History Of Heart Transplantation Research Articles

Effect of body mass index on exercise capacity following pediatric heart transplantation.

Obesity and impaired exercise tolerance following heart transplantation increase the risk of post-transplant morbidity and mortality. The aim of this study was to evaluate the effect of body mass index on markers of exercise capacity in pediatric heart transplant recipients and compare this effect with a healthy pediatric cohort. A retrospective analysis of cardiopulmonary exercise test data between 2004 and 2022 was performed. All patients exercised on a treadmill using the Bruce protocol. Inclusion criteria included patients aged 6-21 years, history of heart transplantation (transplant cohort) or no cardiac diagnosis (control cohort) at the time of testing, and a maximal effort test. Patients were further stratified within these two cohorts as underweight, normal, overweight, and obese based on body mass index groups. Two-way analyses of variance were performed with diagnosis and body mass index category as the independent variables. A total of 250 exercise tests following heart transplant and 1963 exercise tests of healthy patients were included. Heart transplant patients across all body mass index groups had higher resting heart rate and lower maximal heart rate, heart rate recovery at 1 min, exercise duration, and peak aerobic capacity (VO2peak). Heart transplant patients in the normal and overweight body mass index categories had higher VO2peak and exercise duration when compared to underweight and obese patients. Underweight status and obesity are strongly associated with lower VO2peak and exercise duration in heart transplant patients. Normal and overweight heart transplant patients had the best markers of exercise capacity.

PO-05-024 LEADLESS CARDIAC PACEMAKER IMPLANTATION IN HEART TRANSPLANT RECIPIENTS: SAFETY AND OUTCOMES

Leadless cardiac pacemakers (LCP) appear to have lower risk of device infection and bacteremia compared to transvenous systems. Heart transplant patients are at increased risk for cardiovascular implantable electronic device complications, but there is limited data on the safety and effectiveness of LCPs in these patients; this is especially true when repeat myocardial biopsies are necessary. To report outcomes and device performance of LCPs in heart transplant recipients, some of whom also underwent endomyocardial biopsies Retrospective analysis of all heart transplant recipients at a single institution who received a leadless pacemaker, either tine-affixed (Micra – Medtronic Inc.) or helix- affixed (Aveir – Abbott Inc.), between April 2018 thru September 2022. Data on demographics, clinical characteristics, pacemaker interrogations, device changes, endomyocardial biopsies, and cardiac allograft vasculopathy screening was collected. Pacemaker interrogations were performed and data collected as the patients were followed. Twelve heart transplant patients underwent LCP implantation (tine-affixed: 11 patients; helix-affixed: 1 patient): mean age was 59.5±12.5 years, gender 8 males/4 females. The indication for LCP implantation was sinus node disease (n=2) or high-degree AV block (n=10). The mean follow-up time was 473±518 days. A total of 12 endomyocardial (mean 1±1.3) biopsies were performed in 6 patients (see Swimmer plot figure). Pacemaker interrogation showed no clinically significant differences in pacing thresholds, sensing or impedance over time, and following biopsies. Two patients underwent upgrade to CRT-P during follow-up. Leadless cardiac pacemakers are a viable treatment option for symptomatic bradycardia in patients after heart transplant. Endomyocardial biopsies do not appear to affect LCP function. Further research is needed to confirm the safety and efficacy of leadless pacemakers in patients with history of heart transplantation.

PSUN249 Starvation Ketoacidosis as the Presenting Finding of Glycogen Storage Disorder Type III

Abstract A 30-year woman with a history of heart transplantation and a history of post-transplant diabetes mellitus presents with severe euglycemic ketoacidosis. Initial concern was for diabetes related ketoacidosis given history of diabetes however blood glucose was within goal range in the absence of pregnancy and use of sodium glucose transporter type 2 inhibitor use. Initial laboratory evaluation showed a serum glucose of 177 mg/dL, a serum beta hydroxybutyrate of greater than 12.66 mmol/L, serum lactate of 0.6 mmol/L, with a calculated anion gap of greater than 30 mmol/L. C-peptide levels were repeatedly normal and appropriate for blood glucose level ranging between 2.8 ng/mL and 7.5 ng/mL ruling out insulin deficiency as causative. It was discovered that the patient had intentionally fasted for greater than 18 hours and thus had not used the premixed insulin regimen prescribed. Metabolic disturbance corrected with a dextrose and insulin infusion. Evaluation for inborn error of metabolism showed: acylcarnitine panel returned normal making an inborn error of fatty acid metabolism unlikely. The mitochondrial DNA mutation panel was negative for point mutations or deletions. Further testing for urinary organic acids did not reveal elevation of 3-hydroxybutyric acid suggesting SCOT enzyme levels were sufficient in our patient. Review of pathology records for heart transplantation completed for idiopathic cardiomyopathy 20 years prior to this presentation showed high levels of glycogen deposition prompting evaluation for glycogen storage disorders. Results revealed a heterozygous abnormality at exon 9 of the AGL gene; AGL gene c.1139A>G (p.Glu380Gly). This is not a reported pathologic mutation, and given the heterozygosity of the mutation in this autosomal recessive condition, it is felt that either environmental factors or the presence of other unrecognized genetic mutations allowed for the phenotypic expression of GSDIII with euglycemic starvation ketoacidosis. The patient was treated with dietary counseling regarding ketone suppression with basal insulin during periods of fasting and preference to avoid periods of prolonged fasting with frequent carbohydrate-containing meals. Conclusion This case represents a novel, potentially pathologic defect in the AGL gene. This finding should prompt further investigations into genetic variabilities that may lead to this phenotypic expression. Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m.

Breakthrough COVID-19 Infections in Heart Transplant Recipients: A Case Series

PurposeHeart transplant (HT) recipients are at high risk for Covid-19 infection, and data are limited about the efficacy of vaccination in this unique population. We sought to describe the presentation and outcomes of a cohort of HT patients with Covid-19 infection despite prior vaccination.MethodsRetrospective chart review of 250 adult HT recipients followed at the University of Southern California identified 7 individuals with PCR-proven Covid-19 infection after full vaccination (no patients received booster doses) between December 1st, 2020 and October 1st, 2021. Baseline clinical characteristics, serial echocardiographic parameters, laboratory testing, medication regimens, clinical presentation and clinical course were collected.ResultsA total of 237 were vaccinated with an incidence of 7 breakthrough infections (3%). Patients were predominantly male (71%) with a median age of 50 years old. The average BMI was 32.89. Hypertension (86%), diabetes (57%), and hyperlipidemia (43%) were common. Nearly all (71%) of patients were on 3 classes of immunosuppressive therapy, and nearly half (43%) had a history of rejection. Five patients (72%) received Pfizer-BioNTech, 1 patient (14%) received Moderna, and 1 patient (14%) received the Johnson & Johnson vaccination. One patient had a prior history of Covid-19 infection before vaccine availability. Patients were on average 6.74 (3.8-8.4) years out from transplantation. The most common presentation was dyspnea (71%), cough (57%), and fever (43%). Seventy one percent were hospitalized, and 29% were admitted to the ICU. Treatments varied, with equal rates of antibiotics (29%), steroids (29%), and remdesevir (29%). However the most common treatment was monoclonal antibody therapy (57%). One patient, vaccinated with a single Johnson & Johnson shot, died lending an 86% survival rate for breakthrough infections after Covid-19 vaccination.ConclusionIn a single center experience 7 patients with a history of heart transplant and breakthrough Covid-19 infection were identified and found to have an 86% survival rate. Further investigation is needed assessing the efficacy of the Covid-19 vaccination in this population, as well as evaluation for differential outcomes between the various vaccine options.

National Trends and Outcomes of Surgical Aortic Valve Replacement With Concomitant Mitral Valve Surgery

BackgroundIncidence of multivalvular heart disease is increasing, with aortic stenosis and mitral regurgitation being the most common. Data are limited on outcomes of patients undergoing multivalvular surgery. The purpose of this study was to evaluate contemporary trends and in-hospital outcomes for combined surgical aortic valve replacement (SAVR) and mitral valve repair (MVr) or replacement (MVR). MethodsWe identified patient hospitalizations aged ≥18 years who underwent SAVR + MVr or MVR between 2004 and 2018 using the National Inpatient Sample. Data were weighted to estimate national estimates of the entire US hospitalized population. Exclusion criteria included endocarditis, history of heart transplant or left ventricular assist device, and any other concomitant valve surgery. ResultsBetween January 1, 2004, and December 31, 2018, there were 68,882 weighted admissions for SAVR with concomitant mitral valve surgery. Overall, in-hospital mortality was 8.34% with significantly higher inpatient mortality in SAVR + MVR group compared with SAVR + MVr group (9.91% vs 5.57%, p < 0.001). During the study period, adjusted in-hospital mortality decreased in both SAVR + MVr group (p-trend 0.004) and SAVR + MVR group (p-trend <0.001). Age ≥70 years was associated with higher in-hospital mortality compared to those < 70 years (9.95% vs 6.70%, p < 0.001). ConclusionCombined aortic and mitral valve surgery is associated with a high risk of in-hospital mortality, especially in patients ≥ 70 years of age. Further research is needed to assess the role of transcatheter approaches in the treatment of multivalvular heart disease.

