Repair Of Complete Atrioventricular Septal Defect Research Articles

Management of Left Ventricular Intramural Hematoma- an Unusual Complication of Complete Atrioventricular Septal Defect Repair in a Child.

Postoperative survival for complete atrioventricular septal defect (cAVSD) is excellent. Common complications of surgery include valvular stenosis/regurgitation, left ventricular outflow tract obstruction, arrythmias, and residual defects. We report a challenging case of a 7-month old girl with Trisomy 21, preoperative obstructive sleep apnea with severe pulmonary hypertension, who underwent AVSD repair and required veno-arterial extracorporeal membrane oxygenation (V-A ECMO) while unable to come off cardiopulmonary bypass and developed left ventricular intramural hematoma during the course. This case highlights the challenges in management of an unusual complication.

Use of glutaraldehyde-treated autologous pericardial patch in complete atrioventricular septal defect repair.

Synthetic patch materials, such as polytetrafluoroethylene and knitted polyester, are commonly used for the repair of complete atrioventricular septal defects using the double-patch technique. In our study, we investigated the impact of glutaraldehyde-treated autologous pericardial patch on postoperative atrioventricular valve function. In this retrospective study, patients who underwent repair of complete atrioventricular septal defects using the double-patch technique between January 1, 2018, and May 15, 2023, at our pediatric cardiac center were included in the study. Patients were divided into two groups based on the type of patch used for the repair of the ventricular component of the atrioventricular septal defect: autologous pericardial patch (group 1) and polytetrafluoroethylene patch (group 2). Postoperative data of the patients were retrospectively analyzed. Thirty-four patients (57.6%) were female, and 25 (42.4%) were male, with a median age of 7 (5-18) months. The median body weight at the time of surgery was 5.5 (4.1-10) kilograms. Down syndrome was present in 45 (76.3%) patients. The autologous pericardium was used for ventricular septal defect closure in 44 (74.6%) patients (Group 1), while polytetrafluoroethylene was used in 15 (25.4%) patients (Group 2). Permanent cardiac pacemaker placement was required in 4 (6.8%) patients due to postoperative atrioventricular block. Hospital mortality was 11.9% (n=7). Among these cases, 4 (6.8%) patients died from low cardiac output syndrome, and 3 (5.1%) patients died from sepsis. Postoperative echocardiographic evaluation revealed insignificant residual ventricular septal defects in 17 (28.8%) patients. No significant differences were observed between the groups in terms of mortality and postoperative atrioventricular valve insufficiency. The median follow-up duration for discharged patients was 5 months (7 days to 3.9 years). Throughout the follow-up period, there were no reoperations due to valve insufficiency or left ventricular outflow tract obstruction. Glutaraldehyde-treated autologous pericardium is a safe material that can be confidently used for the repair of the ventricular component of complete atrioventricular septal defects. This patch material does not adversely affect atrioventricular valve function and allows for the successful closure of ventricular septal defects.

Timing of surgical repair and resource utilisation in infants with complete atrioventricular septal defect.

Variation exists in the timing of surgery for balanced complete atrioventricular septal defect repair. We sought to explore associations between timing of repair and resource utilisation and clinical outcomes in the first year of life. In this retrospective single-centre cohort study, we included patients who underwent complete atrioventricular septal defect repair between 2005 and 2019. Patients with left or right ventricular outflow tract obstruction and major non-cardiac comorbidities (except trisomy 21) were excluded. The primary outcome was days alive and out of the hospital in the first year of life. Included were 79 infants, divided into tertiles based on age at surgery (1st = 46 to 137 days, 2nd = 140 - 176 days, 3rd = 178 - 316 days). There were no significant differences among age tertiles for days alive and out of the hospital in the first year of life by univariable analysis (tertile 1, median 351 days; tertile 2, 348 days; tertile 3, 354 days; p = 0.22). No patients died. Fewer post-operative ICU days were used in the oldest tertile relative to the youngest, but days of mechanical ventilation and hospitalisation were similar. Clinical outcomes after repair and resource utilisation in the first year of life were similar for unplanned cardiac reinterventions, outpatient cardiology clinic visits, and weight-for-age z-score at 1 year. Age at complete atrioventricular septal defect repair is not associated with important differences in clinical outcomes or resource utilisation in the first year of life.

