SD): 8.7] and 35.8% (SD: 9.1), respectively. Fontan completion was achieved in 60% (18/30). Survival at 4 years after birth was 69.7% (SD: 8.5) in 30 patients with CAVV surgery, whereas it was 83% (SD: 6.3) in 36 patients without CAVV surgery (P = 0.32). CONCLUSIONS CAVV surgery in patients with FSV is associated with substantial mortality and a high incidence of reoperation.

AV canal defects (AVCD) are caused by maldevelopment of the endocardial cushions and typically include a primum atrial septal defect (ASD), an inlet ventricular septal defect (VSD), and a common atrioventricular valve. The variations in deformities provide the basis for the many terms used in the anatomical classifications: partial, transitional, intermediate, and complete common AVCD (balanced or unbalanced). The balanced complete common AVCDs are classified as Rastelli A, B, C depending on the anomaly of the anterior bridging leaflet division and attachments. Unbalanced complete AVCDs occur when the common AV valve leads primarily into the RV or LV. Echocardiographic apical, subcostal, and parasternal views are the best views to image AV canal defects. These views can help determine the type of repair required for the various AV canal defects.

A Melody valve was successfully placed across a very stenotic right-sided component of a common atrioventricular valve because of ongoing troublesome arrhythmias in a young woman with an unbalanced atrioventricular septal defect, a very dilated right atrium and a hypoplastic right ventricle. Four years later, she remains well.

Background The incidence of ventricular tachycardia (VT) in patients following Fontan operation is reported as 3.5%. Furthermore, in patients with repaired double outlet right ventricle (DORV), scar-related VT and outflow tract VT have been reported; however, Purkinje-related VT has not previously been reported. In this report, we present the case of idiopathic left VT (ILVT) in a patient with DORV who underwent Fontan operation. Case summary A 31-year-old man was diagnosed as having DORV with complete atrioventricular defect at birth. When he was 17 years old, he underwent surgical repair, including extracardiac Fontan operation and common atrioventricular valve replacement. Five years later, VT was detected. Since some medications were ineffective in suppressing VT, he was referred to our hospital for definitive treatment. Ventricular tachycardia was induced by atrial and ventricular programmed electrical stimulations. The mechanism of the VT was determined to be re-entry. The earliest activation site was located at the mid-inferior septum of the hypoplastic left ventricle, in which Purkinje potentials were observed before the local ventricular electrogram. Radiofrequency catheter ablation (RFCA) was performed at this site to eliminate VT. Discussion Most VTs originate from surgical scars in patients with congenital heart disease. Catheter ablation was feasible in scar-related VT. To the best of our knowledge, this is the first report of ILVT treated successfully with RFCA in a DORV patient who had undergone Fontan operation.

Double-outlet right atrium (DORA) is a rare clinical entity associated with atrioventricular septal defects. Although it is easily correctable by surgery, it may be misdiagnosed during echocardiographic evaluation. Awareness and a thorough knowledge of this entity, its associations, echocardiographic appearances, surgical techniques, and its embryogenesis can alert the clinician to identify this rare but correctable condition. A young patient with DORA is described in this clinical report.

Transthoracic echocardiography is the principal imaging modality for assessment of patients with atrioventricular septal defects. Three-dimensional echocardiography streamlines and simplifies data acquisition offering a unique realistic en-face display of heart valves and septal defects and enables accurate evaluation of the cardiac anatomy, dynamic, and function. We demonstrated an added value of three-dimensional echocardiography in assessment of an adult patient with atrioventricular septal defect and its advantages over conventional echocardiography.

The association of complete atrioventricular canal with transposition of the great arteries is rare, with a prevalence of less than 3–5%. We present an 18-month-old patient with a complete atrioventricular canal, side-by-side transposition of the great arteries, and anomalous coronary anatomy, managed initially with pulmonary banding and then by arterial switch with complete atrioventricular canal repair at early infancy.

