Interstage Survival for Patients with Hypoplastic Left Heart Syndrome After ECMO

Abstract

There is a reported 5-20 % incidence of extracorporeal membrane oxygenation (ECMO) following stage I palliation for hypoplastic left heart syndrome (HLHS). This study compares the interstage mortality of HLHS patients supported with ECMO (HLHS-ECMO) to those who were not supported with ECMO (HLHS-nECMO) using the National Pediatric Cardiology Quality Improvement Initiative database. Patients with HLHS who survived to hospital discharge after stage I palliation were analyzed. HLHS-ECMO patients were compared to HLHS-non-ECMO patients with respect to demographics, surgical variables, and interstage survival. A total of 931 patients were identified in the database. Sixty-six (7.1 %) patients were supported with ECMO during their stage I palliation admission. There were no statistically significant differences between the groups with respect to demographics or anatomic subtype. HLHS-ECMO patients were more likely to have a preoperative risk factor identified (62 vs. 48 %, p = 0.03) or require ECMO prior to stage I palliation (3 vs. 0.5 %, p = 0.03). HLHS-ECMO patients had a significantly higher incidence of death or transplant versus the HLHS-nECMO group (18 vs. 9 %, p = 0.03). Despite survival to discharge, patients with HLHS requiring ECMO after their palliation continue to have an increased risk of death/cardiac transplant versus patients that do not require ECMO. ECMO use is likely a marker for a high-risk patient group. These patients may benefit from closer follow-up during the interstage period.

Neonatal Cardiac ECMO in 2019 and Beyond

“…Although incidence is low, mortality is very high (69). Neonates who survive to discharge following ECMO post-Norwood have been shown to have an increased risk of death or cardiac transplant, when compared to patients who did not receive ECMO post-Norwood (45, 61, 63, 70).…”

Improved outcomes in neonates who require venoarterial extracorporeal membrane oxygenation after the Norwood procedure

“…Survival to hospital discharge for the 29 neonates and infants in which VA-ECMO was employed after their Norwood procedure at our institution over the past 9 years was 72%, notably higher than the previously published literature. 2,5,6,8 Furthermore, though the survival rate of children placed on VA-ECMO was still significantly lower than the 92% survival rate for children who were not placed on VA-ECMO, the gap between the two populations has narrowed. Debrunner and colleagues previously reported the outcomes of children who underwent the Norwood operation at our institution during the 15-year time period (1994-2008) preceding this study, where survival in patients who were and were not placed on VA-ECMO postoperatively was 31% and 79%, respectively.…”

“…1,2,[5][6][7] Intermediate-term survival has also been shown to be negatively impacted by the use of postoperative VA-ECMO, with an increased rate of attrition following discharge when compared to neonates who recovered from the Norwood procedure without VA-ECMO. 3,4,8 Risk factors that directly influence operative mortality following the Norwood procedure have been described including, but not limited to, gestational age, birth weight, genetic or chromosomal anomalies, and extracardiac abnormalities. 3,6,[8][9][10][11] Analysis of the landmark Single Ventricle Reconstruction (SVR) Trial also identified delayed sternal closure and perioperative VA-ECMO support as independent risk factors for hospital mortality.…”

“…3,4,8 Risk factors that directly influence operative mortality following the Norwood procedure have been described including, but not limited to, gestational age, birth weight, genetic or chromosomal anomalies, and extracardiac abnormalities. 3,6,[8][9][10][11] Analysis of the landmark Single Ventricle Reconstruction (SVR) Trial also identified delayed sternal closure and perioperative VA-ECMO support as independent risk factors for hospital mortality. 3 Though many studies have reported outcomes of patients who have received VA-ECMO after the Norwood procedure, most contain patients who underwent surgery prior to the current decade.…”

Mechanical Circulatory Support Following Norwood Palliation

“…Attempts have also been made to "unload" the ventricle with salvage bidirectional Glenn palliation, but rarely has this approach been successful enough to avoid transplantation. Overall poor outcomes 2,3 following these approaches have led to a generally pessimistic view of ECMO and any other measures to rescue the failing single-ventricle patient with a shunt-dependent circulation. In a recent review of data from the investigational device exemption trial of the Berlin Heart EXCOR device (the only circulatory support device other than ECMO currently approved for use in children), only 1 out of 9 infants with single-ventricle physiology and shuntdependent pulmonary blood flow survived to transplantation.…”