The influence of age and weight on the outcomes of complete atrioventricular septal defect repair

BackgroundThe appropriate age and weight for surgical repair of atrioventricular septal defect (AVSD) is an area of controversy. We aimed to study the effect of weight and age at the time of surgical repair for complete AVSD in children less than 2 years of age on postoperative outcomes. A retrospective data review was performed for patients who underwent the AVSD repair from 2012 to 2019 at our institutions. Our primary outcome was the postoperative in-hospital length of stay (LOS). Secondary outcomes included total positive pressure ventilation (PPV), ventilation time, maximum vasoactive–inotropic score (max VIS), and other postoperative complications.ResultsThe study included fifty patients. The median age was 191 days, and the median weight was 4.38 kg at the time of surgery. Weight < 4 kg was associated with longer PPV time and postoperative in-hospital LOS (p value of 0.033 and 0.015, respectively). Additionally, they had higher max VIS at 24 h and 48 h than the other groups with bodyweight 4–5.9 kg or ≥ 6 kg (p value of 0.05 and 0.027, respectively). Patients with older age or lower weight at operation had a longer in-hospital LOS and total length of PPV after surgery. There were no postoperative in-hospital deaths.ConclusionsOlder age and lower weight at the time of surgical repair of atrioventricular septal defect could be independent predictors of prolonged postoperative in-hospital length of stay and total length of positive pressure ventilation.

Long-Term Outcome Up To 40 Years after Single Patch Repair of Complete Atrioventricular Septal Defect in Infancy or Childhood.

Objectives Patients with repaired complete atrioventricular septal defect (CAVSD) represent an increasing portion of grown-ups with congenital heart disease. For repair of CAVSD, the single-patch technique has been employed first. This technique requires division of the bridging leaflets, thus, among other issues, long-term function of the atrioventricular valves is of particular concern. Methods Between 1978 and 2001, 100 consecutive patients with isolated CAVSD underwent single-patch repair in our institution. Hospital mortality was 11%. Primary endpoints were clinical status, atrioventricular valve function, and freedom from reoperation in long term. Follow-up was obtained contacting the patient and/or caregiver, and the referring cardiologist. Results Eighty-three patients were eligible for long-term follow-up (21.0 ± 8.7, mean ± standard deviation [21.5; 2.1–40.0, median; min–max] years after surgical repair). Actual long-term mortality was 3.4%. Quality of life (QoL; self- or caregiver-reported in patients with Down syndrome) was excellent or good in 81%, mild congestive heart failure was present in 16%, moderate in 3.6% as estimated by New York Heart Association classification. Echocardiography revealed normal systolic left ventricular function in all cases. Regurgitation of the right atrioventricular valve was mild in 48%, mild–moderate in 3.6%, and moderate in 1.2%. The left atrioventricular valve was mildly stenotic in 15% and mild to moderately stenotic in 2%; regurgitation was mild in 54%, mild to moderate in 13%, and moderate in 15% of patients. Freedom from left atrioventricular-valve-related reoperation was 95.3, 92.7, and 89.3% after 5, 10, and 30 years, respectively. Permanent pacemaker therapy, as an immediate result of CAVSD repair ( n = 7) or as a result of late-onset sick sinus syndrome ( n = 5), required up to six reoperations in single patients. Freedom from pacemaker-related reoperation was 91.4, 84.4, and 51.5% after 5, 10, and 30 years, respectively. Conclusion Up to 40 years after single-patch repair of CAVSD, clinical status and functional results are promising, particularly, in terms of atrioventricular valve function. Permanent pacemaker therapy results in a life-long need for surgical reinterventions.

Correlation of ventricular septal defect height and outcomes after complete atrioventricular septal defect repair.

