Severe Left Atrioventricular Valve Regurgitation Research Articles

Predictors of post-operative left atrioventricular valve regurgitation in pediatric patients with complete atrioventricular canal defects.

In infants with complete atrioventricular canal (CAVC) defects, post-operative left atrioventricular valve regurgitation (LAVVR) is a known major cause of morbidity and mortality and a common indication for re-operation. However, there is scarce data to identify risk factors for poor outcomes. Our study aims to find echocardiographic characteristics that predict post-operative LAVVR at discharge and 1-year follow-up. Retrospective cohort study of patients with initial CAVC repair at our hospital who were followed for 1 year between 2013 and 2022. Patients with major co-morbid conditions were excluded. Serial echocardiograms were reviewed. Anatomic details, quantitative and qualitative measure of LAVVR including the number of regurgitant jets, regurgitant jet length and vena contracta width, and ventricular function were collected. The time points measured include pre-operative transthoracic echocardiogram (TTE), post-operative transesophageal echocardiogram (PO-TEE), routine protocol based post-operative day 1 (POD1) TTE, discharge TTE and 1-year post-operative (1yPO) TTE. Paired t-tests, chi-square analysis, and linear regression analysis were performed comparing measured variables to LAVVR outcomes. Fifty-two patients were included; 92% had Trisomy 21. The majority were classified as Rastelli A (71%), others Rastelli C (29%). Only two patients had moderate or greater LAVVR pre-operatively. The mean age at repair was 125 ± 44 days. Pre-operative LAVVR was the only significant predictor of LAVVR severity at 1 year after backward stepwise regression. Of those with<moderate LAVVR on PO-TEE, 20% had worsening to≥moderate at discharge, but only 9% remained that way at 1 year. Of those with≥moderate LAVVR on PO-TEE, 40% improved to<moderate by 1 year. Two patients who worsened at 1 year, both secondary to likely cleft suture dehiscence. Only one patient required reoperation in the immediate post-operative period secondary to severe LAVVR due to suture dehiscence. Routine protocol-based POD1 echo did not have any association with altered outcomes. Pre-operative LAVVR was the only significant predictor of LAVVR severity at 1 year. A significant percentage (40%) of patient with≥moderate LAVVR on PO-TEE improved to<moderate by 1 year. Furthermore, routine protocol-based POD1 echo did not have any association with altered outcomes.

Middle to long-term outcomes of surgical repair for atrioventricular septal defect: a single-center study.

The exact incidence and predictors of mortality and left atrioventricular valve (LAVV) re-operation in congenital atrioventricular septal defect (AVSD) repair are still unclear. This study analyzed the middle to long-term outcomes of surgical repair for AVSD. A total of 150 patients (69 males and 81 females) who underwent AVSD repair at Children's Hospital of Fudan University from January 2013 to December 2021 were divided into complete defect group (C-group, 67 cases), transitional defect group (T-group, 26 cases), and partial defect group (P-group, 57 cases). Outcomes during the peri-operative and 10-year follow-up periods were evaluated. The total mortality was 5.33% (8/150), including seven early deaths (10.4%) and no late deaths in the C-group, no early deaths (0%) and one late death (1.8%) in the P-group, and no early or late deaths in the T-group. Up to the last follow-up, severe LAVV regurgitation had occurred in 27 patients, including 16 in the C-group, four in the T-group, and seven in the P-group. In total, 12 (12/150, 8.0%) patients received LAVV re-operation, including seven in the C-group, three in the T-group, and two in the P-group. Cox regression analysis showed that pre-operative severe pulmonary hypertension (P=0.006) and severe LAVV regurgitation within 24 hours after the first surgery (P=0.023) were independent risk factors for mortality. ≥ Moderate LAVV regurgitation within the first 24 hours after surgery (P=0.014) was an independent risk factor for LAVV re-operation. Complete AVSD repair increased the risk of early death, severe LAVV regurgitation and re-operation. Pre-operative severe pulmonary hypertension and residual severe LAVV regurgitation indicated high risk for mortality. ≥ Moderate LAVV regurgitation within 24 hours after the first surgery predicted a high probability of LAVV re-operation.

