Congenital Heart Defects in Patients with Classic Bladder Exstrophy: A Hitherto Neglected Association?

Abstract

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.

Keywords

bladder exstrophy - congenital heart defects - primary closure

Publication History

Received: 03 August 2020

Accepted: 14 December 2020

Article published online:
07 March 2021

• References

• 1 Grady RW, Mitchell ME. Complete primary repair of exstrophy. J Urol 1999; 162 (04) 1415-1420
• 2 Jayachandran D, Bythell M, Platt MW, Rankin J. Register based study of bladder exstrophy-epispadias complex: prevalence, associated anomalies, prenatal diagnosis and survival. J Urol 2011; 186 (05) 2056-2060
• 3 Ebert AK, Reutter H, Jenetzky E. et al. First results of the German CURE-Network in newborns with the exstrophy-epidpadias complex. J Pediatr Urol 2012; S6: 1
• 4 Franklin RCG, Béland MJ, Colan SD. et al. Nomenclature for congenital and paediatric cardiac disease: the International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Iteration of the International Classification of Diseases (ICD-11). Cardiol Young 2017; 27 (10) 1872-1938
• 5 Ebert AK, Zwink N, Jenetzky E. et al. Association between exstrophy-epispadias complex and congenital anomalies: a German multicenter study. Urology 2019; 123: 210-220
• 6 Reutter H, Boyadjiev SA, Gambhir L. et al. Phenotype severity in the bladder exstrophy-epispadias complex: analysis of genetic and nongenetic contributing factors in 441 families from North America and Europe. J Pediatr 2011; 159 (05) 825-831
• 7 Cervellione RM, Mantovani A, Gearhart J. et al. Prospective study on the incidence of bladder/cloacal exstrophy and epispadias in Europe. J Pediatr Urol 2015; 11 (06) 337.e1-337.e6
• 8 Lindinger A, Schwedler G, Hense HW. Prevalence of congenital heart defects in newborns in Germany: results of the first registration year of the PAN Study (July 2006 to June 2007). Klin Padiatr 2010; 222 (05) 321-326
• 9 Ramamoorthy C, Haberkern CM, Bhananker SM. et al. Anesthesia-related cardiac arrest in children with heart disease: data from the Pediatric Perioperative Cardiac Arrest (POCA) Registry. Anesth Analg 2010; 110 (05) 1376-1382
• 10 Christensen RE, Lee AC, Gowen MS, Rettiganti MR, Deshpande JK, Morray JP. Pediatric Perioperative Cardiac Arrest, Death in the Off Hours: a report from Wake Up Safe, The Pediatric Quality Improvement Initiative. Anesth Analg 2018; 127 (02) 472-477
• 11 Jacobson Z, Glickstein J, Hensle T, Marion RW. Further delineation of the Opitz G/BBB syndrome: report of an infant with complex congenital heart disease and bladder exstrophy, and review of the literature. Am J Med Genet 1998; 78 (03) 294-299
• 12 Tulloh RM, Tansey SP, Parashar K, De Giovanni JV, Wright JG, Silove ED. Echocardiographic screening in neonates undergoing surgery for selected gastrointestinal malformations. Arch Dis Child Fetal Neonatal Ed 1994; 70 (03) F206-F208
• 13 Lindinger A, Kozlik-Feldmann R, Cesnjevar R. Leitlinie Pädiatrische Kardiologie: Persistierender Ductus arteriosus. DEUTSCHE GESELLSCHAFT FÜR PÄDIATRISCHE KARDIOLOGIE UND ANGEBORENE HERZFEHLER e.V. Leitlinien. 2015 . Available at: www.dgpk.org
• 14 Gorenflo M, Schirmer KR, Dewald O. Leitlinie Pädiatrische Kardiologie: Der isolierte Ventrikelseptumdefekt. DEUTSCHE GESELLSCHAFT FÜR PÄDIATRISCHE KARDIOLOGIE UND ANGEBORENE HERZFEHLER e.V. Leitlinien 2019. Available at: www.dgpk.org
• 15 Jux C, Vogel M, Scheewe J. Leitlinie Pädiatrische Kardiologie: Vorhofseptumdefekt (ASD). DEUTSCHE GESELLSCHAFT FÜR PÄDIATRISCHE KARDIOLOGIE UND ANGEBORENE HERZFEHLER e.V. Leitlinien. 2010 . Available at: www.dgpk.org
• 16 Bravo-Valenzuela NJ, Peixoto AB, Araujo Júnior E. Prenatal diagnosis of congenital heart disease: a review of current knowledge. Indian Heart J 2018; 70 (01) 150-164
• 17 Goldstein I, Shalev E, Nisman D. The dilemma of prenatal diagnosis of bladder exstrophy: a case report and a review of the literature. Ultrasound Obstet Gynecol 2001; 17 (04) 357-359
• 18 Bang SR. Neonatal anesthesia: how we manage our most vulnerable patients. Korean J Anesthesiol 2015; 68 (05) 434-441