Complete Atrioventricular Septal Defect Research Articles

Comparison of the prenatal ultrasonic features between left and right isomerism

Objective To compare the prenatal ultrasonic characteristics between left and right isomerism as referring to autopsy outcomes.Methods Between November 2007 and July 2010,fetuses with isomerism that were confirmed by autopsy,were identified from 1200 prenatal ultrasonic scans.Their abnormal spectrums and ultrasonic features were analyzed and comparied between left and right isomerism.Results A total of 18 fetuses with right isomerism and 4 fetuses with left isomerism were detected and confirmed.The major findings of the right isomerism were;viscerocardiac heterotaxy (17,94%),anomalous pulmonary venous connection (16,89%),complete atrioventricular septal defect (15,83%),juxtaposition of the descending aorta and inferior vena cava(12,67%),left persistent superior vena cava (11,61%),right aortic arch (10,56%) and univentricle (10,56%).As for the left isomerism,all had viscerocardiac heterotaxy and interruption of inferior vena cave,three of them had heart block,complete atrioventricular septal defect and hypoplasia of aorta,two of them had univentricle.Conclusions Viscerocardiac heterotaxy,complete atrioventricular septal defect,univentricle are the most common anomalies detected.Right isomerism cases usually exist with anomalous pulmonary venous connection,juxtaposition of the descending aorta and inferior vena cava as well as right aortic arch.The left isomerism cases usually have interruption of inferior vena cave,heart block and hypoplasia of aorta. Key words: Ultrasonography,prenatal; Heart defects,congenital; Atrium isomerism

Real-Time 3-Dimensional Transesophageal Echocardiography of the Atrioventricular Septal Defect

Being able to view the morphological details of the heart malformation in any patient with the diagnosis of atrioventricular septal defect (AVSD) is invaluable in surgical planning, especially in the current era of modified 1-patch versus 2-patch techniques. Real-time 3-dimensional transesophageal echocardiography (RT 3D TEE) is a recently developed technique that allows real-time high-quality images akin to viewing the heart specimen. We present the principal pathomorphological features of AVSD as visualized by RT 3D TEE. The hallmark of AVSD is a common atrioventricular junction guarded by a common atrioventricular valve.1 In the normal heart, when seen from atrial perspective, the aortic valve is wedged between the mitral and tricuspid valve. In AVSD, because of the common AV junction, the aortic valve is in an “unwedged” position (Figure 1). The valve consists of 5 leaflets: the superior and inferior bridging leaflets, each of which overrides the ventricular septum, a left mural leaflet, a right anterior, and a right inferior leaflet (Figure 2). Whether the bridging leaflets are joined together distinguishes the complete form from the partial form. In partial AVSD (also termed “ostium primum ASD”), a tongue of leaflet tissue connects the bridging leaflets and the undersides of these leaflets …

