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Background Childhood cancer survivors have increased risk of dyslipidemia and atherosclerotic cardiovascular disease (CVD). The aim of this study was to evaluate the prevalence and associated cardiovascular risks of specific lipid abnormalities among childhood cancer survivors. Methods Comprehensive lipid panel measurements were obtained from 4115 5-year survivors, with 3406 (mean age at evaluation = 35.2 years, SD = 10.4 years) not having previous dyslipidemia diagnosis, as well as 624 age, sex, and race and ethnicity matched community controls. Results Previously undiagnosed dyslipidemia with abnormal low-density lipoprotein (LDL) cholesterol (>160 mg/dL), non–high density lipoprotein (HDL) cholesterol (>190 mg/dL), HDL cholesterol (<40 mg/dL for men, <50 mg/dL for women), and triglycerides (>150 mg/dL) were identified in 4%, 6%, 30%, and 17%, respectively. Survivors without previous dyslipidemia diagnosis had higher LDL cholesterol and non-HDL cholesterol and lower HDL cholesterol than community controls. Cranial radiotherapy (relative risk [RR] = 2.2, 95% confidence interval [CI] = 1.6 to 3.0 for non-HDL cholesterol) and total body irradiation for hematopoietic cell transplantation (RR = 6.7, 95% CI = 3.5 to 13.0 for non-HDL cholesterol; RR = 9.9, 95% CI = 6.0 to 16.3 for triglycerides) were associated with greater risk of dyslipidemia. Diagnoses of low HDL cholesterol (hazard ratio [HR] = 2.9, 95% CI = 1.8 to 4.7) and elevated triglycerides (HR = 3.1, 95% CI = 1.9 to 5.1) were associated with increased risk for myocardial infarction, and diagnoses of high LDL cholesterol (HR = 2.2, 95% CI = 1.3 to 3.7), high non-HDL cholesterol (HR = 2.2, 95% CI = 1.3 to 3.7), low HDL cholesterol (HR = 3.9, 95% CI = 2.8 to 5.4), and elevated triglycerides (HR = 3.8, 95% CI = 2.7 to 5.5) were associated with increased risk for cardiomyopathy. Conclusions Previously undiagnosed dyslipidemia among childhood cancer survivors was associated with increased risk for myocardial infarction and cardiomyopathy. Comprehensive dyslipidemia evaluation and treatment are needed to reduce cardiovascular morbidity in this population. [ABSTRACT FROM AUTHOR]

Background: Limited data exist regarding left ventricular remodeling patterns observed in adult survivors of childhood cancer after therapy. Methods: Among 1190 adult survivors diagnosed with childhood cancer (median age at diagnosis, 9 years [interquartile range (IQR), 3.8‐14.4 years]; age at evaluation, 35.6 years [IQR, 29.5‐42.8 years]), treatment exposures included anthracyclines (n = 346), chest radiotherapy (n = 174), both (n = 245), or neither (n = 425). Prospective echocardiographic assessment compared survivors with 449 noncancer controls classified according to left ventricle geometric patterns. Associations between left ventricle geometric patterns and decreased exercise tolerance were assessed. Results: Overall, 28.2% of survivors (95% confidence interval [CI], 25.6%‐30.8%) exhibited concentric remodeling, 2.4% (95% CI, 1.6%‐3.5%) exhibited eccentric hypertrophy, and 1.1% (95% CI, 0.6%‐1.9%) exhibited concentric hypertrophy. A greater proportion of survivors who received only chest radiotherapy (41%) had concentric remodeling compared with those who received only anthracyclines (24%), both (27%), or neither (27%; all P <.001), and all were greater than the proportions in noncancer controls (18%; all P <.05). Concentric remodeling was associated with radiation exposure, but not with anthracycline exposure, in multivariable models. Survivors who had concentric remodeling were more likely to have a maximal oxygen uptake peak <85% compared with those who had normal geometry (81.0% vs 66.3%; odds ratio, 1.75; 95% CI, 1.15‐2.68). Conclusions: Chest radiation therapy, but not anthracycline therapy, increased the risk for concentric remodeling in survivors of childhood cancer. The presence of concentric remodeling was associated with increased exercise intolerance. Chest radiation therapy increases the risk of concentric left ventricular remodeling as assessed by echocardiography, but anthracycline therapy does not increase the risk of concentric remodeling. Adult survivors of childhood cancer who have concentric remodeling are more likely to have a relative peak oxygen uptake <85% compared with survivors who have normal left ventricular geometry. [ABSTRACT FROM AUTHOR]

