Atrioventricular Valve Repair Research Articles

Optimal timing of bridging annuloplasty and patch augmentation for heterotaxy syndrome associated with functional single ventricles.

Although the challenging prognosis of functional single ventricles with common atrioventricular valves due to complex morphology and uncontrollable regurgitation by valvuloplasty has been highlighted, reports on when and how these extremely complicated atrioventricular valves should be repaired are few. This study investigated the timing and risk factors for valve intervention in these patients. Between April 2006 and March 2023, 40 patients with heterotaxy syndrome associated with functional single ventricles underwent surgery. Valve intervention was performed in 14 of the 40 patients with moderate or severe atrioventricular valve regurgitation. The timing of the first valve intervention varied, with four, five, three, and two patients undergoing valve intervention before the cavopulmonary shunt, simultaneously with the cavopulmonary shunt, before total cavopulmonary connection, and simultaneously with total cavopulmonary connection, respectively. Mechanical valve replacements were performed in three patients. Among the 14 patients undergoing valve intervention, four died. Three of the four patients underwent valvuloplasty before the cavopulmonary shunt, including two who could undergo the cavopulmonary shunt but died after the procedure. Eight of the fourteen patients completed total cavopulmonary connection. The cumulative survival rate was not significantly different between the 14 patients who underwent and 26 who did not undergo intervention (hazard ratio, 2.52; 95% confidence interval, 0.56-11.24; P = 0.23). Our surgical strategies provide a chance for the next staged repair of common atrioventricular valves in patients with both heterotaxy and valvular regurgitation. Including patch augmentation, advanced valve intervention is possible at or after the cavopulmonary shunt.

Atrioventricular valve surgery in patients with univentricular heart and two separate atrioventricular valves.

Atrioventricular valve regurgitation in patients with univentricular heart is a well-known risk factor for adverse outcomes and atrioventricular valve repair remains a particular surgical challenge. We reviewed all surgical atrioventricular valve procedures in patients with univentricular heart and two separate atrioventricular valves who underwent surgical palliation. Endpoints of the study were reoperation-free survival and cumulative incidence of reoperation. Between 1994 and 2021, 202 patients with univentricular heart and two separate atrioventricular valve morphology underwent surgical palliation, with 15.8% (32/202) requiring atrioventricular valve surgery. Primary diagnoses were double inlet left ventricle (n = 14, 43.8%), double outlet right ventricle (n = 7, 21.9%), and congenitally corrected transposition of the great arteries (n = 7, 21.9%). Median weight at valve surgery was 10.6 kg (interquartile range, 7.9-18.9). Isolated left or right atrioventricular valve surgery was required in nine (28.1%) and 22 patients (68.8%), respectively. Concomitant left and right atrioventricular valve surgery was performed in one patient (3.1%). Closure of the left valve was conducted in four patients (12.5%) and closure of the right valve in three (9.4%). Operative and late mortality were 3.1% and 9.7%, respectively. Reoperation-free survival and cumulative incidence of reoperation at 10 years after surgery were 62.3% (standard error of the mean: 6.9) and 30.9% (standard error of the mean: 9.6), respectively. In patients with univentricular heart and two separate atrioventricular valves, surgical intervention on these valves is required in a minority of patients and is associated with low mortality but high incidence of reoperation.

Staged repair of borderline hypoplastic heart disease with early biventricular conversion

