Causes-Mechanisms of Death Following Stage I Repair for Hypoplastic Left Heart Syndrome (HLHS)
PB Baker, A Hughes. Nationwide Childrens Hospital, Colunbus, OH; The Ohio State University, Columbus, OH
Background: Staged hybrid repair is a recent advancement in treatment of HLHS. Stage I (of 3 stages) includes balloon atrial septostomy and ductus arteriosus stenting (by catheterization) as well as surgical pulmonary artery banding. The causes-mechanisms of death between the stage I and II procedures have not been well-documented at autopsy.
Design: Autopsy reports and microscopic slides were reviewed from 13 consecutive patients (August 2002 - August 2009) who died after first stage repair for HLHS (6 males and 7 females). Causes-mechanisms of death as well as nonfatal complications were recorded.
Results: The mean age at death was 60 days (range 9 to 180 days). Six deaths occurred at < 30 days following stage I repair (group 1) and 7 deaths occurred at > 30 days (group 2). Eleven patients had the complete stage I repair, one had only atrial septostomy and one had only ductus arteriosus stenting. Autopsy permit included no restrictions (n=7), chest and abdomen only (n=2) or chest only (n=4). The causes-mechanisms of death in group 1 were ductus arteriosus closure (n=1, patient had only atrial septostomy), left atrial appendage tear (n=1, occurred during atrial septostomy), arrhythmias that developed during the procedure (n=3) and pneumonia (n=1). Two of the patients with arrhythmias had a myocardial substrate that may have predisposed to arrhythmia (fibrosis and myocyte disarray). The causes-mechanisms of death in group 2 were intestinal necrosis (n=1), myocardial necrosis due to insufficient retrograde flow into the aortic arch (n=1), pneumonia (n=2), pulmonary emboli (n=1), pulmonary vein stenosis (n=1) and sudden death without documented arrhythmia (n=1). Aorta and/or vena cava thrombosis or a significant thromboembolic event were found in four group 2 patients.
Conclusions: Intraprocedural events (arrhythmia, left atrial tear) accounted for 67% of deaths in group 1 patients. Pneumonia and ischemic necrosis (heart, intestines) accounted for the majority of deaths (57%) in group 2. Thrombotic / thromboembolic events found in 57% of group 2 patients were not observed in group 1. Autopsy results demonstrated differences in causes-mechanisms of death and rates of thrombotic / thromboembolic events in patients who died at < 30 days and > 30 days following stage I repair for HLHS.
Wednesday, March 24, 2010 1:00 PM
Poster Session VI # 38, Wednesday Afternoon