Abstract 14426: Comparing Performance of MARKER-HF, a Machine Learning Derived Risk Score, and the Seattle Heart Failure Model in Patients With Heart Failure in a Large, Integrated Health System

Introduction: Patients with heart failure (HF) who advance from Stage C to Stage D experience progressively higher morbidity and mortality rates. Cardiovascular societies recommend using 1-year mortality risk (≥20%) as a trigger for evaluation by a HF specialist. Application of available risk scores to identify patients at increased risk using electronic health records (EHR) is challenging. Consequently, we compared the performance of MARKER-HF, a machine-learning risk model that uses readily available structured data in the EHR, to the Seattle Heart Failure Model (SHFM), which requires elements derived from both structured and unstructured data, for estimating 1-year mortality risk. Methods: We analyzed an EHR database for ambulatory patients with HF seen in primary care and cardiology clinics from 1/1/10-12/31/19 at Northwestern Medicine. Eligibility criteria included ages ≥18 years old, at least 1-year of follow-up or death within 1-year, no history of heart transplantation or left ventricular assist device implantation, and availability of the 8 variables used to calculate MARKER-HF (diastolic blood pressure, creatinine, blood urea nitrogen, hemoglobin, white blood cell count, platelets, albumin, and red blood cell distribution width). Unavailable or missing variables required for SHFM were imputed. In all HF patients and in those with reduced ejection fraction (HFrEF), we evaluated model discrimination for 1-year mortality using area under the curve (AUC) and compared AUCs with the DeLong test. Results: In a sample of 7130 patients, of whom 1012 died within 1-year, 46.9% were female and median age was 71 (IQR 60-82). The AUC for MARKER-HF and SHFM were 0.69 and 0.64, respectively, for all HF patients and 0.69 and 0.67, respectively, for patients with HFrEF (n=1899). The AUCs for MARKER-HF and SHFM were different for the entire HF population (DeLong p-value=0.01) but not in the HFrEF sub-group. Conclusion: In patients with ambulatory HF at an integrated health system, MARKER-HF produced 1-year mortality estimates with equal (HFrEF) or higher (all HF) discrimination than SFHM using fewer and more readily available variables. These findings suggest that MARKER-HF may facilitate the identification of patients with advanced HF using EHR data.

Evaluation of Quality of Care for US Veterans With Recent-Onset Heart Failure With Reduced Ejection Fraction

Multiple guideline-recommended therapies for heart failure with reduced ejection fraction (HFrEF) are available and promoted by performance measures. However, contemporary data on the use of these therapies are limited. To evaluate trends in guideline-directed medical therapy, implantable cardioverter-defibrillator (ICD) use, and risk-adjusted mortality among patients with recent-onset HFrEF. This cohort study analyzed claims and electronic health record data of patients with recent-onset HFrEF diagnosed at US Department of Veterans Affairs (VA) health care system facilities from July 1, 2013, through June 30, 2019. Veterans who had a history of heart transplant or used a ventricular assist device were among the patients who were excluded. Guideline-directed medical therapy (any β-blocker, guideline-recommended β-blocker [bisoprolol, carvedilol, or metoprolol succinate], angiotensin-converting enzyme inhibitor, angiotensin receptor blocker, angiotensin receptor-neprilysin inhibitor, mineralocorticoid receptor antagonist, and hydralazine plus nitrate) and ICD. Treatment rates for guideline-directed medical therapies and ICDs were calculated within 6 months of the index HFrEF date using medication fills, procedural codes for implantation and monitoring, and diagnosis codes. Risk-adjusted mortality was calculated after adjusting for baseline patient characteristics. For both treatment rates and risk-adjusted mortality, we evaluated the change over 3 periods (period 1: July 1, 2013, to June 30, 2015; period 2: July 1, 2015, to June 30, 2017; and period 3: July 1, 2017, to June 30, 2019) and variation across VA facilities. The final cohort comprised 144 074 eligible patients with incident HFrEF that was diagnosed between July 1, 2013, and June 30, 2019. The cohort had a mean (SD) age of 71.0 (11.4) years and was mostly composed of men (140 765 [97.7%]). Overall, changes in medical therapy rates were minimal over time, with the use of a guideline-recommended β-blocker increasing from 64.2% in 2013 to 72.0% in 2019. Rates for mineralocorticoid receptor antagonist therapy increased from 23.9% in 2013 to 26.9% in 2019, and rates for hydralazine plus nitrate therapy remained stable at 24.2% over the study period. Rates for angiotensin receptor-neprilysin inhibitor therapy increased since its introduction in 2015 but only to 22.6% in 2019. Among patients with an ICD indication, early use rates decreased over time. Substantial variation in medical therapy rates persisted across VA facilities. Risk-adjusted mortality decreased over the study period from 19.9% (95% CI, 19.6%-20.2%) in July 1, 2013, to June 30, 2015, to 18.4% (95% CI, 18.0%-18.7%) in July 1, 2017, to June 30, 2019 (OR, 0.96 per additional year; 95% CI, 0.96-0.97). This study found only marginal improvement between 2013 and 2019 in the guideline-recommended therapy and mortality rates among patients with recent-onset HFrEF. New approaches to increase the uptake of evidence-based HFrEF treatment are urgently needed and could lead to larger reductions in mortality.

