Biventricular Circulation Research Articles

Transcatheter atrial septal defect closure late after completion of biventricular circulation in patients with pulmonary atresia intact ventricular septum or critical pulmonary stenosis.

This study aimed to explore anatomical and hemodynamic features of atrial septal defect, which was treated by transcatheter device closure late after completion of biventricular circulation in patients with pulmonary atresia and intact ventricular septum (PAIVS) or critical pulmonary stenosis (CPS). We studied echocardiographic and cardiac catheterization data, including defect size, retroaortic rim length, single or multiple defects, the presence of malalignment atrial septum, tricuspid and pulmonary valve diameters, and cardiac chamber sizes, in patients with PAIVS/CPS who underwent transcatheter closure of atrial septal defect (TCASD), and compared to control subjects. A total of 173 patients with atrial septal defect, including 8 patients with PAIVS/CPS, underwent TCASD. Age and weight at TCASD were 17.3 ± 18.3 years and 36.6 ± 13.9 kg, respectively. There was no significant difference in defect size (13.7 ± 4.0 vs. 15.6 ± 5.2 mm, p = 0.317) and the retro-aortic rim length (3.7 ± 4.3 vs. 3.6 ± 0.3.1 mm, p = 0.948) between the groups; however, multiple defects (50% vs. 5%, p < 0.001) and malalignment atrial septum (62% vs. 14%. p < 0.001) were significantly frequent in patients with PAIVS/CPS compared to control subjects. The ratio of pulmonary to systemic blood flow in patients with PAIVS/CPS was significantly lower than that in the control patients (1.2 ± 0.4 vs. 2.0 ± 0.7, p < 0.001); however, four out of eight patients with atrial septal defect associated with PAIVS/CPS had right-to-left shunt through a defect, who were evaluated by the balloon occlusion test before TCASD. The indexed right atrial and ventricular areas, the right ventricular systolic pressure, and mean pulmonary arterial pressure did not differ between the groups. After TCASD, the right ventricular end-diastolic area remained unchanged in patients with PAIVS/CPS, whereas it significantly decreased in control subjects. Atrial septal defect associated with PAIVS/CPS had more complex anatomy, which would be a risk for device closure. Hemodynamics should be individually evaluated to determine the indication for TCASD because PAIVS/CPS encompassed anatomical heterogeneity of the entire right heart.

A Single-Centre Retrospective Review ofModified Blalock-Taussig Shunts: A22-YearExperience.

This single-centre retrospective study explores demographics and outcomes of patients who underwent a modified Blalock-Taussig shunt (MBTS) over a 22-year period. The predominant surgical approach in this study is a lateral thoracotomy, in contrast to a midline sternotomy. Risks and outcomes of this approach are compared with national and international literature. Demographic, anatomical, clinical, surgical and outcome data of all patients who underwent a MBTS between 2000 and 2022 were collected and analysed, excluding Norwood procedures, which are not performed at this institution. Short- and long-term morbidity and mortality is described. Over the 22-year study period, 185 MBTS were performed in 162 patients, at a median age of 16 days (interquartile range [IQR] 5-59 days) and weight of 3.47 kg (IQR 3-4.25 kg, minimum weight 2 kg). Of these, 79% of patients had a biventricular circulation. Cardiac diagnoses included both univentricular and biventricular anatomy; tetralogy of Fallot (TOF) (36%), transposition of the great arteries/ventricular septal defect/pulmonary stenosis (TGA/VSD/PS) (11%), pulmonary atresia with intact ventricular septum (PA/IVS) (23%), pulmonary atresia with ventricular septal defect (PA/VSD) (14%), other (16%). The most common size of MBTS was 4 mm (71%); 93% were performed via a lateral thoracotomy. There were 47 cases of major operative morbidity, which did not differ significantly with cardiac diagnosis. Overall all-cause mortality was 13.5%. Early operative mortality was 4.3%. Mortality varied with cardiac diagnosis, 6% with TOF and 19% with PA/IVS. There was no era effect on mortality rates, however a lower frequency of major morbidity (23% vs 7%, p=0.03) was observed in the most recent third of the study period. Risk factors for shunt reintervention or mortality included weight <2.5 kg (HR=2.79 [1.37, 5.65], p=0.005), and pre- (HR=3.31 [1.86, 5.9], p<0.001) or postoperative lactic acidosis (HR=1.37 [1.25,1.5], p<0.001). These rates are comparable to those in the literature, with the predominant approach a midline sternotomy. Hobbes etal. from Royal Children's Hospital in Melbourne, Australia reported an overall and operative mortality rate of 12.8% and 5.2%, respectively, while Sasikumar etal. from The Hospital for Sick Children in Toronto, Canada reported an overall and operative mortality of 16% and 10%, respectively, for MBTS procedures over the same time period, however including smaller weight infants. Petrucci etal. described outcomes from the multi-institutional Society of Thoracic Surgeons Congenital Heart Surgery database reporting an overall mortality in MBTS of 7.2% in 1,273 patients with both single and double ventricle anatomy. Mortality rates and risk factors for adverse outcomes are comparable to those previously reported for both univentricular and biventricular groups. These results highlight that outcomes of MBTS performed via lateral thoracotomy are comparable to those by midline sternotomy as reported in the literature. Operating via the lateral approach may be advantageous as it avoids the complications of a midline sternotomy.

