Severe Atrioventricular Valve Regurgitation Research Articles

Predictors of spontaneous pregnancy loss in single ventricle physiology

BackgroundPregnant patients with single ventricle (SV) physiology carry a high risk of spontaneous pregnancy loss (SPL), yet the clinical factors contributing to this risk are not well defined.MethodsSingle-centre retrospective study...

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.

Double outlet right ventricle accompanied by complex congenital anomalies: A rare association

Double outlet right ventricle (DORV) comprises a heterogeneous series of associated cardiac anomalies that involve the RV outflow tract in which both of the great arteries arise entirely or predominantly from the RV. The anatomic dysmorphology is variable, and there may be associated cardiac anomalies. We report here a case of a complex cyanotic heart disease in a 14 year old adolescent comprising of DORV accompanied by multiple congenital cardiac anomalies: single atrium, complete AV canal defect, severe AV valve regurgitation, D-malposition of great arteries, subaortic obstruction and pulmonary valvular stenosis.

Severity of tricuspid regurgitation predicts risk of atrial fibrillation recurrence after pulmonary vein isolation

Abstract Background Atrioventricular valve regurgitation (AVVR) results in atrial remodeling, thus promoting the formation of a substrate for atrial fibrillation (AF). On the other hand, AF may cause functional AVVR by annular dilatation of the atria. In this vicious circle, the identification of the leading pathomechanism and consequently the optimization of treatment can be challenging. Purpose Our aim was to investigate whether the presence of significant AVVR and especially of tricuspid regurgitation (TR) affects the efficacy of catheter-based pulmonary vein isolation (PVI) for AF. Furthermore, we evaluated whether rhythm control for AF through PVI could lead to an improvement of AVVR severity. Finally we examined the effect of concomitant right atrial flutter on the arrhythmic outcome in patients with significant TR . Methods We enrolled patients undergoing first-time PVI for symptomatic AF. We compared the severity of AVVR at the time of the procedure as well as after 6 months, using transthoracic echocardiography. Moderate to severe AVVR was defined as significant. Moreover, we evaluated whether the presence of significant AVVR, at baseline and 6 months after the procedure, could serve as an independent predictor for AF recurrence. Results Out of 320 consecutive patients (mean age 66.3 ± 10 years, 61.6% male, 60% paroxysmal AF) , significant TR at baseline was documented in 13.1%, while significant mitral regurgitation (MR) in 10.9%. Six months after PVI, the proportion of patients with moderate to severe TR (13.1% to 7.2%; p<0.001) and MR (10.9% to 6.6%; p<0.001) decreased significantly. Compared to patients with post-interventional improvement of significant TR, right atrial (RA) dilatation at baseline was more pronounced in patients with persistent significant TR (RA area 20.2 ± 4.4 vs. 26.6 ± 8.3 cm2; p=0.002). The presence of significant TR, particularly without improvement during the follow-up, independently increased the risk of atrial fibrillation recurrences. Even after propensity-score matching, TR at baseline remained an independent risk predictor regarding recurrent atrial fibrillation (HR 2.2 [95% CI, 1.1 - 4.9]; p=0.045). Among patients with significant TR, those with documented right atrial flutter were at high risk for recurrence of atrial arrhythmia (HR 2.69 [95% CI, 1.0 – 6.9]; p=0.039), despite the additional ablation of the cavo-tricuspid isthmus. Significant MR was not associated with increased risk of atrial arrhythmias. Conclusion In patients with symptomatic AF undergoing PVI, the presence of moderate to severe TR, particularly without improvement 6 months after the procedure, was associated with a worse arrhythmic outcome. Further investigations to enhance the understanding of the underlying electrophysiological substrate and the need for tailored ablation strategies are warranted.TR as predictor for AF(propensity score)AVVR improvement after PVI

Early and long-term outcomes following cardiac surgery for patients with heterotaxy syndrome

