A subset of newborns with the congenital heart condition pulmonary atresia with intact ventricular septum (PA-IVS) are at risk of myocardial infarction (MI) and sudden death because of abnormal coronary circulation. To avoid this, such patients may undergo single ventricle palliation and ultimately the Fontan procedure.
Data are lacking, however, on the survivors’ risk of long-term myocardial ischemia. Adam Small, MD, Therese Giglia, MD, and colleagues performed a retrospective review of CHOP patients who underwent the Fontan procedure for PA-IVS between 1985 and 2013. There were 46 patients who met inclusion criteria for the study, with median follow-up of 3.5 years (range 0-27.9y) after the Fontan. There were two early deaths after Fontan. Five patients had evidence of late ischemia: 2 had non-fatal MI at 16.9 and 18.8 years post-Fontan and three additional patients had evidence of ischemia on stress test and/or cardiac MRI without clinical evidence of MI. No patient had traditional risk factors for atherosclerotic coronary disease, and there were no late deaths or sustained ventricular tachycardia. Patients with PA-IVS and single-ventricle palliation remain at risk for long-term myocardial ischemia. Further study is needed to define risk factors for ischemic outcomes.
Therese Giglia, Adam Small et al, Late Myocardial Infarction After Fontan in Pulmonary Atresia with Intact Ventricular Septum
Contact: Joey McCool Ryan, The Children’s Hospital of Philadelphia, 267-426-6070 or [email protected] (link sends e-mail)