Long-term outcomes of pacemaker implantation in children with univentricular versus complex biventricular surgical repair.

Pacing in a univentricular circulation has been associated with worsened outcomes. We investigated the long-term outcomes of pacing in children with a univentricular circulation compared to a complex biventricular circulation. We also identified predictors of adverse outcomes. A retrospective study of all children with major congenital heart disease who underwent pacemaker implantation under the age of 18 years between November 1994 and October 2017. Eighty-nine patients were included; 19 with a univentricular and 70 with a complex biventricular circulation. A total of 96% of pacemaker systems were epicardial. Median follow up was 8.3 years. The incidence of adverse outcome was similar between the two groups. Five (5.6%) patients died and two (2.2%) underwent heart transplantation. Most adverse events occurred within the first 8 years after pacemaker implantation. Univariate analysis identified five predictors of adverse outcomes in the patients in the biventricular but none in the univentricular group. The predictors of adverse outcome in the biventricular circulation were a right morphologic ventricle as the systemic ventricle, age at first congenital heart disease (CHD) operation, number of CHD operations, and female gender. The nonapical lead position was associated with a much higher risk of an adverse outcome. Children with a pacemaker and a complex biventricular circulation have similar survival to the ones with a pacemaker and a univentricular circulation. The only modifiable predictor was the epicardial lead position on the paced ventricle, emphasizing the importance of apical placement of the ventricular lead.

Successful Pregnancy in A Patient with Fontan Surgery: A Case Report

The Fontan operation is a palliative procedure for patients with several forms of congenital heart disease unsuitable for biventricular circulation, especially tricuspid valve atresia. In this procedure, the vena cava flow is directed to the pulmonary arteries bypassing the ventricle. Pregnant patients with Fontan surgery in their post-surgical history have more maternal risks. We should counsel patients with saturations &lt;85%, depressed ventricular function, refractory arrhythmia, or protein-losing enteropathy against pregnancy (mWHO; IV). In this study, we review a pregnant case that has successful pregnancy and Fontan palliation and lv systolic heart failure (LVEF: 35%) in her past medical history.

Outcomes of different types of pulmonary atresia in neonates treated by ductus arteriosus stenting

Objective: To summarize the outcomes of different types of pulmonary atresia in neonates treated by ductus arteriosus stenting. Methods: This study was a retrospective cohort study. A total of 19 neonates who had pulmonary atresia treated by ductus arteriosus stenting in Xinhua Hospital, Shanghai Jiao Tong University School of Medicine from April 2014 to June 2021 were included. They were divided into the intact ventricular septum (PA-IVS) group and the ventricular septal defect (PA-VSD) group. Ductus arteriosus stents were implanted by different approaches. These children were followed up regularly at the 1, 3, 6, and 12 months after the surgery and annually since then to evaluate the outcome. Independent sample t-test was used for the statistical analysis. Results: There were 12 children in PA-IVS group and 7 in PA-VSD group. All of them were full term in fants. The gestational age of the PA-IVS group and the PA-VSD group was (38.8±1.1) and (37.7±1.8) weeks, the birth weights were (3.2±0.4) and (3.4±1.1) kg, and the age at operation was (10±9) and (12±7) days, respectively, without significant difference (all P>0.05). Among the 12 children with PA-IVS, 9 had stents successfully implanted through the femoral artery and 3 through the femoral vein. Of the 7 children with PA-VSD, 2 had the stents successfully implanted via the femoral artery and 2 failed, and the remaining 3 had stents successfully implanted via the left carotid artery. There was no postoperative thromboembolism, arteriovenous fistula, pseudoaneurysm or other vascular complications. Five children with PA-VSD who had successful operations were followed up at 6 months of age. They all had the operation for pulmonary atresia, repair of the ventricular septal defect, removal of arterial duct stents, and ligation of the arterial duct. All children survived without any stent displacement or stenosis and biventricular circulation was achieved during the follow-up. Conclusions: Ductus arteriosous stenting can be the first-stage treatment for children with PA-IVS and PA-VSD. In addition to the traditional femoral vein and femoral artery approach, the carotid artery can be used as a route for stent placement.