ObjectiveHeterotaxy syndrome is a complex multisystem abnormality historically associated with high morbidity and mortality. We sought to evaluate the early and long-term outcomes after cardiac surgery in heterotaxy syndrome. MethodsThis is a single-center retrospective review of patients with heterotaxy syndrome undergoing single-ventricle palliation or primary or staged biventricular repair from 1998 to 2018. Patients were stratified by single ventricle versus biventricular physiology, and the severity of atrioventricular valve regurgitation. Demographics, anatomic characteristics, and early and late outcomes, including the length of stay, mortality, and surgical or catheter reinterventions, were analyzed. ResultsAmong 250 patients, 150 (60%) underwent biventricular repair. In-hospital mortality was 7.6% (n = 19). Median follow-up was 5.2 (range, 0-16) years. Among survivors to discharge, mortality was 19% (n = 44) and reintervention was 52% (n = 120). Patients with moderate/severe atrioventricular valve regurgitation were older (32 vs 16 months, P = .02), were more likely to experience adverse events during their index surgical admission (72% vs 46%, P < .001), and had longer in-hospital length of stay (20 vs 12 days, P = .009). Among patients with moderate to severe atrioventricular valve regurgitation, single-ventricle palliation is associated with a greater risk of unplanned reintervention compared with patients undergoing biventricular repair (hazard ratio, 2.13; CI, 1.10-4.12; P = .025). ConclusionsThere was no significant difference in early or late outcomes in single-ventricle versus biventricular repair strategies in heterotaxy. In the subgroup of patients with moderate/severe atrioventricular valve regurgitation, patients who underwent single-ventricle palliation were 2.5 times more likely to need a late reintervention compared with those undergoing biventricular repair.

Introduction of transcatheter edge-to-edge repair in patients with congenital heart disease at a children's hospital.

Atrioventricular valve regurgitation (AVVR) is a devastating complication in children and young adults with congenital heart disease (CHD), particularly in patients with single ventricle physiology. Transcatheter edge-to-edge repair (TEER) is a rapidly expanding, minimally invasive option for the treatment of AVVR in adults that avoids the morbidity and mortality associated with open heart surgery. However, application of TEER in in CHD and in children is quite novel. We describe the development of a peri-procedural protocol including image-derived pre-intervention simulation, with successful application to four patients. To describe the initial experience using the MitraClip system for TEER of dysfunctional systemic atrioventricular valves in patients with congential heart disease within a pediatric hospital. A standardized screening and planning process was developed using cardiac magnetic resonance imaging, three dimensional echocardiography and both virtual and physical simulation. Procedures were performed using the MitraClip G4 system and patients were clinically followed post-intervention. A series of fourCHD patients with at least severe AVVR were screened for suitability for TEER with the MitraClip system: three patients had single ventricle physiology and Fontan palliation, and one had repair of a common atrioventricular canal defect. Each patient had at least severe systemic AVVR and was considered at prohibitively high risk for surgical repair. Each patient underwent a standardized preprocedural screening protocol and image-derived modeling followed by the TEER procedure with successful clip placement at the intended location in all cases. The early results of our protocolized efforts to introduce TEER repair of severe AV valve regurgitation with MitraClip into the CHD population within our institution are encouraging. Further investigations of the use of TEER in this challenging population are warranted.

Hybrid stage 1 palliation with simultaneous off-pump ventricular assist device placement in neonates with high-risk single ventricle anatomy: Initial experience

