Systemic Ventricular Dysfunction Research Articles

Long-term Outcomes of Surgical Repair for Corrected Transposition of the Great Arteries

BackgroundThis study investigated the long-term outcomes of physiological and anatomical repair for corrected transposition of the great arteries and double-outlet right ventricle with discordant atrioventricular connection. MethodsThis single-center retrospective study included 146 patients who underwent biventricular repair of corrected transposition of the great arteries or double-outlet right ventricle with discordant atrioventricular connections from 1972 to 2023. Survival rate, freedom from reoperation, New York Heart Association (NYHA) Functional Classification, and incidence of systemic ventricular dysfunction in the long-term were compared between physiological repair (PR) and anatomical repair (AR) groups. ResultsThe PR group consisted of 55 patients, with median age at repair of 10.3 years. Thirty-one patients underwent conventional Rastelli procedure, and 24 patients underwent atrial and/or ventricular septal defect closure. The AR group consisted of 91 patients, with median age at repair of 5.8 years. Seventy-two patients underwent atrial switch plus Rastelli procedure and 19 patients underwent atrial plus arterial switch operation. The 30-year survival was 63.5% in the PR group and 72.3% in the AR group (P = .448). The 30-year freedom from reoperation was 71.9% in the PR group and 62.2% in the AR group (P = .220). There was a significant difference in the incidence of systemic ventricular dysfunction between the groups (87.5% in the PR group and 35.3% in the AR group, P < .001) and in the NYHA classification of long-term survivors (mean NYHA class of 1.9 in the PR group and 1.5 in the AR group, P = .009). ConclusionsThe systemic ventricular function and general status in the long-term were significantly better in AR patients, suggesting the potential advantage of AR.

Morphology of the native ascending aorta after the Norwood procedure for aortic atresia: impact on survival and right ventricular dysfunction.

To evaluate the impact of variable morphology of the native ascending aorta after the Norwood I procedure in patients with hypoplastic left heart syndrome/aortic atresia on long-term survival and systemic right ventricular dysfunction. Of 151 survivors of the Norwood procedure for hypoplastic left heart syndrome/aortic atresia at our institution between January 2001 and December 2020, we included patients with available and measurable aortography prior to stage II palliation. Diameter of the native ascending aorta, length of the native ascending aorta, and the angle between the the native ascending aorta and the proximal pulmonary artery were measured. We investigated the impact of these morphologic parameters on the mortality and the right ventricular dysfunction (defined as at least moderate). Angiography was available in 78 patients. Median diameter of native ascending aorta was 3.2 mm (2.6-3.7), median length of native ascending aorta was 15.4 mm (13.3-17.9), and median angle between the native ascending aorta and the proximal pulmonary artery was 44° (35° - 51°). During median follow-up of 6.5 years, eight (10%) patients died and systemic right ventricular dysfunction occurred in 19 patients (24%). No significant association between the aortic morphology and mortality could be detected. Right ventricular function was negatively affected by a larger angle between the native ascending aorta and the proximal pulmonary artery and (odds ratio 1.07 [1.01-1.14], P= 0.02). In survivors of the Norwood procedure for hypoplastic left heart syndrome/aortic atresia with available angiography, no significant association between the native aortic morphology and mortality could be demonstrated after stage II palliation, within the scope of this limited study. A larger anastomosis angle between the native ascending aorta and the proximal pulmonary artery emerged as a risk factor for right ventricular dysfunction.

Role and Applications of Experimental Animal Models of Fontan Circulation.

Over the last four decades, the Fontan operation has been the treatment of choice for children born with complex congenital heart diseases and a single-ventricle physiology. However, therapeutic options remain limited and despite ongoing improvements in initial surgical repair, patients still experience a multiplicity of cardiovascular complications. The causes for cardiovascular failure are multifactorial and include systemic ventricular dysfunction, pulmonary vascular resistance, atrioventricular valve regurgitation, arrhythmia, development of collaterals, protein-losing enteropathy, hepatic dysfunction, and plastic bronchitis, among others. The mechanisms leading to these late complications remain to be fully elucidated. Experimental animal models have been developed as preclinical steps that enable a better understanding of the underlying pathophysiology. They furthermore play a key role in the evaluation of the efficacy and safety of new medical devices prior to their use in human clinical studies. However, these experimental models have several limitations. In this review, we aim to provide an overview of the evolution and progress of the various types of experimental animal models used in the Fontan procedure published to date in the literature. A special focus is placed on experimental studies performed on animal models of the Fontan procedure with or without mechanical circulatory support as well as a description of their impact in the evolution of the Fontan design. We also highlight the contribution of animal models to our understanding of the pathophysiology and assess forthcoming developments that may improve the contribution of animal models for the testing of new therapeutic solutions.