Long-term outcomes in children with atrioventricular septal defect (AVSD) and univentricular palliation are of concern, with 1 Common atrioventricular valves (AVV) often become insufficient in patients with univentricular physiology, leading to heart failure. 1 , 2 We have recently observed that outcomes of children with AVSD who reach Fontan circulation are not as bad as previously thought, provided that the AVV remains competent. 1 Common AVV surgery is associated with substantial mortality and reoperation rates. 3 Although successful AVV repair is associated with better survival and freedom from reoperation, good quality repair is difficult to achieve in univentricular circulation, 3 especially in patients with dextrocardia. 4 Herein, we report a patient with unbalanced AVSD and dextrocardia who underwent AVV repair using the “polytetrafluoroethylene (Gore-Tex, W.L. Gore & Associates, Flagstaff, AZ) bridge” technique 5 with excellent early outcome.

Unbalance in atrioventricular septal defect can be found in more than one anatomic level and in different degrees at each level. The definition of “unbalance” has historically been focused in comparing the dimensions of main cardiac structures, such as the atrioventricular valve and the ventricles. However, the hemodynamic aspects of unbalance need to be considered as having, at least, similar relevance. New concepts and already described parameters must be combined and understood as a whole to help the surgical decision-making process.

Ellis-van Creveld syndrome is a rare genetic disorder, characterized by chondral dysplasia, ectodermal dysplasia, polydactyly and congenital heart defects. Patients with this syndrome rarely survive into adulthood. The syndrome has also been rarely reported in the Indian population. We present the case of a 56 year old female who presented with post-menopausal bleeding, and was diagnosed with Ellis van Creveld syndrome, with a common atrium and common atrioventricular valve.

Objectives To determine a clinical standard for post-repair common atrioventricular valve orifice area based on mid- to long-term valve function in patients with a functional single ventricle. Methods The medical records of 19 single-ventricle patients who underwent common atrioventricular valve plasty from July 1988 to January 2013 were retrospectively reviewed. Bivalvation valvuloplasty was performed in 7 patients with relatively hypoplastic leaflets. The relationship between the orifice area of the repaired common atrioventricular valve measured intraoperatively and valve function and ventricular volume in the early postoperative period (median, 9.5 months) and at mid- to long-term follow-up (median, 4 years) were analysed. Results Post-repair valve area was significantly positively correlated with valve regurgitation severity in the early postoperative period (P = 0.001, r = 0.69) and at mid- to long-term follow-up (P = 0.02, r = 0.57). Patients who did not undergo bivalvation had favourable valve function at mid- to long-term follow-up and in the early postoperative period when the post-repair valve area was 96-136% of the normal mitral valve area. Bivalvation patients had significantly more valve regurgitation in the early postoperative period than patients without bivalvation, despite equivalent repaired valve area (P = 0.02). Conclusions The post-repair orifice area of the common atrioventricular valve is significantly related to postoperative valve function. The clinical standard of post-repair valve orifice area might be 96-136% of the normal mitral valve area in patients undergoing repair without bivalvation. Patients undergoing bivalvation require greater reduction to obtain favourable mid- to long-term valve function.

OBJECTIVES: Common atrioventricular valve (CAVV) regurgitation is widely known as a risk factor for mortality and Fontan completion in patients with functional single ventricle. Hence, we reviewed our surgical experience with CAVV plasty in Fontan candidates. METHODS: Staged Fontan strategy and extracardiac total cavopulmonary connection as Fontan modification were our principal approaches in 1995. Since then, 38 consecutive Fontan candidates (21 males, median weight at operation was 7.0 kg and median age was 17 months old) underwent CAVV plasty. Right atrial isomerism was associated with 24 patients. The initial CAVV plasty was performed before inter-stage bidirectional Glenn (BDG) in 3 patients, at BDG in 23, before Fontan in 4 and during Fontan in 8. Since 1995, the modified Alfieri technique with a tailed, expanded, polytetrafluoroethylene tube as a bridging strip was the procedure for repair and 27 patients underwent the procedure. The mean follow-up period was 7.1 years (range 0–17 years). RESULTS: Actuarial survival and freedom from CAVV replacement rates at 1, 5 and 10 years were 81, 70 and 67% and 89, 85 and 75%, respectively. Seven patients ultimately underwent CAVV replacement with one death. Twenty-three of the 38 patients completed Fontan operation (61%). Association with total anomalous pulmonary venous connection (P=0.01) and CAVV plasty before BDG (P= 0.05) were risk factors for mortality. CONCLUSIONS: CAVV plasty for patients with functional single ventricle is still challenging; however, the aggressive and repeated surgical intervention may contribute to provide better life-prognosis. The ventricular volume unloading effect of BDG without additional pulmonary blood flow or Fontan operation did not contribute to maintain CAVV function. Therefore, there would not be any hesitation for CAVV replacement to control CAVVR in the setting of systemic ventricular failure. Although the statistically significant therapeutic superiority of the modified Alfieri technique was not shown so far, further follow-up may reveal the advantage of this easy and simple technique.