Pediatric Heart Failure: Cardiac Ejection Fraction with Cardiomyopathy Decreased to 21% in Iron Deficient from 37% in Iron Sufficient Children

▪Introduction: The overall aim of our study was to determine if iron deficiency has harmful effects on cardiac function in children with chronic heart failure. Heart failure in children is a complex, heterogeneous disorder leading to a final common pathway of cardiomyocyte dysfunction and attrition. Cellular, animal, and human studies have shown that iron deficiency causes cardiomyocyte dysfunction that can be reversed with iron treatment. Cellular studies of human iron-deficient cardiomyocytes have shown that supplemental iron restores impaired contractility and relaxation. Animal studies have found that cardiomyocyte-specific deletions or alterations of critical iron proteins (transferrin receptor 1, hepcidin, ferroportin) produce cardiomyocyte iron deficiency (without anemia) and result in ultimately fatal cardiac dysfunction that can be rescued with intravenous (IV) iron. Over half of adults with chronic heart failure are iron deficient. Meta-analysis of small randomized clinical trials has shown that IV iron significantly reduces recurrent hospitalization, cardiovascular mortality, and all-cause mortality in iron deficient adults with heart failure. European Society of Cardiology, American College of Cardiology and American Heart Association guidelines recommend consideration of IV iron therapy for adult iron-deficient patients with heart failure.The prevalence and consequences of iron deficiency in children with heart failure have not been established. Previously, two small retrospective studies of children with heart failure have reported that 56% to 96% were iron deficient, with increased morbidity and mortality. The goals of our study of children with heart failure were to determine (i) how often iron status is assessed, (ii) the prevalence of iron deficiency, and (iii) the effects of iron deficiency on cardiac function in patients with cardiomyopathy.Methods: We retrospectively reviewed electronic medical records to identify pediatric patients ages 1-21 years old seen at Columbia University Irving Medical Center Pediatric Heart Failure clinic with absolute iron deficiency during 2010-2020. Heart failure was defined as presence of symptoms or systolic dysfunction by echocardiography. Patients were excluded with a history of heart transplant, isolated diastolic failure, or renal failure requiring dialysis. In adults with heart failure, a transferrin saturation <20% has a sensitivity of 94% and a specificity of 84% in identifying absolute iron deficiency, as determined from a bone marrow aspirate, and iron stores were present in 100% of patients with a transferrin saturation ≥30% (Circ Heart Fail. 2018;11:e0045). In children with heart failure, we used these criteria to define absolute iron deficiency as a transferrin saturation <20% and iron sufficiency as a transferrin saturation ≥30%.Patients with an intermediate transferrin saturation are likely a mixture of absolute and functional iron deficiency, and of iron sufficiency and were excluded from our analysis of cardiomyopathy. Cardiac ejection fraction was evaluated by an echocardiogram performed within 3 months of measurement of transferrin saturation.Results: Of 579 patients with heart failure, only 159 (27%) had any type of laboratory iron studies. Of patients with iron studies, 81 (51%) were evaluated as outpatients; 49% as inpatients. The cause of heart failure was cardiomyopathy (52%), congenital heart disease (34%), acute myocarditis (6%), and other (8%).In the 82 patients with heart failure due to cardiomyopathy, 39 (48%) were iron deficient and 16 (20%) iron sufficient. In the iron deficient children with cardiomyopathy, the left ventricular ejection fraction was lower than in the iron sufficient patients (median 21% vs. 37%; p=0.03 (Mann-Whitney); Figure). The groups did not differ significantly with respect to hemoglobin (Figure), sex, age, or New York Heart Association class.Conclusion: We report a clinically important decrease in cardiac ejection fraction in children with heart failure due to cardiomyopathy who have absolute iron deficiency. Potentially, iron treatment could safely and effectively reverse the harmful effect of iron deficiency on heart function and prospective randomized trials of oral and intravenous iron therapy are urgently needed. Measurement of iron status should routinely be included in the evaluation of children with heart failure. [Display omitted] DisclosuresNeunert: Novartis: Research Funding.