Efficacy of transcatheter pulmonary valve perforation in neonates with pulmonary atresia with intact ventricular septum

Objective: To explore the efficacy and safety of transcatheter pulmonary valve perforation in the treatment of neonatal pulmonary atresia with intact ventricular septum (PA-IVS). Methods: The clinical data on surgical treatment and follow-up in 16 patients with PA-IVS who underwent transcatheter pulmonary valve perforation in Women and Children's Hospital, Qingdao University from October 2018 to October 2021 were analyzed retrospectively. The right ventricular systolic pressure and percutaneous oxygen saturation (SpO2) were compared before and after operation. In addition, the SpO2 and echocardiographic data at preoperative and the last follow-up were compared. Comparisons between groups were performed using paired-samples t test. Results: Among the 16 patients (10 males and 6 females) with the age at operation of 19 (14, 26) days, 12 cases underwent transcatheter pulmonary valve perforation successfully, 2 cases were transferred to surgery department for open-heart pulmonary valvulotomy, and the remaining 2 cases were transmitted to surgery department for transthoracic pulmonary valve perforation. The age at operation of the 12 patients who underwent transcatheter pulmonary valve perforation was 18 (14, 27) days, and the weight was (3.6±0.4) kg. The immediate postoperative right ventricular systolic pressure decreased significantly ((57±16) vs. (95±19) mmHg (1 mmHg=0.133 kPa), t=7.49, P<0.001), and the postoperative SpO2 was improved effectively (0.90±0.48 vs.0.75±0.09, t=-5.61, P<0.001). The follow-up time was 22 (7, 33) months for 12 patients who underwent transcatheter pulmonary valve perforation successfully. At the last follow-up, the ratio of right to left ventricular transverse diameter was significantly higher than that before operative (0.55±0.05 vs. 0.45±0.05, t=-3.27,P=0.007). Furthermore, the Z-scores of pulmonary valvular diameter (-0.78±0.23 vs. -1.73±0.56, t=-8.52, P<0.001) and the tricuspid valvular diameter (-0.52±0.12 vs. -1.46±0.38, t=-10.40, P<0.001) were all significantly higher than preoperative data. At last, all the patients achieved biventricular circulation without death or major complications. Conclusion: Transcatheter pulmonary valve perforation is a safe and effective therapy for neonatal PA-IVS, and its curative effect has been confirmed by the medium follow-up data.

Cardiac magnetic resonance parameters associated with successful conversion from a single ventricular to a one-and-a-half or biventricular circulation in patients with a hypoplastic right ventricle

BackgroundSome patients with pulmonary atresia with an intact ventricular septum (PA/IVS) or a left ventricle dominant atrioventricular canal defect (LDAVC) with a hypoplastic right ventricle (RV) and univentricular (1 V) circulation may be candidates for conversion to either a complete biventricular (2 V) repair or a one-and-a-half ventricle repair (1.5 V). We sought to identify pre-operative cardiovascular magnetic resonance (CMR) findings associated with successful conversion from 1V to 1.5V or 2V circulation. MethodsIn this single center retrospective study, subjects with PA/IVS or LDAVC and no conotruncal abnormalities were included if they had a 1 V circulation at the time of CMR followed by a surgical intervention intended to convert them to a 1.5 V or 2 V circulation. Conversion failure was defined as any of the following: (1) oxygen saturation < 90% at the most recent follow-up, (2) conversion back to a 1.5 V or 1 V circulation, or (3) death. ResultsIn the PA/IVS cohort (n = 15, median age 1.32 years), 10 patients underwent surgical conversion to a 1.5 V circulation and 5 to a 2 V circulation. In the attempted 1.5 V group, there were 2 failures, and these cases had a lower RV mass (p = 0.04). In the attempted 2 V group, there was 1 failure, and no CMR parameters were significantly different compared to the successes. Among the successful 2 V group patients, the minimum RV end-diastolic volume (EDV) was 27 ml/m2. In the LDAVC cohort (n = 15, median age 1.0 years), 1 patient underwent surgical conversion to a 1.5 V circulation and 14 patients to a 2 V circulation. In the attempted 1.5 V group, the 1 conversion was a failure and had an RV EDV of 15 ml/m2. In the attempted 2 V group, there were 2 failures, and these cases had a smaller RV:LV stroke volume ratio (p = 0.05) and a lower RV ejection fraction (p = 0.05) compared to the successes. Among the successful 2 V group patients, the minimum RV EDV was 22 ml/m2. ConclusionsWe identified multiple CMR parameters associated with successful conversion from 1 V circulation to 1.5 V or 2 V circulation in patients with PA/IVS and LDAVC. This information may improve patient selection for conversion procedures and encourage larger studies to better define the role of CMR.