ObjectiveIn select patients with borderline ventricular hypoplasia, we adopted a strategy of initial single-ventricle palliation followed by staged or direct biventricular conversion by 2 years of age. MethodsBetween 2018 and 2023, 14 newborns with borderline hypoplastic heart disease deemed high risk for primary biventricular repair underwent palliative procedures as a neonate/infant, followed by staged or direct biventricular conversion. ResultsOf the 14 patients, 6 had borderline left ventricles and 8 had borderline right ventricles. Index neonatal operations were performed in 12 patients and included the Norwood operation (n = 5), pulmonary artery band (n = 3), ductal stent (n = 3), and hybrid Norwood (n = 1). Five patients underwent direct biventricular conversion, and the remaining 9 patients underwent staged ventricular recruitment operations at a mean age of 6 months (range, 3-11 months). Ventricular recruitment operations included atrial septation with or without ventricular rehabilitation, atrioventricular valve repair, or outflow tract operations. At a mean duration of 8 months (range, 4-10 months) after ventricular recruitment, there was a significant increase in chamber volume, aortic valve, and mitral valve size in patients with borderline left ventricles, and a normalization of the right ventricle:left ventricle end-diastolic volume ratio in patients with borderline right ventricles. To date, 13 of 14 patients have undergone successful biventricular conversion at a mean age of 16 months (range, 4-31 months). ConclusionsIn select newborns with borderline hypoplastic heart disease, single-ventricle palliation followed by staged or direct biventricular conversion may increase infant survival while allowing for early attainment of a biventricular circulation.

Discussion to: Left atrioventricular valve repair after primary atrioventricular canal surgery: Predictors of durability

Discussion to: Left atrioventricular valve repair after primary atrioventricular canal surgery: Predictors of durability

Surgical outcomes of double-orifice mitral valve repair in patients with atrioventricular canal defects: a systematic review and meta-analysis.

Double-orifice mitral valve or left atrioventricular valve is a rare congenital cardiac anomaly that may be associated with an atrioventricular septal defect. The surgical management of double-orifice mitral valve/double-orifice left atrioventricular valve with atrioventricular septal defect is highly challenging with acceptable clinical outcomes. This meta-analysis is aimed to evaluate the surgical outcomes of double-orifice mitral valve/double-orifice left atrioventricular valve repair in patients with atrioventricular septal defect. A total of eight studies were retrieved from the literature by searching through PubMed, Google Scholar, Embase, and Cochrane databases. Using Bayesian hierarchical models, we estimated the pooled proportion of incidence of double-orifice mitral valve/double-orifice left atrioventricular valve with atrioventricular septal defect as 4.88% in patients who underwent surgical repair (7 studies; 3295 patients; 95% credible interval [CI] 4.2-5.7%). As compared to pre-operative regurgitation, the pooled proportions of post-operative regurgitation were significantly low in patients with moderate status: 5.1 versus 26.39% and severe status: 5.7 versus 29.38% [8 studies; 171 patients]. Moreover, the heterogeneity test revealed consistency in the data (p < 0.05). Lastly, the pooled estimated proportions of early and late mortality following surgical interventions were low, that is, 5 and 7.4%, respectively. The surgical management of moderate to severe regurgitation showed corrective benefits post-operatively and was associated with low incidence of early mortality and re-operation.

Catheter-Based Techniques for Addressing Atrioventricular Valve Regurgitation in Adult Congenital Heart Disease Patients: A Descriptive Cohort.

Increasing survival of adult congenital heart disease (ACHD) patients comes at the price of a range of late complications-arrhythmias, heart failure, and valvular dysfunction. Transcatheter valve interventions have become a legitimate alternative to conventional surgical treatment in selected acquired heart disease patients. However, literature on technical aspects, hemodynamic effects, and clinical outcomes of percutaneous atrioventricular (AV) valve interventions in ACHD patients is scarce. This is a descriptive cohort from CAHAL (Center of Congenital Heart Disease Amsterdam-Leiden). ACHD patients with severe AV valve regurgitation who underwent a transcatheter intervention in the period 2020-2022 were included. Demographic, clinical, procedural, and follow-up data were collected from patient records. Five ACHD patients with severe or torrential AV valve regurgitation are described. Two patients underwent a transcatheter edge-to-edge repair (TEER), one patient underwent a valve-in-valve procedure, one patient received a Cardioband system, and one patient received both a Cardioband system and TEER. No periprocedural complications occurred. Post-procedural AV valve regurgitation as well as NYHA functional class improved in all patients. The median post-procedural NYHA functional class improved from 3.0 (IQR [2.5-4.0]) to 2.0 (IQR [1.5-2.5]). One patient died 9 months after the procedure due to advanced heart failure with multiorgan dysfunction. Transcatheter valve repair is feasible and safe in selected complex ACHD patients. A dedicated heart team is essential for determining an individualized treatment strategy as well as pre- and periprocedural imaging to address the underlying mechanism(s) of AV regurgitation and guide the transcatheter intervention. Long-term follow-up is essential to evaluate the clinical outcomes of transcatheter AV valve repair in ACHD patients.