Pregnancy-related cardiac outcomes among patients with congenital heart disease after formalization of a cardio-obstetrics program

The prevalence of pregnant patients with congenital heart disease (CHD) is increasing, and these patients are at high risk for cardiac morbidity. This study aimed to examine the pregnancy outcomes in patients with congenital heart disease before and after the establishment of formal cardio-obstetrics collaboration between adult congenital heart disease and maternal-fetal medicine programs. This was a retrospective cohort study of pregnant patients with congenital heart disease from 2002 to 2020 at a single urban academic institution in the United States. This study included patients with a singleton pregnancy who continued a pregnancy beyond 20 weeks of gestation. The primary outcome was a composite adverse maternal cardiac outcome, compared before (2002-2010) and after (2011-2020) the program. The secondary outcomes included gestational age at delivery, mode of delivery, rate of labor induction, use of diuresis after delivery, and a composite maternal morbidity outcome. The number of pregnant patients with congenital heart disease increased after formalization of the cardio-obstetrics program (200 [postprogram group] vs 84 [preprogram group]; 0.48% of all deliveries in the postprogram group vs 0.25% of all deliveries in the preprogram group; P<.001). The postprogram group was more likely to undergo labor induction than the preprogram group (126 [63%] vs 34 [41%], respectively; P<.001). There were fewer patients in the postprogram group than in the preprogram group who were New York Heart Association class II to IV (23 [12%] vs 17 [22%], respectively; P=.04) or with systemic ventricular dysfunction (8 [4%] vs 12 [16%], respectively; P=.001). There was no difference in the primary outcome (38 [19%] in the postprogram group vs 14 [17%] in the preprogram group; P=.64), even after adjusting for confounders, including New York Heart Association class >I and systemic ventricular dysfunction (adjusted odds ratio, 2.3; 95% confidence interval, 0.96-5.4). Patients in the postprogram group were more likely to receive diuresis after delivery than patients in the preprogram group, even in the absence of heart failure or pulmonary edema (9 [4.5%] vs 0 [0.0%], respectively; P=.04). In the period after the establishment of a formal cardio-obstetrics program between adult congenital heart disease and maternal-fetal medicine, the number of patients with congenital heart disease delivering at our institution increased significantly. Overall, fewer patients entered pregnancy with advanced-stage heart failure or systemic ventricular dysfunction, possibly suggesting improved prepregnancy cardiac care or improved preconception counseling. Composite maternal cardiac outcomes were similar, but the rates of postpartum diuresis increased significantly, suggesting increased attention to volume status in the postpartum period. Formalized collaboration between congenital heart disease and maternal-fetal medicine may help better optimize patients' care before conception, during pregnancy, and after delivery.

Low-grade systemic inflammation and left ventricular dysfunction in hypertensive compared to non-hypertensive hypertrophic cardiomyopathy

BackgroundArterial hypertension (HTN) is associated with excess mortality in hypertrophic cardiomyopathy (HCM), but underlying mechanisms are largely elusive. The objective of this study was to investigate the association between HTN and markers of left ventricular (LV) dysfunction and low-grade systemic inflammation in a HCM cohort. MethodsThis was a single-center cross-sectional case-control study comparing echocardiographic and plasma-derived indices of LV dysfunction and low-grade systemic inflammation between 30 adult patients with HCM and HTN (HTN+) and 30 sex- and age-matched HCM patients without HTN (HTN-). Echocardiographic measures were assessed using post-processing analyses by blinded investigators. ResultsMean age of the study population was 55.1 ± 10.4 years, 30% were women. Echocardiographic measures of systolic and diastolic dysfunction, including speckle-tracking derived parameters, did not differ between HTN+ and HTN-. Moreover, levels of N-terminal pro B-type natriuretic peptide were balanced between cases and controls. Compared with HTN-, HTN+ patients exhibited a higher white blood cell count [8.1 ± 1.8 109/l vs. 6.4 ± 1.6 109/l; p < 0.001] as well as higher plasma levels of interleukin-6 [2.8 pg/ml (2.0, 5.4) vs. 2.1 pg/ml (1.5, 3.4); p = 0.008] and high-sensitivity C-reactive protein [2.6 mg/l (1.4, 6.5) vs. 1.1 mg/l (0.9, 2.4); p = 0.004]. ConclusionThis study demonstrates that HTN is associated with indices of low-grade systemic inflammation among HCM patients. Moreover, this analysis indicates that the adverse impact of HTN in HCM patients is a consequence of systemic effects rather than alterations of cardiac function, as measures of LV systolic and diastolic dysfunction did not differ between HTN+ and HTN-.