OBJECTIVESThere are limited data available on the height of the ventricular component of the septal deficiency (VSD) in patients undergoing complete atrioventricular septal defect (CAVSD) repair. VSD height may influence optimal choice of repair strategy with potential consequences for long-term outcomes. We aimed to measure VSD height using 2-dimensional echocardiography and review its association with postoperative outcomes.METHODSWe retrospectively reviewed the preoperative echocardiograms of 45 consecutive patients who underwent CAVSD repair between May 2010 and December 2015 at a single centre. VSD height and left ventricular length on the four-chamber view were measured. Demographic details and early and late outcomes including reoperation and long-term survival were studied.RESULTSTwenty patients underwent modified single-patch repair and 25 patients underwent double-patch repair of CAVSD. VSD height in the modified single-patch group ranged from 4.2 to 11.7 mm and in the double-patch group ranged from 5.1 to 14.9 mm. Nine patients had a deep ‘scoop’ with a VSD height of >10 mm, (7 double patch, 2 modified single patch). VSD height did not correlate with a specific Rastelli classification. There was no significant difference in the VSD height (P = 0.51) or the VSD height-to-left ventricular length ratio (P = 0.43) between the 2 repair groups. There was no 30-day mortality. Eight patients required reoperation; however, VSD height was not a significant predictor of reoperation (hazard ratio 0.95, 95% confidence interval 0.69–1.33; P = 0.08).CONCLUSIONSThere was no correlation between VSD height and risk of reoperation after CAVSD repair. A deep ventricular scoop is uncommon in CAVSD patients.

Propensity Score Matched Analysis of Cleft Closure in Complete Atrioventricular Septal Defect Repair

BackgroundRepair of complete atrioventricular septal defect (cAVSD) is achieved with low mortality. However, there is a high rate of reoperation on the left atrioventricular valve (LAVV), which is often attributed to nonclosure of the cleft. Although nonclosure of the cleft has been reported to be a risk factor for reoperation, no randomized controlled or propensity-matched trials have ever been performed. We investigated the effect of cleft closure on outcomes after cAVSD repair. MethodsWe reviewed 455 patients who underwent cAVSD repair between 1990 and 2019. To determine the effect of cleft closure, propensity score matching was performed on risk factors for reoperation after cAVSD repair. ResultsMedian age was 3.6 months (mean, 9.6 ± 20.4 months), median weight was 4.3 kg (mean, 4.7 ± 4.3 kg) and 41.9% (191 of 455) were male. Early mortality was 2.9% (13 of 455), and survival was 89.8% ± 1.9% at 20 years. Early reoperation was a risk factor for mortality (P = .004). Freedom from reoperation was 72.5% ± 4.0% at 20 years. Freedom from LAVV reoperation was 74.1% ± 4.0% at 20 years. Preoperative severe LAVV regurgitation (P < .001) and early postoperative moderate or greater LAVV regurgitation (P = .007) were risk factors for reoperation, while trisomy 21 (P = .03) and recent era of surgery (P = .02) were protective. Propensity score matching yielded 106 pairs. There were no differences in long-term survival (P = .71) or reoperation (P = .26) between the 2 groups. ConclusionsRepair of cAVSD can be achieved with low mortality and good long-term survival, although the reoperation rate remains high. Similar freedom from reoperation can be achieved with or without closure of the LAVV cleft.

Impact of concomitant complex cardiac anatomy in nonsyndromic patients with complete atrioventricular septal defect

ObjectiveWe studied a cohort of patients with nonsyndromic complete atrioventricular septal defect with and without concomitant complex cardiac anatomy and compared the outcomes after surgical repair. MethodsBetween 1993 and 2018, 62 nonsyndromic patients underwent complete atrioventricular septal defect repair. Sixteen patients (26%) had complex complete atrioventricular septal defect with variables representing concomitant cardiac anatomic complexity: tetralogy of Fallot, double outlet right ventricle, total anomalous pulmonary venous return, concomitant aortic arch reconstruction, multiple ventricular septal defects, staged repair of coarctation of the aorta, and a persisting left superior vena cava. The mean follow-up was 12.7 ± 7.9 years. Baseline variables were retrospectively evaluated and analyzed using univariable logistic regression. Survival was studied using Kaplan–Meier estimates, and group comparisons were performed using the log-rank test. A competing-risk analysis estimated the risk of reoperation with death as the competing event. A Gray's test was used to test equality of the cumulative incidence curves between groups. ResultsThe perioperative mortality was 3.2% (2/62). Actuarial survival was 100% versus 66.7% ± 14.9% at 10 years in the noncomplex and complex groups, respectively (P < .01). There was no significant difference in the overall reoperation rate between the noncomplex group (7/46; 15%) and the complex group (4/16; 25%) (odds ratio, 1.86; 95% confidence interval, 0.46-7.45; P = .30). The competing-risk analysis demonstrated no significant difference in reoperation between the groups (P = .28). ConclusionsOur data show that nonsyndromic patients without complex cardiac anatomy have a good long-term survival and an acceptable risk of reoperation similar to contemporary outcomes for patients with complete atrioventricular septal defect with trisomy 21. However, the corresponding group of nonsyndromic patients with concomitant complex cardiac lesions are still a high-risk population, especially regarding mortality.

