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Congenital Heart Defects in Patients with Classic Bladder Exstrophy: A Hitherto Neglected Association?
Martin Promm1, Stephan Gerling2, Carsten P Schepp3
1Department of Pediatric Urology, Clinic St. Hedwig, University Medical Center of Regensburg, Regensburg, Germany.
Insights
Congenital heart defects (CHDs) are more common in bladder exstrophy (BE) patients than previously thought. Early echocardiography is recommended for safe patient care, even without observed clinical differences.
Area of Science:
- Pediatric Surgery
- Cardiology
- Medical Genetics
Background:
- Bladder exstrophy (BE) is typically considered an isolated defect.
- Emerging evidence suggests a higher prevalence of cardiac anomalies in BE patients.
- This study investigates the incidence and clinical significance of congenital heart defects (CHDs) in BE.
Purpose of the Study:
- To determine the prevalence of CHDs in patients with bladder exstrophy.
- To assess the clinical relevance of CHDs in the peri- and postoperative management of BE patients.
- To evaluate the utility of transthoracic echocardiography (TTE) in this population.
Main Methods:
- Prospective cohort study of 39 BE patients undergoing primary closure between March 2012 and January 2019.
- Transthoracic echocardiography (TTE) performed on 37 patients prior to surgery.
- Assessment of demographic, perioperative, and postoperative data.
Main Results:
- Congenital heart defects (CHDs) were detected in 7 out of 39 (18.9%) BE patients.
- No significant clinical differences in peri- or postoperative outcomes were observed between patients with and without CHDs.
- Median age at delayed primary bladder closure was 61 days.
Conclusions:
- The prevalence of CHDs in bladder exstrophy (BE) is higher than previously recognized.
- While not impacting immediate outcomes in this cohort, TTE is crucial for comprehensive patient management.
- Systematic echocardiographic screening is recommended for safe short- and long-term care of BE patients with CHDs.
Introduction:
Classic bladder exstrophy (BE) is regarded as an isolated malformation without any further anomalies, but some studies have indicated a higher incidence of cardiac anomalies. This cross-sectional study is planned to evaluate the prevalence of congenital heart defects (CHDs) and the clinical relevance for patients with BE admitted for primary closure.
Materials And Methods:
Patients were prospectively recruited between March 2012 and January 2019. Patients' profiles including demographic data, results of transthoracic echocardiography (TTE), as well as essential peri- and postoperative data were assessed.
Results:
Thirty-nine (25 boys and 14 girls) patients with BE (median age 61 days) underwent delayed primary bladder closure. Thirty-seven (24 boys and 13 girls) patients had received TTE 1 day before surgery. CHD was detected in 7 (18.9%) out of the 39 patients, but no clinical differences between patients with and without CHD were observed peri- or postoperatively.
Discussion And Conclusion:
This prospective systematic evaluation shows an even higher rate of CHD in patients with BE than assumed previously. Although peri- and postoperative outcome did not differ between patients with and without CHD, we consider TTE an important additional method for ensuring a safe peri- and postoperative courses and a short- and long-term care for patients with CHD.
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