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Heart failure hospitalization in adults with congenital heart diseases: What predicts it and how does it affect mortality?
10-Pediatrics and congenital cardiopathies 510
Electrocardiographic and echocardiographic findings in a cohort of patients with Costello syndrome and Cardiofaciocutaneous syndrome N. Welté , M. Michaud , X. Iriart ∗ , J. Thambo CHU de Bordeaux, Pessac, France ∗ Corresponding author. E-mail address: [email protected] (X. Iriart) Background Costello syndrome (CS) and Cardiofaciocutaneous syndrome (CFCS) are both RASopathies, a group of developmental disorders caused by a mutation in genes encoding in the RAS/MAPK pathway. RASopathies are prone to cardiac malformations and sudden cardiac death. Description of electrocardiographic findings in these patients is rare. The objective of this study was to describe electrocardiogram and correlation with echocardiographic findings in a cohort of CS and CFC. Method A 12-lead electrocardiogram (ECG) and a trans-thoracic echocardiography (TTE) were performed for all patients during the same period in July 2016, and were analyzed by two different cardiologists. Results Twenty-four patients were included, 14 with CFCS and 10 with CS. The mean age was 10.3 ± 7.0 years. In 14 patients (58%), at least one abnormality was noticed on the electrocardiogram: axis deviation in 11 patients (46%); atrial hypertrophy in 5 patients (21%); left ventricular hypertrophy in 2 patients (8%); incomplete bundle-branch block in 3 patients (13%) and a significant Q-wave in 5 patients (21%). All patients with left axis deviation and/or left ventricular hypertrophy at ECG had a hypertrophic cardiomyopathy (sub-aortic septal, apical or concentric hypertrophy) on TTE. The patients with a significant Q wave (among them 2 had an extreme right axis deviation) had in fact a minimal hypertrophic cardiomyopathy (septal thickness < 3 Z-score), suggesting the presence of myocardial fibrosis. Despite a normal ECG, 5 patients had a hypertrophic cardiomyopathy at TTE. Finally, 5 patients (21%) had a morphologically normal heart at TTE and all had a normal ECG. Conclusion Significant Q-wave or extreme right axis deviation on the ECG may suggest the potential presence of myocardial fibrosis. Detection of fibrosis by MRI may play an important role for the risk stratification of sudden cardiac death in these patients. Disclosure of interest The authors declare that they have no competing interest. https://doi.org/10.1016/j.acvdsp.2017.11.144
January 19th , Friday 2018 368
Heart failure hospitalization in adults with congenital heart diseases: What predicts it and how does it affect mortality? S. Cohen ∗ , A. Liu , F. Wang , L. Guo , J. Therrien , A. Marelli McGill adult unit for congenital heart disease excellence, Montreal, Canada ∗ Corresponding author. E-mail address: [email protected] (S. Cohen) Background Adults with congenital heart disease (ACHD) are not cured and residual abnormalities predispose them to heart failure (HF).
137 Aim To calculate the cumulative incidence of HF and assess the impact of HF on mortality. To identify predictors of one-year risk of HF. Methods This population-based retrospective cohort of 27,975 ACHD aged 18—65 in 1995—2010 was based on the Quebec CHD database. We calculated the cumulative probability of HF hospitalization using the practical incidence estimator macro to adjust for the competing risk of death. To assess the impact of HF on mortality, we first used propensity score matching to select random controls for each HF hospitalized patient. We then compared the mortality rates between cases and their matched controls. Finally, we applied nested-case control study and conducted logistic regression analyses to identify the predictors of one-year risk of HF hospitalization. We further used the regression model to construct a risk scoring system (RAAID-HF) for HF hospitalization to identify patients at high-risk of 1-year HF hospitalization. Results The lifetime cumulative risk of HF hospitalization by age 65 was 33.2%. HF hospitalization was associated with a 5-fold increase in mortality risk (HR = 5.4, 95% CI: 3.5, 8.3). Age, sex, CHD severity, HF hospitalization history and comorbidities (arrhythmia, pulmonary hypertension, coronary heart disease, diabetes, hypertension) were significant predictors of one-year HF hospitalization. The RAAID-HF had excellent predictive performance for HF hospitalization (C-statistics = 0.92). Conclusion HF is a common comorbid condition in ACHD patients and is strongly associated with an risk of death in ACHD population. We developed a convenient clinical risk score for predicting the risk of HF hospitalization within a year. These data enable targeting patients at high risk for 1-year HF hospitalization for accelerated referral to CHD centers. Disclosure of interest The authors declare that they have no competing interest. https://doi.org/10.1016/j.acvdsp.2017.11.145 084