We present the case of a 54-year-old male with an intermediate atrioventricular septal defect (AVSD). He was referred to our institute for treatment of shortness of breath. Two-dimensional transthoracic echocardiography demonstrated an ostium primum defect of the atrial septum, a common atrioventricular valve with mild regurgitations, and an interventricular communication, which are the features of complete AVSD. However, three-dimensional transthoracic echocardiography (3D-TTE) could clearly visualize the tricuspid pouch (i.e., persistence of the tissue in the endocardial cushion), a feature of partial AVSD. 3D-TTE appears to be a useful modality for diagnosis of intermediate AVSD.

A 6-year-old girl with unbalanced atrioventricular septal defect, hypoplastic right ventricle, and severe common atrioventricular valve regurgitation developed patient-prosthetic mismatch. At 6 months old, she underwent primary one and one-half ventricle repair and replacement of left side atrioventricular valve. A catheter examination showed that her right ventricular end-diastolic volume increased from 39.4 mL/m2 1 year after to 70 mL/m2 3.5 years after the previous surgery. Thus, at the timing of redo left side atrioventricular valve replacement, she was converted successfully to biventricular circulation. The postoperative course was uneventful, and the right atrial pressure was 7 mm Hg before discharge.

Objectives To review long-term outcomes of patients with right atrial (RA) isomerism who underwent common atrioventricular valve (CAVV) plasty. Methods We retrospectively analysed 59 patients with RA isomerism operated on between January 2004 and April 2016. We divided patients into those with CAVV plasty (CAVV plasty (+), 29 patients) and without CAVV plasty (CAVV plasty (-), 30), and we compared the outcome between the groups. We further divided patients into those with CAVV plasty before bidirectional cavopulmonary shunt (BCPS) operation (group before BCPS, 13 patients) or CAVV plasty with or after BCPS (group with or after BCPS, 16), and we compared the outcome between these groups. We reviewed the outcomes of 7 neonatal patients who underwent CAVV plasty. Results Kaplan-Meier estimated survival rates at 10 years were 70 ± 10% and 69 ± 9% in the CAVV plasty (+) and CAVV plasty (-) groups, respectively ( P = 0.45). Kaplan-Meier estimated survival rates at 10 years were 47 ± 17% and 85 ± 10% in the group before BCPS and group with or after BCPS, respectively ( P = 0.01). Among 7 neonates in the group before BCPS, 4 are alive; Kaplan-Meier estimated survival rates at 1 year and 5 years were 60 ± 20% and 30 ± 24%, respectively. Conclusions Patients who underwent CAVV plasty with or after BCPS had good outcomes; the outcome of patients with CAVV plasty was the same as that of those without CAVV plasty. Treatment for patients who require CAVV plasty before BCPS, especially neonates, is challenging.

Biventricular repair in right atrial isomerism is rarely feasible due to associated anomalies of venous connection, ventricular imbalance, nonroutabilty of the interventricular communication, a common atrioventricular junction, and inadequate pulmonary arterial branches. These patients are also often not ideal for univentricular repair due to some of the above associations. We describe a novel surgical technique that was utilized in such a patient for biventricular repair of a child with right atrial isomerism with total anomalous pulmonary venous connection, regurgitant common atrioventricular valve, hypoplastic left ventricle, nonroutable ventricular septal defect, and pulmonary stenosis.