BackgroundInfants with single ventricle heart disease and severe atrioventricular valve regurgitation have poor outcomes following conventional staged palliation. As such, ventricular assist device (VAD) placement along with hybrid stage 1 palliation has been proposed as a bridge to heart transplant. We present a novel surgical technique for VAD implantation concurrent with hybrid stage 1 that avoids cardiopulmonary bypass. MethodsWe performed a retrospective review of our institutional experience with this novel surgical technique. ResultsThree patients (weight, 2.7-3.5 kg; age, 3 to 5 days) underwent hybrid stage 1 with VAD placement, consisting of bilateral 3.5-mm expandable polytetrafluoroethylene (PTFE) pulmonary artery bands, a ductal stent, a 6-mm Berlin Heart outflow cannula onto the main pulmonary trunk with a 10-mm graft, a 6-mm Berlin Heart outflow cannula onto the right atrium, and a 10-mL Berlin Heart pump. In patients with severe aortic arch hypoplasia or coarctation, a 4-mm PTFE graft was sewn from the VAD outflow graft to the innominate artery to protect coronary and cerebral perfusion. Procedures were performed off bypass with minimal blood product use. Patients were extubated on postoperative days 2, 2, and 5. There were no procedural complications. All patients were transferred out of the intensive care unit and demonstrated appropriate weight gain. Anticoagulation strategy was bivalirudin and antiplatelet therapy. The patients underwent transplantation after 149 days, 157 days, and 288 days of support. ConclusionsOff-pump single ventricle VAD placement is technically feasible and can be done at the time of hybrid stage 1 palliation with minimal operative morbidity as a bridge to transplant.

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.

Indocyanine green clearance predicts outcome in patients undergoing transcatheter valve intervention for severe atrio-ventricular valve regurgitation.

Severe mitral regurgitation (MR) and tricuspid regurgitation (TR) aggravate haemodynamic stress leading to congestive heart failure with impaired hepatic function, also known as cardiohepatic syndrome (CHS). Current perioperative risk calculators do not sufficiently consider CHS and serum liver function parameters lack sensitivity to diagnose CHS. Indocyanine green and its elimination (measured by the LIMON® test) represent a dynamic and non-invasive test which correlates with the hepatic function. Nevertheless, its utility in the setting of transcatheter valve repair/replacement (TVR) to predict CHS and outcome remains unknown. We analysed liver function and outcomes of patients undergoing TVR for MR or TR between August 2020 and May 2021 at the Munich University Hospital. Out of a total of 44 patients treated at the University Hospital of Munich, 21 (48%) were treated for severe MR, 20 (46%) for severe TR and 3 (7%) for both diseases. Procedural success defined as MR/TR ≤2+ was 94% among MR patients and 92% among TR patients. While classical serum liver function parameters did not change after TVR, there was a significant improvement in liver function as assessed by the LIMON® test (P ≤ 0.001). Patients with baseline indocyanine green plasma disappearance rate <12.95%/min showed significantly increased 1-year mortality (hazard ratio: 1.54, 95% confidence interval: 1.05-2.25, P = 0.027) and lower New York Hear Association class improvement (P = 0.05). Especially in the context of the recently stressed importance of a careful patient selection prior to the interdisciplinary treatment of valvular heart disease, the LIMON® test may provide further real-time information on the patients' cardiohepatic injury and prognosis.

Early prediction of failure to progress in single ventricle palliation: A step toward personalizing care for severe congenital heart disease.

Advances in surgical technique and medical surveillance have improved outcomes of single ventricle (SV) palliation, particularly during the first interstage period. However, there remains a considerable mortality risk beyond this period. Patients born between January 2004 and December 2011 who required SV palliation were retrospectively identified. Patients who survived stage 1 palliation, were discharged home, and then were evaluated for Glenn candidacy, and continued care at our institution were included. Perioperative echocardiographic, hemodynamic, and operative data were analyzed at each surgical stage. The primary outcome was death or need for transplant. Univariate and multivariate analysis was completed using Cox proportional-hazards modeling. A total of 175 patients were included. Three patients died after pre-operative evaluation before Glenn. Glenn was completed in 168 patients, 16 died before Fontan. Fontan was completed in 149 patients; 117 were alive without need for transplant, 17 died post-Fontan, and 1 required transplantation. Twenty-one patients were lost to follow-up throughout the study period and were censored at time of last follow-up. Pre-Glenn moderate or severe atrioventricular valve regurgitation (AVVR) was an independent risk factor for death/transplant (HR 2.41; p-value .026). Pre-Glenn moderate ventricular dysfunction was also an independent risk factor (HR 5.29; p-value .012). Other risk factors included right ventricular (RV) dominant morphology and perinatal acidosis. Despite advances in SV palliation, a subset of these children remains at increased risk for poor outcomes. Early risk factors include RV dominant morphology and perinatal acidosis. Patients with substantial AVVR or ventricular dysfunction before Glenn palliation are also at significantly higher risk for death or requirement of transplantation later in childhood.