Heart failure in systemic right ventricle: Mechanisms and therapeutic options.

d-loop transposition of the great arteries (d-TGA) and congenitally corrected transposition of the great arteries (cc-TGA) feature a right ventricle attempting to sustain the systemic circulation. A systemic right ventricle (sRV) cannot support cardiac output in the long run, eventually decompensating and causing heart failure. The burden of d-TGA patients with previous atrial switch repair and cc-TGA patients with heart failure will only increase in the coming years due to the aging adult congenital heart disease population and improvements in the management of advanced heart failure. Clinical data still lags behind in developing evidence-based guidelines for risk stratification and management of sRV patients, and clinical trials for heart failure in these patients are underrepresented. Recent studies have provided foundational data for the commencement of robust clinical trials in d-TGA and cc-TGA patients. Further insights into the multifactorial nature of sRV failure can only be provided by the results of such studies. This review discusses the mechanisms of heart failure in sRV patients with biventricular circulation and how these mediators may be targeted clinically to alleviate sRV failure.

Support with Single Ventricle-Ventricular Assist Device (sVAD) in Patients with Functionally Univentricular Circulation Prior to Fontan Operation.

Some patients with functionally univentricular circulation develop cardiac failure refractory to maximal management and are supported with a ventricular assist device (VAD). The purpose of this manuscript is to summarize our previous publications related to single ventricle-ventricular assist device (sVAD) support in patients with functionally univentricular circulation and to describe our current institutional approach at University of Florida to sVAD support in neonates, infants, and children prior to Fontan. Our programmatic philosophy at University of Florida is to strive to identify the minority of neonates with functionally univentricular circulation who are extremely high-risk prior to initiating staged palliation and to stabilize these neonates with primary preemptive sVAD in preparation for cardiac transplantation; our rationale for this approach is related to the challenges associated with failed staged palliation and subsequent bail-out sVAD support and transplantation. A subset of extremely high-risk neonates and infants with functionally univentricular ductal-dependent circulation undergo primary preemptive sVAD insertion and subsequent cardiac transplantation. Support with VAD clearly facilitates survival on the waiting list during prolonged wait times and optimizes outcomes after Norwood (Stage 1) by providing an alternative pathway for extremely high-risk patients. Therefore, the selective utilization of sVAD in extremely high-risk neonates facilitates improved outcomes for all patients with functionally univentricular ductal-dependent circulation. At University of Florida, our programmatic approach to utilizing sVAD support as a bridge to transplantation in the minority of neonates with functionally univentricular circulation who are extremely high-risk for staged palliation is associated with Operative Mortality after Norwood (Stage 1) Operation of 2.9% (2/68) and a one-year survival of 91.1% (82/90) for all neonates presenting with hypoplastic left heart syndrome (HLHS) or HLHS-related malformation with functionally univentricular ductal-dependent systemic circulation. Meanwhile, at University of Florida, for all 82 consecutive neonates, infants, and children supported with pulsatile paracorporeal VAD: Kaplan-Meier survival estimated one year after VAD insertion = 73.3% (95% confidence interval [CI] = 64.1-83.8%), and Kaplan-Meier survival estimated five years after VAD insertion = 68.3% (95% CI = 58.4-79.8%). For all 48 consecutive neonates, infants, and children at University of Florida with biventricular circulation supported with pulsatile paracorporeal VAD: Kaplan-Meier survival estimated one year after VAD insertion = 82.7% (95% CI = 72.4-94.4%), and Kaplan-Meier survival estimated five years after VAD insertion = 79.7% (95% CI = 68.6-92.6%). For all 34 consecutive neonates, infants, and children at University of Florida with functionally univentricular circulation supported with pulsatile paracorporeal sVAD: Kaplan-Meier survival estimated one year after VAD insertion = 59.7% (95% CI = 44.9-79.5%), and Kaplan-Meier survival estimated five years after VAD insertion = 50.5% (95% CI = 35.0-73.0%). These Kaplan-Meier survival estimates for patients supported with pulsatile paracorporeal VAD are better in patients with biventricular circulation in comparison to patients with functionally univentricular circulation both one year after VAD insertion (P=0.026) and five years after VAD insertion (P=0.010). Although outcomes after VAD support in functionally univentricular patients are worse than in patients with biventricular circulation, sVAD provides a reasonable chance for survival. Ongoing research is necessary to improve the outcomes of these challenging patients, with the goal of developing strategies where outcomes after sVAD support in functionally univentricular patients are equivalent to the outcomes achieved after VAD support in patients with biventricular circulation.