Abstract 11829: Association of Adult Congenital Heart Disease and Serial Echocardiographic Parameters With Adverse Cardiac, Obstetric, and Neonatal Outcomes

Background: Risk factors for adverse cardiac, obstetric and neonatal outcomes in pregnant women with adult congenital heart disease (ACHD) are not well-defined. Objective: To assess the association of ACHD and serial echo parameters with adverse cardiac, obstetric and neonatal outcomes. Methods: ACHD patients with prenatal care and delivery at a tertiary care center from 2007-2022 were included. Serial echos during pregnancy and adverse cardiac outcomes (arrhythmia, heart failure, ICU admission) were reviewed. Adverse obstetric (pregnancy-induced hypertension, abruption, hemorrhage) and neonatal outcomes (small-for-gestational age, preterm delivery, NICU admission, fetal/neonatal demise) were compared between the ACHD cohort and an age- and gravidity-matched non-ACHD cohort. Logistic regression was used to calculate the odds ratio (OR) for adverse outcomes. Results: Of 188 ACHD patients (mean age 30.9 ± 5.9 years), 74.4% had moderate or greater anatomic complexity. 11.7% of ACHD patients had an adverse cardiac outcome. Adverse obstetric and neonatal outcomes were more common in ACHD patients (23.9% vs 10.4%, p &lt; 0.001; 35.3% vs 17.0%, p &lt; 0.001, respectively). Systemic ventricular dysfunction, systemic atrioventricular valve dysfunction, and decline in systemic ventricular function during pregnancy were associated with increased risk of adverse cardiac outcomes. ACHD was associated with increased risk of adverse obstetric and neonatal outcomes (OR 2.8, 95% Confidence Interval [CI] 1.7 - 4.8; OR 2.8, 95% CI 1.8 - 4.5, respectively). Echo predictors of adverse cardiac outcomes were not associated with increased risk of adverse obstetric or neonatal outcomes. Conclusion: ACHD is associated with adverse cardiac, obstetric and neonatal outcomes. Echo parameters are predictive of adverse cardiac, but not obstetric or neonatal outcomes. Large-scale studies are needed to identify drivers of adverse obstetric and neonatal outcomes in this population.

Association between systemic inflammatory response index and left ventricular remodeling and systolic dysfunction in atrial fibrillation patients

BackgroundCardiac remodeling and dysfunction can be caused by atrial fibrillation (AF). The aim of this research is to investigate the relationship between the systemic inflammatory response index (SIRI) and left ventricular (LV) remodeling and systolic function in individuals with AF.Methods416 patients with AF who were admitted to the Second Department of Cardiology in the East Ward of the Qingdao Municipal Hospital between January 2020 and May 2022 were included in the present retrospective research. The relationship between SIRI and various cardiac parameters was analyzed. The patients’ left atrial (LA) enlargement and left ventricular (LV) hypertrophy and systolic dysfunction were evaluated. SIRI was calculated by the formula: neutrophil × monocyte/lymphocyte.ResultsSIRI significantly correlated with LV end-diastolic diameter (LVDd), LV posterior wall thickness at end-diastole (LVPWTd), interventricular septal thickness at end-diastole (IVSTd), LV mass index (LVMI), LV ejection fraction (LVEF), LA diameter (LAD), C-reactive protein (CRP), and N-terminal pro-B-type natriuretic peptide (NT-proBNP) in patients with AF. In multivariate linear regression analyses, SIRI was discovered to be significantly related to LVMI (ln-transformed) (p = 0.025), LVEF (ln-transformed) (p = 0.005), and LAD (ln-transformed) (p = 0.007). In multivariate logistic regression, the highest quartile of SIRI (SIRI > 1.62) was significantly associated with LV hypertrophy (p = 0.026), impaired LV systolic function (p = 0.002), and LA enlargement (p = 0.025).ConclusionsSIRI was significantly associated with LV remodeling and systolic function impairment in patients with AF. SIRI may serve as a reliable and convenient inflammatory biomarker for detecting impaired cardiac structure and systolic function in patients with AF.

Pacemaker lead insertion sites contribute to abnormalities of myocardial function and histopathology

Ventricular pacing can cause myocardial dysfunction, but how lead anchoring to the myocardium affects function has not been studied. The purpose of this study was to evaluate patterns of regional and global ventricular function in patients with a ventricular lead using cine cardiac computed tomography (CCT) and histology. This was a single-center retrospective study with 2 groups of patients with a ventricular lead: (1) those who underwent cine CCT from September 2020 to June 2021 and (2) those whose cardiac specimen was analyzed histologically. Regional wall motion abnormalities on CCT were assessed in relation to lead characteristics. For the CCT group, 122 ventricular lead insertion sites were analyzed in 43 patients (47% female; median age 19 years; range 3-57 years). Regional wall motion abnormalities were present at 51 of 122 lead insertion sites (42%) in 23 of 43 patients (53%). The prevalence of a lead insertion-associated regional wall motion abnormality was higher with active pacing (55% vs 18%; P < .001). Patients with lead insertion-associated regional wall motion abnormalities had a lower systemic ventricular ejection fraction (median 38% vs 53%; P < .001) than did those without regional wall motion abnormalities. For the histology group, 3 patients with 10 epicardial lead insertion sites were studied. Myocardial compression, fibrosis, and calcifications were commonly present directly under active leads. Lead insertion site-associated regional wall motion abnormalities are common and associated with systemic ventricular dysfunction. Histopathological alterations including myocardial compression, fibrosis, and calcifications beneath active leads may explain this finding.

