International Journal of Cardiovascular Imaging, May 29, 2021
Abnormal atrial mechanics in biventricular circulations have been associated with elevated left h... more Abnormal atrial mechanics in biventricular circulations have been associated with elevated left heart filling pressures. Similar associations in the Fontan circulation are unknown. The aim of this study was to examine the relationship between atrial mechanics and invasively assessed hemodynamic parameters late after the Fontan operation. Thirty-nine Fontan patients with echocardiographic and invasive hemodynamic studies done within 48 h were included and were compared to 40 age-matched healthy controls. Atrial and ventricular strain measurements were measured offline using 2-dimensional speckle-tracking. Mean age was 10.2 ± 6.7 years and 24 (62%) were male. Atrial strain measures were lower in Fontan patients compared to healthy controls. There was no significant association between atrial strain measurements and Fontan systemic ventricular filling pressures (SVFP) as indicated by pulmonary artery occlusion pressures, direct left atrial pressure or systemic ventricular end-diastolic pressure. Global atrial strain was not correlated with segmental atrial strain in the pulmonary venous atrium. Global atrial reservoir strain was positively correlated with pulmonary vascular resistance (r = 0.508, p = 0.045). Global atrial conduit strain was positively correlated with E/A ratio of the AV valve inflow (r = 0.555, p = 0.002). Atrial and ventricular strain measurements were not significantly correlated. In patients with a Fontan, global atrial function is significantly depressed, and is uncoupled from segmental left lateral atrial function. Global as well as segmental atrial mechanics are not significantly associated with SVFPs in Fontan patients. Instead, global atrial reservoir function appears to parallel pulmonary vascular resistance.
Background: Systemic light chain (AL) amyloidosis is considered a rare disease, but knowledge of ... more Background: Systemic light chain (AL) amyloidosis is considered a rare disease, but knowledge of its exact incidence and prevalence is based on old data involving limited populations. Aim: To determine the incidence and prevalence of AL amyloidosis in the general population. Patients and Methods: The national reference center for AL amyloidosis is located in the CHU de Limoges in Limoges (France), the administrative center of the Limousin region (738,110 inhabitants in 2014). A comprehensive and exhaustive database of AL amyloidosis patients diagnosed in this region has been computerized since January 2007. All patients living in the Limousin region and with a first diagnosis of systemic AL amyloidosis between January 1, 2012, and December 31, 2016, were retrospectively included to determine the disease incidence. All departments and laboratories of pathology were contacted in the region, and any possible missing cases diagnosed during the same period were collected. Prevalence was determined as all living patients with AL amyloidosis during 2016 regardless of the diagnosis date. Cardiac involvement was defined according to current definition (ie, mean wall thickness >12 mm in the absence of other causes of left ventricular hypertrophy). Renal involvement was defined as proteinuria >0.5 g/L (predominantly albumin) in the absence of other etiology. Results: Over the 5-year period, 46 new patients in the Limousin region had a confirmed diagnosis of AL amyloidosis (70% men; median age, 72.5 years), corresponding to a crude yearly incidence of 12.5 (95% CI, 5.6-19.4) cases per million inhabitants. The calculated prevalence of AL amyloidosis was 58 (95% CI, 43-73) cases per million inhabitants. Cardiac and renal involvement was found in 70% and 72% of cases, respectively. Mayo Clinic stages I, II, IIIA (NT-proBNP ≤8500 ng/L), and IIIB (NT-proBNP >8500 ng/L) based on cardiac biomarkers were found in 21%, 29%, 29%, and 21% of patients respectively. The underlying disease was multiple myeloma in 19 patients (41%), including smoldering myeloma in 14 patients, MGUS (monoclonal gammopathy of undetermined significance) in 22 patients (48%), IgM in 2 patients, Waldenstrom disease in 1 patient, low-grade lymphoma in 3 patients, plasmocytoma in 1 patient, and unknown in 1 patient. In this 5 year-period, 16 patients (35%) died. Conclusion: This is the firstreport of contemporary incidence and prevalence of systemic AL amyloidosis in France. Incidence was slightly higher than the incidence (adjusted to sex and age) previously reported by Kyle et al (Blood, 1992;7:1817-1822), probably corresponding to the progress in the past 20 years in diagnosing this rare disease. Disclosures Mohty: Prothena Biosciences: Honoraria. Jaccard: Amgen: Honoraria; Celgene: Honoraria, Other: Travel expenses, Research Funding; Janssen: Honoraria, Membership on an entity9s Board of Directors or advisory committees, Other: Travel expenses, Research Funding.