Myxomatous Degeneration of Atrioventricular Valves in a Crab-eating fox (Cerdocyon thous) - Echodopplercardiography Diagnosis

Background: The myxomatosis degeneration is a degenerative cardiac valve disease, with a higher incidence in male and senile canids. The diagnosis is made by a doppler echocardiography exam. Although there are few reports on the occurrence of cardiopathies in wild dogs (Cerdocyon thous), some studies on their cardiological parameters can be found. Considering this, and knowing that the cardiopathies in wild canids are common post mortem findings, the objective of this study is to describe the echocardiography diagnosis of a case of myxomatous degeneration of the atrioventricular valves in 1 wild dog (Cerdocyon thous) living in captivity.Case: It was treated at the Diagnostic Imaging Department of the Veterinary Hospital of the Federal University of Mato Grosso (HOVET-UFMT), 1 wild dog (C. thous), male, living in captivity with approximately 10-year-old, directed by the Center of Medicine and Research in Wild Animals of Cuiabá, to perform echocardiography exam. The patient was submitted to anesthesia for proper examination, which was used Esaote® machine model MyLabFive VET with sector scan transducer (4.0 -7.5 MHz). The longitudinal, transverse and apical scan planes were obtained through left and right parasternal windows. The evaluation of M mode exposed ejection fraction and shortening increased, of 81% and 47%, respectively, however it showed no increase in systolic and diastolic values of left ventricle, nor in right cavities on subjective evaluation. The relation between the left atrium (LA) and the aorta (Ao) remained normal, at 1.2 mm, with dimensions of 13.4 mm from the AO and 16.3 mm from LA, compatible with species parameters or domestic canines. The atrioventricular valves showed thickening and irregularities in their cusps, with great intensity in the left atrioventricular valve (LAV). The Doppler mode analysis revealed a turbulent systolic flow into the left atrium and right atrium, constituting transvalvular LAV and right atrioventricular valve- (RAV) regurgitation, both observed through the blood flow in colored Doppler and measured through the reflux velocity of 4.02 m/s of LAV and 2.17 m/s of RAV by the continuous Doppler, showing insufficiency of intense degree of LAV and moderate degree of RAV, no evidence of pulmonary hypertension. On the other hand, the relation between wave E and wave A (E/A) was 1.0, with increased transvalvular velocities and values of 0.95 m/s for wave E and A. The isovolumetric mitral relaxation time was approximately 76 m/s. The value of the pressure derivative (dp) in relation to time (dt) dp/dt measured from the LAV reflux was 1257 mmHg, within the limit considered normal for canines. Four months after the diagnosis, the patient died due to complications of chronic renal failure.Discussion: Despite being a commonly diagnosed pathology in domestic canids, the myxomatous degeneration of atrioventricular valves is still little reported in wild canids. The evaluation of the results showed that although there was severe LAV regurgitation, there was no hypertrophy or compensatory dilation of the left cavities. However, there was a compensatory increase in the shortening fraction together with the ventricular relaxation deficit. The diagnosis of this condition in Cerdocyon thous demonstrates that the pathology can affect animals of advanced age and that its incidence needs to be determined in these captive species, in order to understand the real impact of this disease in these populations. Keywords: cardiopathies, cardiac valve disease, degenerative disease, cardiological parameters, wild dog. Título: Degeneração mixomatosa das válvulas atrioventriculares em cachorro-do-mato (Cerdocyon thous) - diagnóstico ecocardiográfico Descritores: cardiopatias, doença valvular cardíaca, doença degenerativa, parâmetros cardiológicos, canídeo selvagem.

Incidence, Risk Factors, and Outcomes of Atrial Arrhythmias in Adult Patients With Atrioventricular Septal Defect.

This study aimed to assess the incidence, associated factors, and outcomes of atrial arrhythmias in adults with atrioventricular septal defect (AVSD). Data regarding atrial arrhythmias in adults with AVSD are particularly scarce. Data were analyzed from a multicentric cohort of adult patients with AVSD. Lifetime cumulative incidences of atrial arrhythmias were studied. Multiple logistic regression models were used to identify risk factors. A total of 391 patients (61.6% women) were enrolled with a mean age of 36.3 ± 16.3 years and a mean follow-up of 17.3 ± 14.2 years after initial surgical repair. Overall, 98 patients (25.1%) developed at least 1 episode of atrial arrhythmia at a mean age of 39.2 ± 17.2 years. The mean ages of patients at first episode of intra-atrial re-entrant tachycardia (IART)/ focal atrial tachycardia (FAT) and atrial fibrillation were 33.7 ± 15.3 and 44.3 ± 16.5 years, respectively. The lifetime risks for developing atrial arrhythmia to ages 20, 40, and 60 years were 3.7%, 17.8%, and 55.3%, respectively. IART/FAT was the leading arrhythmia until the age of 45, then atrial fibrillation surpassed IART/FAT. Age (odds ratio [OR]: 1.4; 95% confidence interval [CI]: 1.2-1.6), number of cardiac surgeries (OR: 4.1; 95%CI: 2.5-6.9), left atrial dilatation (OR: 3.1; 95%CI: 1.4-6.8), right atrial dilatation (OR: 4.1; 95%CI: 1.7-10.3), and moderate or severe left atrioventricular valve regurgitation (OR: 3.7; 95%CI: 1.2-11.7) were independently associated with a higher risk of atrial arrhythmias, whereas the type of AVSD and the age at repair were not. The occurrence of atrial arrhythmias was associated with pacemaker implantation (41.8% vs. 8.5%; P< 0.001), heart failure (24.5% vs. 1.0%; P< 0.001), and cerebrovascular accidents (11.2% vs. 3.4%; P = 0.007). The lifetime risk of atrial arrhythmias in patients with AVSD is considerable with more than half of patients developing≥1 atrial arrhythmia by the age of 60 and is associated with a significant morbidity. The risk in partial/intermediate AVSD is as high as in complete AVSD and is not impacted by age at repair.

