Long-Term Outcome of LVAD in Duchenne Population with End Stage Cardiomyopathy

Abstracts (PGD) grade at 72 hours were Grade 0-1, n = 6; Grade 2, n = 2; Grade 3, n = 0. Estimated LW gain during the entire perfusion period significantly correlated with LW gain at the back table (n = 10, r = 0.95, p < 0.01, Fig. 1A). Furthermore, initial LW gain (0-60 min) had a significant correlation with back table LW gain, compared with LW gain after 60 min of EVLP (n = 10, r = 0.79, p < 0.01, Fig. 1B). Non-suitable group had significantly higher initial LW gain compared to suitable group (120 § 30 vs -17 § 56 g, p = 0.02, Fig. 1C). Unfavorable outcome group (defined as non-suitable or PGD grade 2-3) group had significantly higher initial LW gain compared to favorable outcome group (defined as PGD grade 0-1, 75 § 56 vs -32 § 58 g, p = 0.02, Fig. 1D). Conclusion: The data suggest that LW gain calculated by real-time LW measurement might be useful to assess pulmonary edema during EVLP. Initial LW gain at 0-60 min of EVLP might be a predictor of transplant suitability and clinical outcomes.

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(525) Impact of Mechanical Circulatory Support on Pediatric Heart Transplant Candidates with Elevated Pulmonary Vascular Resistance K. Thangappan,1 D.L. Morales,1 D. Lehenbauer,1 C. Villa,2 A. Lorts,2 S. Wittekind,2 and F. Zafar.1 1Cardiovascular Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH; and the 2Cardiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.

(524) Post-Transplant Outcomes of Novel Surgical Procedures to Deal with Graft Size Mismatch in Living-Donor Lobar Lung Transplantation D. Nakajima, Y. Yutaka, M. Hamaji, S. Tanaka, Y. Yamada, A. Ohsumi and H. Date. Thoracic Surgery, Kyoto University, Kyoto, Japan. Purpose: Various living-donor lobar lung transplant (LDLLT) procedures have been developed in order to resolve the serious issue of graft size mismatch. The purpose of this study was to compare the outcomes of newly developed transplant techniques with those of the standard LDLLT approach. Methods: In Kyoto University, the procedures of “native upper lobe-sparing transplant” and “right-left inverted transplant” have been employed as strategies for managing undersized grafts, and single lobar transplant has been used to manage oversized grafts. Between June 2008 and May 2018, we performed 47 standard LDLLT procedures (Standard group), 22 native upper lobe-sparing transplants and/or right-left inverted transplants for undersized grafts (Under group), and 11 single lobar transplants for oversized grafts (Over group). Results: The LDLLT patients showed great early post-transplant outcomes: the ECMO requirement rate was 8.8%, 30-day mortality was 2.5%, and hospital mortality was 5.0%. The pulmonary function at 1 year after LDLLT was similar among the three groups: %FVC of 59%§22% in the Standard group, 63%§19% in the Under group and 61%§21% in the Over group (P = 0.81), and %FEV1 of 59%§20% in the Standard group, 62%§ 15% in the Under group and 58%§20% in the Over group (P = 0.84). The LDLLT patients had a favorable exercise capacity at 1 year after transplant, as demonstrated by the 6-minute walking distance (Standard: 474§ 132 m; Under: 525§114 m; Over: 399§81 m, P = 0.08). The incidence of CLAD per graft did not differ markedly among the groups (Standard: 17.4%; Under: 14.3%; Over: 20%, P = 0.87). The overall 5-year survival rate of LDLLT was 79%, and each group showed an excellent 5-year survival rate (Standard: 77%; Under: 74%; Over: 91%, P = 0.67). Conclusion: These novel LDLLT procedures may have resolved the critical size-matching problems between living-donor lobar lungs and recipients, demonstrating favorable post-transplant outcomes.

Purpose: With the new era of more effective medicines and increasing use of mechanical circulatory support (MCS) in children, seemingly more patients with elevated pulmonary vascular resistance (PVR) are having positive outcomes. The purpose of this study is to define the effect of MCS on pediatric patients listed for heart transplant with an elevated PVR. Methods: The United Network for Organ Sharing (UNOS) database was used to identify patients ages 0-18 at time of listing for heart transplant between 2010 and 2019 who had PVR documented. Patients were divided into MCS (LVAD, RVAD, BiVAD, and TAH) and NoMCS groups, then divided by indexed PVR (PVRi) at time of listing: <3, 3-6, and >6 Wood units. Positive waitlist outcome was defined as reaching transplant or delisted because of clinical improvement. Negative waitlist outcome was defined as death or delisted because too sick to transplant. Results: 2081 pediatric patients were listed for heart transplant between 2010 and 2019. MCS was used in 20% overall (n=426); 57% of those with PVRi <3, 27% with PVRi 3-6, and 16% with PVRi >6. Median MCS duration was 68 days (IQR 30-142). In all three PVRi groups, MCS patients had a significantly higher incidence of positive waitlist outcome compared to No-MCS patients (Table 1). Within the No-MCS group, patients with a PVRi >6 had a higher incidence of negative waitlist outcome compared to PVRi <3 (17 vs 10%, p=0.002). This was not true in the MCS group (5 vs 6%, p=0.693). No significant differences in post-transplant survival were found between the subgroups. Conclusion: Patients supported with MCS had a significantly higher chance of a positive waitlist outcome than those without such support regardless of PVR status. With better waitlist survival and equivalent posttransplant survival compared to No-MCS patients, MCS patients had better overall survival from listing regardless of PVR.