Severity of hepatic fibrosis after Fontan operation is associated with early postoperative inferior vena cava pressure and time from surgery B. Lefort 1,∗,2 , A. Jaillais 2,3 , B. Aupy 1 , F. Dion 1 , N. Soulé 1 , J.M. El Arid 1 , P. Neville 1 , L. D’alteroche 3 , A. Chantepie 1,2 1 Unité cardiologie pédiatrique et congénitale adulte, CHU de Tours 2 Université Francois-Rabelais 3 Service d’hépato-gastro-entérologie, CHU de Tours, Tours, France ∗ Corresponding author. E-mail address: [email protected] (B. Lefort) Background Hepatic fibrosis increasing over time after a Fontan procedure is well documented, but the others potential risk factors of the severity of hepatopathy are still lacking. Method Since 2016, we started a systematic hepatic evaluation of patients undergoing a total cavo-pulmonary connection (TCPC) for at least 10 years, including clinical features, blood sample, abdominal ultrasound, liver elastography and liver biopsy. We look for clinical, biological and hemodynamic factors potentially associated with greater degree of fibrosis at biopsy. Results Fourteen patients had a complete evaluation with liver biopsy. Median time from TCPC to hepatic assessment was 15.9 years (range 3.0—25.9). Seventy one percent had biological cholestasis, and only 14% had cytolysis. Abdominal ultrasound revealed hepatomegaly in 4 patients and splenomegaly in 3, but no liver nodularity. METAVIR score of liver fibrosis at biopsies showed precirrhotic or cirrhotic lesions (F3—4) in 9 patients out the 14 (F1 = 3; F2 = 2; F3 = 5; F4 = 4). The degree of fibrosis was significantly correlated to inferior vena cava pressure (IVCP) obtained by cardiac
Electrocardiographic and echocardiographic findings in a cohort of patients with Costello syndrome and Cardiofaciocutaneous syndrome N. Welté , M. Michaud , X. Iriart ∗ , J. Thambo CHU de Bordeaux, Pessac, France ∗ Corresponding author. E-mail address: [email protected] (X. Iriart) Background Costello syndrome (CS) and Cardiofaciocutaneous syndrome (CFCS) are both RASopathies, a group of developmental disorders caused by a mutation in genes encoding in the RAS/MAPK pathway. RASopathies are prone to cardiac malformations and sudden cardiac death. Description of electrocardiographic findings in these patients is rare. The objective of this study was to describe electrocardiogram and correlation with echocardiographic findings in a cohort of CS and CFC. Method A 12-lead electrocardiogram (ECG) and a trans-thoracic echocardiography (TTE) were performed for all patients during the same period in July 2016, and were analyzed by two different cardiologists. Results Twenty-four patients were included, 14 with CFCS and 10 with CS. The mean age was 10.3 ± 7.0 years. In 14 patients (58%), at least one abnormality was noticed on the electrocardiogram: axis deviation in 11 patients (46%); atrial hypertrophy in 5 patients (21%); left ventricular hypertrophy in 2 patients (8%); incomplete bundle-branch block in 3 patients (13%) and a significant Q-wave in 5 patients (21%). All patients with left axis deviation and/or left ventricular hypertrophy at ECG had a hypertrophic cardiomyopathy (sub-aortic septal, apical or concentric hypertrophy) on TTE. The patients with a significant Q wave (among them 2 had an extreme right axis deviation) had in fact a minimal hypertrophic cardiomyopathy (septal thickness < 3 Z-score), suggesting the presence of myocardial fibrosis. Despite a normal ECG, 5 patients had a hypertrophic cardiomyopathy at TTE. Finally, 5 patients (21%) had a morphologically normal heart at TTE and all had a normal ECG. Conclusion Significant Q-wave or extreme right axis deviation on the ECG may suggest the potential presence of myocardial fibrosis. Detection of fibrosis by MRI may play an important role for the risk stratification of sudden cardiac death in these patients. Disclosure of interest The authors declare that they have no competing interest. https://doi.org/10.1016/j.acvdsp.2017.11.144
January 19th , Friday 2018 368
Heart failure hospitalization in adults with congenital heart diseases: What predicts it and how does it affect mortality? S. Cohen ∗ , A. Liu , F. Wang , L. Guo , J. Therrien , A. Marelli McGill adult unit for congenital heart disease excellence, Montreal, Canada ∗ Corresponding author. E-mail address: [email protected] (S. Cohen) Background Adults with congenital heart disease (ACHD) are not cured and residual abnormalities predispose them to heart failure (HF).
