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.
Abstract

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