Marie-A Chaix’s research while affiliated with Montreal Heart Institute and other places

Background The univentricular heart with a predominant right ventricle morphology (uRV) has been associated with a higher rate of adverse cardiovascular events. It remains to be determined whether the specific type of uRV influences outcomes. Methods and Results A North American multicenter retrospective cohort study was conducted by the Alliance for Adult Research in Congenital Cardiology on individuals with a uRV and total cavopulmonary connection Fontan. The incidence of a composite outcome consisting of all‐cause mortality, cardiac transplantation, atrial arrhythmias, or thromboembolic events was compared among patients with Fontan palliation who had hypoplastic left heart syndrome (HLHS) versus other forms of uRV (non‐HLHS). All components of the composite outcome were classified by a blinded adjudicating committee. Competing risks were taken into account in time‐to‐event analyses. A total of 171 patients with uRV of whom 76 (44.4%) had HLHS were followed for 10.2±5.7 years. The composite outcome occurred in 7.1 versus 2.1 cases per 100 person‐years in patients with HLHS versus non‐HLHS ( P <0.0001). In multivariable analyses, HLHS was associated with a significantly higher risk of the composite outcome (hazard ratio [HR], 6.13 [95% CI, 2.92–12.69], P <0.001). Moreover, HLHS was associated with significantly higher rates of all components of the primary outcome. Conclusions Among patients with a uRV and Fontan palliation, HLHS is associated with a significantly higher rate of adverse cardiovascular events.

Background There is a paucity of data regarding sex-related differences on cardiac outcomes in the context of transposition of the great arteries (TGA) with a systemic right ventricle and biventricular physiology (sRV-biV). Moreover, the long-term impact of pregnancy on cardiac outcomes remains unknown. Objectives The purpose of this study was to identify sex-related differences and the influence of pregnancy on cardiac outcomes in TGA sRV-biV population. Methods A retrospective cohort study was conducted on 213 adults with TGA sRV-biV, 82 (38.4%) women, age 42.6 ± 12.8 years, with a median follow-up of 16 years. Cardiac events, interventions, last follow-up sRV-biV dysfunction, and heart failure (HF) medications were compared between men vs women, and women with vs without pregnancies resulting in live births. Results Women had a lower incidence of nonsustained ventricular tachycardia (HR: 1.80; 95% CI: 1.04-3.09, P = 0.035) and nonsignificantly fewer HF-related hospitalizations than men (HR: 2.10; 95% CI: 0.95-4.67, P = 0.069) in univariable analysis. At the last follow-up, women had a lower prevalence of moderate to severe sRV-biV dysfunction than men (P < 0.001) and were less frequently prescribed HF therapy. Women had fewer implantable cardioverter-defibrillators for primary prevention than men (P = 0.016), with no difference for secondary prevention. Women who had pregnancies resulting in live births (N = 47), had a high prevalence of cardiac events in the 15 (IQR: 9-28) years following pregnancy with no significant differences with those without (N = 32) pregnancies. Conclusions Women with a sRV-biV have fewer adverse cardiovascular events than men. Due to sRV-biV, pregnancy remains with high maternal risk but is not associated with worse long-term cardiac outcomes under rigorous multidisciplinary cardio-obstetrical care.

Background There is a paucity of data on long-term outcomes after Fontan palliation in patients with a dominant morphological univentricular right (uRV) vs left (uLV) ventricle. Objectives The purpose of this study was to compare the incidence of atrial arrhythmias, thromboembolic events, cardiac transplantation, and death following Fontan palliation in patients with uRV vs uLV. Methods The Alliance for Adult Research in Congenital Cardiology conducted a multicenter retrospective cohort study on patients with total cavopulmonary connection Fontan palliation across 12 centers in North America. All components of the composite outcome, that is, atrial arrhythmias, thromboembolic events, cardiac transplantation, and death, were reviewed and classified by a blinded adjudicating committee. Time-to-event analyses were performed that accounted for competing risks. Results A total of 384 patients were followed for 10.5 ± 5.9 years. The composite outcome occurred in 3.7 vs 1.7 cases per 100 person-years for uRV (N = 171) vs uLV (N = 213), respectively (P < 0.001). In multivariable analyses, uRV conferred a >2-fold higher risk of the composite outcome (HR: 2.17, 95% CI: 1.45-3.45, P < 0.001). In secondary analyses of components of the primary outcome, uRV was significantly associated with a greater risk of cardiac transplantation or death (HR: 9.09, 95% CI: 2.17-38.46, P < 0.001) and atrial arrhythmias (HR: 2.17, 95% CI: 1.20-4.00, P = 0.010) but not thromboembolic events (HR: 1.64, 95% CI: 0.86-3.16, P = 0.131). Conclusions Fontan patients with uRV vs uLV morphology have a higher incidence of adverse cardiovascular events, including atrial arrhythmia, cardiac transplantation, and all-cause mortality.