Complete atrioventricular septal defect: anatomy, pathology and surgical timing

Komplet atriyoventrikuler septal defekt ostiyum primum atriyal septal defekt, ortak atriyoventrikuler kapak ve posteriyor ventrikuler septumun degisik derecelerdeki defekti ile karakterize bir dogustan kalp hastaligidir. Komplet atriyoventrikuler septal defekt dogustan kalp hastaliklarinin yaklasik %3’unu olusturur ve 10.000 canli dogumda iki oraninda gorulur. Bu durum Down sendromu ile yakin iliskilidir ve hastalarin %40-45’inde Down sendromu vardir. Rastelli siniflamasina gore, ortak atriyoventrikuler kapagin superiyor bridging yaprakcigin morfolojisine bagli olarak uc komplet atriyoventrikuler septal defekt tipi tanimlanmistir (Rastelli tip A, B ve C). Komplet atriyoventrikuler septal defektteki atriyal ve ventrikuler defektler sag ventrikulde yuksek basinc ve volum yukune neden olur ve bunun sonucu olarak hastalarda pulmoner hipertansiyon ve konjestif kalp yetersizligi gelisir. Konjestif kalp yetersizligi belirtileri erken sut cocuklugu doneminde ortaya cikar. Tani ve anatomik detay degerlendirmesi ekokardiyografik inceleme ile saglanir. Pulmoner hipertansiyon zaman icinde geri cevrilemez hale gelir ve cerrahi tedaviyi imkansiz kilar. Medikal tedavinin yeri azdir ve medikal tedavideki amac cerrahiye kadar hastanin iyi kondisyonunu saglamaktir. Cerrahi pulmoner vaskuler hastalik gelismeden once gerceklestirilmelidir ve genel tercih ilk 3-6 ay civaridir. Anah tar soz cuk ler: Anatomi; komplet atriyoventrikuler septal defekt; patoloji; cerrahi zamanlama. Complete atrioventricular septal defect is a congenital heart disease characterized by an ostium primum atrial septal defect, a common atrioventricular valve and a variable degree of posterior ventricular septum defect. Complete atrioventricular septal defect accounts for about 3% of congenital heart diseases and occurs in two out of every 10.000 live births. It is closely associated with Down syndrome and 40-45% of patients have Down syndrome. According to Rastelli’s classification, three types of complete atrioventricular septal defect have been described (Rastelli type A, B, and C) based on the morphology of the superior bridging leaflet of the common atrioventricular valve. The atrial and ventricular defects in complete atrioventricular septal defect lead to a high pressure and volume load in right ventricle, resulting in the development of pulmonary hypertension and congestive heart failure in these patients. Congestive heart failure symptoms appear in early infancy The diagnosis and the evaluation of anatomical details are performed by echocardiographic examination. Over time, pulmonary hypertension becomes irreversible, thus precluding the surgical therapy. Medical treatment plays only a minor role and the goal of the medical treatment is to maintain the condition of the patients in a good level until surgery. The surgery should be performed before the development of pulmonary vascular disease and the first 3-6 months is generally preferred.

Complete atrioventricular septal defect: long-term results and reoperations

Complete atrioventricular septal defect: long-term results and reoperations

Rastelli one-patch procedure for complete atrioventricular septal defect repair

Repair of the complete atrioventricular septal defect, as with many other congenital heart defects, has changed with time from the one-patch technique (Rastelli) to the two-patch technique and then to the “modified” onepatch technique. In this review, we have reported our experience with one-patch Rastelli type correction in babies under one-years of age. Our experience confirmed that the classical one-patch Rastelli technique is a safe, reproducible, teachable technique giving satisfactory late results. Diger bircok dogustan kalp defektlerinde oldugu gibi, komplet atriyoventrikuler septal defeklerin tamiri de zaman icinde tek yama tekniginden (Rastelli) cift yama teknigine ve daha sonra “modifiye” tek yama teknigine degisim sergilemistir. Bu incelemede Rastelli tipi tamir

Simplified single-patch (Australian) technique for the surgical repair of complete atrioventricular septal defects

Simplified single-patch (Australian) technique for the surgical repair of complete atrioventricular septal defects

Prematurity, Low Birth Weight, and Cardiovascular Malformations

Over the past 20 years the survival rate of infants with cardiovascular anomalies has improved dramatically. Repair of complex critical malformations such as transposition of the great arteries, tetralogy of Fallot, complete atrioventricular septal defect, and coarctation of the aorta is now undertaken with a surgical mortality rate that approaches zero in otherwise normal term infants. The improvement in outcome has focused attention on malformations that remain more of a challenge (such as hypoplastic left heart, other single-ventricle malformations, and pulmonary atresia) or on infants whose other problems (such as prematurity, low birth weight, and coexisting noncardiac malformations) put them at higher risk. A recent study from Costello et al1 and a study by Archer et al,2 reported on in this issue of Pediatrics , draw attention to some of these risk factors. Archer et al2 report the details of almost 100 000 very low birth … Address correspondence to Christopher Wren, MBChB, PhD, Department of Paediatric Cardiology, Freeman Hospital, Newcastle Upon Tyne NE7 7DN, United Kingdom. E-mail: christopher.wren{at}nuth.nhs.uk

Progress in CRELD1 gene on atrioventricular septal defect

Congenital heart disease(CHD) is a series of birth defect that can actually or potentially impact heart function , occurring 4‰～ 12‰ of live birth. Atrioventricular septal defect (AVSD) is a frequently CHD affecting the atrioventricular valves and septa. AVSD refers to a clinical spectrum of defects ranging from a complete AVSD to cleft mitral valve. AVSD occurs most frequently in Trisomy 21 syndrome. Research shows that the development defect of endocardial cushion caused by heredity and environment during different period of heart development leads to all kinds of AVSD. CRELD1 is the first identified gene associated with morbidity of AVSD.The research on this gene has important significance to find out the pathogenesis of AVSD and provide better prevention and cure methods. Key words: Atrioventricular septal defect; CRELDI gene; Trisomy 21 syndrome; Endocardial cushion

Single patch repair of complete atrioventricular septal defect in neonates within the first three months of age suffering from therapy refractory heart failure. Is it feasible?