An atrioventricular septal defect with an Ebsteinoid right atrioventricular valve

Abstract Funding Acknowledgements Type of funding sources: None. The combination of an atrioventricular septal defect (AVSD) with an Ebsteinoid atrioventricular valve is incredibly rare with only a handful of cases being described in the literature (1, 2). In this poster we describe the diagnosis of this anatomy in a neonate born at our centre. The baby was antenatally diagnosed with a chromosome 8p23.1 microdeletion which is associated with congenital heart disease and developmental delay (3). Although fetal echo had revealed a high suspicion of congenital heart disease, maternal BMI had prevented a precise diagnosis. The baby was born in good condition at thirty-nine weeks gestation weighing 2.4kg. She had been admitted to the neonatal unit where she was self-ventilating in room air with saturations above 90%. The echocardiogram was performed at one hour of age. The initial echo revealed a normal atrial arrangement with the heart in the left chest and the apex pointing to the left. There was normal pulmonary and systemic venous drainage. There was a complete AVSD with sizeable atrial and ventricular components. The left atrioventricular valve was dysplastic with at least moderate regurgitation. The right atrioventricular valve was displaced caudally and rotated into the right ventricular outflow tract. The right atrioventricular valve was also regurgitant. The right ventricle above the displaced atrioventricular valve was atrialized with the true right ventricle limited to the outlet portion. The great arteries were normally related. The ventricular component of the AVSD opened into the sub-pulmonary area along with two smaller muscular ventricular septal defects. These findings raised many clinical concerns including the insufficiency of the right ventricle to support a biventricular repair. Similarly, the severe left atrioventricular valve regurgitation precluded single ventricle palliative surgical techniques. After discussion between the multidisciplinary team and a second opinion sought from another centre, the family was counselled that this was a complex and life limiting form of congenital heart disease. Although surgical options were discussed with the family, the family opted for comfort care and the baby passed away at three weeks of age. Image 1: A complete AVSD with an Ebsteinoid right atrioventricular valve displaced caudally (a) resulting in an atrialized right ventricle (b). In addition, the left atrioventricular valve which straddles the ventricular septum is severely dysplastic (c) and regurgitant (d). Abstract Figure. Image 1: An Ebsteinoid AVSD

Characteristics of the pulmonary circulation in infants with complete atrioventricular septal defect.

Infants with complete atrioventricular septal defect occasionally accompany pulmonary hypertension; however, the pulmonary circulation can be altered by pulmonary vascular conditions as well as the left heart lesions. This study aimed to explore whether the left heart lesions were related to the pulmonary circulation among them. We performed echocardiography and cardiac catheterisation in 42 infants with complete atrioventricular septal defect and studied relationships between the pulmonary haemodynamic parameters and the left heart morphology. Age and weight at preoperative evaluation were 65 days (47-114) (the median following interquartile range) and 5.5 kg (4.0-7.1), respectively. There were 27 individuals with Down syndrome. Gestational age was 38 weeks (37-39). Catheterisation showed mean pulmonary arterial pressure: 36 (29-46) mmHg, the ratio of pulmonary to systemic blood flow: 3.45 (2.79-4.98), pulmonary vascular resistance: 2.20 Wood units·m2 (1.53-3.65), and pulmonary arterial compliance: 2.78 (1.86-4.10) ml/Hg/m2. Echocardiography showed the Rastelli classification type A in 28 and type C in 14, moderate or severe left atrioventricular valve regurgitation in 19 patients (45%), atrioventricular valve index of 0.67 (0.56-0.79), left ventricular end-diastolic volume z score of 4.46 (1.96-7.78), and aortic valve diameter z score of -0.70 (-1.91 to 0.20). Multivariable regression analysis revealed that preoperative pulmonary vascular resistance was significantly correlated to gestational age (p = 0.002), and that preoperative pulmonary arterial compliance was significantly correlated to gestational age (p = 0.009) and Down syndrome (p = 0.036). The pulmonary circulation does not depend upon the presence of left heart lesions but gestational age and Down syndrome in infants with complete atrioventricular septal defect.

