The field of heart transplantation continues to evolve, with major changes in allocation systems and the increasing use of extended-criteria donor hearts, including hearts from donation after circulatory death donors, hearts supported with ex-vivo perfusion, and hearts from hepatitis C viremic donors. The use of such non-traditional donor hearts has made transplantation available to a larger number of recipients, but the demand continues to outpace the supply. Given this persistent donor heart shortage, much attention has been paid to the topic of donor-recipient size matching. Prior studies have challenged traditional criteria for size matching, 1-3 while others have attempted to identify the optimal metric for matching donor and recipient heart size by comparing criteria, such as height, weight, body mass index (BMI), body surface area (BSA), and predicted heart mass (PHM). [4][5][6][7] This 36th annual adult heart transplant report is based on data submitted to the International Society for Heart and Lung Transplantation (ISHLT) Thoracic Organ Transplant Registry on 146,975 heart transplants in recipients of all ages (including 131,249 adult heart transplants)
This 36 th adult lung and heart-lung transplant report summarizes data from 69,200 adult lung and 4,128 adult heart-lung transplants performed through June 30, 2018 and reported to the International Thoracic Organ Transplant Registry. With each year's report, we now provide more detailed analyses on a particular focus theme important to patient outcomes. Since 2013, these have been donor and recipient age; retransplantation; early graft failure; indication for transplant; allograft ischemic time; and multiorgan transplantation. Although widely accepted as critical to decision making at the time of receipt of an organ donor offer, there is surprisingly little literature outlining current practice and the impact of size (mis-)matching on outcomes. Hence, this year's report focuses on an overall theme of donor and recipient size matching. In addition to reporting donor and recipient height and weight difference for all adult lung and heart-lung transplant recipients stratified by transplant type (bilateral or single) and indication, we report historical trends and associations between size match and survival. The Registry's online slide sets include results from additional analyses and complementary information not included in this publication (see https:// ishltregistries.org/registries/slides.asp).
BACKGROUND
Peripartum cardiomyopathy shares some clinical features with idiopathic dilated cardiomyopathy, a disorder caused by mutations in more than 40 genes, including TTN, which encodes the sarcomere protein titin.
METHODS
In 172 women with peripartum cardiomyopathy, we sequenced 43 genes with variants that have been associated with dilated cardiomyopathy. We compared the prevalence of different variant types (nonsense, frameshift, and splicing) in these women with the prevalence of such variants in persons with dilated cardiomyopathy and with population controls.
RESULTS
We identified 26 distinct, rare truncating variants in eight genes among women with peripartum cardiomyopathy. The prevalence of truncating variants (26 in 172 [15%]) was significantly higher than that in a reference population of 60,706 persons (4.7%, P = 1.3×10−7) but was similar to that in a cohort of patients with dilated cardiomyopathy (55 of 332 patients [17%], P = 0.81). Two thirds of identified truncating variants were in TTN, as seen in 10% of the patients and in 1.4% of the reference population (P = 2.7×10−10); almost all TTN variants were located in the titin A-band. Seven of the TTN truncating variants were previously reported in patients with idiopathic dilated cardiomyopathy. In a clinically well-characterized cohort of 83 women with peripartum cardiomyopathy, the presence of TTN truncating variants was significantly correlated with a lower ejection fraction at 1-year follow-up (P = 0.005).
CONCLUSIONS
The distribution of truncating variants in a large series of women with peripartum cardiomyopathy was remarkably similar to that found in patients with idiopathic dilated cardiomyopathy. TTN truncating variants were the most prevalent genetic predisposition in each disorder.
This continues to be an exciting time for pediatric heart transplantation. Since the first orthotopic heart transplantation a little over 50 years ago, the field has grown tremendously, including many advancements in the application of this therapy to children. Today, heart transplantation is performed routinely in many pediatric centers throughout the world with over 100 centers reporting to the International Thoracic Transplant Registry (Figure 1, eSlide H(p) 4). The Registry is the largest source of worldwide data on pediatric heart transplantation with over 14,000 transplants reported in children. The focus theme for this year's report is donor and recipient size match. Statistical methods Data collection, conventions, and statistical methods National and multinational organ and data exchange organizations and individual centers submit data to the International Society for Heart and Lung Transplantation (ISHLT)
BACKGROUND
Peripartum cardiomyopathy (PPCM) remains a major cause of maternal morbidity and mortality.
OBJECTIVES
This study sought to prospectively evaluate recovery of the left ventricular ejection fraction (LVEF) and clinical outcomes in the multicenter IPAC (Investigations of Pregnancy Associated Cardiomyopathy) study.
METHODS
We enrolled and followed 100 women with PPCM through 1 year post-partum. The LVEF was assessed by echocardiography at baseline and at 2, 6, and 12 months post-partum. Survival free from major cardiovascular events (death, transplantation, or left ventricular [LV] assist device) was determined. Predictors of outcome, particularly race, parameters of LV dysfunction (LVEF), and remodeling (left ventricular end-diastolic diameter [LVEDD]) at presentation, were assessed by univariate and multivariate analyses.
RESULTS
The cohort was 30% black, 65% white, 5% other; the mean patient age was 30 ± 6 years; and 88% were receiving beta-blockers and 81% angiotensin-converting enzyme inhibitors or angiotensin receptor blockers. The LVEF at study entry was 0.35 ± 0.10, 0.51 ± 0.11 at 6 months, and 0.53 ± 0.10 at 12 months. By 1 year, 13% had experienced major events or had persistent severe cardiomyopathy with an LVEF <0.35, and 72% achieved an LVEF ≥0.50. An initial LVEF <0.30 (p = 0.001), an LVEDD ≥6.0 cm (p < 0.001), black race (p = 0.001), and presentation after 6 weeks postpartum (p = 0.02) were associated with a lower LVEF at 12 months. No subjects with both a baseline LVEF <0.30 and an LVEDD ≥6.0 cm recovered by 1 year post-partum, whereas 91% with both a baseline LVEF ≥0.30 and an LVEDD <6.0 cm recovered (p < 0.00001).
CONCLUSIONS
In a prospective cohort with PPCM, most women recovered; however, 13% had major events or persistent severe cardiomyopathy. Black women had more LV dysfunction at presentation and at 6 and 12 months post-partum. Severe LV dysfunction and greater remodeling at study entry were associated with less recovery. (Investigations of Pregnancy Associated Cardiomyopathy [IPAC]; NCT01085955)
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