Single patch repair of complete atrioventricular septal defect in neonates within the first three months of age suffering from therapy refractory heart failure. Is it feasible?

Surgical management of complete atrioventricular septal defect: Associations with surgical technique, age, and trisomy 21

We sought to evaluate the contemporary results after repair of a complete atrioventricular septal defect and to determine the factors associated with suboptimal outcomes. The demographic, procedural, and outcome data were obtained within 1 and 6 months after repair of a complete atrioventricular septal defect in 120 children in a multicenter observational study from June 2004 to 2006. The median age at surgery was 3.7 months (range, 9 days to 1.1 years). The type of surgical repair was a single patch (18%), double patch (72%), and a single atrial septal defect patch with primary ventricular septal defect closure (10%). The incidence of residual septal defects and the degree of left atrioventricular valve regurgitation (LAVVR) did not differ by repair type. The median interval of intensive care stay were 4 days, ventilation use 2 days, and total hospitalization 8 days. All were independent of the presence of trisomy 21 (80% of the cohort). The in-hospital mortality rate was 2.5% (3/120). The overall 6-month mortality rate was 4% (5/120). The presence of associated anomalies and younger age at surgery were independently associated with a longer hospital stay. The age at repair was not associated with residual ventricular septal defect or moderate or greater LAVVR at 6 months. Moderate or greater LAVVR occurred in 22% at 6 months, and the strongest predictor for this was moderate or greater LAVVR at 1 month (odds ratio, 6.9; 95% confidence interval, 2.2-21.7; P<.001). The outcomes after repair of complete atrioventricular septal defect did not differ by repair type or the presence of trisomy 21. An earlier age at surgery was associated with increased resource use but had no association with the incidence of residual ventricular septal defect or significant LAVVR.

618 Significance of Murmurs in Infancy

Aim: To find out the significance of murmurs heard during infancy.Methods: Retrospective audit of all infants with newly heard murmurs referred from April 2007 to October 2009 to cardiac clinics in district general hospital run by a Paediatrician with special expertise in cardiology.Results: Over 30 month period, 246 infants were newly referred for murmurs. No murmurs were heard in clinic in 36% (88/246). Of 158 persistent murmurs, 54 were discharged following normal echocardiogram. Abnormalities excluding Patent Foramen Ovale (PFO) on echocardiogram were found in 91 (58%). These were Ventricular Septal Defect (VSD) 34 (37%), Pulmonary Branch Stenosis 24 (26%), Patent ductus arteriosus (PDA) 7, Atrial Septal Defect 7, PFO & PDA 6, Aortic Stenosis 5, VSD & PDA 1 and Tetralogy of Fallot 2, Pulmonary Stenosis 1, Transposition of great arteries (TGA) 1, Congenitally Corrected TGA 1, Double Outlet Right ventricle 1, Complete Atrio-Ventricular Septal defect 1. 24 of the 91 infants (26%) were referred to the nearest tertiary cardiac centre for further management. Abnormalities in ECG were seen in 20/24 infants. 80 infants with less significant cardiac defects did not require any intervention and were followed up locally.Conclusion: More than half of infants with murmur have a cardiac defect and one-fourth of these defects are significant ones. Asymptomatic infants with persistent murmurs and/or abnormal ECGs should be referred for definitive diagnosis to cardiac clinics. District general hospital clinics with echocardiographic facility provide a good screening place and in reducing the work load of tertiary cardiac centres.

