Key result
Among children with Ebstein's anomaly, cyanosis (64.6%) and exertional dyspnea (56.3%) were the most frequent symptoms, while arrhythmias (60.4%) and heart failure (60.4%) were the most common clinical features.
Why the study?
Clinical features and echocardiographic findings among children presenting with Ebstein's anomaly needed to be determined.
Observational (n=48)
No
In a Pakistani cohort of children with Ebstein's anomaly, the majority presented late with severe symptoms including cyanosis, arrhythmias, and heart failure, highlighting the need for earlier diagnosis and neonatal screening.
Describes pediatric EA features in one center; hypothesis-generating and should not yet change practice.
Objective: To determine the clinical features and echocardiographic findings among children presenting with Ebstein’s anomaly (EA). Study Design: Descriptive Case-series. Setting: Department of Pediatric Cardiology, National Institute of Cardiovascular Diseases (NICVD), Karachi Pakistan. Period: July 2021 to June 2022. Material & Methods: Children of both genders aged above 1 month up to 18 years, having echocardiographic confirmed EA were analyzed. Demographic data along with clinical features, presenting symptoms and echocardiographic findings were noted in all case. Results: In a total of 48 cases of EA, 38 (79.2%) were female. The mean age was 8.4±4.21 years. Cyanosis, exertional dyspnea, easy fatigue ability and exercise intolerance were the most frequent symptoms reported in 31 (64.6%), 27 (56.3%), 26 (54.2%) and 24 (50.0%) patients respectively. Arrhythmias and heart failure were the most frequent clinical features noted in 29 (60.4%) patients each. Small atrial septal defect (ASD) was noted in 27 (56.3%) patients while ventricular septal defect in 19 (39.6%) cases. There were 11 (22.9%) patients who presented timely with diagnosis of EA while 37 (77.1%) patients had late diagnosis. Conclusion: Majority of the patients with Ebstein’s anomaly were female. Cyanosis, exertional dyspnea, easy fatigue ability and exercise intolerance were the most frequent symptoms whereas arrhythmias and heart failure were the most frequent clinical features observed. ASD was the most commonly noted associated CHDs observed in almost half of the Ebstein’s anomaly case. Almost 3/4th of the patients had late diagnosis.
No takes yet. Share an insight, caveat, or question.
Jokio et al. (2023) conducted an observational in Ebstein's anomaly (n=48). Ebstein's anomaly was evaluated on Clinical features and echocardiographic findings. Among children with Ebstein's anomaly, cyanosis (64.6%) and exertional dyspnea (56.3%) were the most frequent symptoms, while arrhythmias (60.4%) and heart failure (60.4%) were the most common clinical features.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: