Objective: Surgery for heart defects in children with trisomy 13 or 18 is controversial. We analyzed our 20-year experience. Methods: Since 2002, we performed 21 operations in 19 children with trisomy 13 (n = 8) or trisomy 18 (n = 11). Age at operation was 4 days to 12 years (median, 154 days). Principal diagnosis was ventricular septal defect in 10 patients, tetralogy of Fallot in 7 patients, arch hypoplasia in 1 patient, and patent ductus arteriosus in 1 patient. Results: The initial operation was ventricular septal defect closure in 9 patients, tetralogy of Fallot repair in 7 patients, pulmonary artery banding in 1 patient, patent ductus arteriosus ligation in 1 patient, and aortic arch/coarctation repair in 1 patient. There were no operative or hospital deaths. Median postoperative intensive care and hospital stays were 189 hours (interquartile range, 70-548) and 14 days (interquartile range, 8.0-37.0), respectively, compared with median hospital stays in our center for ventricular septal defect repair of 4.0 days and tetralogy of Fallot repair of 5.0 days. On median follow-up of 17.4 months (interquartile range, 6.0-68), 1 patient was lost to follow-up after 5 months. Two patients had reoperation without mortality. There have been 5 late deaths (4 with trisomy 18, 1 with trisomy 13) predominately due to respiratory failure from 4 months to 9.4 years postoperatively. Five-year survival was 66.6% compared with 24% in a group of unoperated patients with trisomy 13 or 18. Conclusions: Cardiac operation with an emphasis on complete repair can be performed safely in carefully selected children with trisomy 13 or trisomy 18. Hospital resource use measured by postoperative intensive care and hospital stays is considerably greater compared with nontrisomy 13 and 18.
Coronary artery fistulas (CAFs) are exceedingly rare.1 They are defined as an abnormal communication between a coronary artery and another cardiac structure or major thoracic vessel. While largely presumed to be asymptomatic, a recent study showed that a majority of neonates/infants presented with heart failure–type symptoms, albeit many of them had a larger fistula size.2 Indications for intervention on CAFs have been controversial because many patients are asymptomatic; however, delaying intervention is associated with a significantly greater risk of death preoperatively and an increased risk of morbidity and mortality postoperatively.3 Therefore, the majority of CAFs are addressed when they are identified. Transcatheter and surgical approaches have been both been used with good success rates; however, the number of neonates undergoing repair by either method remains low.2,4 Here, we present a case of surgical repair of a large CAF in a neonate after an attempted transcatheter closure resulted in iatrogenic tricuspid regurgitation (TR). The institutional review board of Children's Healthcare of Atlanta approved the study protocol and publication of data (approval number: STUDY00001462, date approved: July 18, 2022). Patient written consent for the publication of the study data was waived by the institutional review board, as it was determined to be research not involving human subjects.