More Than 30 Years' Experience With Surgical Correction of Atrioventricular Septal Defects

Outcome of surgical correction of atrioventricular septal defects (AVSD) still varies despite enhanced results. We reviewed our 30-year experience with AVSD repair and identified risk factors for mortality and reoperation. Between 1975 and 2006, 312 patients underwent surgery for complete AVSD (n = 209; 67.0%), partial AVSD (n = 76; 24.4%), or intermediate AVSD (n = 27; 8.6%). Mean age was 2.4 ± 3.9 years; 142 patients (45.5%) were younger than 6 months. Follow-up was 99.0% complete. There were 26 in-hospital deaths (8.3%) and 6 late deaths (2.1% of 283). Estimated overall survival for the total study population was 91.3%, 90.6%, and 88.6% at 1, 5, and 15 years, respectively. In the multivariable logistic regression analysis, surgical era 1975 to 1995 (p < 0.001) and younger age (p = 0.004) were found to be independent risk factors for early mortality, whereas preoperative AV valve insufficiency showed a tendency toward statistical significance (p = 0.052). Of the hospital survivors, 43 patients required a late reoperation. Estimated freedom from late reoperation was 96.4%, 89.3%, and 81.8% at 1, 5, and 15 years, respectively. Multivariable Cox regression analysis showed associated cardiovascular anomalies (p < 0.001), left AV valve dysplasia (p < 0.001), and absence of cleft closure (p = 0.003) to be independent risk factors for late reoperation. AVSD repair can be accomplished with good long-term results. Early surgical era, associated cardiovascular anomalies, left AV valve dysplasia, and absence of cleft closure negatively influence survival and risk of reoperation.

Echocardiographic Definition and Surgical Decision-Making in Unbalanced Atrioventricular Septal Defect

Although identification of unbalanced atrioventricular septal defect (AVSD) is obvious when extreme, exact criteria to define the limits of unbalanced are not available. We sought to validate an atrioventricular valve index (AVVI) (left atrioventricular valve area/total atrioventricular valve area, centimeters squared) as a discriminator of balanced and unbalanced forms of complete AVSD and to characterize the association of AVVI with surgical strategies and outcomes. Diagnostic echocardiograms and hospital records of 356 infants with complete AVSD at 4 Congenital Heart Surgeons' Society (CHSS) institutions (2000-2006) were reviewed and AVVI measured (n=315). Patients were classified as unbalanced if AVVI≤0.4 (right dominant) or ≥0.6 (left dominant). Surgical strategy and outcomes were examined across the range of AVVI. Competing risks analysis until the time of commitment to a surgical strategy examined 4 end states: biventricular repair (BVR), univentricular repair (UVR), pulmonary artery banding (PAB), and death before surgery. A prediction nomogram for surgical strategy based on AVVI was developed. The majority of patients had balanced AVSD (0.4<AVVI<0.6) and underwent BVR. Patients with AVVI<0.19 uniformly underwent UVR. Heterogeneous repair strategies were found when 0.19≤AVVI≤0.39 (UVR and BVR), with a disproportionate number of deaths in this range. AVVI≥0.6 (left dominant) was less common. The proportion of subjects predicted for the end states at 12 months after diagnosis are: BVR, 86%; UVR, 7%; PAB, 1%; death without surgery, 1%; alive without surgery, 5%. AVVI effectively characterizes the transition between balanced and unbalanced AVSD with important correlation to anatomic substrate and selected surgical strategy.

Primary Biventricular Repair of Atrioventricular Septal Defects: An Analysis of Reoperations

The purpose of this study was to analyze the factors affecting reoperation after primary biventricular atrioventricular septal defect (AVSD) repair. Between April 1997 and April 2007, 93 consecutive patients underwent surgery for biventricular correction of AVSD with a median age of 5.8 months (range, 9 days to 68.9 years). Fifty-three patients had complete AVSD, 6 patients had an intermediate type, and 29 patients had partial AVSD; 4 patients had a complete AVSD with associated tetralogy of Fallot, and 1 patient had a complete AVSD with double-outlet right ventricle. There was no in-hospital mortality. There were 2 late deaths (2.2%). Forty-three reoperations were performed in 23 patients (24.7%), of which 18 were for repair of significant left atrioventricular valve regurgitation and 8 were mitral valve replacements. Seven patients (7.5%) required insertion of a permanent pacemaker. The overall 5-year freedom from reoperation after AVSD repair was 73.6% +/- 4.8%. In the multivariate analysis for complete AVSDs, Down syndrome (p = 0.01) and the presence of right ventricular dominance (p = 0.03) were independent predictors of reoperation. At last follow-up, 76 patients (83.5%) were in New York Heart Association class I, and 68 patients (74.7%) were not taking any heart failure medications. Echocardiographic examination showed absent to mild left atrioventricular valve regurgitation in 76.5%; moderate, in 19.8%; and severe, in 3.7% of patients. Down syndrome and right ventricular dominance are independent predictors of reoperation after complete AVSD repair. Biventricular repair of isolated AVSD with a small left ventricle can be successfully accomplished with no mortality.