Echocardiographic Strain to Predict Need for Transplant or Mortality in Fontan Patients with Hypoplastic Left Heart Syndrome.

Despite recent advances, hypoplastic left heart syndrome (HLHS) patients subsequent to the Fontan still have significant morbidity and mortality. Some require heart transplant due to systemic ventricular dysfunction. Limited data exist on timing for transplant referral. This study aims to correlate systemic ventricular strain by echocardiography to transplant-free survival. HLHS patients who had Fontan palliation at our institution were included. Patients were divided into: 1) Required transplant or experienced mortality (composite end point); 2) Did not require transplant or survived. For those who experienced the composite endpoint, the last echocardiogram prior to the composite outcome was used, while for those who did not experience the composite endpoint the last echocardiogram obtained was used. Several qualitative and quantitative parameters were analyzed with focus on strain parameters. Ninety-five patients with HLHS Fontan palliation were identified. Sixty-six had adequate images and eight (12%) experienced transplant or mortality. These patients had greater myocardial performance index by flow Doppler (0.72 versus 0.53, p = 0.01), higher systolic/diastolic duration ratio (1.51 versus 1.13, p = 0.02), lower fractional area change (17.65 versus 33.99, p < 0.01), lower global longitudinal strain (GLS) (-8.63 versus -17.99, p < 0.01), lower global longitudinal strain rate (GLSR) (-0.51 versus -0.93, p < 0.01), lower global circumferential strain (GCS) (-6.68 versus -18.25, p < 0.01), and lower (GCSR) global circumferential strain rate (-0.45 versus -1.01, p < 0.01). ROC analysis demonstrated predictive value for GLS -7.6 (71% sensitive, 97% specific, AUC 81%), GLSR -0.58 (71% sensitive, 88% specific, AUC 82%), GCS -10.0 (86% sensitive, 91% specific, AUC 82%), and GCSR -0.85 (100% sensitive, 71% specific, AUC 90%). GLS and GCS can help predict transplant-free survival in patients with hypoplastic left heart syndrome having undergone Fontan palliation. Higher strain values (closer to zero) may be a helpful tool in determining when transplant evaluation is warranted in these patients.

Single Center Experience With Durable Continuous Flow Single Ventricle Assist Device: A Viable Option in Fontan Circulatory Failure.

Ventricular assist devices are increasingly used for patients with single ventricle physiology. We describe the use of durable, continuous flow, single ventricular assist device (SVAD) therapy in Fontan circulatory failure patients. Retrospective, single-center review of patients with Fontan circulation implanted with a SVAD between 2017 and 2022. Patient characteristics and outcomes were obtained by chart review. Nine patients underwent SVAD implantation (median age 24 years). Most patients had a total cavopulmonary connection; one had an atriopulmonary Fontan. Five patients had a systemic right ventricle. SVAD was most often utilized as bridge to candidacy (67%). Eight patients had at least moderate systemic ventricular systolic dysfunction. SVAD support continued for a median of 65 days (longest duration, 1,105 days) and one patient remains on support at time of submission. Of five patients discharged home, median length of stay after SVAD was 24 days. Six patients were transplanted (median 96 days from SVAD). Two patients died from pretransplant multisystem organ failure before transplant. All transplanted patients remain alive (median time since transplant 593 days). Continuous flow SVAD therapy can be effective for patients with Fontan circulatory failure and systolic dysfunction. Further studies should investigate feasibility and optimal SVAD timing with more advanced Fontan associated end-organ dysfunction.

Longitudinal analysis of systemic ventricular function and atrioventricular valve function after the Fontan procedure.