Outcomes of Complete Atrioventricular Septal Defect Surgical Repair in Down Patients

Background: Atrioventricular septal defect (AVSD) is commonly associated with chromosomal abnormalities, especially trisomy 21 or Down syndrome (DS). Surgical repair of complete AVSD (CAVSD) is a complex procedure that carries risks of postoperative morbidity and mortality. Objective: To evaluate the surgical outcomes and to identify the risk factors for hospital mortality and reoperation after repair of CAVSD in DS patients. Patients and Methods: This retrospective cohort study included 65 consecutive DS patients who underwent surgical correction for the complete form of AVSD with or without associated congenital heart diseases during the period from 1st January 2014 to the end of June 2020. Patients with associated other major cardiac anomalies were excluded. Results: In-hospital mortality was documented in 3 (4.6%) patients, whereas 4 (6.2%) patients needed second unplanned operation for valve/shunt correction. Heart block that needed permanent pacemaker insertion was recorded in 3 (4.6%) patients. In-hospital mortality was significantly associated with prolonged cardiopulmonary bypass (CPB) time (p = 0.008) and the development of renal dysfunction that required dialysis or sepsis (p = 0.004). We found a significant association between the need for second unplanned operation and type A CAVSD (p = 0.041) and the presence of preoperative moderate/severe atrioventricular (AV) valve regurgitation as detected in the transesophageal echocardiography (TEE) (p=0.035). Conclusions: In view of the incidence of the hospital mortality, reoperation, and other postoperative morbidities, we suggest that our outcomes are accepted for surgical repair of CAVSD in DS patients. The CPB time and the development of renal dysfunction that required dialysis and sepsis during the ICU care significantly contributed to the hospital mortality.

Complete atrioventricular septal defect with absent or diminutive primum component: Incidence, anatomic characteristics, and outcomes

BackgroundRepair of complete atrioventricular septal defect with absent or diminutive primum defect is challenging because of atrial septal malposition and abnormal anatomy of the left atrioventricular valve. We sought to define the incidence, anatomy, and surgical outcomes of this entity. MethodsWe identified all patients in our institutional database presenting for complete atrioventricular septal defect repair from 2006 to 2018. Operative reports and echocardiograms were reviewed to determine the presence and size of the primum defect, atrioventricular valve anatomy, degree of atrioventricular valve regurgitation, repair method, and complications, including reoperation for atrioventricular valve regurgitation. Functionally univentricular patients and those receiving repair at an outside institution were excluded. ResultsOf 183 patients with complete atrioventricular septal defect, absent/diminutive primum defect occurred in 16 patients (8.7%; 10 absent, 6 diminutive). Six patients (38%) had leftward malposition of the atrium septum on the common atrioventricular valve. The rate of reoperation for left atrioventricular valve regurgitation was 31% (3 early, 2 late), for which preoperative predictors included leftward malposition of the atrial septum onto the common atrioventricular valve (4/6 patients with malposition required reoperation, P = .036, Fisher exact test). One patient exhibiting this risk factor died. The overall rate of moderate or greater left atrioventricular valve regurgitation on the most recent postoperative echocardiogram was 13% (2/16 patients; median follow-up, 141 days; range, 3-2236 days). ConclusionsComplete atrioventricular septal defect with absent or diminutive primum defect is a unique variant of complete atrioventricular septal defect for which the risk of reoperation for left atrioventricular valve regurgitation after complete repair is high and risk factors include leftward malposition of the atrial septum on the common atrioventricular valve.