(526) Long-Term Outcome of LVAD in Duchenne Population with End Stage Cardiomyopathy R. Adorisio,1 N. Cantarutti,1 A. D'Amico,1 E. Bertini,1 M. Catteruccia,1 G. Perri,1 D. D'Amario,2 S. Filippelli,1 F. Drago,1 and A. Amodeo.1 1Bambino Gesu Hospital, Rome, Italy; and the 2Fondazione Policlinico Universitario A. Gemelli, Rome, Italy. Purpose: The aim of this study was to report the effect of long-term use of VAD in patients affected by Duchenne Muscular Distrophy and end stage cardiomyopathy.

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The Journal of Heart and Lung Transplantation, Vol 39, No 4S, April 2020

Methods: We collected data of DMD patients with end-stage cardiomyopathy implanted with LVAD in our center (group 1) and the ones in regular follow-up not eligible for LVAD implantation (group 2). Each patient of group 1 underwent extensive pre-operative assessment, in-hospital treatment and was enrolled in a dedicated DMD HF (heart failure) clinic during the post-discharge period. Patients of group 2 were followed in a dedicated DMD HF clinic or as inpatient in case of need of inotropic support. During long-term follow-up all data were recorded including echocardiography, ECG examination, laboratory blood tests, and respiratory function. Survival was represented by Kaplan Meier analysis and compared by log rank. Results: A total of 8 DMD patients (group 1) were implanted with LVAD and treated at our center during the period from 2011 until 2018 (mean age at implantation was 16.9 § 2.9 years)¸c whilst 4 patients (group 2) were not eligible for LVAD implantation. At the time of the last follow-up, 4 out of 8 patients were alive and 2 out of 8 patients did not have post-operative complications. The principal causes of 3 deaths at the median follow-up of 22 months were sepsis, tracheal bleeding, and cerebral hemorrhage. After 1 year from implantation we noticed a significant decrease in heart rate (p=0.002), in LV volumes and diameters (LVEDD p=0.03, LVESD p=0.02, EDV p=0.01 and ESV p=0.02) and significant increase in ejection fraction (p=0.0036). However, relative wall thickness did not change over time, showing an eccentric remodeling pattern before and after LVADs. Analysis on short and long-term follow-up showed significant improvement of survival in DMD patients treated with LVAD compared to the group that was not implanted (log rank<0.001), with a 5-year increase in life expectancy. Conclusion: Our data showed that cardiac atrophy is persistent in Duchenne cardiomyopathy despite the improvement of EF due to a significant ventricular unloading coupled with chronic therapy. LVAD improved outcomes in patients with LVAD when compared to those without. An integration of outpatient DMD HF clinic and patient-centered palliative care will most likely increase the quality of life and delivery of care for this population. (527) Preparing for Discharge in Pediatric Ventricular Assist Device Supported Patients B. Elias,1 H.P. Tunuguntla,2 L. Smyth,3 P. Krack,3 K. Fields,3 M. McQueen,4 M.M. Brickler,5 M. Mehegan,6 and J. Conway.7 1Congenital Heart Surgery, Texas Children's Hospital, Houston, TX; 2Pediatric Cardiology, Texas Children's Hospital, Houston, TX; 3Cincinnati Children's Hospital Medical Center, Cincinnati, OH; 4 Transplant Families, Phoenix, AZ; 5Children's Hospital of Wisconsin, Milwaukee, WI; 6St. Louis Children's Hospital, St. Louis, MO; and the 7 Stollery Children's Hospital, Edmonton, AB, Canada. Purpose: While the use of durable ventricular assist device (VAD) support with potential for discharge in pediatric patients has increased over time, there is limited knowledge of individual centers’ approach to preparation and education for patients and families around discharge. Methods: The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) was surveyed in September 2018 to determine current discharge practices in preparation for a quality improvement project. Results: Surveys were sent to 28 sites, with 22 sites responding (response rate 78.5%). The majority of programs (86.4%, 18/22) reported having a standardized discharge protocol. Discharge teaching was performed in a majority of centers by a VAD coordinator (95.5%). Frequently utilized practices in preparation for discharge included field trips with and without medical chaperones, assessment of home environment (without an actual visit), rooming-in process, establishment of an EMS plan, and education about using a shower bag. The three main topics that patient and families received education on was: dressing change teaching, VAD equipment teaching and emergency planning. In terms of community outreach, programs offered education to EMS (72.7%), school staff (63.6%) and local fire stations (50%). In addition, 50% notified the local utility company in case of an electricity outage. Conclusion: The majority of programs caring for pediatric patients on durable VADs have a standardized approach to discharge with extensive education of patients, families, and community providers. As most programs discharge a small number of patients, a collaborative effort can allow for the development of shared educational materials that best address the needs of patients and their families.

