Practice variability in management of infectious issues in heterotaxy: A survey of pediatric cardiologists.

BACKGROUND: Splenic dysfunction is common in heterotaxy syndrome, and increases the risk of bacteremia and bacteremia related mortality. Despite the risks associated with bacteremia in this setting, best practice guidelines for management of infectious concerns are lacking. We conducted a survey of pediatric cardiologists to characterize practice regarding the diagnosis of splenic dysfunction, approach to antibiotic prophylaxis, and management of possible bacterial infection.

METHODS: A 22-item web-based survey was distributed via email to pediatric cardiologists in North America.

RESULTS: We received 230 responses from 63 centers, for a response rate of 22%. The majority (83%) always obtain abdominal ultrasound to define splenic anatomy in the neonate with heterotaxy. Despite a normal ultrasound result, 43% perform additional splenic functional testing. In addition, 21% report prescribing antibiotic prophylaxis regardless of testing results. There was wide variability in timing of stopping of prophylaxis, with 36% responding "never" and 24% "not sure." Those with more years in practice were more likely to obtain functional testing, to indefinitely continue antibiotic prophylaxis once started, and to recommend the 23-valent pneumococcal vaccination.

CONCLUSION: In a survey of North American cardiologists, significant variability exists in the management of infectious issues in heterotaxy syndrome. The development of practice guidelines for diagnosis of splenic dysfunction, indications for and duration of antibiotic prophylaxis, and management of possible bacterial illness may lead to improved outcomes in this complex patient population.