Abstract 15099: Limitations of Predicting Postoperative Left Atrioventricular Valve Regurgitation by Transesophageal Echocardiography

Introduction: After atrioventricular septal defect (AVSD) repair, the degree of post-repair left atrioventricular valve regurgitation (LAVVR) at discharge is associated with need for re-intervention. Knowledge of the relationship between post-repair transesophageal echocardiogram (TEE) and discharge transthoracic echocardiogram (TTE) LAVVR could influence intraoperative decision-making. At present, this relationship has not been well-defined and to assess it, we undertook a retrospective study of a large cohort of AVSD patients. Methods: Between 11/2012-12/2018, 96 AVSD patients underwent surgical repair. Data included patient demographics, clinical characteristics, preoperative LAVV anomalies, surgical procedure and outcomes. LAVVR was graded as less than moderate or moderate and greater with statistical blinding techniques employed. Cohen’s kappa statistics were calculated to measure interobserver variability. Association between parameters was tested using univariate analysis. Results: Median age and weight at surgery for complete AVSD patients (68%) was 5 months and 5.6 kg respectively; for partial AVSD (32%), median age and weight was 27 months and 11.9 kg respectively. The median interval between echo studies was 6 days (IQR 4-12 days) with median length of stay of 8 days (IQR 5-16 days). There were no deaths. Post-repair TEE revealed less than moderate LAVVR in 86 patients (90%) and moderate or greater in 10 (10%). Overall, there was a discrepancy in LAVVR seen at discharge in 25 patients (26%) with 23/25 (92%) demonstrating a higher grade of LAVVR. Two patients underwent reoperation before discharge due to severe LAVVR. In univariate analysis, significant predictors for LAVVR discrepancy were younger age (p=0.032) and lower weight (p=0.032) at repair; preoperative LAVVR grade was a significant predictor for worsening LAVVR (p=0.038). Conclusions: Discrepancy between LAVVR grade on post-repair TEE and discharge TTE was present in 26% of AVSD patients with the vast majority changing to a higher grade. This important finding with implications for intraoperative decision-making should prompt further investigations into preoperative LAVV anomalies, surgical technique and postoperative factors influencing valvar function.

Early and midterm outcomes of a modified single-patch technique for repairing complete atrioventricular septal defect in children and adults.

The "modified single-patch" technique (Nunn's technique) has become more widely adopted, especially in small infants with common atrioventricular septal defect (CAVSD). In this study, we retrospectively reviewed our surgical experiences of the "modified single-patch" technique with CAVSD patients, including newborns and adults, to reveal the early and midterm clinical results. There were 233 cases diagnosed with CAVSD undergoing biventricular repair in our hospital from June 2009 to 2016. A total of 197 cases (84.5%) with the "modified single-patch" technique were enrolled in the final analysis, including type A in 106 cases (53.8%), type B in 13 cases (6.6%), type C in 15 cases (7.6%), and intermediate type in 63 cases (32%). Major associated cardiovascular malformations included five cases of unroofed coronary sinus syndrome with LSVC (UCS), five cases of coarctation of the aorta and one case of translocation of the great artery. The median age at operation of the studied 197 cases was 44.3 ± 103.3 months (ranging from 18 days to 58 years old), including two newborns. Detailed information on the "modified single-patch" procedure was described previously, and the other cardiac malformations were repaired simultaneously with surgical repair. There were 14 (7.1%) early deaths and 3 (1.5%) late deaths at 3 months and 1 year after the initial operation, respectively. The total mortality rate was 8.1%, and there was no significant difference in mortality among the different age groups (P = .291). Five patients (2.5%) underwent reoperation because of severe mitral insufficiency (MI), and another patient was reoperated for left ventricular outflow tract obstruction 3 years after the initial surgery. Long-term survival in the <3 months group was significantly lower than that in the group of older children (1-18 years old). Of the 180 survivors who were followed up for at least 1 year, the proportion of severe MI began to drop from 26.2% before the operation to 13.8% just 1-week postoperation and continued to decline to the lowest level, 3.4%, at 3 months postoperation. However, MI began to rebound at 3 months and reached 20.8% at 1-year postoperation. The rebound of MI was evident in the (3 months, 6 months) age group and the >18 years age group. However, compared to the preoperation situation, the proportion of severe tricuspid insufficiencies decreased each month from 24.8% to 0.0% until 3 months after the operation and remained at a relatively low level within 1 year after surgery. In our experience, age is not a limitation of the modified single-patch technique, and patients even in adults with surgical indications can apply this technique. In older children and adults, we should pay more attention to atrioventricular valve annuloplasty. Infants may suffer from severe residual left atrioventricular valve regurgitation, especially in the <3 months age group, so we should improve postoperative follow-up work during infancy.