Predictors of protein losing enteropathy after Fontan completion: An 8-year retrospective study at Sheikh Khalifa Medical City.

The Fontan procedure is the final stage of a three-stage palliation process in patients born with a univentricular heart as part of hypoplastic left heart syndrome (HLHS) or other pathologies with a univentricular heart. As essential as this procedure has proven to be for such cases, the Fontan physiology diminishes cardiac output and expands systemic venous pressure, which then leads to venous congestion that can be complicated by protein-losing enteropathy (PLE). This retrospective study aimed to identify the predictors of such complications in all patients who underwent completion of the Fontan procedure at our center (Sheikh Khalifa Medical City/SKMC) in the past eight years. This study examined the medical records of patients who underwent completion of Fontan repair at our center since the inauguration of the cardiac surgery program of SKMC in the United Arab Emirates (UAE) - 01 Jan 2012 to 31 Dec 2020. Exclusion criteria included the absence of any of the undermentioned data in patient files. Patients were divided into two groups: those who developed PLE and those who did not. For each group, the following data were collected: demographics data (current age and age at completion of Fontan), clinical and laboratory data (oxygen saturation, serum albumin), echocardiographic data (classification of original cardiac diagnosis, degree of atrio-ventricular valve regurgitation, ventricular functions), hemodynamic data (mean pressures of superior vena cava and pulmonary arteries before Fontan completion), operative data (type of initial palliation, type of Fontan, presence of fenestrations and its size) and the need for any cardiac intervention prior to Fontan completion, such as atrio-ventricular valve repair, peripheral pulmonary stenting and arch balloon dilatation. Of the 48 included patients,13 (25%) developed PLE. Multivariate regression analysis proved that the best predictors of PLE were superior vena cava mean pressure (P=0.012) and the degree of atrio-ventricular valve regurgitation (P=0.013). An oxygen saturation <83% prior to Fontan completion was 92% sensitive in predicting PLE after Fontan completion. This is a single-center study of the predictors of PLE after Fontan procedure and, as expected from similar studies, SVC pressure higher than 11 mmHg and moderate-to-severe atrio-ventricular valve regurgitation were predictors of Fontan failure. The higher prevalence of PLE in our cohort, as well as lower cut-offs of SVC pressure that can predict complications, may be related to the predominance of hypoplastic left heart in the operated patients, which has been the main referral center for cardiac surgeries in UAE in the last decade.

Left atrioventricular valve repair after primary atrioventricular canal surgery: Predictors of durability

ObjectiveAcute outcomes after atrioventricular canal defects (AVCD) surgery in the current era are excellent; yet despite surgical advances, ∼15% of patients require future left atrioventricular valve (LAVV) repair. Among patients with AVC who undergo LAVV repair after primary AVC surgery, we sought to characterize the durability of these repairs. Specifically, we aimed to determine predictors for reintervention following an LAVV repair in patients with repaired AVCD, with a focus on postoperative transesophageal echocardiography (TEE). MethodsWe reviewed all patients undergoing LAVV repair (after a primary AVCD surgery) at Boston Children's Hospital between 2010 and 2020. Competing risk analysis was performed to evaluate cumulative incidence of LAVV reinterventions. Predictors of LAVV reintervention were evaluated using multivariable Cox regression. ResultsA total of 137 LAVV repairs following primary AVCD surgery were performed in 113 patients. Median age and weight at LAVV repair were 25 months (interquartile range, 12-76 months) and 11.1 kg (interquartile range, 7.8-19.4 kg). Original anatomy was complete AVCD in 87 (63%), transitional AVCD in 27 (20%), and partial AVCD in 23 (17%) cases. Over a median follow-up of 12 months (interquartile range, 1.3 months-4 years), 47 (34%) of the LAVV repairs required LAVV reintervention. Reinterventions included a total of 27 LAVV re-repairs and 20 LAVV replacements. In multivariable analysis, age at LAVV repair younger than 72 months, partial AVCD anatomy, left ventricle dysfunction, mean LAVV stenosis gradient ≥5 mm Hg, and multiple jets of regurgitation on postoperative LAVV repair TEE were associated with LAVV reintervention. Grade of LAVV regurgitation on postoperative TEE was not an independent risk factor, but reintervention rates were high when residual LAVV stenosis gradient was ≥5 mm Hg and residual mild LAVV regurgitation was present on postoperative TEE (47%) and even higher when residual LAVV stenosis gradient was ≥5 mm Hg and LAVV regurgitation was greater than mild (73%). ConclusionsReintervention rates remain high for LAVV repairs that occur after primary AVCD surgery, particularly for patients with LAVV stenosis gradient ≥5 mm Hg and mild or greater LAVV regurgitation on postoperative TEE.