Staged ventricular recruitment and biventricular conversion following single-ventricle palliation in unbalanced atrioventricular canal defects

ObjectiveRestoration of biventricular circulation is an alternative management strategy in unbalanced atrioventricular canal defects (uAVCDs), especially in patients with risk factors for single-ventricle palliation (SVP) failure. When ventricular volume is inadequate for biventricular circulation, recruitment procedures may accommodate its growth. In this study, we review our uAVCD experience with biventricular conversion (BIVC) after prior SVP. MethodsThis is a single-institution, retrospective cohort study of uAVCD patients who underwent BIVC after SVP, with staged recruitment (staged) or primary BIVC (direct) between 2003 to 2018. Mortality, unplanned reinterventions, imaging, and catheterization data were analyzed. ResultsSixty-five patients underwent BIVC from SVP (17 stage 1, 42 bidirectional Glenn, and 6 Fontan). Decision for conversion was based on poor SVP candidacy (n = 43) or 2 adequately sized ventricles (n = 22). Of the 65 patients, 20 patients underwent recruitment before conversion. The staged group had more severe ventricular hypoplasia than the direct group, reflected in prestaging end-diastolic volume z scores (–4.0 vs –2.6; P < .01), which significantly improved after recruitment (–4.0 to –1.8; P < .01). Median follow-up time was 1.0 years. Survival and recatheterizations were similar between both groups (hazard ratio, 0.9; 95% CI, 0.2-3.7; P = .95 and hazard ratio, 1.9; 95% CI, 0.9-4.1; P = .09), but more reoperations occurred with staged approach (hazard ratio, 3.1; 95% CI, 1.3-7.1; P = .01). ConclusionsBiventricular conversion from SVP is an alternative strategy to manage uAVCD, particularly when risk factors for SVP failure are present. Severe forms of uAVCDs can be converted with staged BIVC with acceptable mortality, albeit increased reinterventions, when primary BIVC is not possible.

Abstract 11015: Pacemaker Lead Insertion Sites Contribute to Regional and Global Ventricular Dysfunction

Introduction: Ventricular pacing can cause dysfunction but how the lead is anchored to the myocardium has not been studied. We evaluated patterns of regional and global ventricular function in patients with a ventricular pacemaker or defibrillator using cine cardiac CT (CCT). Methods: This was a single center retrospective study of patients with a ventricular pacemaker or defibrillator who underwent a cine CCT from September 2020 to June 2021. Regional wall motion abnormalities (RWMAs) were assessed in relation to lead insertion site (sew-on vs screw-in) and pacing activity (active vs. inactive). Results: A total of 122 ventricular lead insertion sites were analyzed in 43 patients (53% male, median age 20 years, range 3-57 years). Thirteen patients (30%) had palliated single ventricle physiology, twenty-nine patients had congenital heart disease with biventricular circulation (67%), and one patient had a structurally normal heart (2%). RWMAs were present in 51/122 (42%) lead insertion sites among 23/43 (53%) patients. The prevalence of a lead insertion site associated RWMA was higher with active pacing (54% vs 19%; p &lt; 0.001) and highest for active epicardial sew-on sites ( Figure 1 ). RWMAs were not associated with position of the insertion site relative to the chest wall (p = 0.85) or the distance between the two heads of a bipolar lead (p = 0.23). Patients with lead insertion site associated RWMAs had a lower systemic ventricular ejection fraction (mean of 37.5% vs 52.5%, p &lt; 0.001) compared to those without a RWMA. Conclusions: Pacemaker lead insertion site RWMAs are common and associated with systemic ventricular dysfunction. The mechanism and clinical significance of this regional and global dysfunction warrant further study.

Abstract 14737: Right Heart Remodeling After Pulmonary Valve Replacement in Patients With Pulmonary Atresia With Intact Ventricular Septum and Critical Pulmonary Stenosis