The study objective was to determine the mechanisms of atrioventricular valve regurgitation in single-ventricle physiology and their influence on outcomes after total cavopulmonary connection.Among 460 patients who underwent a total cavopulmonary connection, 101 (22%) had atrioventricular valve surgery before or coincident with total cavopulmonary connection.Atrioventricular valve morphology showed 2 separated in 33 patients, mitral in 11 patients, tricuspid in 41 patients, and common in 16 patients. Patients with a tricuspid and a common atrioventricular valve underwent atrioventricular valve surgery frequently, 27% and 36%, respectively. Atrioventricular valve regurgitation was due to 1 or more of the following mechanisms: dysplastic leaflet (62), prolapse (53), annular dilation (27), cleft (22), and chordal anomaly (14). Structural anomalies were observed in 89 patients (88%). The procedure was atrioventricular valve repair in 81 patients, atrioventricular valve closure in 16 patients, and atrioventricular valve replacement in 4 patients. Among 81 patients who underwent initial repair, repeat repair was required in 20 patients, atrioventricular valve replacement was required in 7 patients, and atrioventricular valve closure was required in 3 patients. Among patients undergoing atrioventricular valve surgery, overall survival after total cavopulmonary connection (88% vs 95% at 15 years, P = .01), freedom from atrioventricular valve reoperation after total cavopulmonary connection (75% vs 99% at 15 years, P .01), and grade of atrioventricular valve regurgitation at a median follow-up of 6.6 years (P .01) were worse than in those who did not require atrioventricular valve surgery.Atrioventricular valve regurgitation in univentricular heart is more frequently associated with a tricuspid or a common atrioventricular valve, and structural anomalies are the primary cause. Significant atrioventricular valve regurgitation requiring surgery influences survival after total cavopulmonary connection, especially when atrioventricular valve replacement was needed. Surgical management based on mechanisms of regurgitation is mandatory.

The clinical detail, echocardiographic features, angiographic evaluation, and surgical repair of double-outlet left atrium (LA) in a 2-year-old boy are discussed. In a solitus position and normally connected systemic and pulmonary venous drainage setting, the only outlet of the right atrium was a secundum atrial septal defect. The LA thus received the entire venous return and then drained into both ventricles through a common atrioventricular valve.

A complete atrioventricular canal defect occurs when an embryonic common atrioventricular valve fails to divide completely into 2 common atrioventricular valves. Tetralogy of Fallot is an abnormal embryologic development in which an unequal conotruncal division results in a small pulmonary artery and a large aortic artery. We report a case of prenatal diagnosis of a complete atrioventricular canal defect associated with tetralogy of Fallot. © 2007 Wiley Periodicals, Inc. J Clin Ultrasound, 2008

Double inlet left ventricle (LV) is a type of atrioventricular connection in which the morphologically LV receives more than 50% of the atrioventricular valves when they are separate, or more than 75% of a common atrioventricular valve. The aim of this study was to establish an anatomoechocardiographic correlation between the morphologic features of equivalent anatomic specimens and the echocardiographic images of patients to provide a means of interpreting the image correctly and a more precise diagnosis of the cardiac defect. Echocardiography was used to study 18 patients with LV double inlet who were seen in a congenital heart disease clinic. The morphology of 17 hearts with this malformation from the department of embryology was analyzed to compare the anatomic features with their echocardiographic images. Echocardiography proved to be a noninvasive diagnostic tool that allowed characterization of anatomic and functional aspects of double inlet LV.

We describe an atypical case of an atrioventricular septal defect with a common atrioventricular junction in which the right-sided component of the common atrioventricular valve was imperforate, producing tricuspid atresia with a severely hypoplastic right ventricle and an ostium primum defect. We discuss the implications of the anatomic findings with regard to concepts of cardiac development, drawing a comparison with similar cases previously reported.