BACKGROUND: The clinical care of adults with a systemic right ventricle with biventricular circulation (sRV-biV) is influenced by cardiorespiratory fitness (CRF) evaluation. However, prognostic markers for CRF are derived from analyses of adults with a systemic left ventricle (sLV). While some studies suggest that CRF is lower in adults with sRV-biV compared to those with a sLV, they have not controlled for key clinical characteristics and ventricular function of the systemic ventricle. RESEARCH QUESTION: Is CRF lower in adults with a sRV-biV compared to adults with sLV matched for clinical characteristics and systemic ventricular function? METHODS: A retrospective cross-sectional comparison of exercise stress test results was performed comparing 24 adults with sRV-biV to 24 adults with a sLV matched for sex (4 females), age (sRV-biV: 46 ± 9 years, sLV: 46 ± 11 years), body mass index (sRV-biV: 28 ± 4 kg/m2, sLV: 27 ± 4 kg/m2), ejection fraction of the systemic ventricle (normal: 2, mild: 4, moderate: 11, severe: 7), NYHA class (I: 6, II: 17, III: 1), and doses of diuretics (spironolactone ≤ 25 mg: 24, furosemide ≤ 80 mg: 24 ). Peak VO2 was compared between groups with Wilcoxon signed-rank tests. Effect sizes are reported as Cohen’s d. RESULTS: Peak VO2 did not differ significantly between groups (sRV-biV: 20.3 ± 4.2 vs. sLV: 20.9 ± 6.0 mL/kg/min, p=0.43, d=0.18). When patients were dichotomized according to the Weber criterion for optimal versus suboptimal CRF (sLV peak VO2 > or ≤ 20 mL/kg/min), peak VO2 was lower in sRV-biV in those with optimal CRF (sRV-biV: 21.0 ± 4.8 vs sLV: 26.6 ± 5.1 mL/kg/min, p=0.006, d=0.72). In contrast, peak VO2 was greater in sRV-biV in those with suboptimal CRF (sRV-biV: 19.3 ± 3.5 vs. sLV: 16.9 ± 2.0 mL/kg/min, p=0.12, d=NA). CONCLUSION: Although peak VO2 values are similar among patients with a sRV-biV and matched controls with a sLV, differences are observed when stratified according to the Weber criteria for optimal CRF. These results may reflect different pathophysiological adaptive mechanisms of a sRV vs sLV. Further studies are required to establish specific prognostic thresholds for patients with sRV-biV.

Background: The clinical care of adults with a systemic right ventricle with biventricular circulation (sRV-biV) is influenced by cardiorespiratory fitness (CRF) evaluation. However, prognostic markers for CRF are derived from analyses of adults with a systemic left ventricle (sLV). While some studies suggest that CRF is lower in adults with sRV-biV compared to those with a sLV, they have not controlled for key clinical characteristics and ventricular function of the systemic ventricle. Research Question: Is CRF lower in adults with a sRV-biV compared to adults with sLV matched for clinical characteristics and systemic ventricular function? Methods: A retrospective cross-sectional comparison of exercise stress test results was performed comparing 24 adults with sRV-biV to 24 adults with a sLV matched for sex (4 females), age (sRV-biV: 46 ± 9 years, sLV: 46 ± 11 years), body mass index (sRV-biV: 28 ± 4 kg/m ² , sLV: 27 ± 4 kg/m ² ), ejection fraction of the systemic ventricle (normal: 2, mild: 4, moderate: 11, severe: 7), NYHA class (I: 6, II: 17, III: 1), and doses of diuretics (spironolactone ≤ 25 mg: 24, furosemide ≤ 80 mg: 24 ). Peak VO 2 was compared between groups with Wilcoxon signed-rank tests. Effect sizes are reported as Cohen’s d . Results: Peak VO 2 did not differ significantly between groups (sRV-biV: 20.3 ± 4.2 vs. sLV: 20.9 ± 6.0 mL/kg/min, p=0.43, d =0.18). When patients were dichotomized according to the Weber criterion for optimal versus suboptimal CRF (sLV peak VO 2 > or ≤ 20 mL/kg/min), peak VO 2 was lower in sRV-biV in those with optimal CRF (sRV-biV: 21.0 ± 4.8 vs sLV: 26.6 ± 5.1 mL/kg/min, p=0.006, d =0.72). In contrast, peak VO 2 was greater in sRV-biV in those with suboptimal CRF (sRV-biV: 19.3 ± 3.5 vs. sLV: 16.9 ± 2.0 mL/kg/min, p=0.12, d =NA). Conclusion: Although peak VO 2 values are similar among patients with a sRV-biV and matched controls with a sLV, differences are observed when stratified according to the Weber criteria for optimal CRF. These results may reflect different pathophysiological adaptive mechanisms of a sRV vs sLV. Further studies are required to establish specific prognostic thresholds for patients with sRV-biV.