Outcomes of COVID-19 infection in heart transplant recipients

Abstract Background Heart transplant recipients may be at increased risk for infection and adverse outcomes from infection with COVID-19. Management of these patients is complex, with limited data and no standard of care. Purpose We sought to describe the incidence and outcomes of COVID-19 infection in patients with a history of heart transplant. Methods Retrospective review of 235 heart transplant patients at a large academic medical center identified 31 patients with laboratory confirmed COVID-19 infection. Laboratory and clinical data were collected from our institution as well as other local hospitals patients had been treated at. Outcomes were followed from March 15, 2020 to March 15, 2021. All data was deidentified. Results In a cohort of 235 heart transplant patients, 31 patients were identified after testing positive for COVID-19 infection. The mean and median age of patients was 51.9 and 56.0 years respectively. 71% of patients were male and 29% of patients were female. The mean and median time from transplant to diagnosis was 6.54 and 6.50 years. Average ejection fraction prior to infection was 60.4%. Seventeen patients (55%) had atherosclerosis, ten patients (32%) had diabetes, fifteen patients (48%) had hypertension, and twelve patients (39%) had a history of smoking. 84% of patients were on three types of immunosuppression and 16% were on two. Eight patients (26%) were asymptomatic, six patients (19%) had acute hypoxic respiratory failure, and two patients (7%) had GI symptoms. Two patients had evidence of graft injury with mean AlloSure (donor derived cell free DNA) levels of 0.61%. Immunosuppressive regimens were decreased in 32% of patients and COVID-19 directed therapies were given in 32% of patients. The most common treatments were remdesevir (5 patients), steroids (3 patients), and bamlanivimab (2 patients). Thirteen patients (42%) were hospitalized and two patients (7%) died. Conclusions In a single center case series 31 patients were identified with COVID-19 infection. Nearly half of the patients required hospitalization, with one third of patients decreasing their immunosuppressive regimen and 7% mortality. Further investigation is needed to determine long term outcomes and optimal therapeutic regimen for COVID-19 in this unique population. Funding Acknowledgement Type of funding sources: None.

Poor efficacy of oral iron replacement therapy in pediatric patients with heart failure.

Iron deficiency is associated with worse outcomes in children and adults with systolic heart failure. While oral iron replacement has been shown to be ineffective in adults with heart failure, its efficacy in children with heart failure is unknown. We hypothesised that oral iron would be ineffective in replenishing iron stores in ≥50% of children with heart failure. We performed a single-centre retrospective cohort study of patients aged ≤21 years with systolic heart failure and iron deficiency who received oral iron between 01/2013 and 04/2019. Iron deficiency was defined as ≥2 of the following: serum iron <50 mcg/dL, serum ferritin <20 ng/mL, transferrin >300 ng/mL, transferrin saturation <15%. Iron studies and haematologic indices pre- and post-iron therapy were compared using paired-samples Wilcoxon test. Fifty-one children with systolic heart failure and iron deficiency (median age 11 years, 49% female) met inclusion criteria. Heart failure aetiologies included cardiomyopathy (51%), congenital heart disease (37%), and history of heart transplantation with graft dysfunction (12%). Median dose of oral iron therapy was 2.9 mg/kg/day of elemental iron, prescribed for a median duration of 96 days. Follow-up iron testing was available for 20 patients, of whom 55% (11/20) remained iron deficient despite oral iron therapy. This is the first report on the efficacy of oral iron therapy in children with heart failure. Over half of the children with heart failure did not respond to oral iron and remained iron deficient.

Clinical course and surgical outcomes in middle-age adults with anomalous aortic origin of a coronary artery

Abstract Funding Acknowledgements Type of funding sources: None. Background Patients with anomalous aortic origin of a coronary artery (AAOCA) present with a wide range of clinical manifestations, including ischemic symptoms (chest pain or dyspnea) and sudden cardiac death (SCD). Studies have identified coronary anatomic characteristics associated with a higher risk of SCD. However, most of the published literature consists of studies in adolescents and young adults. There is a paucity of data regarding outcomes in middle-aged patients. Current guidelines reveal gaps in evidence for identification of adults are at risk for SCD, and for whom surgery is beneficial. Purpose To study the clinical course and rate of major adverse cardiac events (MACE) in middle-aged adults with AAOCA based on presenting symptoms, coronary anatomy on coronary computed tomography angiography (CCTA), stress test results, and surgical management. Methods We included all patients from January 2013 to December 2019 age &amp;gt; 18 at our institution who were found to have AAOCA. Patients with the following were excluded to minimize confounding factors which could cause MACE: coronary artery disease (CAD) with &amp;gt;50% stenosis in any coronary vessel, CAD requiring revascularization, heart failure with ejection fraction &amp;lt;40%, history of heart transplant, and non-AAOCA congenital heart disease. All patient charts were reviewed for demographics, coronary anatomy on CCTA, presenting symptoms, rationale for pursuing stress testing and CCTA, nature of surgical interventions, post-surgical course, and MACE (cardiovascular death, myocardial infarction, and need for coronary revascularization). All patients underwent PET as well as treadmill stress testing. Results Of 19,367 patients who underwent CCTA, 47 met inclusion criteria, with median age at diagnosis of 54 and median follow-up of 48 months. No patients suffered MACE. Twenty-five patients had AAORCA and 22 had AAOLCA (Table 1). Ten patients with AAORCA and 8 patients with AAOLCA presented with ischemic symptoms and had coronary anatomy characteristics associated with higher risk of SCD, as well as ischemia corresponding to the anomalous artery on stress testing and did not undergo surgery due to personal preference. Five symptomatic patients with stress-induced ischemia corresponding to the anomalous artery underwent surgery and all achieved symptom relief over a median follow up of 5 years. Conclusion As AAOCA is a significant cause of SCD in young adults, it is compelling to observe this adult cohort in which no patients experienced MACE, including 18 symptomatic patients with high-risk anatomy and stress-induced ischemia, as well as a Class I recommendations for surgery. The results suggest that although surgery may be beneficial for symptom relief, it does not necessarily improve mortality over an intermediate follow-up period. Future studies should examine surgical outcomes in middle-aged cohorts with larger sample sizes.