The Fontan procedures – early outcomes and predictive factors

Objectives: the aim of this study was to describe the early outcomes after the extracardiac conduit Fontan procedure and evaluate predictive factors related to these results.&#x0D; Methods: a cross – sectional study. We retrospectively reviewed the perioperative medical records of 71 patients with Fontan surgery at Hanoi Heart Hospital between January 2017 and December 2020. Data processing using SPSS 22 software&#x0D; Results: the early mortality and early failure rates with Fontan circulation were 4.2%. High postoperative pulmonary artery pressure was a risk factor for early mortality and early Fontan failure. The mean duration of mechanical ventilation was 62.86±108.17 hours; female, short height, and common atrioventricular valve or double­inlet atrioventricular connection increased the risk of prolonged mechanical ventilation. The mean intensive care unit (ICU) length of stay was 6.46±6.32 days, female, short height, and moderate to severe postoperative atrioventricular valve regurgitation increased the risk of prolonged duration in ICU. The mean time of pleural drainage was 16.96±10.23 days, low weight was a risk factor for prolonged pleural drainage. The mean postoperative hospital stay was 23.89±11.83 days, age at the Fontan surgery, preoperative oxygen saturation, concomitant atrioventricular valve repair, and postoperative atrioventricular valve regurgitation were factors associated with prolonged postoperative hospital stay.&#x0D; Conclusion: the early outcomes after Fontan surgery have been consistent with our condition. There have been many risk factors that influenced early results after surgery, and identifying those factors may help with our treatment.

Application of the Alfieri technique for the resolution of tricuspid dysplasia in 7 canines

The application of the Alfieri technique in humans is a well-known and pioneering thechnique that was replaced by modern atrioventricular valve repair strategies.1,2 This technique in canines and also in humans is yet a valid alternative, particullary to canine tricuspid dysplasia.1 The “strategy” consists to transform the “monorificial” dysplastic tricuspid valve insufficiency into a “biorificial” one by means of a suturing at the level of the valve apposition face in the site of greatest regurgitant flow. The surgical field is cleared from blood by means of occlusion of both venae cavae. With this strategy the reduction of the insufficiency jet was achieved in 7 canine carriers of said congenital pathology. The technique demonstrated its anatomical reliability, repeatability, low mortality, and low cost. Of the total number of cases operated on, one patient died the day after surgery, another patient a year later due to non-cardiac causes, and the rest continue under clinical follow-up at the time of presenting this series.

