Comments Ebstein's Anomaly(EA)is a rare congenital heart disease(CHD)with an incidence of approximately 1 in 20,000.The pathognomonic feature involves apical displacement of the septal and posterior leaflets,resul...Comments Ebstein's Anomaly(EA)is a rare congenital heart disease(CHD)with an incidence of approximately 1 in 20,000.The pathognomonic feature involves apical displacement of the septal and posterior leaflets,resulting in valvular insufficiency and right ventricular(RV)remodeling.Untreated patients exhibit a cumulative mortality rate of up to 25% within the first decade,with heart failure(HF)and arrhythmias constituting the predominant causes of death.Current guidelines suggest that asymptomatic patients with accessory pathways may benefit from prophylactic ablation,though robust evidence specific to EA remains limited[1-3].展开更多
Background:Patients with unrepaired Ebstein’s anomaly experience exercise intolerance,heart failure and premature mortality.Volumetric assessment of right ventricular function is difffcult due to the complex anatomy ...Background:Patients with unrepaired Ebstein’s anomaly experience exercise intolerance,heart failure and premature mortality.Volumetric assessment of right ventricular function is difffcult due to the complex anatomy of the right ventricle and tricuspid valve.Myocardial deformation indices are an early marker in other cardiac pathologies of ventricular dysfunction.Objectives:1.Assess myocardial deformation in unrepaired Ebstein’s compared to healthy controls.2.Investigate the relationships between myocardial deformation and exercise capacity.Methods:Myocardial deformation parameters(strain)were calculated using feature tracking from standard cardiac magnetic resonance cine images.Cardiopulmonary exercise results were included where available.Results:36 patients with unrepaired Ebstein’s and 36 matched controls were included.Right ventricular,right atrial,and left ventricular global longitudinal,as well as left ventricular circumferential strain were impaired in Ebstein’s patients compared to controls(p<0.05).In Ebstein’s patients right atrial peak strain correlated with their percentage predicted VO_(2) max(r=−0.448,p=0.022)and VE/VCO_(2) slope(r=0.435,p=0.026).There were no correlations between right ventricular ejection fraction and exercise parameters.When Ebstein’s patients were divided by severity into mild or severe according to the median total right/left index,those with severe demonstrated signiffcantly impaired right ventricular global longitudinal strain compared to those in the mild category(−17.5±5.4%vs.−21.4±4.4%,p=0.0017).Conclusions:Myocardial deformation parameters for both the right and left ventricle are impaired in patients with unrepaired Ebstein’s compared to healthy controls.Right atrial peak strain is related to impaired exercise capacity and warrants further investigation as an early prognostic marker in this patient cohort.展开更多
基金funded by E Fund Congenital Heart Disease Medical Talent Cultivation and Education Fund,grant number 2023QT0009the Science and Technology Planning Project of Guangdong Province,grant number 2023B03J1255.
文摘Comments Ebstein's Anomaly(EA)is a rare congenital heart disease(CHD)with an incidence of approximately 1 in 20,000.The pathognomonic feature involves apical displacement of the septal and posterior leaflets,resulting in valvular insufficiency and right ventricular(RV)remodeling.Untreated patients exhibit a cumulative mortality rate of up to 25% within the first decade,with heart failure(HF)and arrhythmias constituting the predominant causes of death.Current guidelines suggest that asymptomatic patients with accessory pathways may benefit from prophylactic ablation,though robust evidence specific to EA remains limited[1-3].
基金This observational retrospective study was approved by the local Clinical Governance Committee(RRK6237)conformed to the principles of Good Clinical Practice guidelines.Additionally,healthy controls were identiffed from a national ethics board approved observational CMR study(NCT01028703).
文摘Background:Patients with unrepaired Ebstein’s anomaly experience exercise intolerance,heart failure and premature mortality.Volumetric assessment of right ventricular function is difffcult due to the complex anatomy of the right ventricle and tricuspid valve.Myocardial deformation indices are an early marker in other cardiac pathologies of ventricular dysfunction.Objectives:1.Assess myocardial deformation in unrepaired Ebstein’s compared to healthy controls.2.Investigate the relationships between myocardial deformation and exercise capacity.Methods:Myocardial deformation parameters(strain)were calculated using feature tracking from standard cardiac magnetic resonance cine images.Cardiopulmonary exercise results were included where available.Results:36 patients with unrepaired Ebstein’s and 36 matched controls were included.Right ventricular,right atrial,and left ventricular global longitudinal,as well as left ventricular circumferential strain were impaired in Ebstein’s patients compared to controls(p<0.05).In Ebstein’s patients right atrial peak strain correlated with their percentage predicted VO_(2) max(r=−0.448,p=0.022)and VE/VCO_(2) slope(r=0.435,p=0.026).There were no correlations between right ventricular ejection fraction and exercise parameters.When Ebstein’s patients were divided by severity into mild or severe according to the median total right/left index,those with severe demonstrated signiffcantly impaired right ventricular global longitudinal strain compared to those in the mild category(−17.5±5.4%vs.−21.4±4.4%,p=0.0017).Conclusions:Myocardial deformation parameters for both the right and left ventricle are impaired in patients with unrepaired Ebstein’s compared to healthy controls.Right atrial peak strain is related to impaired exercise capacity and warrants further investigation as an early prognostic marker in this patient cohort.