Anatomical repair of a persistent left superior vena cava into the left atrium

The anatomy of a persistent left superior vena cava (SVC) to the left atrium (LA) without the innominate vein can make it challenging to complete intracardiac repair. We reviewed our five cases of the direct end-to-side anastomosis of SVCs to facilitate anatomical repair of SVC-right atrial connection for biventricular repair. Diagnoses were two partial atrioventricular septal defect with left isomerism, one complete atrioventricular septal defect (CAVSD) with left isomerism, one CAVSD without isomerism and one atrioventricular discordance and double outlet right ventricle with right isomerism. Mean age at the operation was 20+/-23 months (4-58 months) and body weight was 7.8+/-3.4 kg (4.8-12.7 kg). After completion of intracardiac repair, the SVC to LA was divided and end-to-side anastomosed to the SVC to the right atrium during cardiopulmonary bypass. No early or late death occurred during follow-up of 14.4+/-6.9 months (7-23 months). None of the patients developed an obstruction at the anastomosis site of the SVCs. The direct end-to-side anastomosis of SVCs achieved an excellent anatomical SVC-right atrium connection in complex congenital heart diseases.

Recognising heart disease in children with Down syndrome

There is an increased incidence of congenital heart disease among children with Down syndrome. The most common lesion is complete atrioventricular septal defect (cAVSD). Failure to recognise this defect early...

Variations of Atrioventricular Septal Defects Predisposing to Regurgitation and Stenosis

An additional malformation of the atrioventricular valve is occasionally encountered in patients with complete atrioventricular septal defect, and this may compromise accurate correction. We reviewed 138 patients undergoing complete repair with two-patch technique between 1992 and 2008. The mean age was 7.1 + or - 8.3 months, and the mean body weight was 5.1 + or - 2.1 kg. Preceding pulmonary arterial banding was performed in 23 patients. The operative record delineated additional malformations of the atrioventricular valve that posed difficulty in positioning the ventricular septal patch and in accurately approximating the cleft in 45 patients. Of them, four types (n = 40) were associated with increased incidence of postoperative left valvular problems (moderate or worse regurgitation or stenosis). These included abnormalities of the papillary muscles that accompanied hypoplastic mural leaflet or incomplete opening of one commissure in (n = 15; p = 0.0054), dense insertion of the chords of the superior leaflet that obscured the right side of the ventricle septal crest in (n = 13; p = 0.0004), double orifice valve (n = 7; p = 0.0225), and severe length disparities of the cleft that resulted from either disproportional size of superior against inferior leaflets or redundant chord supporting the left extremity of one of these leaflets (n = 5; p < 0.0001). Neither greater age at operation (more than 6 months) nor preceding pulmonary arterial band reduced the incidence of left valvular problems in the malformation group. An individualized technique is required to maintain coaptation of the atrioventricular valve, but in many cases, they are not completely correctable. Deferring complete repair by placing a pulmonary arterial band did not reduce left valvular problems.

Complete atrioventricular septal defect: Outcome of pulmonary artery banding improved by adjustable device

We sought to evaluate pulmonary artery banding in infants with complete atrioventricular septal defects. From 2000 to 2009, 20 infants with complete atrioventricular septal defects underwent pulmonary artery banding because of unsuitable anatomy (unbalanced ventricles, associated lesions, or both) or clinical condition (infection, chronic lung disease, or noncardiac malformation). Patients were divided into 2 groups: the conventional PAB group (n = 13 [65%]; mean age, 74 ± 56 days [range, 6-187 days]; mean weight, 3.3 ± 1.1 kg [range, 2.1-5.8 kg]) and the FloWatch-PAB group (n = 7 [35%]; mean age, 111 ± 40 days [range, 81-187 days]; mean weight, 4.3 ± 1.2 kg [range, 3.2-6.1 kg]). There was no statistical difference in age or weight. Preoperative mechanical ventilation was required in 3 (23%) of 13 infants in the conventional PAB group and 5 (71%) of 7 infants in the FloWatch-PAB group (P < .05). Ten (77%) of 13 infants in the conventional PAB group died versus 0 (0%) of 7 infants in the FloWatch-PAB group (P < .001). Sternal closure was delayed in 6 (46%) of 13 infants in the conventional PAB group and 0 (0%) of 7 infants in the FloWatch-PAB group (P < .05). The mean duration of mechanical ventilation, intensive care unit stay, and hospital stay was significantly longer (P < .05) in the conventional PAB group than in the FloWatch-PAB group (21 ± 17 days [range, 4-61 days] vs 3 ± 2 days [range, 1-8 days], 22 ± 18 days [range, 5-61 days] vs 7 ± 6 days [range, 2-21 days], and 54 ± 12 days [range, 40-71 days] vs 29 ± 25 days [range, 9-81 days], respectively). Left atrioventricular valve regurgitation increased (mild to moderate) in 2 infants in the conventional PAB group and decreased (severe to moderate) in 2 infants in the FloWatch-PAB group. Six of 10 survivors (1 in the conventional PAB group and 5 in the FloWatch-PAB group) underwent pulmonary artery debanding and repair after a median interval of 125 days (range, 34-871 days); 4 of 10 are awaiting repair. In selected patients with complete atrioventricular septal defects, pulmonary artery banding followed by late repair is a viable alternative strategy. In our study the FloWatch-PAB device resulted in improved survival and made later repair possible in a better clinical state.