137 Aim To calculate the cumulative incidence of HF and assess the impact of HF on mortality. To identify predictors of one-year risk of HF. Methods This population-based retrospective cohort of 27,975 ACHD aged 18—65 in 1995—2010 was based on the Quebec CHD database. We calculated the cumulative probability of HF hospitalization using the practical incidence estimator macro to adjust for the competing risk of death. To assess the impact of HF on mortality, we first used propensity score matching to select random controls for each HF hospitalized patient. We then compared the mortality rates between cases and their matched controls. Finally, we applied nested-case control study and conducted logistic regression analyses to identify the predictors of one-year risk of HF hospitalization. We further used the regression model to construct a risk scoring system (RAAID-HF) for HF hospitalization to identify patients at high-risk of 1-year HF hospitalization. Results The lifetime cumulative risk of HF hospitalization by age 65 was 33.2%. HF hospitalization was associated with a 5-fold increase in mortality risk (HR = 5.4, 95% CI: 3.5, 8.3). Age, sex, CHD severity, HF hospitalization history and comorbidities (arrhythmia, pulmonary hypertension, coronary heart disease, diabetes, hypertension) were significant predictors of one-year HF hospitalization. The RAAID-HF had excellent predictive performance for HF hospitalization (C-statistics = 0.92). Conclusion HF is a common comorbid condition in ACHD patients and is strongly associated with an risk of death in ACHD population. We developed a convenient clinical risk score for predicting the risk of HF hospitalization within a year. These data enable targeting patients at high risk for 1-year HF hospitalization for accelerated referral to CHD centers. Disclosure of interest The authors declare that they have no competing interest. https://doi.org/10.1016/j.acvdsp.2017.11.145 084
Severity of hepatic fibrosis after Fontan operation is associated with early postoperative inferior vena cava pressure and time from surgery B. Lefort 1,∗,2 , A. Jaillais 2,3 , B. Aupy 1 , F. Dion 1 , N. Soulé 1 , J.M. El Arid 1 , P. Neville 1 , L. D’alteroche 3 , A. Chantepie 1,2 1 Unité cardiologie pédiatrique et congénitale adulte, CHU de Tours 2 Université Francois-Rabelais 3 Service d’hépato-gastro-entérologie, CHU de Tours, Tours, France ∗ Corresponding author. E-mail address: [email protected] (B. Lefort) Background Hepatic fibrosis increasing over time after a Fontan procedure is well documented, but the others potential risk factors of the severity of hepatopathy are still lacking. Method Since 2016, we started a systematic hepatic evaluation of patients undergoing a total cavo-pulmonary connection (TCPC) for at least 10 years, including clinical features, blood sample, abdominal ultrasound, liver elastography and liver biopsy. We look for clinical, biological and hemodynamic factors potentially associated with greater degree of fibrosis at biopsy. Results Fourteen patients had a complete evaluation with liver biopsy. Median time from TCPC to hepatic assessment was 15.9 years (range 3.0—25.9). Seventy one percent had biological cholestasis, and only 14% had cytolysis. Abdominal ultrasound revealed hepatomegaly in 4 patients and splenomegaly in 3, but no liver nodularity. METAVIR score of liver fibrosis at biopsies showed precirrhotic or cirrhotic lesions (F3—4) in 9 patients out the 14 (F1 = 3; F2 = 2; F3 = 5; F4 = 4). The degree of fibrosis was significantly correlated to inferior vena cava pressure (IVCP) obtained by cardiac