Outcomes of Intracorporeal Continuous and Paracorporeal Pulsatile Ventricular Assist Devices in Pediatric Patients 10-30 kg.

Ventricular assist devices (VADs) have been increasingly implanted in pediatric patients. Paracorporeal VADs are generally chosen when intracorporeal continuous (IC) devices are too large. Superiority between IC and paracorporeal pulsatile (PP) devices remains unclear in smaller pediatric patients. Our study analyzes outcomes of IC and PP VADs in pediatric patients who could be considered for either of these options. Using the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) database, we identified children between 10 and 30 kg who received a VAD between June 2018 and September 2021. Survival and stroke outcomes were analyzed based on VAD type. There were 41 patients in the IC group and 54 patients in the PP group. Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) profile at the time of implant was higher in the PP cohort (p < 0.02). The PP cohort was younger (p < 0.001) and smaller (p < 0.001) than the IC cohort. The diagnosis was similar between cohorts. Overall survival was similar between groups. Stroke was more common in the PP cohort, but did not reach statistical significance (p = 0.07). Discharge was possible only in the IC group, but the discharge rate was low (9.5%). Direct comparisons remain challenging given differences in INTERMACS profiles, age, and size.

Outcomes after initial heart failure consultation in Fontan patients.

BACKGROUND: Patients with Fontan failure are high-risk candidates for heart transplantation and other advanced therapies. Understanding the outcomes following initial heart failure consultation can help define appropriate timing of referral for advanced heart failure care. METHODS: This is a survey study of heart failure providers seeing any Fontan patient for initial heart failure care. Part 1 of the survey captured data on clinical characteristics at the time of heart failure consultation, and Part 2, completed 30 days later, captured outcomes (death, transplant evaluation outcome, and other interventions). Patients were classified as "too late" (death or declined for transplant due to being too sick) and/or "care escalation" (ventricular assist device implanted, inotrope initiated, and/or listed for transplant), within 30 days. "Late referral" was defined as those referred too late and/or had care escalation. RESULTS: Between 7/2020 and 7/2022, 77 Fontan patients (52% inpatient) had an initial heart failure consultation. Ten per cent were referred too late (6 were too sick for heart transplantation with one subsequent death, and two others died without heart transplantation evaluation, within 30 days), and 36% had care escalation (21 listed ± 5 ventricular assist device implanted ± 6 inotrope initiated). Overall, 42% were late referrals. Heart failure consultation < 1 year after Fontan surgery was strongly associated with late referral (OR 6.2, 95% CI 1.8-21.5, p=0.004). CONCLUSIONS: Over 40% of Fontan patients seen for an initial heart failure consultation were late referrals, with 10% dying or being declined for transplant within a month of consultation. Earlier referral, particularly for those with heart failure soon after Fontan surgery, should be encouraged.

End-of-Life in Pediatric Patients Supported by Ventricular Assist Devices: A Network Database Cohort Study.

OBJECTIVES: Most pediatric patients on ventricular assist device (VAD) survive to transplantation. Approximately 15% will die on VAD support, and the circumstances at the end-of-life are not well understood. We, therefore, sought to characterize patient location and invasive interventions used at the time of death. DESIGN: Retrospective database study of a cohort meeting inclusion criteria. SETTING: Thirty-six centers participating in the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) Registry. PATIENTS: Children who died on VAD therapy in the period March 2012 to September 2021. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Of the 117 of 721 patients (16%) who died on VAD, the median (interquartile range) age was 5 years (1-16 yr) at 43 days (17-91 d) postimplant. Initial goals of therapy were bridge to consideration for candidacy for transplantation in 60 of 117 (51%), bridge to transplantation in 44 of 117 (38%), bridge to recovery 11 of 117 (9%), or destination therapy (i.e., VAD as the endpoint) in two of 117 (2%). The most common cause of death was multiple organ failure in 35 of 117 (30%), followed by infection in 12 of 117 (10%). Eighty-five of 92 (92%) died with a functioning device in place. Most patients were receiving invasive interventions (mechanical ventilation, vasoactive infusions, etc.) at the end of life. Twelve patients (10%) died at home. CONCLUSIONS: One-in-six pediatric VAD patients die while receiving device support, with death occurring soon after implant and usually from noncardiac causes. Aggressive interventions are common at the end-of-life. The ACTION Registry data should inform future practices to promote informed patient/family and clinician decision-making to hopefully reduce suffering at the end-of-life.

Initial multicenter experience with ventricular assist devices in children and young adults with muscular dystrophy: An ACTION registry analysis.

PURPOSE: Cardiac disease results in significant morbidity and mortality in patients with muscular dystrophy (MD). Single centers have reported their ventricular assist device (VAD) experience in specific MDs and in limited numbers. This study sought to describe the outcomes associated with VAD therapy in an unselected population across multiple centers. METHODS: We examined outcomes of patients with MD and dilated cardiomyopathy implanted with a VAD at Advanced Cardiac Therapies Improving Outcomes Network (ACTION) centers from 9/2012 to 9/2020. RESULTS: A total of 19 VADs were implanted in 18 patients across 12 sites. The majority of patients had dystrophinopathy (66%) and the median age at implant was 17.2 years (range 11.7-29.5). Eleven patients were non-ambulatory (61%) and 6 (33%) were on respiratory support pre-VAD. Five (28%) patients were implanted as a bridge to transplant, 4 of whom survived to transplant. Of 13 patients implanted as bridge to decision or destination therapy, 77% were alive at 1 year and 69% at 2 years. The overall frequencies of positive outcome (transplanted or alive on device) at 1 year and 2 years were 84% and 78%, respectively. Two patients suffered a stroke, 2 developed sepsis, 1 required tracheostomy, and 1 experienced severe right heart failure requiring right-sided VAD. CONCLUSIONS: This study demonstrates the potential utility of VAD therapies in patients with muscular dystrophy. Further research is needed to further improve outcomes and better determine which patients may benefit most from VAD therapy in terms of survival and quality of life.

Decreased Risk of Strokes in Children with Ventricular Assist Devices Within ACTION.

We sought to characterize strokes in children with ventricular assist devices. Of 407 patients in the ACTION registry (4/1/18-5/3/2021), 45 (11%) experienced 52 strokes (45 ischemic and 7 hemorrhagic). Median time to stroke was 23.5 days and 19/52 (37%) occurred ≤ 10 days. Stroke rate was 0.09 and 0.63 strokes per patient-year for implantable continuous and paracorporeal devices, respectively. Patients with stroke were younger, more likely to have congenital heart disease and have been on extracorporeal membrane oxygenation at time of VAD. Based on these data, ACTION is now focused on decreasing strokes in these higher-risk patients with particular attention to the peri-implant period.