This study aimed to determine the longitudinal change of systemic ventricular function and atrioventricular valve (AVV) regurgitation after total cavopulmonary connection (TCPC). In 620 patients who underwent TCPC between 1994 and 2021, 4219 longitudinal echocardiographic examinations of systemic ventricular function and AVV regurgitation were evaluated retrospectively. The most frequent primary diagnosis was hypoplastic left heart syndrome in 172, followed by single ventricle in 131, tricuspid atresia in 95 and double inlet left ventricle (LV) in 91 patients. Dominant right ventricle (RV) was observed in 329 (53%) and dominant LV in 291 (47%). The median age at TCPC was 2.3 (1.8-3.4) years. Transplant-free survival at 5, 10 and 15 years after TCPC was 96.3%, 94.7% and 93.6%, respectively, in patients with dominant RV and 97.3%, 94.6% and 94.6%, respectively, in those with dominant LV (P = 0.987). Longitudinal analysis of systemic ventricular function was similar in both groups during the first 10 years postoperatively. Thereafter, systemic ventricular function worsened significantly in patients with dominant RV, compared with those with dominant LV (15 years: P = 0.007, 20 years: P = 0.03). AVV regurgitation was more frequent after TCPC in patients with dominant RV compared with those with dominant LV (P < 0.001 at 3 months, 3 years, 5 years, 10 years and 15 years, P = 0.023 at 20 years). There was a significant correlation between postoperative systemic ventricular dysfunction and AVV regurgitation (P < 0.001). There were no transplant-free survival difference and no difference in ventricular function between dominant RV and dominant LV for the first 10 years after TCPC. Thereafter, ventricular function in dominant RV was inferior to that in dominant LV. The degree of AVV regurgitation was significantly higher in dominant RV, compared with dominant LV, and it was positively associated with ventricular dysfunction, especially in dominant RV.

Three-dimensional electroanatomical mapping guided right bundle branch pacing in congenitally corrected transposition of great arteries.

The ideal pacing strategy has been the Achilles' heel for patients with congenitally corrected transposition of great arteries (ccTGA) with bradycardia. Various pacing modalities were documented in the literature. This article describes a novel pacing strategy and its feasibility in ccTGA with an intact ventricular septum. We prospectively recruited three patients with ccTGA who presented with symptomatic complete heart block to our institute and were evaluated. All patients were planned for conduction system pacing. Those who had more than moderate or severe systemic atrioventricular regurgitation and systemic ventricular dysfunction were planned for conduction system pacing with an additional lead in the coronary sinus (CS) tributary, i.e. bundle branch pacing optimized cardiac resynchronization therapy with the intention to achieve incremental benefit. Since right bundle pacing is not described previously and in view of anatomical complexity in location, three-dimensional (3D) anatomical mapping was done with the EnSite system and later right bundle capture is identified conventionally as that of a left bundle in a normal heart. All three patients have stable lead positions and adequate thresholds at short-term follow-up. In this report, we demonstrated the feasibility of permanent physiological pacing of the systemic ventricle by capturing the right bundle with 3D anatomical mapping guidance, which results in physiological activation of the systemic ventricle.

Outcome after stage 1 palliation in non-hypoplastic left heart syndrome patients as a univentricular palliation.

Studies focused on infants with univentricular heart undergoing stage I palliation other than the Norwood procedure remain a topic of great interest. This study evaluated the outcome of infants who underwent systemic to pulmonary shunt (SPS) or pulmonary artery banding (PAB). Infants who underwent SPS or PAB as stage I palliation between 1994 and 2019 were included. Survival and late systemic ventricular function were evaluated. Out of 242 patients, 188 underwent SPS (77.7%) and 54 PAB (22.3%). Main diagnosis includedtricuspid atresia, unbalanced atrioventricular septal defects, double inlet left ventricles and single ventricles with other morphology. Thirty-eight patients died before stage II palliation (15.7%). Stage II palliation was performed in 182 patients (75.2%), and mortality between stages II and III was 11 (5.6%). Stage III palliation was performed in 160 (66.1%) patients. Survival at 1, 5 and 15 years after stage I procedure was 81.9, 77.1 and 76.2%, respectively, and similar between both procedures (P = 0.97). Premature birth [P = 0.03, hazard ratio (HR) = 2.1], heterotaxy (P = 0.006, HR = 2.4) and dominant right ventricle (P = 0.015, HR = 2.2) were factors associated to mortality. Unbalanced atrioventricular septal defect (P = 0.005, HR = 4.6) was a factor associated to systemic ventricular dysfunction. In patients with univentricular heart who underwent SPS and PAB as stage I palliation, survival at 15 years was 76%, regardless of th chosen approach. Premature birth, heterotaxy and dominant right ventricle wereassociated to mortality.

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 9972: Sex-Related Differences and the Influence of Pregnancy on Cardiac Outcomes in Adults With a Systemic Right Ventricle and Biventricular Physiology