Propensity-matched comparison of the long-term outcome of the Nunn and two-patch techniques for the repair of complete atrioventricular septal defects.

To compare the long-term performance of the Nunn and 2-patch techniques for the repair of complete atrioventricular septal defects. Between January 1995 and December 2015, a total of 188 patients (Nunn n = 41; 2-patch n = 147) were identified from hospital databases. Univariable Cox regression was performed to calculate the risk of reintervention in each group. Propensity score matching was used to balance the Nunn group and the 2-patch group. Baseline characteristics including age at surgery, weight, trisomy 21, other cardiac anomalies, previous operations and preoperative atrioventricular valve regurgitation did not differ between the 2 groups. Overall, there was no difference in mortality between the 2 groups (P = 0.43). Duration of cardiopulmonary bypass (CPB) and myocardial ischaemia time were 29 min (P < 0.001) and 28 min (P < 0.001) longer, respectively, in the 2-patch group. Median follow-up was 10.8 years (2-21 years). Unadjusted Cox regression did not reveal a significant difference in the risk of reoperation for either group 9 years after initial surgery [hazard ratio (HR) (Nunn) 0.512, 95% confidence interval 0.176-1.49; Nunn 89%; 2-patch 82%]. This finding was reiterated from Cox regression performed on the propensity-matched sample (31 pairs). The probability of freedom from moderate or worse left atrioventricular valve regurgitation or left ventricular outflow obstruction was similar in the 2 groups. The Nunn and 2-patch techniques are comparable in terms of the long-term mortality and probability of freedom from reoperation, moderate or severe left atrioventricular valve regurgitation and left ventricular outflow obstruction. However, the duration of CPB and myocardial ischaemia is longer in the 2-patch group.

Surgical outcome of atrioventricular septal defect: A 10 years review

Background: Surgical management of patients with Atrioventricular Septal Defect (AVSD) has advanced over the last decades. The aims of this study were to evaluate the early and late outcomes in patients undergoing operation at a single heart center. Methods: Between April 2006 and April 2016, 337 consecutive patients underwent Atrioventricular Septal Defect (AVSD) repair at the Shaheed Rajaie Heart Center. The patients were subtyped as 73 (21.7%) partial, 67 (19.9%) transitional and 197 (58.5%) complete. Results: At the time of preoperative evaluation, there were 39 cases (11.7%) with moderate to severe or severe Left Atrioventricular Valve Regurgitation (LAVVR). Previous Pulmonary Artery banding (PA banding) had been performed in 144 (42.7%) patients and associated malformations were found in 115 (34.1%). Type of surgical repair was single patch 193 (57.3%), double patch 144 (42.7%), and single atrial septal defect patch with primary ventricular septal defect closure in TAVSD 64 (95.5%). 141 patients (71.6%) with CAVSD underwent definitive early repair by using a two patch technique and complete cleft closure. Annuloplasty and/or commissuroplasty were performed for 60 (17.8%) patients and debanding was also implemented in all patients. Mean hours for ventilation were determined as 26.8 ± 2.8. Mean days for intensive care unit was defined to be 4.4 ± 0.2, and total hospitalization time was as 26.8 ± 0.5. A 1 month, 21.9% (72 of 328) had ejection fraction less than 55%; 33 (10.9%) had more than moderate LAVVR; 73 (24.2%) had residual Ventricular Septal Defect (VSD), followed by 14 cases (4.6%) with residual Atrial Septal Defect (ASD) and 2 cases (0.6%) with Left Ventricular Outlet Tract Obstruction (LVOTO). Conclusions: Definitive early repair for AVSD can be performed with acceptable results. Despite a complete cleft closure, LAVVR remains the most common residual defect that more frequently required reoperation in the future.