Predictors of reoperation and mortality after complete atrioventricular septal defect repair.

Previous studies investigating risk factors associated with reoperation or mortality after repair of complete atrioventricular septal defect (CAVSD) often have not included sizeable cohorts undergoing modified single-patch repair. Both double patch and modified single-patch techniques have been widely used in Australia since the 1990s. Using a large multi-institutional cohort, we aimed to identify risk factors associated with reoperation or mortality following CAVSD repair. Between January 1990 and December 2015, a total of 829 patients underwent biventricular surgical repair of CAVSD in Australia at 4 centres. Patients with associated tetralogy of Fallot and other conotruncal abnormalities were excluded. Demographic details, postoperative outcomes including reoperation and survival, and associated risk factors were analysed. Fifty-six patients (6.8%) required early reoperation (≤30 days) for significant left atrioventricular valve regurgitation or residual septal defects. Freedom from reoperation at 10, 15 and 20 years was 82.7%, 81.1% and 77%, respectively. Patients without Down syndrome and moderate left atrioventricular valve regurgitation on postoperative echocardiogram were found to be independent risk factors for reoperation. Operative mortality was 3.3%. Overall survival at 10, 15 and 20 years was 91.7%, 90.7% and 88.7%, respectively. Prior pulmonary artery banding was a predictor for mortality, while later surgical era (2010-2015) was associated with a reduction in mortality risk. Improved survival in the contemporary era is in keeping with improvements in surgical management and higher rates of primary CAVSD repair over time. The presence of residual moderate left atrioventricular valve regurgitation on postoperative echocardiography is an important factor associated with reoperation and close surveillance is essential to allow timely reintervention. Primary CAVSD repair at age <3 months should be preferenced to palliation with pulmonary artery banding due to the association of pulmonary artery banding with mortality in the long-term.

Impact of Major Residual Lesions on Outcomes After Surgery for Congenital Heart Disease

BackgroundMany factors affect outcomes after congenital cardiac surgery. ObjectivesThe RLS (Residual Lesion Score) study explored the impact of severity of residual lesions on post-operative outcomes across operations of varying complexity. MethodsIn a prospective, multicenter, observational study, 17 sites enrolled 1,149 infants undergoing 5 common operations: tetralogy of Fallot repair (n = 250), complete atrioventricular septal defect repair (n = 249), arterial switch operation (n = 251), coarctation or interrupted arch with ventricular septal defect (VSD) repair (n = 150), and Norwood operation (n = 249). The RLS was assigned based on post-operative echocardiography and clinical events: RLS 1 (trivial or no residual lesions), RLS 2 (minor residual lesions), or RLS 3 (reintervention for or major residual lesions before discharge). The primary outcome was days alive and out of hospital within 30 post-operative days (60 for Norwood). Secondary outcomes assessed post-operative course, including major medical events and days in hospital. ResultsRLS 3 (vs. RLS 1) was an independent risk factor for fewer days alive and out of hospital (p ≤ 0.008) and longer post-operative hospital stay (p ≤ 0.02) for all 5 operations, and for all secondary outcomes after coarctation or interrupted arch with VSD repair and Norwood (p ≤ 0.03). Outcomes for RLS 1 versus 2 did not differ consistently. RLS alone explained 5% (tetralogy of Fallot repair) to 20% (Norwood) of variation in the primary outcome. ConclusionsAdjusting for pre-operative factors, residual lesions after congenital cardiac surgery impacted in-hospital outcomes across operative complexity with greatest impact following complex operations. Minor residual lesions had minimal impact. These findings may provide guidance for surgeons when considering short-term risks and benefits of returning to bypass to repair residual lesions.

Long-Term Follow-Up of Pericardium for the Ventricular Component in Atrioventricular Septal Defect Repair.