Specific patterns of executive functioning weaknesses among children after heart transplant.

Children with a history of heart transplant (HT) are at risk of executive functioning weaknesses secondary to heart disease and associated morbidity. However, specific executive functioning weaknesses have not been identified. The present study, anchored in Anderson's (2002) Developmental Model of Executive Functioning, provides a detailed, retrospective analysis of executive functioning in the areas of goal setting, cognitive flexibility, attentional control, and information processing for a clinically referred sample of 53 pediatric HT recipients who underwent neuropsychological evaluations as part of typical clinical care. Broadly, the sample demonstrated mild-to-moderate deficits across cognitive, adaptive behavior, executive functioning, and academic domains, as well as elevated parent-reported concerns for depression and anxiety. Executive functioning weaknesses, while global, persisted after controlling for the effects of depression and anxiety and were most prominent in cognitive flexibility. In addition, poor cognitive flexibility predicted lower adaptive behavior, IQ, and academic outcomes among this population, placing them at considerable risk of extensive impairment in several domains of their lives. Taken together, children with a history of HT demonstrated broad difficulties across several areas of functioning, with particular concerns for working memory. As such, interventions and accommodations specifically targeting working memory may help provide the most optimal outcomes for this population.

Caring for Adolescents and Young Adults in an Adult Hospital Setting with a History of Heart Transplant: Nurses' Experience

Purpose Congenital heart disease represents the largest number of birth defects in Ireland with 95% of children now surviving to adulthood (HSE, 2018). With improving long-term survival after paediatric heart transplant, there is an increased number of adolescent heart transplant recipients transitioning to the adult health services. Unique challenges occur during this transition. There is however a dearth of literature on heart transplant adolescents transitioning to adult cardiac services. It is necessary to explore nurses’ experiences of caring for adolescents and young adults with a history of heart transplant in an adult hospital setting. The overall study aim was to explore nurse's experiences of caring for adolescents and young adults in an adult hospital setting with a history of heart transplant. Methods A descriptive phenomenological study using one-to-one interviews was used to conduct this study. Nurses and nurse managers with experience of caring for adolescents with a history of heart transplant in a large urban tertiary referral adult hospital took part in the study. Colizzi's (1978), seven step phenomenological framework was used for data analysis. Results Five key themes were identified: support for adolescents, support for families, support for staff, communication and maturity. Nurses identified that moving from the familiar paediatric setting to the adult setting is a huge change which affects the adolescents and their family. Ideas were put forward to ensure young people settle into their new hospital environment such as meal menus, ward decor, visiting hours. The need for a ‘Transition Co-ordinator’ was identified. Nurses also expressed feelings of stress. Further education and service training was recommended. Inter-professional communication and collaboration is also required for successful transition. Conclusion The findings of this study highlight further improvements required to aid the seamless transition of adolescents with a history of heart transplant to adult cardiac services.

Evolution of heart transplantation since Barnard's first.

