Cause, timing, and location of death in the Single Ventricle Reconstruction trial

Abstract

Objectives The Single Ventricle Reconstruction trial randomized 555 subjects with a single right ventricle undergoing the Norwood procedure at 15 North American centers to receive either a modified Blalock-Taussig shunt or right ventricle-to-pulmonary artery shunt. Results demonstrated a rate of death or cardiac transplantation by 12 months postrandomization of 36% for the modified Blalock-Taussig shunt and 26% for the right ventricle-to-pulmonary artery shunt, consistent with other publications. Despite this high mortality rate, little is known about the circumstances surrounding these deaths. Methods There were 164 deaths within 12 months postrandomization. A committee adjudicated all deaths for cause and recorded the timing, location, and other factors for each event. Results The most common cause of death was cardiovascular (42%), followed by unknown cause (24%) and multisystem organ failure (7%). The median age at death for subjects dying during the 12 months was 1.6 months (interquartile range, 0.6 to 3.7 months), with the highest number of deaths occurring during hospitalization related to the Norwood procedure. The most common location of death was at a Single Ventricle Reconstruction trial hospital (74%), followed by home (13%). There were 29 sudden, unexpected deaths (18%), although in retrospect, 12 were preceded by a prodrome. Conclusions In infants with a single right ventricle undergoing staged repair, the majority of deaths within 12 months of the procedure are due to cardiovascular causes, occur in a hospital, and within the first few months of life. Increased understanding of the circumstances surrounding the deaths of these single ventricle patients may reduce the high mortality rate. (J Thorac Cardiovasc Surg 2012;144:907-14)

Risk Factors for Death or Heart Transplant for Patients with Right Ventricular Dysfunction After the Norwood Procedure: A Secondary Analysis of the Single Ventricle Reconstruction Trial

“…Finally, six (4 %) patients died from the stage II hospital discharge and the 12-month follow-up. Evaluating the composite outcome of death and heart transplant, 144 patients (26 % of the 555 enrolled infants) died or received a heart transplant between the randomization and the stage II surgery (Ohye et al 2012). Out of the survivors, there were 393 (71 %) infants who received a stage II procedure, which was associated with 17 (4 %) further deaths and three (1 %) heart transplants (Schwartz et al 2014).…”

Infant deaths in the UK community following successful cardiac surgery: building the evidence base for optimal surveillance, a mixed-methods study

“…26,37 Only three studies 29,36,37 included a prospective element. Although study designs differed, all studies were rated as good quality ( Table 1); studies that were assigned a lower rating failed to address some potential confounding factors.…”

“…Although study designs differed, all studies were rated as good quality ( Table 1); studies that were assigned a lower rating failed to address some potential confounding factors. Ten reports were of patients with a functional single-ventricle (SV) diagnosis, 23,24,[26][27][28]30,33,[35][36][37] which was most often HLHS. Fourteen papers involved patients who underwent cardiac surgery during the first year of life and the remaining study 31 included cardiac patients operated up to the age of 18 years with results provided separately for each age group.…”

“…Two further studies focused on 'unexpected' interstage deaths, defined as acute events or sudden cardiovascular collapse, and reported 4-5% unexpected deaths in neonates discharged home between stages 1 and 2. 28,37 Carlo et al 24 and Hansen et al 27 reported interstage mortality of 9% for children discharged home between second (superior cavopulmonary anastomosis or bidirectional Glenn) and third stage (Fontan) surgery. Two studies investigated postdischarge mortality after all types of cardiac surgery: Chang et al 31 reported a low mortality rate of 0.62% at 1 year after any cardiac surgery undertaken in children up to age 18 years, whereas Pinto et al 29 found a higher mortality rate of 8% in the 2 years following discharge after neonatal congenital heart surgery.…”

“…Children undergoing more complex operations, based on the Risk Adjustment in Congenital Heart Surgery (RACHS) -1 system, 9,38 were at greater risk. 25,29,34 Several studies reported that Norwood procedures, 31 specific shunt operations, 23,26,31,37 total anomalous pulmonary venous connection (TAPVC) repair 31 and truncus arteriosus repair 31 were associated with significantly higher mortality, whereas palliative operations 34 increased the risk of unplanned readmission. There was insufficient evidence to suggest that intraoperative characteristics, such as cardiopulmonary bypass or circulatory arrest time, had a negative impact on outcome.…”