Survival for pediatric patients diagnosed with cancer has improved significantly. This achievement has been made possible due to new treatment modalities and the incorporation of a systematic multidisciplinary approach for supportive care. Understanding the distinctive cardiovascular characteristics of children undergoing cancer therapies has set the underpinnings to provide comprehensive care before, during, and after the management of cancer. Nonetheless, we acknowledge the challenge to understand the rapid expansion of oncology disciplines. The limited guidelines in pediatric cardio-oncology have motivated us to develop risk-stratification systems to institute surveillance and therapeutic support for this patient population. Here, we describe a collaborative approach to provide wide-ranging cardiovascular care to children and young adults with oncology diseases. Promoting collaboration in pediatric cardio-oncology medicine will ultimately provide excellent quality of care for future generations of patients. [ABSTRACT FROM AUTHOR]

Background: Survivors of childhood cancer have an increased risk of therapy-related cardiovascular disease. It is not known whether family history of cardiovascular disease further increases risk of adverse cardiovascular outcomes among survivors. Methods: Family history of cardiovascular disease was collected from 1,260 survivors [median age at diagnosis, 8 years (range, 0-23); age at last follow-up, 35 years (range, 18-66)] of childhood cancer in the St. Jude Lifetime Cohort Study. Multivariable risk models evaluated associations with cardiovascular disease (Common Terminology Criteria for Adverse Events grade 2-4 events) and cardiovascular risk factors. Results: Among survivors exposed to chest-directed radiation and/or anthracycline chemotherapy (n = 824), 7% reported a first-degree family history of heart failure, 19% myocardial infarction, 11% stroke, 26% atherosclerotic disease (myocardial infarction and/or stroke), 62% hypertension, and 31% diabetes mellitus. Eighteen percent of exposed survivors developed heart failure, 9% myocardial infarction, 3% stroke, 11% atherosclerotic disease, 30% hypertension, and 9% diabetes mellitus. Having a first-degree family history of atherosclerotic disease was independently associated with development of treatment-related heart failure [RR, 1.38; 95% confidence interval (CI), 1.01-1.88; P = 0.04] among exposed survivors. Risk for hypertension was increased among exposed survivors with a first-degree family history of hypertension (RR, 1.55; 95% CI, 1.26-1.92; P < 0.0001) or of any cardiovascular disease [myocardial infarction, stroke, or heart failure (RR, 1.30; 95% CI, 1.06-1.59; P = 0.01)]. Conclusions: Family history of cardiovascular disease and cardiovascular risk factors independently increased risk of heart failure and hypertension among survivors of childhood cancer exposed to cardiotoxic therapies. Impact: These data show the importance of cardiovascular family history as a risk factor for cardiovascular disease in survivors of childhood cancer. [ABSTRACT FROM AUTHOR]

Thromboembolic events and bleeding are major sources of morbidity among pediatric patients supported on a ventricular assist device (VAD). Pharmacokinetics and pharmacodynamics of enteral antiplatelet agents are affected and variable due to erratic enteral absorption in end‐stage heart failure and VAD circulation. Additionally, 20%‐40% of the population are poor metabolizers of clopidogrel, a prodrug, making cangrelor an alternative when antiplatelet therapy is crucial. Cangrelor has been used effectively and safely for short durations in adults during percutaneous coronary interventions, but the use of cangrelor is still under investigation in pediatrics. This case series utilized cangrelor, a novel short‐acting, reversible, intravenous P2Y12 platelet inhibitor in managing pediatric patients supported with a VAD. We performed a retrospective, single‐center review of patients admitted to a tertiary medical center with end‐stage heart failure requiring mechanical circulatory support and concomitant cangrelor administration between January 2019 and March 2020. Platelet function testing, cangrelor dose, bleeding complications, thromboembolic events, and frequency of circuit interventions during the use of cangrelor were recorded. Optimal platelet reactivity, defined as P2Y12 < 180 platelet reaction units (PRU), was measured with serial point‐of‐care testing (VerifyNow). Seven patients, median age of 4.9 years, met the above criteria. Three patients had a diagnosis of complex congenital heart disease. Four patients had dilated or restrictive cardiomyopathy. All patients were on continuous flow VADs. The median VAD duration was 84.5 days (IQR 61.5‐103). The median duration on cangrelor was 43 days (IQR 8‐70). The median cangrelor dose to reach the therapeutic threshold was 0.75 μg/kg/min with the mean P2Y12, while on cangrelor of 164.75 PRU. Bleeding complications included mild gastrointestinal bleeding and hematuria. There was one patient with pump thrombosis requiring intervention. There were no cerebrovascular events while on cangrelor. We report the first successful long‐term use of cangrelor in pediatric patients. The reversibility and short half‐life of cangrelor make it a feasible antiplatelet agent in selected patients. This data supports the use of cangrelor in children as a viable antiplatelet option; with minimal bleeding complications and no cerebrovascular events demonstrated in this cohort. [ABSTRACT FROM AUTHOR]