Despite the improved outcome in complete atrioventricular septal defect (AVSD) repair, reoperations for left atrioventricular valve (LAVV) dysfunction are common. The aim of this study was to evaluate the effect of fresh untreated autologous pericardium for ventricular septal defect (VSD) closure on atrioventricular valve function and compare the results with the use of treated bovine pericardial patch material. Clinical and echocardiographic data were collected of patients with complete AVSD with their VSD closed with either untreated autologous pericardial or treated bovine pericardial patch material between January 1, 1996, and December 31, 2003. Evaluation closed in September 2019. A total of 77 patients were analyzed (untreated autologous pericardial VSD patch: 59 [77%], treated bovine pericardial VSD patch: 18 [23%]). Median age at surgery was 3.6 (interquartile range [IQR]: 2.7-4.5) months, and median weight was 4.5 (IQR: 3.9-5.1) kg. Trisomy 21 was present in 70 (91%) patients. Median follow-up time was 17.5 (IQR: 12.6-19.8) years. Death <30 days occurred in two (3%) patients. Reinterventions occurred in eight patients (early [within 30 days] in two, early and late in one, and late in five), all in the autologous pericardium group. Log-rank tests showed no significant difference in mortality (P = .892), LAVV reinterventions (P = .228), or LAVV regurgitation (P = .770). In AVSD, the VSD can safely be closed with either untreated autologous pericardium or xeno-pericardium. We found no difference in LAVV regurgitation or the need for reoperation between the two patches.

Atrioventricular Valve Function Predicts Reintervention in Complete Atrioventricular Septal Defect.

Objective:Technical performance score (TPS) has been associated with both early and late outcomes across a wide range of congenital cardiac procedures. A previous study has shown that the presence of residual lesions before discharge, as measured by TPS, is accurately able to identify patients who required postdischarge reinterventions after complete atrioventricular septal defect (CAVSD) repair. The aim of this study is to determine which subcomponents of TPS best predict postdischarge reinterventions after CAVSD repair.Methods:This was a single-center retrospective review of patients with CAVSD after repair between January 2000 and March 2016. We assigned TPS (class 1, no residua; class 2, minor residua; class 3, major residua or reintervention before discharge for residua) based on subcomponent scores from discharge echocardiograms. Outcome of interest was postdischarge reintervention.Results:Among 344 patients, median age was 3.2 months (interquartile range [IQR], 2.4-4.2). There were 34 (10%) postdischarge reinterventions. Median follow-up was 2.6 years (IQR, 0.09-7.9). Trisomy 21 and concomitant procedure were associated with postdischarge reinterventions. After adjusting for these factors, among the subcomponents, left atrioventricular valve stenosis and regurgitation, right atrioventricular valve regurgitation, residual ventricular septal defect, and abnormal conduction at discharge were significantly associated with postdischarge reinterventions.Conclusions:We demonstrated the ability of TPS to predict postdischarge reinterventions in patients who underwent CAVSD repair. Residual left and right atrioventricular valve regurgitation and abnormal conduction at discharge were among the subcomponents strongly associated with postdischarge reinterventions. Thus, TPS may aid clinicians in identifying children at higher risk for reintervention.

Modified-Single Patch vs Double Patch Repair of Complete Atrioventricular Septal Defects

Biventricular repair of complete atrioventricular septal defect (CAVSD) is largely achieved using the double-patch (DP) or modified single-patch (MSP) techniques in the current era; however, long-term results following MSP repair are not well defined. We aimed to compare long-term outcomes including reoperation and mortality after CAVSD repair using DP and MSP techniques, and identify the risk factors associated with adverse outcomes. A retrospective cohort study was performed including all patients who underwent CAVSD repair using DP and MSP techniques at our institution between 17 May 1990 and 14 December 2015. Demographic details, early (≤30 days) and late (>30 days) outcomes (reoperation, mortality) were studied. Competing risks analysis with cumulative incidence function was used for survival analyses. Overall, 273 consecutive patients underwent CAVSD repair (120 DP and 153 MSP) and 41 patients required reoperation during follow-up. Competing risks analysis showed no association between repair technique and reoperation (P = 1.0) or mortality (P = 0.9). Considering competing risks due to mortality, the cumulative incidence of reoperation at 5, 10, and 15 years was 14%, 17%, and 17% for DP and 12%, 13%, and 16% for MSP, respectively. Non-Down syndrome and moderate or greater left atrioventricular valve regurgitation were predictors for reoperation. Pulmonary artery banding was predictive of mortality, though strongly associated with earlier surgical era. Median follow-up duration was 8.0 years (interquartile range 3.9-20.8) for DP and 11.6 years (interquartile range 5.4-16.1) for MSP (P = 0.4). Event-free survival is similar after DP and MSP repair of CAVSD indicating either repair technique can be safely utilized.