Repair of Partial Atrioventricular Septal Defect: Age and Outcomes

We evaluated the outcomes of patients undergoing surgical repair of partial atrioventricular septal defect (AVSD) and analyzed the effect of age on outcome. In this single-center retrospective study, we included all children who underwent repair of partial AVSD between 1990 and 2014. We divided the patients into 4 age quartiles (first quartile: 0-0.75 years, n= 22; second quartile: 0.75-1.5 years, n= 21; third quartile: 1.5-3.75 years, n= 22; and fourth quartile: >3.75 years, n= 21). These quartiles were evaluated for their association with the time-to-event outcomes of survival, freedom from left atrioventricular valve regurgitation (LAVVR), and freedom from reoperation using log-rank analysis. During the study period, 86 patients underwent partial AVSD repair at a median age of 1.5 years. There were no operative deaths and 2 late deaths (unknown cause and trauma). There were 13 reoperations. The most common cause of reoperation was left ventricular outflow tract obstruction (LVOTO) (5 patients [first quartile, 2 cases; second quartile, 1 case; third quartile, 2cases, and fourth quartile, 0 cases]). LAVV reoperation for insufficiency or stenosis was performed in 4 patients (first quartile, 1 case; second quartile, 1 case; third quartile, 1 case; and fourth quartile, 1 case). Two patients underwent pacemaker placement (second quartile, 1 case and fourth quartile, 1 case). There were no statistically significant differences in the most common complications-LVOTO, LAVVR, and AV heart block-between the 4 age quartiles. Median follow-up was 7.1 years (interquartile range [IQR],0.8-11.4 years). On echocardiography, 72 patients (84%) had less than or equal to mild LAVVR, 8 (9%) patients had mild to moderate LAVVR, 5(6%) patients hadmoderate LAVVR, and 1 (1%) patient had severe LAVVR. Age at repair had no significant association withdegree of late AV valve insufficiency. Results of partial AVSD repair at a median age of 1.5 years are excellent. Operating at this age isnot associated with increased mortality, reoperation, orLAVVR.

Factors associated with moderate or severe left atrioventricular valve regurgitation within 30 days of repair of complete atrioventricular septal defect.

IntroductionLeft atrioventricular valve regurgitation is the most concerning residual lesion after surgical correction of atrioventricular septal defects.ObjectiveTo determine factors associated with moderate or severe left atrioventricular valve regurgitation within 30 days of surgical repair of complete atrioventricular septal defect.MethodsWe assessed the results of 53 consecutive patients 3 years-old and younger presenting with complete atrioventricular septal defect that were operated on at our practice between 2002 and 2010. The following variables were considered: age, weight, absence of Down syndrome, grade of preoperative atrioventricular valve regurgitation, abnormalities on the left atrioventricular valve and the use of annuloplasty. Median age was 6.7 months; median weight was 5.3 Kg; 86.8% had Down syndrome. At the time of preoperative evaluation, there were 26 cases with moderate or severe left atrioventricular valve regurgitation (49.1%). Abnormalities on the left atrioventricular valve were found in 11.3%; annuloplasty was performed in 34% of the patients.ResultsAt the time of postoperative evaluation, there were 21 cases with moderate or severe left atrioventricular valve regurgitation (39.6%). After performing a multivariate analysis, the only significant factor associated with moderate or severe left atrioventricular valve regurgitation was the absence of Down syndrome (P=0.03).ConclusionAbsence of Down syndrome was associated with moderate or severe postoperative left atrioventricular valve regurgitation after surgical repair of complete atrioventricular septal defect at our practice.

Factors Associated with Moderate or Severe Left Atrioventricular Valve Regurgitation within 30 days of Repair of Incomplete Atrioventricular Septal Defect

IntroductionLeft atrioventricular valve regurgitation is the most concerning residual lesion after surgical correction of atrioventricular septal defect.ObjectiveTo determine factors associated with moderate or greater left atrioventricular valve regurgitation within 30 days of surgical repair of incomplete atrioventricular septal defect.MethodsWe assessed the results of 51 consecutive patients 14 years-old and younger presenting with incomplete atrioventricular septal defect that were operated on at our practice between 2002 and 2010. The following variables were considered: age, weight, absence of Down syndrome, grade of preoperative left atrioventricular valve regurgitation, abnormalities on the left atrioventricular valve and the use of annuloplasty. The median age was 4.1 years; the median weight was 13.4 Kg; 37.2% had Down syndrome. At the time of preoperative evaluation, there were 23 cases with moderate or greater left atrioventricular valve regurgitation (45.1%). Abnormalities on the left atrioventricular valve were found in 17.6%; annuloplasty was performed in 21.6%.ResultsAt the time of postoperative evaluation, there were 12 cases with moderate or greater left atrioventricular valve regurgitation (23.5%). The variation between pre- and postoperative grades of left atrioventricular valve regurgitation of patients with atrioventricular valve malformation did not reach significance (P=0.26), unlike patients without such abnormalities (P=0.016). During univariate analysis, only absence of Down syndrome was statistically significant (P=0.02). However, after a multivariate analysis, none of the factors reached significance.ConclusionNone of the factors studied was determinant of a moderate or greater left atrioventricular valve regurgitation within the first 30 days of repair of incomplete atrioventricular septal defect in the sample. Patients without abnormalities on the left atrioventricular valve benefit more of the operation.