Introduction: Transposition of the great arteries (TGA) with a systemic right ventricle (sRV) and biventricular physiology is associated with increased morbidity and mortality. There is a paucity of data regarding sex-related differences in outcomes in the context of a sRV. Moreover, pregnancy has been associated with deterioration of sRV function in short-term post-partum follow-up, but the long-term impact remains largely unknown. Methods and Results: A retrospective cohort study was conducted on 214 adults, age 44.7±12.3 years, with a sRV and biventricular physiology followed for a median of 13 years at an adult congenital heart disease center. No sex-related difference was identified in the prevalence of atrial or ventricular arrhythmias, permanent pacemaker implantation, hospitalization for heart failure, systemic atrio-ventricular valve intervention, heart transplant, or cardiac death. Among the 82 (38.3%) women, age 44.0±12.5 years, 43 (52.4%) had at least one full-term pregnancy. Women had a lower prevalence of moderate to severe sRV dysfunction than men (21% vs 42.6%, p=0.001) despite similar ages. Beta-blockers (p=0.008), furosemide (p=0.012), and mineralocorticoid receptor antagonists (p=0.028) were less frequently prescribed to women than men. Women had fewer implantable cardioverter-defibrillators (ICDs) for primary prevention than men (3.7% vs 13.6%, p=0.016), with no difference in the prevalence of secondary prevention ICDs (1.2% vs 2.3%, p=1). The four women with a prohibitive maternal mortality risk (modified WHO class IV) complied with recommendations to avoid pregnancy. After excluding these 4 women, no differences regarding frequency of adverse cardiac events, age at the time of event, and proportion with moderate or severe sRV dysfunction were observed in women with (N=43) and without (N=35) pregnancies during 14 years of follow-up. Conclusions: Women with TGA and a sRV had a lower prevalence of moderate to severe systemic ventricular dysfunction than men, along with a lower proportion of primary prevention ICDs. Following risk assessment and counselling with contraindication of pregnancy in the highest risk subgroup, pregnancy had no impact on long-term cardiac outcomes.

Renal dysfunction: a predictor of adverse outcomes in ACHD patients with acute decompensated heart failure

Abstract Background Renal dysfunction (RD) is a predictor of adverse outcomes in patients with acquired heart failure (HF). Studies in adult congenital heart disease (ACHD) have demonstrated the link between RD and increased mortality. However, there is a paucity of data regarding the prognostic significance of RD in ACHD and HF. We assessed the impact of RD on outcomes in ACHD patients presenting with acute decompensated HF requiring intravenous (IV) diuresis in a tertiary centre between 2010–2021. Methods This was a retrospective analysis on RD and outcomes during the index hospital admission and after discharge. Chronic kidney disease (CKD) was defined as an eGFR &amp;lt;60mL/min/1.73 m2 using the MDRD equation. Cox regression analysis was used to identify predictors of death after discharge. Results We included 176 HF admissions, 76 (43.2%) female, age 47.7±14.5 years. Complex CHD was present in 50.6%. The most frequent underlying congenital heart defects were: transposition of the great arteries (including congenitally corrected, 19.9%), univentricular (14.2%), and tetralogy of Fallot (13.6%). Eisenmenger syndrome was present in 18.8%, a systemic right ventricle in 22.2%, 40.9% had pulmonary arterial hypertension (PAH), and 38.1% were cyanotic. At the time of the index admission 92 (52.3%) had RD (eGFR &amp;lt;60 mL/min/1.73 m2 and/or serum creatinine &amp;gt;88 μmol/L), 63 (38.2%) had a history of CKD. Patients with RD on admission were older (49.8 [42.3–60.9] vs. 46.0 [33.8–53.6] years, p=0.02) and more likely to have a history of arrhythmia (71.7% vs. 53.6%, p=0.02), but did not differ to those without RD in terms of diabetes mellitus or systemic hypertension. Admission BNP was higher in patients with RD (594 [258–1216] vs. 354 [158–633] ng/L, p=0.01). Patients with RD were more likely to have at least moderate systemic (31.8% vs. 11.8%, p=0.005) or pulmonary (58.4% vs. 36.4%, p=0.01) ventricular dysfunction. They were also more likely to have systemic ventricular dilatation (28.6% vs. 11.8%, p=0.02) or a larger RA area (29 [21–34] vs. 21 [16–31] cm2, p=0.008). Inpatients with RD required higher doses of IV furosemide (160 [80–200] vs. 80 [70–160] mg, p=0.03) and there was a trend for more frequent inotropic support (19.6% vs. 8.3%, p=0.06). In-hospital mortality was relatively low (4.5%), however, 94 (56.0%) patients died and 73 (43.5%) were rehospitalised for HF at a median follow-up of 2.8 [0.01–12.0] years. CKD (HR 2.43, 95% CI: 1.59–3.71, p&amp;lt;0.0001) and RD on admission (HR 1.7, 95% CI: 1.13–2.58, p=0.01) were strong predictors of death (Figure 1). On multivariable Cox analysis, PAH, CKD, and peak inpatient diuretic dose remained predictive of mortality. Conclusions ACHD patients admitted with acute decompensated HF are a high-risk cohort for acute re-admission and mortality. Preexisting RD and need for high dose IV diuretics conveys an even worse prognosis. Earlier interventions based on evidence of RD in ACHD may modulate this spiral trajectory and warrants further investigation. Funding Acknowledgement Type of funding sources: None.