Complete Atrioventricular Septal Defect: Evolution of Results in a Single Center During 50 Years

Operative mortality after complete atrioventricular septal defect (cAVSD) repair has improved vastly. Less improvement has been demonstrated regarding late mortality and reoperation rates, however. There is evident lack of comprehensive population-based studies analyzing the history and progress of the ever-changing operative results. This is a 5-million population-based retrospective study of consecutive 388 cAVSD patients operated in Finland between 1962 and 2014. Data were collected using Children's Cardiac Surgical Registry of Children's Hospital at the Helsinki University Hospital, Finland. Mortality data and reoperation rates were analyzed on a decade-by-decade basis. During the early era, overall mortality was 17.4%, operative mortality constituting 10.9%. The operative results have improved significantly over the decades, and eventually, the last decade showed no mortality. A total of 23 late deaths occurred; of these, 20 were directly heart-related. Half of the late mortality occurred during the first postoperative year. The only significant risk factor for overall mortality was an earlier decade of operation (p < 0.001). Reoperation rates have not decreased but slightly increased over decades (p= 0.621), and reoperations have been performed mainly during the first year after the primary operation. Actuarial freedom from left side atrioventricular valve reoperation at 15 years was 90.9%. There has been an outstanding improvement in surgical results through the years even though the general operative approach has remained the same. Rates of reoperation have not been declining, but the reoperations are dated to early childhood years. The improvement in results has been ongoing.

Surgical Treatment for Congenital Heart Defects in Down Syndrome Patients.

ObjectiveTo analyze data related to surgical treatment in patients with congenital heart defects (CHD) and Down syndrome (DS) based on information from International Quality Improvement Collaborative Database for Congenital Heart Disease (IQIC).MethodsBetween July 1, 2010 and December 31, 2017, 139 patients with CHD and DS underwent surgery at Hospital de Base and Hospital da Criança e Maternidade de São José do Rio Preto (FUNFARME)/Faculdade de Medicina de São José do Rio Preto - SP (FAMERP). A quantitative, observational and cross-sectional study was performed in which the pre, intra and postoperative data were analyzed in an IQIC database. The data included gender, age, prematurity, weight, preoperative procedures, diagnosis, associated cardiac and non-cardiac anomalies, Risk Adjustment for Congenital Heart Surgery (RACHS-1), type of surgery, cardiopulmonary bypass (CPB), perfusion time, aortic clamping time and CPB temperature, bacterial sepsis, surgical site infection and other infections, length of stay in intensive care unit (ICU), length of hospital stay and in-hospital mortality.ResultsThe most prevalent procedures were complete atrioventricular septal defect repair (58 - 39.45%), followed by closure of ventricular septal defect (36 - 24.49%). The RACHS-1 categories 1, 2, 3 and 4 were distributed as 22 (15%); 49 (33.3%); 72 (49%) and 4 (2.7%), respectively. There were no procedures classified as categories 5 or 6. Bacterial sepsis occurred in 10.2% of cases, surgical site infection in 6.1%, other infections in 14.3%. The median length of ICU stay was 5 days and the median length of hospital stay was 11 days. In-hospital mortality was 6.8%.ConclusionSurgical treatment in patients with CHD and DS usually does not require highly complex surgical procedures, but are affected by infectious complications, resulting in a longer ICU and hospital length of stay with considerable mortality.