Kawashima Procedure: The Impact of Age

BackgroundThe results of the Kawashima operation are incompletely defined. Furthermore, optimal timing of Kawashima operation, an important consideration when managing desaturated young infants awaiting surgery, remains unclear. We reviewed our outcomes of Kawashima operation, with a focus on the impact of age. MethodsWe conducted a retrospective review of patients who underwent Kawashima operation from 1990 to 2020. ResultsThirty patients underwent Kawashima operation at a median age of 11.7 months (interquartile range, 4.4-27.4). Left isomerism was present in 27 patients (90%). There were no early deaths. There were 2 patients (6.7%) who had Kawashima takedown, both attributed to hypoxia. Fontan completion was achieved in 25 patients (83%). Overall freedom from death and transplantation at 20 years was 67% (95% CI, 32%-87%). Freedom from death and transplantation at 10 years was 100% for children 3 to 6 months of age at the time of Kawashima, compared with 86.4% for older children (P = 1.0). However, children aged 3 to 6 months had longer ventilation time (P = .01), intensive care stays (P = .03), and hospital stays (P = .05). Freedom from death or transplantation at 20 years among those who had concomitant common atrioventricular valve repair was 33%, vs 79% for those who did not undergo common atrioventricular valve repair (P = .02). ConclusionsKawashima operation can be performed with low operative risk and acceptable long-term outcomes. Performing Kawashima operation on a patient aged less than 6 months does not affect survival, but is associated with increased morbidity. Need for common atrioventricular valve operation carries significant risk of mortality, and more effective techniques for atrioventricular valve repair are required.

Fetal Kadavralarda Plantaris’in Morfometrik ve Morfolojik Analizi

Objective: The plantaris is a muscle in the back of the leg that has a short body and a long, thin tendon. The muscle acts functionally with the gastrocnemius. Due to removal of the muscle does not cause a change in limb function, it is used as a potential source of graft. The aim of the present study was to investigate the morphological features and possible variations of plantaris in fetal cadavers.Methods: This study was carried out on the fetal cadaver collection of University of Necmettin Erbakan, Faculty of Medicine, Anatomy Department. 11 male and 9 female fetal cadavers (ages range: 28 to 40 weeks) were used. The muscle belly length (LB), width (WB) and thickness (TB) were measured. The plantaris insertion was classified into 4 types. Furtermore, the distal part of the tendon was classified as the fan-shaped and flat-shaped.Results: It was determined that plantaris was present in 36 of 40 (90%) lower extremities and absent in 4 (10%). The average muscle belly length (LB), width (WB) and thickness (TB) was found 13.29±3.73 mm, 4.72±1.77 mm and 3.65±1.17 mm, respectively. It was observed that the plantaris tendon often (Type 2, 67.5%) inserts in the anteromedial of the calcaneal tendon. Furthermore, the most common insertion type was observed as flat-shaped (86.11%). Conclusion: We believe that the data obtained from our study will be useful in procedures such as flexor tendinoplasties, reconstruction of hand tendons and lateral ankle ligaments, and repair of atrioventricular valves, which are planned to be performed in infancy and childhood.

Liver Cirrhosis After the Fontan Procedure: Impact of Atrioventricular Valve Failure

BackgroundLiver cirrhosis is now well recognized as a potential complication after the Fontan procedure, although associated risk factors and optimal timing of liver screening remain unclear. MethodsAll patients who underwent an extracardiac conduit Fontan procedure at The Royal Children’s Hospital, Melbourne, were identified using the Australia and New Zealand Fontan Registry. Cirrhosis was diagnosed based on liver biopsy, or a combination of imaging findings and clinical evaluation by a hepatologist. ResultsBetween 1997 and 2020, 398 patients underwent an extracardiac conduit Fontan procedure at our center, and 276 had ongoing follow-up in Victoria. Ninety-five patients (34%) underwent liver assessment at a mean age of 18.2 ± 6.7 years (11.8 ± 5.5 years post-Fontan). Fifteen patients (16%) were diagnosed with cirrhosis at a mean age of 22.7 ± 5.9 years (14.0 ± 5.2 years post-Fontan). The need for prior or concomitant atrioventricular valve repair or replacement was associated with an increased risk of cirrhosis (univariable hazard ratio [HR] 7.09, 95% confidence interval [CI] 2.13-23.61, P = .001). By multivariable analysis, factors associated with development of cirrhosis were atrioventricular valve failure prior to Fontan (HR 3.27, 95% CI 1.15-9.31, P = .026) and older age at Fontan operation (HR 1.13 per year increase, 95% CI 1.01-1.26, P = .034). The proportion of patients alive, nontransplanted, and without cirrhosis at 10, 15, and 20 years was 93.4% (95% CI 88.4%-98.7%), 79.6% (95% CI 69.7%-90.8%), and 64.6% (95% CI 51.0%-81.9%), respectively. ConclusionsEarly commencement of liver screening should be considered for patients with a history of atrioventricular failure during Fontan palliation.