The Fontan procedure in one of its many modifications is applicable to the surgical treatment of those patients with double-outlet right ventricle who are not amenable to bi-ventricular correction. Included in this group are patients with significant hypoplasia of one of the ventricles, patients with distant (non-committed) ventricular septal defect, patients with significant straddling of the atrioventricular valves, patients with abnormal atrioventricular connections (such as absence of one of the valves), or common atrioventricular valve connection. The presence of complex associated lesions such as those seen in the heterotaxia syndromes may, by virtue of the surgical risk of attempts at bi-ventricular repair, make the Fontan approach a suitable option for these patients. The presence of a subaortic conus and the potential for subaortic obstruction, a frequent findings in patients with double outlet right ventricle, plays an important role in the preparation and conduct of the Fontan operation. Copyright © 2000 by W.B. Saunders Company

One patient with major aorto-pulmonary collateral arteries and heterotaxy underwent staged unifocalizations, and eventually the staged Fontan completion. Subsequent to the bidirectional Glenn procedure, the left pulmonary artery was once thrombozed, but successfully treated. The progressively regurgitant common atrioventricular valve needed repair twice during the course. Pulmonary arterial pressure was 11 mm Hg 1 year after the Fontan procedure.

A common atrioventricular valve (CAVV) presents a challenge in single-ventricle palliation (SVP) because of its propensity to leak, with CAVV regurgitation being an important risk factor for repeated operation and mortality. We propose a new technique of CAVV repair in the context of SVP. The method uses the techniques of complete repair of antrioventricular septal defect while maintaining free communication at atrial and ventricular levels necessary for single-ventricle physiology.

Significant common atrioventricular valve insufficiency is associated with increased mortality and morbidity in patients with univentricular physiology. Shortly after bidirectional cavopulmonary anastomosis and concomitant valvuloplasty, valve replacement was carried out using a floating annuloplasty technique reinforced by a Dacron strip in a patient with common atrioventricular valve insufficiency. The technique applied is described in detail.

Introduction Few studies investigated the long-term outcome of patients with univentricular heart and common atrioventricular valve. Method We retrospectively analysed the medical files of all patients univentricular heart with common atrioventricular valve in the setting of heterotaxy or with unbalanced atrioventricular septal defect preventing biventricular repair. Results 117 patients were identified during the study period. 89/117 had a postnatal diagnosis. 28/117 patients never underwent surgery, 25/117 underwent one palliation surgery (Blalock-Taussig-shunt (BTS)/ pulmonary banding), and finally, 61/117 patients entered a sequential cavopulmonary connection program: 37/61 had partial cavopulmonary connection at the time of data analysis while 24/61 had total cavopulmonary connection (TCPC). The average age at TCPC was 7.6 years +/-4 years [1.7-16 years]. Three patients were eventually transplanted. The overall mortality was 59% (69/117): 65% and 30% in heterotaxy and in patients with unbalanced atrioventricular septal defect respectively. Mortality was 85% (24/28) in the subgroup of patients who never underwent surgery, 93% in the subgroup of patients who had a BTS, and 89% after pulmonary banding. In the subgroup planed to have TCPC, 49% died after partial cavopulmonary connection and survival rate was 71.6% [50.7-100] in patients who had TCPC. Conclusion The long-term outcome of univentricular hearts with common atrioventricular valve treated in a tertiary referral center showed a high mortality rate. Patients with this kind of complex congenital heart disease should undergo the sequential univentricular program without delay in order to obtain better long-term survival.

Long-term left atrioventricular valvar function was evaluated in 95 of 110 survivors following surgical repair of atrioventricular septal defect between 1975 and 1984. A common or complete form was present in 40 and a partitioned or partial form in 55 patients. The patients have been followed for three to 13 years with a mean of 8.3 years. Pulmonary arterial banding was performed in 17 patients with a common atrioventricular valve prior to complete repair. The left atrioventricular valvar regurgitation was evaluated by clinical examination, Doppler and/or angiography. Three patients required valvar replacement postoperatively. Previous pulmonary arterial banding, pulmonary hypertension or pulmonary-to-systemic flow ratio did not affect the incidence or severity of left atrioventricular valvar regurgitation postoperatively. In this series left atrioventricular valvar regurgitation increased in the early postoperative period but rarely progressed at late follow-up.