The article describes the case of a male infant of a diabetic mother who has severe hypertrophic obstructive cardiomyopathy associated with persistent pulmonary hypertension. Topics discussed include the medical history of the mother, the decision to proceed with extracorporeal membrane oxygenation (ECMO) cannulation, and the eventual recovery of the patient who showed significant improvement in septal hypertrophy at three months of age.

Background Hypertension and tachycardia (H/T) are frequently found after hemopoietic cell transplantation (HCT) and are often underrecognized and undertreated. Maintenance of appropriate hemodynamic parameters after HCT may prevent cardiac dysfunction and other adverse cardiovascular outcomes. We sought to determine trends of H/T after HCT as well as the resolution of H/T after hemodynamic medication (HM) and the association between H/T and significant adverse cardiac events (CE). Methods We performed a retrospective review of all patients undergoing non-autologous HCT from 2015 through 2017 at a pediatric cancer hospital, examining vital sign data before HCT and at specified intervals within 100 days of HCT. H/T were determined by published criteria, and records were queried for the use of HM—ace inhibitor, beta blocker, or calcium channel blocker—and the presence of CE during the study period. Results 140 patients were examined [79 male, median age: 9.33]. Hypertension (HTN) was found in 107 (76.4%) patients. Tachycardia (TAC) was found in 97 (68.8%) patients. Figure 1 demonstrates the trend of H/T in the sample. Of patients who experienced HTN, 68 (70%) also experienced TAC. Hypertensive patients were more likely to be male and were 2.2 years younger than the median age, Tachycardic patients were more likely to be male and were 1.7 years older than the median age. 71% of the sample received HM during the study period. The table examines the effects of H/T in treated and untreated patients, showing the proportion of H/T resolution, the median time to resolution, and the presence of CE within each group. Patients who underwent treatment for TAC were more likely to have resolution than those without treatment (OR=3.68, 95%CI: 1.49-9.07, p=0.005). TAC resolution occurred 29.9 days sooner in the treated vs untreated group (p=0.003). The lower likelihood of HTN resolution in the treated vs. untreated groups and the shorter duration of such did not reach statistical significance. Given low event rates, there were no associations between groups and CEs. Conclusions In this pediatric HCT sample, H/T were common. Patients treated for TAC had higher rates of TAC resolution and resumption of normal vitals in a shorter duration of time. In this highly treated sample, cardiac events were rare. Careful surveillance and treatment of H/T after HCT may allow for prevention of cardiovascular complications. [ABSTRACT FROM AUTHOR]

We describe a case of pulmonary artery band placement in a 2-month-old infant with dilated cardiomyopathy and moderate mitral regurgitation in impending need of ventricular assist device. Despite minimal change in echocardiographic appearance, pulmonary artery band placement resulted in significantly reduced left atrial pressure and unchanged right atrial pressure. Improved cardiac output has enabled weaning from ventilator support and favorable somatic growth with enteral feeding. The patient has been stable on milrinone for 3 months, awaiting cardiac transplantation on a regular ward. This therapy represents a potential alternative in small children, who are deemed high-risk candidates for mechanical circulatory support. [ABSTRACT FROM AUTHOR]

Cancer survivors who have undergone hematopoietic cell transplantation (HCT) are at risk for myocardial dysfunction. Children who receive allogenic HCT encounter systemic inflammation resulting in tachycardia and hypertension. The effect of these abnormalities on myocardial function is not known. The aim of this study was to determine whether cardiac dysfunction early after HCT can be predicted by tachycardia or hypertension, within a retrospective single-center sample of pediatric HCT recipients. Early tachycardia or hypertension was defined as a majority of values taken from infusion date to 90 days post-infusion being abnormal. Ejection fraction <53% determined systolic dysfunction. A composite score of accepted pediatric diastolic abnormalities determined diastolic dysfunction. Among 80 subjects (median age 8 years), early tachycardia, systolic dysfunction, and diastolic dysfunction were present in 64%, 25%, and 48% of the sample, respectively. In multivariable models, early tachycardia was an independent predictor of early systolic dysfunction (OR = 12.6 [1.4-112.8], p = 0.024) and diastolic dysfunction (OR = 3.9 [1.3-11.5], p = 0.013). Tachycardia and cardiac dysfunction are common and associated with one another in the early period after pediatric HCT. Future studies may elucidate the role of tachycardia and myocardial dysfunction early after HCT as important predictors of future cardiovascular dysfunction., (© 2021. The Author(s), under exclusive licence to Springer Nature Limited.)