(528) Percutaneous Impella Device for Mechanical Circulatory Support in Children with Cardiogenic Shock: Long Term Outcomes S.C. Tume,1 M. Anders,1 W. Dreyer,2 I. Adachi,3 H. Justino,2 and A. Qureshi.2 1Critical Care Medicine, Baylor College of Medicine, Houston, TX; 2Pediatric Cardiology, Baylor College of Medicine, Houston, TX; and the 3Congenital Heart Surgery, Baylor College of Medicine, Houston, TX. Purpose: Use of temporary mechanical circulatory support (MCS) is an alternative to increase systemic blood flow avoiding the possible cardiotoxicity and long-term morbidity of inotropes and vasopressors. It is often the only option to achieve hemodynamic stability. Use of temporary MCS is increasing in frequency in children with evolution of new technologies. Recently, catheter deployed micro-axial percutaneous ventricular assist device (PC-VAD) has been approved for management of cardiogenic shock in adults. We present our experience and long-term outcomes associated with use of this device in a pediatric cardiac intensive care unit at a freestanding children’s hospital. Methods: All patients treated for cardiogenic shock with PC-VAD alone between September 2014 and June 2019 were included in retrospective analysis. The device sizes used included CP device which provides up to 4.0 l/min of flow and 5.0 device which provides 5.0 l/min of flow. Demographic and support data were reviewed and reported using descriptive statistics. Results: A total of 24 PC-VAD devices were implanted in 18 patients: CP (n=17), and 5.0 (n=7). Median patient age was 17 years (IQR 15,20), weight of 61 kg (IQR 54,75), and BSA of 1.72 m2(IQR 1.58,1.96). Median length of CP support was 5 days (IQR 4,7) and 5.0 was 12 days (IQR 11,22). The most common device related complications included site bleeding 9/24 (38%), ventricular arrhythmias 3/24 (17%) and extremity arterial thrombus 1/24 (4%), with no limb ischemia encountered. Acute renal failure requiring CRRT occurred in 5/ 18 (38%) patients. None of the patients sustained neurologic injury. Median ICU length of stay was 24 days (IQR 15,45) with median hospital length of stay of 35 days (26,80). The survival to ICU discharge was 89% (16/18) and survival to hospital discharge was 89% (16/18). Twelve out of eighteen (67%) patients were alive at one year. Conclusion: Temporary MCS for cardiogenic shock with PC-VAD shows favorable short and long-term outcomes in select pediatric patients. This type of circulatory support can be considered for management of older children with acute cardiogenic shock to achieve quicker hemodynamic stability, end-organ recovery and as a temporary bridge to recovery or clinical decision making. The risk profile in our population was low and only minor complications occurred during the treatment. (529) US Trends in Pediatric VAD Utilization - Where are We Now? H. Ahmed, J. Dykes, A. Power, E. Profita, C. Chen, J. Murray and C.S. Almond. Stanford University School of Medicine, Palo Alto, CA. Purpose: Pediatric ventricular assist device (VAD) therapy is a rapidly changing field where devices and applications are rapidly evolving. We sought to describe some of the most recent trends in pediatric VAD use and contemporary outcomes since UNOS began collecting VAD data on all listed patients regardless of whether they survived. Methods: All children ≤21 years of age listed for isolated HT between Jan 2004 and 2019 and underwent VAD placement were identified using OPTN data. Descriptive statistics were used to characterize recent trends in VAD utilization. Waitlist survival in children with cardiomyopathy, CHD and singleventricle heart disease were estimated using the Kaplan-Meier method. Results: Overall, 2059 children met the study inclusion criteria of whom the median age was 11 (IQR 1, 17), median weight 38 (11,66) kg, 26% were black; 18.3 had congenital heart disease (CHD) including 5.1% with single-ventricle (SV) HD. Forty-nine percent were supported with a VAD at listing, 72% at transplant and 13% received a VAD but was not supported at listing or transplant. Overall, 64% received LVADs, 31% BIVADs, 2.5% RVADs 1.7% total artificial hearts (TAH). Implants of the Berlin Heart fell dipped sharply to 34 in 2015 after a peak of 63 but have rebounded since in the era of bivalirudin. As of 2018, the Heartware HVAD was the most commonly implanted VAD in children (N=61) followed by the Berlin Heart (N=48), CentriMag/PediMag (N=18) and