Predictors of liver cirrhosis and hepatocellular carcinoma among perioperative survivors of the Fontan operation

ObjectiveFontan-associated liver disease (FALD) is widely recognised as a common complication in patients long after the Fontan operation. However, data on the predictors of FALD that can guide its screening...

Neonatal ventricular assist device implantation for high-risk hypoplastic left heart syndrome: How we do it.

Neonatal ventricular assist device implantation for high-risk hypoplastic left heart syndrome: How we do it.

Pre-Operative Planning to Mitigate Risk in Complex Single Ventricular Assist Devices

<h3>Introduction</h3> Infants with single ventricle (SV) anatomy are a high-risk group for ventricular assist device (VAD) support. Hybrid stage 1 palliation can be utilized to successfully bridge SV patients to transplantation, such as in the setting of atrioventricular valve regurgitation (AVVR) which precludes long-term SV palliation. Our case highlights how comprehensive pre-procedural planning can mitigate future risk of VAD implantation. <h3>Case Report</h3> A neonate with unbalanced right-dominant atrioventricular canal presented with severe AVVR and acute kidney injury (AKI) precluding traditional SV palliation. She underwent hybrid palliation as bridge to transplant. A 6 mm expandable PTFE graft was anastomosed to the pulmonary artery (PA); ductal stenting was performed through the graft which was then left <i>in situ</i>. She stabilized following initial palliation, but after 10 weeks developed progressive ventricular dysfunction and AKI (Figure 1). A PediMag continuous flow VAD was inserted off bypass using the previous graft for arterial outflow and a right atrial inflow cannula (Figure 2). Renal function recovered and the patient is awaiting transplant with stable hemodynamics. <h3>Summary</h3> Anastomosing a graft to the PA during hybrid palliation enabled placement of VAD off bypass in an infant with SV anatomy and AKI. Detailed pre-operative planning at the time of stage 1 palliation in high-risk infants with potential future VAD support needs may be beneficial as part of a strategy to avoid an early VAD when systemic output is adequate while decreasing subsequent operative risk at time of VAD insertion.

Abdominal CT and MRI Findings of Portal Hypertension in Children and Adults with Fontan Circulation.

Background Portal hypertension in the Fontan circulation is a function of elevated systemic venous pressure and liver fibrosis. Purpose To quantify the prevalence of radiologic evidence of portal hypertension and elevated VAST score (one point each for varices, ascites, splenomegaly, and thrombocytopenia) of 2 or greater in children and adults with Fontan circulation and to determine the association with hemodynamics and adverse outcomes. Materials and Methods This was a retrospective study of individuals with Fontan circulation who underwent abdominal MRI or CT for focal liver lesion surveillance between January 2012 and December 2019. Portal hypertension was defined as the presence of at least two of the following: varices, ascites, or splenomegaly. Fontan deterioration was defined as a composite of heart failure signs or symptoms requiring diuretic escalation, placement of a ventricular assist device, heart transplant, or death. Relationships between variables and the composite end point were assessed using univariable and multivariable logistic regression. Results A total of 123 patients (age range, 9-55 years; 32 children) were evaluated (median age, 23 years; IQR, 17-30 years; 63 male patients). Median time since diagnosis of Fontan circulation was 16 years (IQR, 12-23 years). Twenty-five of the 123 patients (20%) had radiologic evidence of portal hypertension, and 34 (28%) had a VAST score of 2 or greater. Fontan deterioration occurred in 25 of the 123 patients (20%); median follow-up duration was 0.4 year (IQR, 0.1-3.1 years). Compared with patients who had Fontan circulation without deterioration, patients with Fontan deterioration were more likely to have moderate or severe ventricular systolic dysfunction (P < .01), moderate or severe atrioventricular valve regurgitation (P < .01), higher Fontan pressure (P = .01), radiologic evidence of portal hypertension (P < .01), and VAST score of 2 or greater (P < .01). Conclusion Radiologic evidence of portal hypertension at abdominal imaging in children and adults with Fontan circulation was associated with higher venous pressures and an increased risk for Fontan deterioration. These characteristics may be used to identify patients who warrant comprehensive hemodynamic evaluation. © RSNA, 2022.