Background: Patients with pulmonary atresia with intact ventricular septum (PA/IVS) and critical pulmonary stenosis (CPS) managed to a biventricular circulation may eventually require pulmonary valve replacement (PVR) for pulmonary regurgitation (PR). Right ventricular (RV) remodeling after PVR has been well described in tetralogy of Fallot (TOF); we sought to investigate RV volumetric and functional changes in PA/IVS using CMR. Methods: A retrospective cohort of PA/IVS and CPS patients who underwent PVR at Boston Children’s Hospital from 1995-2021 with CMR before and after PVR was matched 1:3 with TOF patients by age at PVR. Patients with initial intervention &gt;2 weeks of age or 1.5 ventricle circulation were excluded. Median regression modeling accounting for matching was performed with reduction in indexed right ventricular end diastolic volume (RVEDVi) post PVR as the primary outcome, adjusting for covariates including tricuspid regurgitation (TR) and PVR valve size. Results: Twenty PA/IVS or CPS patients (cases) were matched with 60 TOF (controls), with median age at PVR 14 years; at median follow-up 8 years post PVR, 95% were NYHA Class I. Pre-PVR RVEDVi was similar between groups (165 vs 167 ml/m 2 , p=0.7), although cases had higher RV EF (51.4 vs 48.6%, p=0.03), TR fraction (23 vs 9%, p=0.008), and a trend toward lower RV mass; PR fraction, LV volumes and EF were similar. Pre-PVR RV free wall and LV longitudinal strain were similar, although LV circumferential strain was worse in cases (-15.6 vs -17.1, p=0.01). At median 2 years after PVR, RVEDVi was similarly reduced from 166 to 118 ml/m 2 (p=0.73), although cases had higher RV EF (52.3% vs 46.9%, p=0.007) with less reduction in indexed RV mass (Δ4.5 vs 9.6 g/m 2 , p=0.02); LV volumes and EF were similar. Post PVR, RV and LV longitudinal strain remained unchanged both within and between groups; LV circumferential strain was similar post PVR and remained lower in cases vs controls. Conclusion: Patients with PA/IVS after PVR demonstrate similar RV remodeling to TOF patients, with lesser reduction in RV mass and comparatively better post RV EF. CMR strain imaging found no significant pre-post differences in RV or LV systolic parameters. Further investigation is needed to evaluate for changes in diastolic parameters.

Ventricular-arterial coupling predicts outcomes in adults with a systemic right ventricle

Abstract Background Patients with a systemic right ventricle (SRV) and biventricular circulation experience high incidence of cardiovascular morbidities and decreased survival [1]. Non-invasive measures of subclinical ventricular dysfunction are needed to appropriately identify patients at increased risk for adverse outcomes. Ventricular-arterial coupling (VAC), the ratio between the effective arterial elastance (Ea) and ventricular end-systolic elastance (Ees), may predict clinical outcomes in patients with SRV [2]. Objectives To assess VAC in adults with SRV and evaluate its correlation with clinical outcomes. Methods Consecutive cardiovascular magnetic resonance (CMR) examinations of adults with D-loop transposition of great arteries (TGA) after atrial switch operation and L-loop TGA performed at Boston Children's Hospital between 2005 and 2019 were analyzed. VAC was calculated as Ea/Ees (Ea = mean arterial blood pressure (MBP)/ventricular stroke volume; Ees = MBP/end-systolic volume). Global myocardial strain was measured by feature tracking analysis on cine steady-state free precession sequences. Cox proportional hazards regression analysis was performed to assess the association of SRV functional parameters with clinical outcomes. The analysis was adjusted for age, sex, and body mass index. The primary outcome was defined as a composite of death, cardiovascular arrest, hospitalizations for heart failure (HF); the secondary outcome as atrial arrhythmias; the tertiary outcome included other causes of cardiovascular hospitalizations (percutaneous or surgical interventions, device implantation, other cardiovascular disease). Cumulative incidence of the study outcomes was estimated using Kaplan-Meier method. Results One hundred sixty-seven adults (mean age 32±10 years, 59% men) with SRV were analyzed. Patients with HF (n=48, 29%) had higher VAC values as compared to those without HF (1.4±0.8 vs. 1.1±0.5, p=0.01). Over a mean follow-up of 6.5±4.2 years, 15 over 139 patients (11%) experienced the primary outcome with an incidence rate of 1.7 per 100 patient-years (95% confidence interval (CI), 1.04–2.85). Higher VAC values were significantly associated with an increased risk of the primary outcome (p for trend = 0.01, Figure 1). VAC was the only functional parameter associated with the primary outcome (hazard ratio (HR) 1.99, 95% CI: 1.06–3.73, p=0.031), secondary outcome (HR 2.33, 95% CI: 1.12–4.82, p=0.023) and tertiary outcome (HR 1.63, 95% CI: 1.09–2.44, p=0.018) in the adjusted analysis (Table 1). Ejection fraction (EF) was not associated with the study outcomes in the adjusted analysis (p&amp;gt;0.05, Table 1) whereas global circumferential and radial strain showed an association limited to the tertiary endpoint (p=0.004, Table 1). Conclusions CMR-derived VAC is associated with adverse outcomes in SRV patients and may improve risk stratification of this unique population. Funding Acknowledgement Type of funding sources: None.