Repair of Common Atrioventricular Valve in Univentricular Circulation Using Adjustable Annular Bridging Technique

The main reason for common atrioventricular valve regurgitation is the enlargement of the valve annulus contralaterally to the aorta and pulmonary artery. Edge-to-edge repair can be used to address the regurgitation; however, there might be potential disadvantage that a fixed bridging leaflet could have a restrictive motion, leading to recurrence of atrioventricular valve regurgitation. To avoid this issue and address the anteroposterior dilatation of the annulus, we have developed an adjustable annular bridging technique. The main reason for common atrioventricular valve regurgitation is the enlargement of the valve annulus contralaterally to the aorta and pulmonary artery. Edge-to-edge repair can be used to address the regurgitation; however, there might be potential disadvantage that a fixed bridging leaflet could have a restrictive motion, leading to recurrence of atrioventricular valve regurgitation. To avoid this issue and address the anteroposterior dilatation of the annulus, we have developed an adjustable annular bridging technique. Commentary: The Adjustable Bridge – Definitely a Useful Technique; HoweverOperative Techniques in Thoracic and Cardiovascular SurgeryVol. 27Issue 3PreviewI am grateful to the authors and commend them for sharing their technique of adjustable annular bridge for patients with single-ventricle (SV) hearts and atrioventricular valve regurgitation (AVVR).1 They have provided clear technical detail and excellent illustrations and video. No one questions the significance of AVVR in these patients, its significant impact on prognosis, and the difficulty in achieving durable, excellent valve competence. Likewise, we acknowledge the numerous technical repair tools at our disposal and that frequently we use most, if not all, of them in any given case (side to side, cleft or scallop closure, various degrees of annuloplasty, etc.). Full-Text PDF Commentary: Atrioventricular Valve Repair in Univentricular Hearts: No Longer a Bridge Too Far?Operative Techniques in Thoracic and Cardiovascular SurgeryVol. 27Issue 3PreviewIt is increasingly apparent that atrioventricular valve (AVV) failure is a major cause of mortality in patients with a Fontan circulation.1 Furthermore, patients with a common AVV are at the highest risk of AVV failure among those with a Fontan circulation.1-4 Atrioventricular valve repair is notoriously difficult in the setting of a common AVV, and failed repair is associated with very high mortality.3 One of the major contributing factors to AVV failure in these patients is the attenuated fibrous skeleton of the heart, which increases the risk of annular dilatation. Full-Text PDF

In-Hospital Outcomes in Fontan Completion Surgery According to Age

Actual timing of the Fontan operation is variable. Our aim was to evaluate the impact of age at the time of Fontan operation on mortality and clinical outcome and characterize patients with worse outcomes. We conducted a retrospective, cross-sectional study on the Fontan operation using nationally representative databases from 2003 to 2016 and categorized the patient into 1 of 5 groups according to their age at the time of surgery: <2, 2, 3, 4, and ≥5years. Survey-weighted logistic regression models were used to compare the outcomes of the different age groups. A total of 6,647 children underwent the Fontan completion procedure during the study period with median age 3 (interquartile range 2 to 4) years. The in-hospital mortality was 2.1%. In logistic regression models, in-hospital mortality, respiratory failure, acute kidney injury, chylothorax, arrhythmias, and sudden cardiac arrest were similar among the 5 age groups. Compared with children >2 years, those <2 years were less likely to be admitted for surgery on an elective basis (73.5% vs 90.4%, p <0.001), more likely to have chromosomal anomalies (2.7% vs 1.7%), and more likely to have repair of atrioventricular valves (8.5% vs 6.0%, p=0.027). Mortality was higher in those with an underlying atrioventricular septal defect (AVSD) adjusted odds ratio 4.3 (2.4 to 7.9, p <0.001). Repair of AV valves was more common in the AVSD group compared with those in non-AVSD (14.3% vs 5.5%, p <0.001). In conclusion, age at Fontan completion does not adversely affect the in-hospital outcomes. Our focus should be on optimizing essential factors that are crucial for successful Fontan completion.