European Journal of Preventive Cardiology, Nov 6, 2014
Data on peripheral artery disease in Africa are sparse and limited to urban areas. Given the urba... more Data on peripheral artery disease in Africa are sparse and limited to urban areas. Given the urban/rural socio-economical gradient in these countries, we sought to determine the prevalence and risk factors of peripheral artery disease in urban and rural areas of two countries in Central Africa. Individuals ≥65 years old living in two urban and rural areas of the Republic of Central Africa (ROC) and the Central African Republic (CAR) were invited. Demographic, clinical and biological data were collected. Ankle-brachial index ≤0.90 defined peripheral artery disease. Among the 1871 participants (age 73 years, 62% female) the prevalence of peripheral artery disease was 14.8%, higher in ROC than in CAR (17.4% vs. 12.2%, p = 0.007) and higher in females than males (16.6% vs. 11.9%, p = 0.012). The prevalence of peripheral artery disease increased with age, respectively at 10.9%, 14.9%, 15.1% and 22.2% for age bands of 65-69, 70-74, 75-79 and 80+years (p < 0.001). Higher rates of peripheral artery disease were found in urban areas in ROC (20.7% vs. 14.4% in rural areas, p = 0.011), but not in CAR (11.5% vs. 12.9%, p = NS). In multivariate analysis, peripheral artery disease was significantly associated with age (odds ratio (OR): 1.03; p = 0.004), dyslipidaemia (OR: 1.88; p = 0.003), smoking (OR: 1.78; p = 0.003), obesity (OR: 1.98; p = 0.034) and underweight (OR: 1.49; p = 0.023). Regular alcohol drinking was associated with decreased risk of peripheral artery disease (OR: 0.73; p = 0.044). The prevalence of peripheral artery disease in the elderly is high in Africa, especially in females. In ROC, with a higher urban-rural socio-economic gradient, peripheral artery disease is more frequent in the urban areas.
Background: Systemic light chain (AL) amyloidosis is considered a rare disease, but knowledge of ... more Background: Systemic light chain (AL) amyloidosis is considered a rare disease, but knowledge of its exact incidence and prevalence is based on old data involving limited populations. Aim: To determine the incidence and prevalence of AL amyloidosis in the general population. Patients and Methods: The national reference center for AL amyloidosis is located in the CHU de Limoges in Limoges (France), the administrative center of the Limousin region (738,110 inhabitants in 2014). A comprehensive and exhaustive database of AL amyloidosis patients diagnosed in this region has been computerized since January 2007. All patients living in the Limousin region and with a first diagnosis of systemic AL amyloidosis between January 1, 2012, and December 31, 2016, were retrospectively included to determine the disease incidence. All departments and laboratories of pathology were contacted in the region, and any possible missing cases diagnosed during the same period were collected. Prevalence was ...
International Journal of Cardiovascular Imaging, May 29, 2021
Abnormal atrial mechanics in biventricular circulations have been associated with elevated left h... more Abnormal atrial mechanics in biventricular circulations have been associated with elevated left heart filling pressures. Similar associations in the Fontan circulation are unknown. The aim of this study was to examine the relationship between atrial mechanics and invasively assessed hemodynamic parameters late after the Fontan operation. Thirty-nine Fontan patients with echocardiographic and invasive hemodynamic studies done within 48 h were included and were compared to 40 age-matched healthy controls. Atrial and ventricular strain measurements were measured offline using 2-dimensional speckle-tracking. Mean age was 10.2 ± 6.7 years and 24 (62%) were male. Atrial strain measures were lower in Fontan patients compared to healthy controls. There was no significant association between atrial strain measurements and Fontan systemic ventricular filling pressures (SVFP) as indicated by pulmonary artery occlusion pressures, direct left atrial pressure or systemic ventricular end-diastolic pressure. Global atrial strain was not correlated with segmental atrial strain in the pulmonary venous atrium. Global atrial reservoir strain was positively correlated with pulmonary vascular resistance (r = 0.508, p = 0.045). Global atrial conduit strain was positively correlated with E/A ratio of the AV valve inflow (r = 0.555, p = 0.002). Atrial and ventricular strain measurements were not significantly correlated. In patients with a Fontan, global atrial function is significantly depressed, and is uncoupled from segmental left lateral atrial function. Global as well as segmental atrial mechanics are not significantly associated with SVFPs in Fontan patients. Instead, global atrial reservoir function appears to parallel pulmonary vascular resistance.
