4. Database of dextrocardiac patients in KFSH & RC: Retrospective analysis of 360 patients over the past 20 years

Abstract

Clinical research. Presentation Type: Oral Presentation Introduction: Dextrocardia is the right-sided positioning of the human heart during embryological development. It may be isolated or associated with visceral dextroposition or malformations. It is usually discovered during infancy because of associated anomalies or incidentally during adulthood. There is limited information on the associated malformations and their clinical outcomes. Methodology: This is a retrospective study that describes patients with dextrocardia at King KFSH & RC. A total of 250,000 echocardiograms since 1990 were reviewed for demographics, associated anomalies, clinical outcomes, and mortality. Results: Three hundred and sixty patients were identified with dextrocardia. Males slightly outnumbered females with a ratio of 193 (53%) to 167 (47%). The majority of subjects (71%) were children and teenagers (2–18 years). Isolated dextrocardia was the most commonly presented type (48%) followed by situs inversus totalis (43%), situs ambiguous (7%), and situs inversus totalis variant with polysplenia or asplenia (2%), respectively. Cyanotic heart defects were present in more than half of the subjects (54%), while acyanotic heart defects accounted for 25%. Non-cardiac anomalies included Scimitar syndrome (8%), polysplenia (7%), and asplenia (4%). Kaplan Meier survival analysis showed highest death rates among patients with cyanotic heart defects in the first 5 years of life (Figure 1). The overall crude mortality rate is 61 per 1000 patients. Conclusion: The most common associated anomalies with dextrocardia are cyanotic heart defects, which also account for the majority of deaths in dextrocardiac patients. Scimitar syndrome was reported to be the most common non-cardiac associated anomaly in this population of interest.