Atrioventricular Valve Repair in Patients with Univentricular Circulation

Atrioventricular valve (AVV) disease remains a challenge in the management of patients with congenital heart disease, particularly those with single ventricle palliation. Within this group, atrioventricular valve (AVV) disease remains the Achilles heel of long-term successful palliation. In recent years we have learned how AVV pathology is detrimental to late survival, as well as how challenging it can be to obtain a competent and durable repair. In this expert review we outline strategies for AVV repair depending on AVV morphology: mitral, tricuspid, or common atrioventricular valve. Given the broad scope of AVV pathology, a large number of tools are needed in a congenital cardiac surgeon's armamentarium to successfully treat these patients. Many of these techniques have been successfully employed in patients with biventricular physiology, but can have applicability to those with single ventricle palliation.

Surgery for Functional Single Ventricle Associated with Heterotaxy Syndrome: Common Atrioventricular Valve Repair

Surgery for Functional Single Ventricle Associated with Heterotaxy Syndrome: Common Atrioventricular Valve Repair

The Failed Bidirectional Glenn Shunt: Risk Factors for Poor Outcomes and the Role of Early Reoperation.

Background: Bidirectional Glenn shunt (BDG) failure carries high morbidity and mortality but the clinical factors associated with failure and the optimal management strategy are understudied. Methods: A total of 217 patients undergoing BDG at our institution between 1989 and 2020 were retrospectively reviewed and categorized as success or failure. Failure was defined as the need for reoperation (BDG takedown, reoperation for correction of cardiac defect, and/or transplantation) at any time postoperatively; operative mortality (death attributable to BDG malfunction occurring during the index hospitalization for BDG or within 30 days of discharge); or late mortality (death directly attributable to BDG malfunction occurring prior to Fontan or next-stage palliation). Univariate and binary logistic regression analyses were performed. Results: BDG failure occurred in 14 (6.5%) patients. Univariate predictors were: hypoplastic left heart syndrome (P = .037), right ventricular (RV) dominance (P = .010), greater pre-BDG pulmonary vascular resistance (PVR) (P = .012), concomitant atrioventricular valve repair (P = .020), prolonged pleural drainage (P = .001), intensive care unit (P<.001) and hospital (P = .002) stays, and extracorporeal membrane oxygenation (ECMO) requirement (P<.001). Multivariate predictors were: RV dominance (P = .002), greater PVR (P = .041), ICU (P<.001) and hospital (P = .020) stays, and need for ECMO (P<.001). As many as 10 of 14 (71%) patients with BDG failure died. Reoperation was performed for 10 patients with BDG failure. Five reoperation patients survived until discharge, with four patients alive at last follow-up (mean 7.9 years). Survivors underwent reoperation earlier than nonsurvivors (36 vs. 94 days). Conclusions: BDG failure carries high mortality, but preoperative predictors and postoperative indicators of failure exist. Early BDG takedown and insertion of aorta-pulmonary shunt may allow survival.