Stepwise Treatment for Heterotaxy Syndrome and Functional Single Ventricle Complicated by Infra-Cardiac Total Anomalous Pulmonary Venous Connection with Ductus Venosus Stent Placement and Subsequent Occlusion.

Even today, when the surgical outcome of congenital heart disease in the neonatal period has improved, the prognosis for heterotaxy syndrome and functional single ventricle complicated with total anomalous pulmonary venous connection (TAPVC), especially the infra-cardiac type, is catastrophic. We describe a strategy that combines percutaneous ductus venosus (DV) stent placement and occlusion after TAPVC repair to ensure survival from initial surgery to bidirectional cavopulmonary shunt (BCPS) procedure and facilitate subsequent treatment. Three consecutive patients with heterotaxy syndrome and functional single ventricle complicated by infra-cardiac TAPVC treated with our own strategy were retrospectively studied. In two infants, DV stent placement was performed on the day of birth. In one case at 11days of age. The risk of pulmonary vein obstruction was reduced, and on-pump surgery, including TAPVC repair, was performed on a standby basis. Since the rapid increase in hepatic enzymes occurred on postoperative day 0 to 1 in all cases, percutaneous stent occlusion was performed until postoperative day 3. The procedure improved liver function. One patient died due to severe atrioventricular valve regurgitation, one case underwent BCPS, and one patient was waiting to undergo. DV stent placement can avoid TAPVC repair in the early neonatal period. After TAPVC repair, the portosystemic shunt remained, resulting in hepatic dysfunction, but this could be improved by stent and vertical vein occlusion. A series of stepwise treatments can be useful to help such critically ill infants survive the high-risk neonatal period and achieve good BCPS circulation.

Heart transplantation for patients with single ventricle physiology.

BackgroundThere is a growing population of palliated and unpalliated single ventricle physiology patients for whom heart transplantation is the only treatment option available. There is a paucity of reports of heart transplantation in this challenging and growing subset of patients from our part of the world. The purpose of the article is to briefly review our experience in the subgroup and compare it with the available literature.MethodsThis was a single-institution retrospective observational study of 16 patients with single ventricle physiology who were transplanted between 2016 and 2019 and their outcomes. The study groups were divided into those with ventricular dysfunction (group 1), who fare substantially better than those with normal ventricular function (group 2) whose short-term outcomes were poorer. Worsening cyanosis, poor candidature for completion Fontan procedure due to severe atrioventricular valve regurgitation or pulmonary artery anatomy, protein-losing enteropathy, plastic bronchitis, and worsening systemic venous congestion are indications in those with normal ventricular function.ResultsPatients with ventricular dysfunction as the main indication had excellent early survival with no early mortality compared to 40% mortality in patients with normal ventricular function. Patients who survived to leave the hospital had however similar long-term outcomes. Two patients with protein-losing enteropathy resolved completely by one month. Normal ventricular function, pulmonary artery stenting, early Fontan failure (6 months), ascites, and need for desensitization were risk factors for early mortality. After the early acute phase of increased risk, the mortality risk plateaued off.ConclusionTransplantation in patients with single ventricle and ventricular dysfunction can be offered with a good early and late outcome. There is a need to have multi-institutional and multi-disciplinary collaboration along with work in basic sciences to better understand the effects of failed Fontan physiology with normal ventricular function.

Outcomes of the Surgical Management of Atrial Isomerism and Functional Single Ventricle: A Single-Centered Cohort From China.

Objectives: The management of atrial isomerism with complex congenital heart disease remains challenging. Experience has been largely obtained in advanced countries. The clinical diversity is greater in China. We evaluated the early- and medium-term outcomes of surgical treatment of these patients.Methods: We reviewed 86 patients of atrial isomerism with complex congenital heart disease undergoing varied surgeries in our center in 2008–2020. Cox regression models were used to analyze the risk factors for mortality.Results: There were 75 cases of right and 11 of left atrial isomerism. Eighty-three (96.5%) patients underwent single-ventricle staged palliation approach, with 10 early and 7 late deaths. The overall 1-, 5-, and 10-year survival rates were 84.7, 79.3, and 79.3%, respectively. Thirty-six (43.4%) patients completed the Fontan procedure with median age of 48 months and freedom from death or Fontan failure at 1-, 5-, and 8-years were 94.4, 87.4, and 80.7%, respectively. Concomitant total anomalous pulmonary venous connection [hazard ratio (HR): 5.15 (1.95–12.94), p = 0.008], more than moderate atrioventricular valve regurgitation [HR: 4.82 (2.42–6.79), p = 0.003], and the need for first-stage palliative surgery [HR: 4.58 (1.64–10.76), p = 0.015] were independent risk factors for mortality.Conclusions: Despite even greater clinical diversity, the surgical outcomes of atrial isomerism with complex congenital heart disease are improving in China. The early and intermediate outcomes are comparable to many previous reports. Concomitant total anomalous pulmonary venous connection, moderate or severe atrioventricular valve regurgitation, and the need for a first-stage palliative surgery are still independent risk factors for mortality.

Relation of Magnetic Resonance Elastography to Fontan Circulatory Failure in a Cohort of Pediatric and Adult Patients.

Elevated magnetic resonance elastography (MRE)-derived liver stiffness may be associated with worse outcomes in people with Fontan circulation. We sought to evaluate the association between liver stiffness and Fontan failure or portal hypertension. Single center cross-sectional retrospective study of people with Fontan circulation who underwent MRE between 2011 and 2020. The cohort was divided into adult (age ≥ 21years) and pediatric (< 21years) groups. Fontan circulatory failure (FF) was defined as any of the following: death, transplantation, ventricular assist device, heart failure symptoms requiring escalation of diuretics. Radiologic portal hypertension was defined as the presence of one or more of the following: splenomegaly, ascites, or gastrointestinal varices. 128 patients were included (average age = 22.6 ± 8.7years) and 58 (45%) were children. Median liver stiffness was 4.3kPa (interquartile range (IQR) 3.8-5.8) for the entire cohort. Thirty patients (23%) developed FF (16 adults, 14 children). Liver stiffness was higher in adults with FF compared to those without FF (4.9 (IQR 4.0-6.0) vs. 4.2 (IQR 3.8-4.7) kPa, p = 0.04). There was no difference in liver stiffness between pediatric patients with and without FF (4.4 (IQR 4.1-5.4) vs. 4.4 (IQR 3.8-5.0), p = 0.5). Adults with radiologic portal hypertension and adults with moderate or severe atrioventricular valve regurgitation had higher liver stiffness than adults without. MRE-derived liver stiffness is associated with atrioventricular valve regurgitation, portal hypertension, and poor clinical outcomes in adults with Fontan circulation. There was no association between liver stiffness and FF in pediatric patients. This difference may be due to the progressive nature of Fontan-associated liver disease.