Reoperations After Repair of Partial Atrioventricular Septal Defect: A 45-Year Single-Center Experience

Patients undergoing repair of partial atrioventricular septal defect (AVSD), are reportedly more likely to require reoperation for subaortic obstruction and left atrioventricular valve abnormalities when compared with repair of complete AVSD. Our objective was to examine reoperations after initial repair of partial AVSD, and determine late outcome. Between 1962 and 2006, 96 patients (63 female) underwent reoperation at our institution after prior repair of partial AVSD (median interval, 10 years; range, 8 days to 48 years). Median age at first reoperation was 26 years (range, 10 months to 71 years). Indications for the first reoperation included left atrioventricular valve (LAVV) regurgitation in 64 patients (67%), subaortic stenosis in 24 (25%), right atrioventricular valve regurgitation in 21 (22%), residual ASD in 11 (11%), and other in 6 (6%). The first reoperation included LAVV repair in 38 patients, LAVV replacement in 35, subaortic fibrous resection-myectomy in 22, right atrioventricular valve regurgitation surgery in 19, and other in 14. There were five early deaths (5.2%) after first reoperation; three were prior to 1983. There was no significant difference in 20-year survival after LAVV repair or replacement (69% vs 55%, p = 0.20). At last follow-up (median, 5.2 years; max, 34 years), 81 of 89 late survivors were in New York Heart Association functional class I or II. The most common indication for reoperation after initial repair of partial AVSD is LAVV pathology, followed by recurrent left ventricular outflow tract obstruction. The need for further reoperation and late survival is similar between patients undergoing valve repair or replacement. When reoperation is required overall late survival is significantly reduced.

Reoperations After Repair of Partial and Complete Atrioventricular Septal Defect

The most common cause of reoperation following repair of atrioventricular septal defect (AVSD) is left atrioventricular valve regurgitation. However, reoperation for subaortic obstruction is required in some, especially after initial repair of partial AVSD. Etiology of reoperation and late outcome were evaluated. Between 1962 and 2007, 146 patients (59 male) underwent reoperation at the authors' institution after prior repair of partial (n = 96) and complete (n = 50) AVSD. Median age at reoperation after repair of partial AVSD was 26 years (range, 10 months to 71 years) and 4.5 years (range, 53 days to 38 years) after repair of complete AVSD. The 3 most common indications for reoperation included left atrioventricular (AV) valve regurgitation in 105 patients, subaortic stenosis in 29, and right AV valve regurgitation in 21. The most common procedures performed included left AV valve repair in 59 (40%) patients, left AV valve replacement in 56 (38%), subaortic fibrous resection/myectomy in 24 (16%), and right AV valve surgery in 19 (13%). Freedom from subsequent reoperation at 10 years was 48% after initial repair of complete AVSD and 84% after initial repair of partial AVSD. During late follow-up, 10-year actuarial survival was 91% and 77% after initial repair of complete and partial AVSD, respectively. The most common indication for reoperation after initial repair of partial or complete AVSD is left AV valve pathology; left ventricular outflow tract obstruction was more common in partial AVSD. Although freedom from subsequent reoperations is higher after initial repair of partial AVSD, these patients have reduced long-term survival when compared with complete AVSD.