First-in-man use of the EXCOR Venous Cannula for combined cavopulmonary and systemic ventricular support in Fontan circulation failure

The Berlin Heart EXCOR system has been developed for mechanical circulatory support (MCS) of pediatric patients with terminal heart failure. A recently introduced iteration of the system (EXCOR Venous Cannula, Berlin Heart GmbH, Berlin, Germany) is dedicated to support patients with univentricular physiologies by facilitating implantation of the EXCOR device into the Fontan pathway. We report the worldwide first successful implantation of the EXCOR Venous Cannula in a biventricular support concept for a 12-year-old boy (140 cm, 42.7 kg, body surface area 1.29 m2, Pedimacs Level 2) with severe systemic ventricle dysfunction and failing Fontan circulation. Surgery comprised of standard Berlin Heart EXCOR implantation to support the failing ventricle (12 mm apex / staged 12/9 mm arterial cannula / 50 ml ventricle). Cannulation for subpulmonary EXCOR support was achieved by performing a total cavopulmonary connection takedown with subsequent anastomosis of a staged 12/9 mm outflow cannula to the pulmonary artery and implantation of a 14/18 mm EXCOR Venous Cannula as subpulmonary inflow graft, which was connected to the superior vena cava and Fontan tunnel using GORE-TEX grafts. In the postoperative course, cardiac output and central venous pressures rapidly improved with hepatic and renal functions restoring to age- and condition-specific norm values. The Berlin Heart EXCOR Venous Cannula is the first system for standardized mechanical support of Fontan circulatory failure. In our patient, subpulmonary support restoring a biventricular circulation combined with systemic MCS normalized hemodynamics and reversed end-organ dysfunction.

Early Experience With Reverse Double Switch Operation for the Borderline Left Heart

This study reviews our early experience with the "reverse" double switch operation (R-DSO) for borderline left hearts. A retrospective review of children with borderline left hearts who underwent R-DSO between 2017 and 2021 was conducted. Patient characteristics and early hemodynamic and clinical outcomes were collected. R-DSO was performed in 8 patients with no operative or postoperative deaths; 5 underwent decompressing bidirectional Glenn. Left ventricular (LV) poor-compliance was the dominant pathophysiology. Four patients had undergone staged LV recruitment but were not candidates for anatomical biventricular circulation due to LV hypoplasia and/or diastolic dysfunction. 7/8 patients had risk factors for Fontan circulation including pulmonary vein stenosis, pulmonary hypertension, and pulmonary artery stenosis. Median age at R-DSO was 3.7 years (19 months-12 years). All patients were in sinus rhythm at discharge. At median follow-up of 15 months (57 days-4.1 years) no mortalities, reoperations or heart transplants had occurred. All patients had normal morphologic LV systolic function. In one patient, pre-existing pulmonary hypertension (HTN) resolved after R-DSO. Reinterventions included transcatheter mitral valve replacement for residual mitral stenosis and neo-pulmonary balloon valvuloplasty. In 4 patients follow-up catheterization done at a median of 519 days (320 days-4 years) demonstrated median cardiac index of 3.2 L/min/m2 (2.2-4); median sub-pulmonary left ventricular end diastolic pressure was 9 mm Hg (7-15); median inferior vena cava/baffle pressure was 8 mm Hg (7-13). R-DSO is an alternative to anatomical biventricular repair or single ventricle palliation in patients with borderline left hearts and can result in low inferior vena cava pressures and favorable early results. This approach can also relieve pulmonary HTN and allow future transplant candidacy.

Paediatrician's guide to post-operative care for biventricular CHD: a review.

Paediatricians play an integral role in the lifelong care of children with CHD, many of whom will undergo cardiac surgery. There is a paucity of literature for the paediatrician regarding the post-operative care of such patients. The aim of this manuscript is to summarise essential principles and pertinent lesion-specific context for the care of patients who have undergone surgery or intervention resulting in a biventricular circulation. Familiarity with common issues following cardiac surgery or intervention, as well as key details regarding specific lesions and surgeries, will aid the paediatrician in providing optimal care for these patients.

The Ventricular Switch: An Alternative Strategy Toward Biventricular Repair in Moderately Hypoplastic Left Ventricles or Complex Connections.

Hypoplastic left heart complex (HLHC) constitutes a spectrum of left ventricular hypoplasia and valvar disease and can be associated with increased morbidity and mortality. Traditionally, management of these lesions involves single ventricle palliation (SVP). However, the SVP may be associated with substantial long-term consequences related to its physiology. Alternative management strategies have been employed to achieve biventricular circulation. We have proposed a novel technique, termed the "ventricular switch" procedure, in which the hypoplastic or unroutable left ventricle can be harnessed to function as the subpulmonic ventricle, and the right ventricle functions as the systemic ventricle. Herein, we describe our rationale for considering biventricular circulation and the ventricular switch procedure in HLHC and in other complex lesions.

Hypoplastic Left Ventricle: Left Ventricular Recruitment With Hybrid Approach.