Background: Systemic light chain (AL) amyloidosis is considered a rare disease, but knowledge of ... more Background: Systemic light chain (AL) amyloidosis is considered a rare disease, but knowledge of its exact incidence and prevalence is based on old data involving limited populations. Aim: To determine the incidence and prevalence of AL amyloidosis in the general population. Patients and Methods: The national reference center for AL amyloidosis is located in the CHU de Limoges in Limoges (France), the administrative center of the Limousin region (738,110 inhabitants in 2014). A comprehensive and exhaustive database of AL amyloidosis patients diagnosed in this region has been computerized since January 2007. All patients living in the Limousin region and with a first diagnosis of systemic AL amyloidosis between January 1, 2012, and December 31, 2016, were retrospectively included to determine the disease incidence. All departments and laboratories of pathology were contacted in the region, and any possible missing cases diagnosed during the same period were collected. Prevalence was determined as all living patients with AL amyloidosis during 2016 regardless of the diagnosis date. Cardiac involvement was defined according to current definition (ie, mean wall thickness >12 mm in the absence of other causes of left ventricular hypertrophy). Renal involvement was defined as proteinuria >0.5 g/L (predominantly albumin) in the absence of other etiology. Results: Over the 5-year period, 46 new patients in the Limousin region had a confirmed diagnosis of AL amyloidosis (70% men; median age, 72.5 years), corresponding to a crude yearly incidence of 12.5 (95% CI, 5.6-19.4) cases per million inhabitants. The calculated prevalence of AL amyloidosis was 58 (95% CI, 43-73) cases per million inhabitants. Cardiac and renal involvement was found in 70% and 72% of cases, respectively. Mayo Clinic stages I, II, IIIA (NT-proBNP ≤8500 ng/L), and IIIB (NT-proBNP >8500 ng/L) based on cardiac biomarkers were found in 21%, 29%, 29%, and 21% of patients respectively. The underlying disease was multiple myeloma in 19 patients (41%), including smoldering myeloma in 14 patients, MGUS (monoclonal gammopathy of undetermined significance) in 22 patients (48%), IgM in 2 patients, Waldenstrom disease in 1 patient, low-grade lymphoma in 3 patients, plasmocytoma in 1 patient, and unknown in 1 patient. In this 5 year-period, 16 patients (35%) died. Conclusion: This is the firstreport of contemporary incidence and prevalence of systemic AL amyloidosis in France. Incidence was slightly higher than the incidence (adjusted to sex and age) previously reported by Kyle et al (Blood, 1992;7:1817-1822), probably corresponding to the progress in the past 20 years in diagnosing this rare disease. Disclosures Mohty: Prothena Biosciences: Honoraria. Jaccard: Amgen: Honoraria; Celgene: Honoraria, Other: Travel expenses, Research Funding; Janssen: Honoraria, Membership on an entity9s Board of Directors or advisory committees, Other: Travel expenses, Research Funding.
European Journal of Preventive Cardiology, Nov 6, 2014
Data on peripheral artery disease in Africa are sparse and limited to urban areas. Given the urba... more Data on peripheral artery disease in Africa are sparse and limited to urban areas. Given the urban/rural socio-economical gradient in these countries, we sought to determine the prevalence and risk factors of peripheral artery disease in urban and rural areas of two countries in Central Africa. Individuals ≥65 years old living in two urban and rural areas of the Republic of Central Africa (ROC) and the Central African Republic (CAR) were invited. Demographic, clinical and biological data were collected. Ankle-brachial index ≤0.90 defined peripheral artery disease. Among the 1871 participants (age 73 years, 62% female) the prevalence of peripheral artery disease was 14.8%, higher in ROC than in CAR (17.4% vs. 12.2%, p = 0.007) and higher in females than males (16.6% vs. 11.9%, p = 0.012). The prevalence of peripheral artery disease increased with age, respectively at 10.9%, 14.9%, 15.1% and 22.2% for age bands of 65-69, 70-74, 75-79 and 80+years (p < 0.001). Higher rates of peripheral artery disease were found in urban areas in ROC (20.7% vs. 14.4% in rural areas, p = 0.011), but not in CAR (11.5% vs. 12.9%, p = NS). In multivariate analysis, peripheral artery disease was significantly associated with age (odds ratio (OR): 1.03; p = 0.004), dyslipidaemia (OR: 1.88; p = 0.003), smoking (OR: 1.78; p = 0.003), obesity (OR: 1.98; p = 0.034) and underweight (OR: 1.49; p = 0.023). Regular alcohol drinking was associated with decreased risk of peripheral artery disease (OR: 0.73; p = 0.044). The prevalence of peripheral artery disease in the elderly is high in Africa, especially in females. In ROC, with a higher urban-rural socio-economic gradient, peripheral artery disease is more frequent in the urban areas.
Background: Systemic light chain (AL) amyloidosis is considered a rare disease, but knowledge of ... more Background: Systemic light chain (AL) amyloidosis is considered a rare disease, but knowledge of its exact incidence and prevalence is based on old data involving limited populations. Aim: To determine the incidence and prevalence of AL amyloidosis in the general population. Patients and Methods: The national reference center for AL amyloidosis is located in the CHU de Limoges in Limoges (France), the administrative center of the Limousin region (738,110 inhabitants in 2014). A comprehensive and exhaustive database of AL amyloidosis patients diagnosed in this region has been computerized since January 2007. All patients living in the Limousin region and with a first diagnosis of systemic AL amyloidosis between January 1, 2012, and December 31, 2016, were retrospectively included to determine the disease incidence. All departments and laboratories of pathology were contacted in the region, and any possible missing cases diagnosed during the same period were collected. Prevalence was ...
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