Cardiomyopathies (CMs) encompass a heterogeneous group of structural and functional abnormalities of the myocardium. The phenotypic characteristics of these myocardial diseases range from silent to symptomatic heart failure, to sudden cardiac death due to malignant tachycardias. These diseases represent a leading cause of cardiovascular morbidity, cardiac transplantation, and death. Since the discovery of the first locus associated with hypertrophic cardiomyopathy 30 years ago, multiple loci and molecular mechanisms have been associated with these cardiomyopathy phenotypes. Conversely, the disparity between the ever-growing landscape of cardiovascular genetics and the lack of awareness in this field noticeably demonstrates the necessity to update training curricula and educational pathways. This review summarizes the current understanding of heritable CMs, including the most common pathogenic gene variants associated with the morpho-functional types of cardiomyopathies: dilated, hypertrophic, arrhythmogenic, non-compaction, and restrictive. Increased understanding of the genetic/phenotypic associations of these heritable diseases would facilitate risk stratification to leveraging appropriate surveillance and management, and it would additionally provide identification of family members at risk of avoidable cardiovascular morbidity and mortality., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Martinez, Beasley, Miller, Goldberg and Jefferies.)

Objective: Anemia is common among adult heart failure patients and is associated with adverse outcomes, but data are lacking in children with heart failure. The purpose of this study was to determine the prevalence of anemia in children hospitalized with acute heart failure and to evaluate the association between anemia and adverse outcomes., Design: Review of the medical records of 172 hospitalizations for acute heart failure., Setting: Single, tertiary children's hospital., Patients: All acute heart failure admissions to our institution from 2007 to 2012., Interventions: None., Outcome Measures: Composite endpoint of death, mechanical circulatory support deployment, or cardiac transplantation., Results: Patients ages ranged in age from 4 months to 23 years, with a median of 7.5 years, IQR 1.2, 15.9. Etiologies of heart failure included: dilated cardiomyopathy (n = 125), restrictive cardiomyopathy (n = 16), transplant coronary artery disease (n = 18), ischemic cardiomyopathy (n = 7), and heart failure after history of congenital heart disease (n = 6). Mean hemoglobin concentration at admission was 11.8 g/dL (±2.0 mg/dL). Mean lowest hemoglobin prior to outcome was 10.8 g/dL (±2.2 g/dL). Anemia (hemoglobin <10 g/dL) was present in 18% of hospitalizations at admission and in 38% before outcome. Anemia was associated with increased risk of death, transplant, or mechanical circulatory support deployment (adjusted odds ratio 1.79, 95% confidence interval = 1.12-2.88, P = .011). For every 1 g/dL increase in the patients' lowest hemoglobin during admission, the odds of death, transplant, or mechanical circulatory support deployment decreased by 18% (adjusted odds ratio = 0.82, 95% confidence interval = 0.74-0.93, P = 0.002)., Conclusions: Anemia occurs commonly in children hospitalized for acute heart failure and is associated with increased risk of transplant, mechanical circulatory support, and inhospital mortality., (© 2016 Wiley Periodicals, Inc.)

Objectives: Sirolimus has been used in pediatric cardiac transplantation for the past decade for chronic renal dysfunction, recurrent rejection, and/or coronary allograft vasculopathy. There has been concern regarding the effect of sirolimus on wound healing and other postoperative complications. To date, the pediatric literature on its use is limited and has not specifically addressed its use in the perioperative period following repeat cardiac transplantation., Methods: We compared the patients in our institution who received sirolimus before repeat cardiac transplantation to those in the same era who did not receive sirolimus., Results: Of the 5 patients in the study group, 5 (100%) developed pleural effusions vs 1 (17%) in the control group (p=0.013). There was no increase in mortality in the sirolimus group, and there were no significant differences in renal dysfunction, serious bacterial infection, rejection, or postoperative length of stay., Conclusions: In this small data set, there was a statistically significant increase in pleural effusions in patients on sirolimus. Further study is needed to develop an appropriate strategy to avoid postoperative complications in this patient population.