Contemporary risk factors for a longer hospital stay following bidirectional cavopulmonary anastomosis.

Despite high survival after bidirectional cavopulmonary anastomosis, a considerable number of patients suffer significant post-operative morbidities related to prolonged length of stay. A single-center retrospective cohort study of all consecutive patients undergoing a first-time bidirectional cavopulmonary anastomosis from 2006 to 2019. Prolonged length of stay was defined as hospital stay greater than the 75th percentile for our cohort. Of 195 patients who met inclusion criteria, the median post-operative length of stay was 8 days (interquartile range, 4-15 days). Prolonged length of stay was defined as greater than 15 days. In multivariate analysis, greater than mild systemic atrioventricular valve regurgitation (odds ratio 3.7, 95% CI 1.05-13.068, p = 0.04), longer length of stay after the initial palliative procedure (odds ratio 1.028, 95% CI 1.004-1.05, p = 0.02), and pre-operative higher superior vena cava oxygen saturation (odds ratio 0.922, 95% CI 0.85-0.99, p = 0.04) maintained statistical significance as independent risk and protective factors for prolonged length of stay. A one-level increase in the severity of pre-operative systemic atrioventricular valve regurgitation was associated with a multiplicative change in the odds ratio of prolonged length of stay of 5.45 (p = 0.005) independent of the severity of systemic ventricular dysfunction. Pre-operative characteristics with greater than mild systemic atrioventricular valve regurgitation, longer length of stay after the initial palliative procedure, and lower superior vena cava oxygen saturation were associated with prolonged length of stay after a first-time bidirectional cavopulmonary anastomosis.

Percutaneous Edge To Edge Repair for the treatment of severe Tricuspid valve Regurgitation in high-surgical risk patients with Systemic Right Ventricle: A Multicentric French Cohort (PETER TRESRIV)

Patients with systemic right ventricle (SRV) (mostly congenitally-corrected transposition of thegreat arteries or transposition of the great arteries corrected by atrial switch) commonly developsignificant systemic tricuspid valve regurgitation and systemic right ventricular dysfunction inadulthood, both of which being a therapeutic dilemma for the care team. Until recently, the onlytherapeutic option has been surgery but the rate of complications is high. Percutaneous edge to edgerepair could be an alternative, especially for high-surgical risk patients. To describe clinical and paraclinical follow up of high-surgical risk patients with SRV and severe TR undergoing PETER. 10 high-risk surgical patients with severe systemic tricuspid regurgitation (TR) undergoing apercutaneous repair with the Mitraclip or Triclip system (Abbott) were included between May 2019and October 2021. We collected and compared clinical (symptoms, quality of life questionnaires), biological (renal function, BNP/ntproBNP levels), VO2 max testing, echocardiographic, and crosssectionnalimaging (tricuspid regurgitation severity, right ventricle dilatation and dysfunction) dataat baseline and 6 months after the procedure. We report a percutaneous implant success of 80%, one early death due to septal leaflet tear and 2early common flutters successfully ablated. Clinically, we describe a median improvement after 6months of functional status as well as physical (+10,3 points) and mental (+12,2 points) quality oflife scores. Biologically, BNP and ntproBNP median blood levels decreased (respectively of 162and 178 μmol/L). VO2 max testing parameters did not change significantly. TTE TR severityparameters EROA and regurgitation volume improved of respectively 20 mm 2 and 11 mL while MRIor TDM SRV volumes decreased in median of 25 ml/m 2 for diastolic and 22 ml/m 2 for systolicvolume, with an increase in RVEF of 18,5%. Percutaneous edge to edge repair of systemic tricuspid regurgitation might be a safe and effectivetherapeutic option in high-risk surgical adult patients.

New Heart, Old Problems: Aortopulmonary Collaterals Cause Diastolic Heart Failure in a Pediatric Heart Transplant Recipient