Long term outcomes for adults with complete atrioventricular septal defects

Abstract Background/Introduction Complete Atrio-Ventricular septal defect (AVSD) is a complex congenital cardiac disease, characterised by malformation at the atrio-ventricular junction and AV valve abnormalities. Long- term outcome data is limited. Purpose We sought to describe the long-term outcomes of adults with repaired complete AVSD in a tertiary adult congenital heart centre. Methods We retrospectively recruited patients with complete AVSD who underwent surgical repair between 1973 and 2001 in our centre. All clinical and echocardiographic data were collected and analysed for evidence of atrio-ventricular valve (AVV) deterioration, or cardiac dysfunction. We also assessed for the requirement for further surgical intervention throughout the follow up period. Results A total of 345 patients with AVSD were identified, partial AVSD 211, unrepaired 82. Fifty-two with repaired complete AVSD formed study group, female 36 (69%) and male 16 (31%), Trisomy 21 (23, 44%). Mean age at initial repair was 44 months (median 12, IQR 31.5). Mean follow up was 25.3 years (Median 24, IQR 9.75). Clinical status: At the latest follow-up, most patient were asymptomatic with NYHA I-II (n=46) Majority (n=36, 69%) required only initial repair and no further intervention. Further surgeries were performed in 16 patients including AVV repair (n=9) and LVOTO relief (n=3). Permanent pacemaker insertion needed in 6 (12%), all for heart block following valve repair. 7 patients (14%) had documented arrythmia, 4 (8%) requiring ablations and only 1 had endocarditis. Echo findings: AV Valve dysfunction was more commonly regurgitant rather than stenotic. More than moderate AVV regurgitation was present in 83% of patients, Left AVV (n=25, 48%), Right AVV (n=18, 35%) compared to 4% stenosis (LAVV n=1, RAVV n=1). Ventricular outflow tract obstruction was present in 8% of patients, RVOT obstruction (2, one native, one secondary to prior banding), LVOT obstruction (2, both native, one requiring surgical intervention). Right ventricular systolic dysfunction was present in 6% of patients (n=3), with mean TAPSE 14.1mm (SD± 3.1mm). Left ventricular dysfunction was present in 4% (n=2), mean LVEF 58.9% (SD±7.1%) and mean LV EDVi 55.06mL/m2 (SD±13.2 mL/m2). Only one patient had significant pulmonary hypertension (Mean PAP 48 mmHg). Conclusion Long-term outcomes of surgically repaired AVSD are highly favourable. Left AVV regurgitation is the most common residual lesion requiring further surgical intervention. Ventricular outflow tract obstruction was much less common, as was ventricular systolic dysfunction. Complete heart block was associated with surgical repair and arrhythmias were potential late complications. Funding Acknowledgement Type of funding sources: None.

The impact of intraoperative pericardial three-dimensional echocardiography for the atrioventricular valve repair in the patient with congenital heart disease

Abstract Background Atrioventricular valve (AVV) regurgitation enormously affected the survival outcome of the patients with congenital heart disease (CHD). However, the image quality by use of transthoracic echocardiography has not reached a level that is sufficient, and also, three-dimensional echocardiography, which is useful to clarify complex AVV anatomy, cannot be applied for the patients less than 15kg, to guide for the AVV repair in pediatric patients. We try to show surgeons more precise three-dimensional images about an AVV by using intraoperative pericardial three-dimensional echocardiography (IP3DE) and improve the surgical outcome. Purpose To determine the efficacy of IP3DE by assessing the surgical outcome of an AVV repair and re-intervention rate. Method Eighty-five patient with CHD who underwent atrioventricular repair with significant regurgitation (Grade 2–4+) before operation were divided into two groups imaged IP3DE or not, in our hospital from 1993 to 2020. We assessed the surgical outcome and re-intervention rate between two arms and re-evaluate AVV images before surgery compared to the IP3DE. Result IP3DE was performed in forty-six patients (IP3DE group) and thirty-nine patients were not (control group). Median age at AVV repair was 3.0/2.8 years, respectively. The AVV was tricuspid (n=25), mitral (n=41), or common (n=19). The IP3DE group had a significantly higher improvement in regurgitation of AVV (IP3DE: Grade 3.2±0.3 → 1.7±0.3 vs Control: Grade 2.8±0.3 → 1.8±0.3, p&amp;lt;0.05). Fifty-nine percent of the IP3DE group was successful outcome (Grade&amp;lt;1+ after repair). There was no significant difference in the rate of re-intervention after surgery between two groups. In multivariate analysis, using IP3DE contributed to successful outcome for AVV repair (OR: 4.66, 95% CI: 1.46–14.8, p&amp;lt;0.01). The different and/or additional anatomical AVV findings were obtained in sixty-one percent of patients by the IP3DE. Conclusion IP3DE contributes to successful outcome for AVV repair by obtaining further information on complicated AVV anatomy in congenital heart disease. IP3DE also enables both cardiovascular surgeons and cardiologists to share the accurate and detail “surgeon's view” in the operating room for planning of AVV repair. Funding Acknowledgement Type of funding sources: None.