A case of an infant with a rare combination of polysplenia syndrome with common atrioventricular canal and persistent truncus arteriosus is presented. In our present case, severe common atrioventricular valve regurgitation was identified, as in previous cases. To our knowledge, echocardiographic and autopsy findings of this association has not been previously reported. The persistent truncus arteriosus is extremely rare in the setting of the polysplenia syndrome, but the present case report demonstrates that these anomalies may, at times, occur.

A 4-month-old infant with trisomy 21 and repaired complete atrioventricular septal defect developed endocarditis with methicillin-resistant Staphylococcus aureus in the early postoperative period. We report the successful replacement of the common atrioventricular valve with a single St. Jude mechanical prosthetic valve, along with an intraluminal pulmonary artery banding to restrict pulmonary flow.

This study was performed to elucidate the anatomic features of double-inlet ventricle in the Chinese population. The echocardiograms of 60 Chinese patients diagnosed as having double-inlet ventricle were reviewed and analyzed. Both atria were connected to a dominant right ventricle in 36 patients (60%), to a dominant left ventricle in 17 (28%) and to an indeterminate ventricle in 7 (12%). Right atrial isomerism was present in 30 patients (83%) with double-inlet right ventricle, in 5 (29%) with double-inlet left ventricle, and in 6 (86%) with double-inlet indeterminate ventricle. Right atrial isomerism was more frequently associated with double-inlet right and indeterminate ventricles (p < 0.01; chi-square). Common atrioventricular valve was frequently present in both double-inlet right and indeterminate ventricles. Double-outlet main chamber was the predominant type of ventriculoarterial connection in double-inlet right and indeterminate ventricles. Total anomalous pulmonary venous connection was found exclusively in 9 patients with right atrial isomerism (5 with double inlet right and 4 with double-inlet indeterminate ventricles). In contrast to other reports, this series found double-inlet right ventricle to be the predominant type of double-inlet ventricle. This discrepancy may be due to a greater prevalence of right atrial isomerism that is usually associated with double-inlet right ventricle in Chinese patients.

Corrected transposition of the great arteries with a common atrioventricular valve is a rare congenital anomaly. In the presence of two well-defined, atrioventricular valves, cross-sectional echocardiography is the method of choice for the diagnosis of this cardiac defect. The presence of a common atrioventricular valve can be a limitation of the method. Angiocardiography provides additional information for the diagnosis. (ECHOCARDIOGRAPHY, Volume 8, September 1991)

The repair of atrioventricular septal defect with a common atrioventricular valve is reconstructive surgery at its best, and hence one of the favourite operations performed by paediatric cardiac surgeons. In the past, the post-operative course from such patients was dominated by the occurrence of pulmonary hypertension crises, which were responsible for significant morbidity and mortality. Nowadays, repair is generally undertaken early in infancy, and this approach has mitigated the problems emanating from pulmonary hypertension. Coupled with a better understanding of the anatomy, and adaptation of the surgical techniques, repair can now be achieved safely at around 2 to 4 months of life, without increasing the risk of postoperative regurgitation across the reconstructed left atrioventricular valve. In this review, we discuss the surgical techniques required for, and clinical results of, such early repair.

Atrioventricular valve regurgitation is one of the predictors of adverse outcomes after the Fontan procedure. We describe our surgical technique of GoreTex (W. L. Gore & Associates, Inc., Flagstaff, AZ, USA) bridge to repair a common atrioventricular valve in single-ventricular circulation. The repair includes a GoreTex strip that is secured to the mid-line of both superior and inferior bridging leaflets and annulus to obtain a better coaptation of the leaflets and prevent further dilatation of the annulus. We have applied this technique for 7 consecutive patients with excellent outcomes.