Decision-making for biventricular repair (BVR) or univentricular palliation is challenging in neonates with hypoplastic left heart complex (HLHC). Hybrid strategy can be used successfully to achieve BVR in some of these patients. Between June 1998 and January 2022, 342 patients with a diagnosis of HLHS/variants, ductal-dependent lower body circulation with two ventricles, or HLHC with borderline left ventricle (LV) underwent initial bilateral pulmonary artery banding and ductal stenting in our institution. Among these 342 patients, 224 patients were defined as HLHS/variants and underwent univentricular palliation. 118 patients were determined to have borderline LV and hypoplastic left ventricular structures (HLHC, n = 48) or ductal-dependent lower body circulation with two ventricles (n = 70) considered suitable for BVR. 48 patients had multilevel obstructions including both aortic and mitral valve hypoplasia, aortic arch hypoplasia, and borderline LV. These were considered to have HLHC. These 48 HLHC patients are the subject of this report. Neonatal hybrid palliation at a median age of seven days was performed in 48 patients and 46 patients underwent BVR at a median age of 156 days. In 46 patients who underwent BVR, hospital mortality was not observed. The median follow-up was 66 months, with no late mortality. Heart transplant-free survival at 5, 10, and 15 years was 95.7%. 12 patients (26%) required reoperation and 9 (19.5%) required catheter reinterventions. Hybrid palliation may increase the chances for biventricular circulation in patients with borderline LV and small left-sided structures. Giessen hybrid approach as a left ventricular recruitment strategy achieves excellent early and long-term results.

Hemodynamic and anatomic changes after fetal aortic valvuloplasty are associated with procedural success and postnatal biventricular circulation.

There are minimal data characterizing the trajectory of left heart growth and hemodynamics following fetal aortic valvuloplasty (FAV). This retrospective study included patients who underwent FAV between 2000 and 2019, with echocardiograms performed pre-FAV, immediately post-FAV, and in late gestation. Of 118 fetuses undergoing FAV, 106 (90%) underwent technically successful FAV, of which 55 (52%) had biventricular circulation. Technically successful FAV was associated with improved aortic valve growth (p<0.001), sustained antegrade aortic arch (AoA) flow (p=0.02), improved mitral valve (MV) inflow pattern (p=0.002), and favorable patent foramen ovale (PFO) flow pattern (p=0.004) from pre-FAV to late gestation. Compared to patients with univentricular outcome, patients with biventricular outcome had less decrement in size of the left ventricle (LV) (p<0.001) and aortic valve (p=0.005), as well as more physiologic PFO flow (p<0.001) and antegrade AoA flow (p<0.001) from pre-FAV to late gestation. In multivariable analysis, echocardiographic predictors of biventricular outcome were less decline in LV end diastolic dimension (p<0.001), improved PFO flow (p=0.004), and sustained antegrade AoA flow (p=0.002) from pre-FAV to late gestation. Stabilization of left heart growth and improved hemodynamics following successful FAV through late gestation are associated with postnatal biventricular circulation.

Status of adult outpatients with congenital heart disease in Japan: The Japanese Network of Cardiovascular Departments for Adult Congenital Heart Disease Registry

BackgroundThe Japanese Network of Cardiovascular Departments for Adult Congenital Heart Disease (JNCVD-ACHD) was founded in 2011 for the lifelong care of adult patients with congenital heart disease (ACHD patients). This network maintains the first Japanese ACHD registry. Methods and resultsFrom 2011 to 2019, the JNCVD-ACHD registered 54 institutions providing specialized care for ACHD patients in 32 of the 47 prefectures in Japan. The registry collected data on the disease profile for 24,048 patients from 50 institutions and the patient characteristics for 9743 patients from 24 institutions. The most common ACHDs were atrial septal defect (20.5 %), ventricular septal defect (20.5 %), tetralogy of Fallot (12.9 %), and univentricular heart (UVH)/single ventricle (SV; 6.6 %). ACHD patients without biventricular repair accounted for 37.0 % of the population. Also examined were the serious anatomical and/or pathophysiological disorders such as pulmonary arterial hypertension (3.0 %) including Eisenmenger syndrome (1.2 %), systemic right ventricle under biventricular circulation (sRV-2VC; 2.8 %), and Fontan physiology (6.0 %). The sRV-2VC cases comprised congenitally corrected transposition of the great arteries without anatomical repair (61.9 %) and transposition of the great arteries with atrial switching surgery (38.1 %). The primary etiology (86.4 %) for Fontan physiology was UVH/SV. In addition, developmental/chromosomal/genetic disorders were heterotaxy syndromes (asplenia, 0.9 %; polysplenia, 0.7 %), trisomy 21 (4.0 %), 22q11.2 deletion (0.9 %), Turner syndrome (0.2 %), and Marfan syndrome (1.1 %). ConclusionsAlthough the specific management of ACHD has systematically progressed in Japan, this approach is still evolving. For ideal ACHD care, the prospective goals for the JNCVD-ACHD are to create local networks and provide a resource for multicenter clinical trials to support evidence-based practice.