Barnard's first human heart transplantation in 1967 has paved the channel to numerous extensive researches and clinical experiences, mostly from the Stanford group, on orchestrating criteria for donor and recipient selection, immunosuppression, distant heart procurement, re-transplantation, and further global performance of the procedure until it became the gold standard therapy for end-stage heart failure, even in the face of an utterly limited organ availability. Much has happened since Barnard's first. There has been a rapid rise to 4,000-5,000 transplants per year. Previously, the number of transplants depended on the capacity of the units. Later on, it depended largely on the availability of donor organs. The widening of indications and reduction of contraindications to heart transplantations to include elderly patients >70 years, systemic diseases such as amyloidosis, has emerged. Indeed, heart transplantation has come a long way, and is considered a fruitful and stimulating episode in modern medicine. It is rather deplorable to watch that in the beginning of 1990s, the yearly number of heart transplantations began to decline-not because the operation had proved unsuccessful, but because donor hearts became scarce. It is rather poignant that although science and technology as well as increased experience accumulated over a long time have paved the way to make such an operation possible, its applicability is limited by lack of public awareness or its unwillingness to agree to organ donation. The average transplant survival is now over 10 years. Its prevalence and success, however, belies the fact that over 52 years ago, no one had ever attempted the procedure in man and that the procedure seemed destined for ignominy and failure just a year after the first transplant. Had it not been for the work of a few scientists and physicians, each who stepped on the broad shoulders of those who had come previously, the history of heart transplantation would be nothing more than a few legends and myths in dusty tomes.

Анализ клинических случаев новой коронавирусной инфекции у пациентов с трансплантацией сердца в анамнезе

There is a description of two clinical cases of a new coronavirus infection COVID-19 in patients with a history of heart transplantation. Patients who receive immunosuppressants for life are at risk of developing infectious diseases. This requires the vigilance of doctors, especially in the face of a pandemic of a new coronavirus infection. Adequate and timely started therapy increases the effectiveness of treatment and reduces the risk of complications. The complex participation of different specialists is necessary to determine the tactics of treatment, correct pathogenetic and suppressive therapy. © 2021 Sovero Press Publishing House. All rights reserved.

25-Year history of heart transplant in India: Lessons learned

25-Year history of heart transplant in India: Lessons learned

Pancytopenia caused by allopurinol and azathioprine interaction in a heart transplant patient: a case report.

BackgroundAzathioprine is an immunosuppressive now less commonly prescribed after orthotopic heart transplantation. Patients with solid organ transplantation are at increased risk for numerous comorbidities including gout. Co-administration of allopurinol for gout prophylaxis and azathioprine increases the risk for severe myelosuppression due to drug–drug interactions.Case summaryA 57-year-old male with a history of heart transplant 6 years prior presented with a month of severe fatigue and shortness of breath. His admission laboratory values were notable for severe pancytopenia. Medical workup revealed no haematologic malignancy, viral infection, or other consumptive process. After extensive review, it was discovered that the patient was taking excessive allopurinol for gout. His haematologic abnormalities resolved following discontinuation of allopurinol and treatment with filgrastim and romiplostim and was able to be discharged from the hospital.DiscussionAzathioprine and allopurinol can potentially cause profound cytopenias due to the increased production of the active metabolites of azathioprine. Given the association between gout and solid organ transplantation, recognition of the risks of medication interaction as well as communication amongst health care providers and between providers and their patients is paramount.

Abnormal coronary anatomy in patients with atrial switch: a predictor of serious cardiac adverse event?

Abstract Introduction Sudden death and heart failure are severe long-term complications after atrial switch for D-transposition of the great arteries (D-TGA). Mechanisms and factors of long-term complications are poorly known. Right ventricle ischemia may be involved in these complications. Since coronary malformations are present in about 30% of D-TGA, we aimed to look for an association between these complications and the presence of coronary anomalies. Methods The study is an observational international study in two tertiary centers. Adults with history of atrial switch and severe rhythmic or hemodynamic complications, history of heart transplantation or cardiovascular death were included. Patients under 18 years old and/or with corrected transposition of great arteries were excluded. A χ2 test was used to compare the proportion of coronary anomalies in this cohort to the one from population of D-TGA reported in previous studies. Results Forty-two patients were included: 11 women (26.2%) and mean age at the time of the study was 43.3±6.4 years. Eight patients (19.0%) died at a mean age 41.3±9.0 years. No ablation for ventricular tachycardia was reported while 15 (35.7%) patients underwent ablation for a SVT (supraventricular tachycardia). Thirty three patients (78.6%) suffered from heart failure and/or severe right ventricle dysfunction and 4 patients (9.5%) were transplanted at a mean age of 29.3±8.3 years. Ten (23.8%) patients suffered from both arrhythmic events and heart failure. Twelve patients (28,6%) had abnormal coronary anatomy. The χ2 test showed no significant difference of coronary anatomy between our population and a pooled population of 647 D-TGA from previous studies (28.7%, p=0.9804). By multivariate analysis, severe pulmonary hypertension and severe tricuspid regurgitation were significantly associated with cardiovascular death and/or heart transplantation (respectively: p=0.0323, OR= 2.5, and p=0.0494, OR= 4.0). SVT ablation tended to be associated to survival (p=0.0928 with OR= 2.2). Conclusions No difference of coronary anomalies ratio in complicated Mustard or Senning patients was seen in comparison with the coronary anomalies ratio of all D-TGA. These results plead against a role of abnormal coronary anatomy in right ventricle dysfunction. Ischemia may be more related with the alteration of micro vascularization and a mismatch between oxygen needs and supply. Funding Acknowledgement Type of funding source: None