Should Repair of Partial Atrioventricular Septal Defect Be Delayed Until Later in Childhood?

Surgical repair of partial atrioventricular septal defects (AVSD) has been successful for more than 60years. However, recent data from the Pediatric Heart Network show that 31% of patients have moderate or severe left atrioventricular valve regurgitation (LAVVR) at follow-up. Previously, our institution found that only 9% of patients had more than moderate LAVVR at the last follow-up. Our objective was to determine the long-term outcomes after repair of partial AVSD in the current era. We reviewed all patients with partial AVSD who had primary biventricular repair from January 1995 to June 2011 at our institution. The Kaplan-Meier method was used to estimate the survival free of an event, and factors were evaluated for an association with each outcome using the log-rank test. All 105 patients with partial AVSD who had surgery during this period were evaluated. The median age at surgery was 7.9years. The overall survival rate at 1 year was 97%. Median follow-up was 5.3years (interquartile range 1.7 to 11.1). At 3years, the survival rate free from reoperation was 89%. Thirteen patients required reoperations with the most common reason being LAVVR. A total of 10 patients developed more than moderate LAVVR with a cumulative incidence of 8% by 2years. The discrepancy with the Pediatric Heart Network data may be due to the later age of operation for patients in our cohort suggesting that elective repair of partial AVSD should be deferred until children are somewhat older (ages 5 to 8years). Neither patient age (p=0.11) nor severity of preoperative LAVVR (p= 0.16) were identified as statistically significant risk factors. In conclusion, there is less morbidity and mortality after surgical repair for partial AVSD.

Surgical Outcome of Reoperation Due to Left Atrioventricular Valve Regurgitation After Previous Correction of Complete Atrioventricular Septal Defect

The aims of this study were to evaluate the early and late outcomes in patients undergoing reoperation due to left atrioventricular valve regurgitation (LAVVR) after initial complete repair (ICR) of complete atrioventricular septal defect (CAVSD). Between January 1990 and April 2013, 45 consecutive patients underwent reoperation due to severe LAVVR. The mean age was 7.5 ± 6.2 years. Associated LAVV malformations were found in 22 (49%) patients and associated cardiac malformations in 18 (40%). The mean follow-up was 6.8 ± 2.6 years. LAVV repair was possible in all patients. There were two hospital deaths (4.5%). Ten patients (22%) required a second reoperation due to severe LAVVR at mean 7.5 ± 8.4 months after the first reoperation. The actuarial overall survival and free-reoperation survival rates at one, three, and five years were 95.4%, 92.8%, and 92.8% and 89%, 80.5%, and 72%, respectively. Multivariate analysis revealed that the associated cardiac malformations, LAVV leaflet prolapse or detachment from the septal patch, associated LAVV malformations, and post-first correction LAVVR grade ≥ 2 were strong predictors for poor overall free-reoperation survival in patients undergoing reoperation due to LAVVR after ICR of various forms of ACVSD. Patients with severe LAVVR post-ICR of CAVSD may undergo reoperation with acceptable postoperative mortality and morbidity; however, they are at an increased risk for developing postoperative LAVVR and subsequent reoperation.

Invited Commentary

Invited Commentary

Infective Endocarditis in Infancy After Complete Atrioventricular Septal Defect Repair

We report a rare case of infective endocarditis after repair of a complete atrioventricular (AV) septal defect in a four-month-old patient. Perforation of a valve leaflet with progressive severe left AV valve regurgitation required surgical intervention. Valve reconstruction using fresh autologous pericardium was successfully accomplished. This reconstruction in association with prolonged antibiotic therapy resulted in complete recovery of the patient.

An Unusual Case of Congenitally Corrected Transposition of the Great Arteries Associated with Noncompaction‐Like Remodeling of the Morphological Right Ventricle

Congenitally corrected transposition of the great arteries is a rare disease characterized by atrio-ventricular and ventriculo-arterial discordance (double discordance) and is associated with other cardiac anomalies in the majority of cases. The important associated anomalies include ventricular septal defect, abnormalities of left atrioventricular valve, subpulmonary stenosis, and conduction abnormalities. However, the noncompaction-like remodeling of the subaortic, morphologic right ventricle is not a commonly reported association. We, report a case of congenitally corrected transposition of the great arteries in a 40-year-old male patient, who had noncompaction-like remodeling of the morphologic right ventricle with severe ventricular dysfunction. He also had, left-sided Ebstein's anomaly, severe left atrioventricular valve regurgitation and a 2:1 atrioventricular conduction block.