Long-term outcomes of atrioventricular septal defect and single ventricle: A multicenter study

ObjectiveThe study objective was to analyze survival and incidence of Fontan completion of patients with single-ventricle and concomitant unbalanced atrioventricular septal defect. MethodsData from 4 Dutch and 3 Belgian institutional databases were retrospectively collected. A total of 151 patients with single-ventricle atrioventricular septal defect were selected; 36 patients underwent an atrioventricular valve procedure (valve surgery group). End points were survival, incidence of Fontan completion, and freedom from atrioventricular valve reoperation. ResultsMedian follow-up was 13.4 years. Cumulative survival was 71.2%, 70%, and 68.5% at 10, 15, and 20 years, respectively. An atrioventricular valve procedure was not a risk factor for mortality. Patients with moderate-severe or severe atrioventricular valve regurgitation at echocardiographic follow-up had a significantly worse 15-year survival (58.3%) compared with patients with no or mild regurgitation (89.2%) and patients with moderate regurgitation (88.6%) (P = .033). Cumulative incidence of Fontan completion was 56.5%, 71%, and 77.6% at 5, 10, and 15 years, respectively. An atrioventricular valve procedure was not associated with the incidence of Fontan completion. In the valve surgery group, freedom from atrioventricular valve reoperation was 85.7% at 1 year and 52.6% at 5 years. ConclusionsThe long-term survival and incidence of Fontan completion in our study were better than previously described for patients with single-ventricle atrioventricular septal defect. A concomitant atrioventricular valve procedure did not increase the mortality rate or decrease the incidence of Fontan completion, whereas patients with moderate-severe or severe valve regurgitation at follow-up had a worse survival. Therefore, in patients with single-ventricle atrioventricular septal defect when atrioventricular valve regurgitation exceeds a moderate degree, the atrioventricular valve should be repaired.

Restriction of Atrial Septal Defect Leads to Growth of Hypoplastic Ventricle in Patients with Borderline Right or Left Heart

Patients with borderline hypoplastic right or left ventricle and VSD may be candidates for either single ventricle palliation or staged ventricular recruitment (SVR) followed by eventual biventricular conversion. Components of SVR include restriction of atrial septal defects (ASD) without ventricular septal defects (VSD) closure and addition of accessory pulmonary blood flow. This study evaluated the impact of ASD restriction on ventricular growth and function. We retrospectively reviewed patients with borderline ventricular hypoplasia and VSD who underwent a staged ventricular recruitment (SVR) procedure from 2012 to June 2019. Pre- and post-recruitment MRI and echocardiogram data were compared and analyzed. We excluded cases in which we intentionally restricted VSD with simultaneous ASD restriction. Forty-six patients (41 with right-dominant ventricle, 25 with risk factors for Fontan procedure) underwent SVR at a median age of 15.1 months' (interquartile range (IQR), 7.2-37.2 months'). The median indexed ventricular end-diastolic volume, end-systolic volume, and stroke volume according to cardiac MRI significantly increased at median 11.0 months' (IQR:7.8~14.1 months') after recruitment. Among them, except 2 operative mortalities after SVR, 26 patients underwent bi-ventricular repair (56.5% including one and a half ventricle repair) at a median of 8.0 months' (IQR: 6.2-12.2 months') after recruitment. Fifteen patients await biventricular completion, and 3 patients underwent single ventricle palliation. Pulmonary blood flow (Qp) tended to increase after recruitment regardless of type of pulmonary blood flow modification without statistical significance. Six patients died at a median duration of 6.5 months' (IQR: 2.9-11.7) after SVR; 3 patients died after biventricular completion, 2 after recruitment, and 1 after returning to single ventricle palliation. All of them were considered poor Fontan candidates due to severe atrioventricular valve regurgitation, pulmonary hypertension, pulmonary vein stenosis, or airway stenosis. Restriction of the atrial septum leads to the growth of hypoplastic ventricle in patients with ventricular septal defects who undergo SVR regardless of the preoperative characteristics, and eventual biventricular repair can be performed in a subgroup of these patients. Future work is necessary to optimize timing of SVR and method of accessory pulmonary blood flow.