PYOGENIC GRANULOMA OF THE TONGUE COINFECTED WITH CYTOMEGALOVIRUS IN AN IMMUNOCOMPROMISED PATIENT: A CASE REPORT

Cytomegalovirus (CMV) is an opportunistic infection that can lead to organ rejection in transplant recipients. In the oral cavity, it usually appears as nonhealing ulcers. For an accurate diagnosis, tissue biopsy has to be performed, and the antiviral drug of choice is ganciclovir. A 9-year-old female patient with a history of heart transplant 3 months prior was receiving prednisone and tacrolimus. Clinical examination revealed a soft, nontender, pink, pedunculated, and ulcerated covered with pseudomembrane mass of the right lateral border of the tongue with a history of trauma during intubation 2 months prior. Excisional biopsy was performed, and anatomopathologic evaluation revealed pyogenic granuloma and CMV infection. The patient was admitted to the hospital for ganciclovir treatment. Prior to the transplant, the patient was CMV(−) and the donor was CMV(+). Although the most common oral CMV manifestation in immunosuppressed patients are nonhealing ulcers, we report the first literature case of oral pyogenic granuloma coinfected with CMV. Cytomegalovirus (CMV) is an opportunistic infection that can lead to organ rejection in transplant recipients. In the oral cavity, it usually appears as nonhealing ulcers. For an accurate diagnosis, tissue biopsy has to be performed, and the antiviral drug of choice is ganciclovir. A 9-year-old female patient with a history of heart transplant 3 months prior was receiving prednisone and tacrolimus. Clinical examination revealed a soft, nontender, pink, pedunculated, and ulcerated covered with pseudomembrane mass of the right lateral border of the tongue with a history of trauma during intubation 2 months prior. Excisional biopsy was performed, and anatomopathologic evaluation revealed pyogenic granuloma and CMV infection. The patient was admitted to the hospital for ganciclovir treatment. Prior to the transplant, the patient was CMV(−) and the donor was CMV(+). Although the most common oral CMV manifestation in immunosuppressed patients are nonhealing ulcers, we report the first literature case of oral pyogenic granuloma coinfected with CMV.

Resting heart rate in ambulatory heart failure with reduced ejection fraction treated with beta‐blockers

AimsCurrent guidelines recommend beta‐blocker therapy in chronic heart failure with reduced ejection fraction (HFrEF) titrated according to tolerated target dose. The efficiency of this strategy to obtain adequate heart rate (HR) control remains unclear in clinical practice. The aim of this study was to determine, in a real‐world setting, the proportion of HFrEF patients who fail to achieve beta‐blocker target doses, whether target doses of beta‐blockers have a relationship with the adequacy in reducing resting HR over time.Methods and resultsBeta‐blocker dose and resting HR of consecutive ambulatory patients with a diagnosis of HFrEF (ejection fraction ≤ 35%) in sinus rhythm were reviewed at the first outpatient contact in the Cleveland Clinic Health System from the year 2000 to 2015. Patients who did not receive beta‐blocker therapy, have congenital heart disease and hypertrophic cardiomyopathy, were not in sinus rhythm, or have a history of heart transplant were excluded. Patients were followed up until their last known visit at the Cleveland Clinic. Median resting HR was 71 b.p.m. [inter‐quartile range (IQR) 60–84 b.p.m.] in 8041 patients (median age 65; 68% male) with 67% on carvedilol, 32% on metoprolol succinate, and 1% on bisoprolol. In 3674 subjects (56%), resting HR was ≥70 b.p.m. At final follow‐up after a median of 21 months (IQR 0.1–7.2 years), resting HR was 72 b.p.m. (IQR 60–84 b.p.m.) in the subset of patients with persistently low ejection fraction ≤ 35%. HR ≥ 70 b.p.m. was observed in 55% of this group. Beta‐blocker target dose was achieved in 19%, 5%, and 15% of those receiving carvedilol, metoprolol succinate, and bisoprolol, respectively. In the subset of patients who experienced beta‐blocker up‐titration, reduced mortality or hospitalization due to heart failure was observed in patients who experienced the lowest HR after titration.ConclusionsIn our single‐centre experience, the majority of patients with chronic HFrEF treated with beta‐blocker therapy did not achieve target doses over time, and a substantial proportion had inadequate control of resting HR. There was no relationship between achieved beta‐blocker target dose and resting HR control.