Associations between pediatric choledochal cysts, biliary atresia, and congenital cardiac anomalies

Background: In our institutional experience treating pediatric choledochal cysts over the past 12 y, we noted that seven of 32 patients (21.9%) had comorbid congenital cardiac anomalies. This association has not been previously described other than in isolated case reports. We aimed to quantify this association on a national level.

Materials and methods: We queried the 2009 Healthcare Cost and Utilization Project Kids' Inpatient Database. We identified patients with a diagnosis of choledochal cyst (ICD-9-CM 75169, 75162, and 75160) or biliary atresia (75161). We defined cardiac anomalies using the Clinical Classification Software code (CCS 213). Comorbid choledochal cysts or biliary atresia and congenital cardiac anomalies were quantified in both infant (<12 mo) and child (1-18 y) subpopulations.

Results: Of 1646 estimated discharges for patients with choledochal cysts, 506 (30.7%) were for patients who also had congenital cardiac anomalies, compared with 2.6% in the general hospitalized population (χ(2); P < 0.001). The frequency of congenital cardiac anomalies was lower in 1973 hospitalizations for biliary atresia (13.8%) than in those for patients with choledochal cysts (χ(2); P < 0.001). We detected cardiac anomalies in 44.9% of choledochal cyst hospitalizations for infants <12 mo (versus 3.44% general hospitalized population; χ(2); P < 0.001), but in 6.9% of children ages 1-18 y (versus 1.3% general hospitalized population; χ(2); P < 0.001).

Conclusions: We observed a strong association between pediatric choledochal cysts and congenital cardiac anomalies that commonly manifests in infancy. When choledochal cysts are diagnosed either prenatally or in infancy, we suggest echocardiographic screening for cardiac anomalies, which may affect the timing of surgery and anesthetic planning.