IntroductionAortopulmonary collaterals (APCs) frequently develop in children with single ventricle (SV) physiology. The prevalence and clinical impact of APCs after pediatric heart transplant (HT) are unknown. We highlight a case of an infant with failed SV palliation with post-HT diastolic heart failure due to a significant burden of APCs.Case ReportA 1-year old infant with hypoplastic left heart syndrome underwent first and second-stage surgical palliation. He developed end-stage heart failure due to systemic right ventricular systolic dysfunction and subsequently underwent HT (recipient/donor weight: 0.89). The extent of APCs was unknown at the time of HT. Post-HT, he had ongoing evidence of diastolic dysfunction with a significant diuretic requirement, persistently elevated BNPs (≈600-800pg/ml), and chronic kidney disease throughout the first year after HT. He had no evidence of graft rejection or coronary allograft vasculopathy. There was suspicion for APCs given holodiastolic flow reversal in the abdominal aorta seen on echocardiography. Cardiac catheterization revealed diastolic dysfunction with left ventricle end-diastolic pressures of 17-23mmHg. There was a step up of saturation from superior vena cava to pulmonary artery (∆15%). Angiography revealed 6 APCs from the right and left internal mammary, right and left thoracic, and right axillary arteries (Figure 1A). Embolization was performed utilizing 33 coils (Figure 1B). Cerebral near infrared spectroscopy immediately improved from 50s to 80s after coiling. Seven months after this procedure, the BNP decreased to 200-300 pg/ml, the LVEDP decreased to 12mmHg, and his diuretic requirement significantly improved.SummaryAPCs may be a cause of persistent diastolic dysfunction and heart failure after HT in patients with previous SV physiology. Interrogation for APCs in these patients should be considered post-HT to aid in early diagnosis, and treatment may yield significant clinical improvement. Aortopulmonary collaterals (APCs) frequently develop in children with single ventricle (SV) physiology. The prevalence and clinical impact of APCs after pediatric heart transplant (HT) are unknown. We highlight a case of an infant with failed SV palliation with post-HT diastolic heart failure due to a significant burden of APCs. A 1-year old infant with hypoplastic left heart syndrome underwent first and second-stage surgical palliation. He developed end-stage heart failure due to systemic right ventricular systolic dysfunction and subsequently underwent HT (recipient/donor weight: 0.89). The extent of APCs was unknown at the time of HT. Post-HT, he had ongoing evidence of diastolic dysfunction with a significant diuretic requirement, persistently elevated BNPs (≈600-800pg/ml), and chronic kidney disease throughout the first year after HT. He had no evidence of graft rejection or coronary allograft vasculopathy. There was suspicion for APCs given holodiastolic flow reversal in the abdominal aorta seen on echocardiography. Cardiac catheterization revealed diastolic dysfunction with left ventricle end-diastolic pressures of 17-23mmHg. There was a step up of saturation from superior vena cava to pulmonary artery (∆15%). Angiography revealed 6 APCs from the right and left internal mammary, right and left thoracic, and right axillary arteries (Figure 1A). Embolization was performed utilizing 33 coils (Figure 1B). Cerebral near infrared spectroscopy immediately improved from 50s to 80s after coiling. Seven months after this procedure, the BNP decreased to 200-300 pg/ml, the LVEDP decreased to 12mmHg, and his diuretic requirement significantly improved. APCs may be a cause of persistent diastolic dysfunction and heart failure after HT in patients with previous SV physiology. Interrogation for APCs in these patients should be considered post-HT to aid in early diagnosis, and treatment may yield significant clinical improvement.

Outcomes of Children with Hypoplastic Left Heart Syndrome and Systemic Ventricular Dysfunction on Guideline Directed Medical Therapy

<h3>Purpose</h3> Right ventricular dysfunction (RV dysfxn) in hypoplastic left heart syndrome (HLHS) is associated with increased mortality. Such patients (pts) are often commenced on heart failure (HF) medications despite a lack of evidence. We hypothesized that guideline directed medical therapy (GDMT) would be associated with improved function (fxn) and reduced mortality. <h3>Methods</h3> The SickKids HF Database is a retrospective cohort of all paediatric HLHS pts from 2001 to 2017, with at least mild RV dysfxn on echocardiogram lasting >30 days, or death or transplant within 30 days onset of dysfxn. Normalization of fxn was defined as qualitatively normal RV fxn on echocardiogram lasting ≥30 days after dysfxn onset. The primary endpoint was normalization of RV fxn with GDMT at 70% of target dosing (TD). Secondary outcomes included transplant-free survival (TFS) with GDMT. Descriptive statistics and time to event analysis were used. <h3>Results</h3> Our cohort included 99 pts with median age at onset of dysfxn 2.6(1.0-5.3) months. Overall TFS was 44% at 5 years after dysfxn onset and 51 (52%) pts had normalization of RV fxn. TFS was 14% at 2 years after dysfxn onset in pts with persistent dysfxn versus 78% in pts with normalization of fxn. TD in ≥1 HF medication was reached in 57(58%) pts by study endpoint. In those with normalization of RV fxn; 24(47%) were on 1 medication at TD versus 13 (27%) with persistent dysfxn (p<0.0001), and 14 (27%) were on 2 medications at TD versus 4(8%) with persistent dysfxn (p=0.0004). Of those in whom fxn normalized, 35 (69%) were on TD ACEi versus 14(29%) with persistent dysfxn, 12(14%) were on TD beta blocker, and 21(41%) were on TD spironolactone. TD ACEi correlated with improved 5-year TFS (25% to 60%, p<0.0001, Figure 1A). Figure 1B delineates changes in TFS with GDMT. <h3>Conclusion</h3> In our cohort of pts with HLHS and RV dysfxn, GDMT was associated with increased rate of normalization of fxn and improved TFS at 5 years after dysfxn onset.