Surgical Interventions for Atrioventricular Septal Defect Subtypes: The Pediatric Heart Network Experience

The influence of atrioventricular septal defect (AVSD) subtype on outcomes after repair is poorly understood. Demographic, procedural, and outcome data were obtained 1 and 6 months after AVSD repair in an observational study conducted at 7 North American centers. The 215 AVSD patients were subtyped as 60 partial, 27 transitional, 120 complete, and 8 with canal-type VSD. Preoperatively, transitional patients had the highest prevalence of moderate or severe left atrioventricular valve regurgitation (LAVVR, p = 0.01). At repair, complete AVSD and canal-type VSD patients, both with the highest prevalence of trisomy 21 (p < 0.001), were younger (p < 0.001), had lower weight-for-age z scores (p = 0.005), and had more associated cardiac defects (p < 0.001). Annuloplasty was similar among subtypes (p = 0.91), with longer duration of ventilation and hospitalization for complete AVSD (p < 0.001). Independent predictors of moderate or severe LAVVR at the 6-month follow-up were older log(age) at repair (p = 0.02) but not annuloplasty, subtype, or center (p > 0.4). Weight-for-age z scores improved in all subtypes at the 6-month follow-up, and improvement was similar among subtypes (p = 0.17). AVSD subtype was significantly associated with patient characteristics and clinical status before repair and influenced age at repair. Significant postoperative LAVVR is the most common sequela, with a similar prevalence across centers 6 months after the intervention. Annuloplasty failed to decrease the postoperative prevalence of moderate or severe LAVVR at 6 months. After accounting for age at repair, AVSD subtype was not associated with postoperative LAVVR severity or growth failure at 6 months. Further investigation is needed to determine if interventional strategies specific to AVSD subtype improve surgical outcomes.

Repair of complete atrioventricular septal defects in patients weighing less than 5 kg

Background The aim of this study was to evaluate the impact of weight less than 5 kg at operation on mortality and morbidity in patients with atrioventricular septal defect (AVSDc) undergoing total correction. Methods Between January 1990 and December 2002, 190 consecutive patients with AVSDc underwent total biventricular correction. They were divided into two groups: group I (n = 64 patients weighing < 5 kg) and group II (n = 126 patients weighing > 5 kg). Associated major cardiac malformations were found in 49 (25.8%) patients. Associated left atrioventricular valve (LAVV) malformations were found in 35 (18.4%) patients. The mean follow-up time was 4.1 ± 2.9 years (range 2 months–10.7 years). Results The in-hospital mortality in group I was 7.8% (5 patients) versus 8.7% (11 patients) in group II ( p = 0.95). Major associated cardiac malformations ( p < 0.001) and pulmonary hypertension ( p = 0.006) were found to be strong predictors for poor postoperative survival. At discharge the mean LAVVR grade in group I was 1.45 ± 1.2 versus 1.2 ± 1 in group II ( p = 0.13). The actuarial overall survival rates at 1, 3, 5, and 7 years were 96.5%, 92.5%, 91.5%, and 89% respectively and the actuarial overall reoperation free survival rates at 1, 3, 5, and 7 years were 95%, 87%, 84%, and 73%. Twenty-three patients underwent reoperation due to severe left atrioventricular valve regurgitation (LAVVR). Strong predictors for overall reoperation free survival were the operation year before 1995 ( p < 0.001), postoperative LAVVR greater than or equal to 2 ( p = 0.006), major associated cardiac malformations ( p = 0.00034), associated LAVV malformations ( p = 0.0044), and non or partial LAVV cleft closure ( p = 0.012). The actuarial survival rates between patients weighing less than 5 kg versus patients weighing more than 5 kg were similar ( p = 0.51); instead the overall reoperation free survival was significantly lower in patients weighing less than 5 kg ( p = 0.022) according to the log-rank test. Weight less than 5 kg ( p = 0.023, β = −0.6) was one of the predictors for reoperation due to severe LAVVR in this series. Conclusions We may conclude that in the current era repair of AVSDc can be carried out successfully in patients less than 5 kg, however, weight less than 5 kg at initial complete repair seems to be a predictor for late reoperation due to LAVVR. Suture separation at the cleft site or between the leaflets of the newly created mitral valve and the patch remain the main causes of postoperative LAVVR in patients weighing less than 5 kg.