Contemporary Outcomes After Repair of Isolated and Complex Complete Atrioventricular Septal Defect

Contemporary outcomes of complete atrioventricular septal defect (CAVSD) repair, particularly for defects with associated abnormalities, is unclear. The goal of this study is to report an all-inclusive experience of CAVSD repair using a consistent surgical approach. All patients undergoing CAVSD repair between 1995 and 2016 at our institution were included. Patients were divided into 2 groups: isolated and complex (tetralogy of Fallot, aortic arch repair, double outlet right ventricle, and total anomalous pulmonary venous return). Survival and reoperation were analyzed using log-rank test and Gray's test, respectively. Multivariable analysis was performed with Cox regression. Overall, 406 patients underwent repair: 350 (86%) isolated and 56 (14%) complex CAVSD (tetralogy of Fallot: 34, double outlet right ventricle: 7, aortic arch repair: 12, total anomalous pulmonary venous return: 3). Median age at repair was 5 months (range, 10 days to 16 years); 339 (84%) had trisomy 21. A 2-patch repair was used in 395 (97%) and the zone of apposition was completely closed in 305 (75%). Perioperative mortality was 2% and 4% in the isolated and complex groups, respectively. Perioperative mortality since 2006 was 0.9%. Median follow-up was 7 years. Overall 10-year survival and incidence of any reoperation were 92% and 11%, respectively. Complex anatomy was not a risk factor for mortality (p= 0.35), but it was for reoperation (hazard ratio [HR]: 2.6; p < 0.01). Risk factors for left atrioventricular valve reoperation were a second bypass run (HR: 2.7) and preoperative moderate or worse regurgitation (HR: 2.3). Mortality after CAVSD repair is low, yet reoperation remains a significant problem. Repair of complex CAVSD can be performed with similar mortality rates.

Vertical Right Axillary Mini-Thoracotomy for Correction of Ventricular Septal Defects and Complete Atrioventricular Septal Defects

Vertical right axillary mini-thoracotomy (VRAMT) is the standard approach for correction of atrial septal defect and partial atrioventricular septal defect at our institution. This observational single-center study compares our initial results with the VRAMT approach for the repair of ventricular septal defect (VSD) and complete atrioventricular septal defect (CAVSD) ininfants and children to an approach using standard median sternotomy (MS). The perioperative courses of patients undergoing VSD and CAVSD correction through either a VRAMT or an MS were analyzed retrospectively. The surgical technique for the VRAMT involved a 4- to 5-cm vertical incision in the right axillary fold. Of 84 patients, 25 (VSD, n= 15; CAVSD, n=10) underwent correction through a VRAMT approach, whereas 59 (VSD, n= 35; CAVSD, n= 24) had repairthrough MS. VSD and CAVSD groups were comparable with respect to age and weight. No significant differences were observed for aortic cross-clamp duration, intensive care unit stay, hospital stay, and echocardiographic follow-up. There was no need for any conversion from VRAMT to MS in any case. Neither wound infectionsnor thoracic deformities were observed in both groups. VRAMT can be considered as a safe and effective approach for the repair of VSD and CAVSD in selected patient groups, and the outcome data appear comparable to those of MS.

Current status of cardiovascular surgery in Japan 2013 and 2014: Areport based on the Japan Cardiovascular Surgery Database. 2: Congenital heart surgery.

We analyzed the mortality and morbidity of congenital heart surgery in Japan using the Japan Cardiovascular Surgery Database (JCVSD). Data regarding congenital heart surgery performed between January 2013 and December 2014 were obtained from JCVSD. The 20 most frequent procedures were selected and the mortality rates and major morbidities were analyzed. The mortality rates of atrial septal defect repair and ventricular septal defect repair were less than 1%, and the mortality rates of tetralogy of Fallot repair, complete atrioventricular septal defect repair, bidirectional Glenn, and total cavopulmonary connection were less than 2%. The mortality rates of the Norwood procedure and total anomalous pulmonary venous connection repair were more than 10%. The rates of unplanned reoperation, pacemaker implantation, chylothorax, deep sternal infection, phrenic nerve injury, and neurological deficit were shown for each procedure. Using JCVSD, the national data for congenital heart surgery, including postoperative complications, were analyzed. Further improvements of the database and feedback for clinical practice are required.