OCT: A Modality for Identifying Stent Failure in Pediatric Patients With Angiographically Silent Coronary Arteries.

We present the case of a 16-year-old patient with anomalous left coronary artery from the left pulmonary artery requiring percutaneous coronary intervention in infancy who presented with ventricular fibrillation arrest. A coronary angiogram revealed 40% narrowing of the stent relative to the remainder of the left main coronary artery. Optical coherence tomography was performed and revealed an area stenosis of 70% relative to the native left main coronary artery. The patient had outgrown the stent. (Level of Difficulty: Advanced.).

Characterization of a Unique Form of Arrhythmic Cardiomyopathy Caused by Recessive Mutation in LEMD2.

Nuclear envelope proteins have been shown to play an important role in the pathogenesis of inherited dilated cardiomyopathy. Here, we present a remarkable cardiac phenotype caused by a homozygous LEMD2 mutation in patients of the Hutterite population with juvenile cataract. Mutation carriers develop arrhythmic cardiomyopathy with mild impairment of left ventricular systolic function but severe ventricular arrhythmias leading to sudden cardiac death. Affected cardiac tissue from a deceased patient and fibroblasts exhibit elongated nuclei with abnormal condensed heterochromatin at the periphery. The patient fibroblasts demonstrate cellular senescence and reduced proliferation capacity, which may suggest an involvement of LEM domain containing protein 2 in chromatin remodeling processes and premature aging.

The Role of Cardiac Magnetic Resonance Imaging in Severe Anorexia Nervosa.

Objective Anorexia nervosa (AN) patients are at an increased risk of developing cardiac complications including bradyarrhythmias, systolic dysfunction, pericardial effusions, and sudden cardiac death. Although previous echocardiographic studies in AN patients have demonstrated a reduction in overall left ventricular (LV) mass, systolic dysfunction, and silent pericardial effusions, little is known about the role of cardiac magnetic resonance imaging (CMR) in assessing this patient population. The objective of this study was to assess cardiac indices and the presence of myocardial fibrosis in AN patients. Methods Between 2014 and 2015, a cross-sectional pilot study of 16 female patients who met the Diagnostic and Statistics Manual of Mental Disorders, fifth edition (DSM-5) criteria for AN was conducted at a single tertiary care center. Baseline characteristics including age, weight, food restriction behavior, over-exercise, self-induced vomiting, and laxative abuse were collected in the study population. Electrocardiography, transthoracic echocardiography (TTE), and CMR were performed. Results The mean age was 17 years (range: 13-22 years). There were no conduction abnormalities as the average PR interval was 152 ms (range: 130-190 ms) and QTc was 413 ms (range: 360-450 ms). Using TTE, the left ventricular ejection fraction (LVEF) was 54 ± 4% with a lower LV mass/body surface area (BSA) of 56 ± 7g/m2 in AN patients as compared to controls. Using CMR, both the mean LVEF of 52 ± 9% and LV mass/BSA of 45 ± 4g/m2 were lower in AN patients as compared to controls. Using CMR, both right ventricular ejection fraction (RVEF) of 50 ± 10% and a right ventricular (RV) mass/BSA of 18 ± 3g/m2 were smaller in AN patients as compared to controls. There was no evidence of late gadolinium enhancement (LGE) in the study population. Conclusions Young patients with AN have lower cardiac mass and volumes with no evidence of myocardial fibrosis.

Neonatal myocardial infarction secondary to umbilical venous catheterization: A case report and review of the literature.

Neonatal acute myocardial infarction (MI) is a rare event, with a high mortality rate. Early recognition and intensive care management can be successful, and it requires a high index of suspicion in cases of unknown cardiogenic shock. The lack of access to cardiac catheterization should not delay management. Furthermore, there is great potential for myocardial regeneration to occur in surviving neonates with MI, emphasizing the importance of early recognition and management. The present report describes a neonate with an anatomically normal heart and coronary arteries who developed clinical, laboratory and electrocardiographic features of MI, and was managed successfully with fluid resuscitation, inotropic drugs and vasodilators.

Should prevention of chronic kidney disease start before pregnancy?

OBJECTIVE: The objective was to evaluate whether there is a role for the prevention of future chronic kidney disease (CKD) in children by improving maternal health at conception, this review addresses: the risk of childhood obesity in the development of CKD, trends in childhood obesity and body composition in children with renal diseases, trends in pre-pregnancy BMI and its association with neonatal outcome, and the effect of pre-pregnancy body mass index (BMI) on blood pressure and body composition in the offspring. INCREASED BMI AND HYPERTENSION AS RISK FACTORS FOR CKD: It is now well established that the presence of hypertension increases the risk of CKD. Increased BMI can also increase the risk of the development of CKD indirectly, through an increase in the prevalence of hypertension, and, possibly, through a direct effect independent of hypertension. TRENDS IN CHILDHOOD OBESITY IN GENERAL, AND SPECIFICALLY AMONG CHILDREN WITH RENAL DISEASES: An unprecedented epidemic of childhood obesity has been witnessed since the 1970s. An estimated 35% of children in North America are reported to be overweight. Children with CKD have even higher BMIs. TRENDS IN PRE-PREGNANCY BMI AND ITS ASSOCIATION WITH NEONATAL OUTCOME: The average BMI of mothers delivering in a single hospital in London, Ontario, rose from 24.3 kg/m2 in 1995 to 25.1 kg/m2 in 2004, whereas the average age of conception of the first child remained unchanged at 28 years. High pre-pregnancy BMIs increased the proportion of large-for-gestational-age newborns, a high proportion of congenital anomalies including renal abnormalities, and the need for Cesarean sections. EFFECT OF PRE-PREGNANCY BMI ON BLOOD PRESSURE AND BODY COMPOSITION IN THE OFFSPRING: Among 1,915 children (mean age 8.3 +/- 5.2 years), studied at the Children's Hospital, London Health Science Centre, BMI z-score correlated significantly with systolic (Spearman r = 0.214, P < 0.0001), and diastolic blood pressure z-scores (Spearman r = 0.143, P < 0.0001). The pre-pregnancy BMI correlated with both BMI z-score (Spearman r = 0.144, P < 0.0001) and blood pressure z-score (Spearman r = 0.13, P = 0.0005) in the children. The birth weight also correlated significantly with a higher BMI z-score (Spearman r = 0.134, P < 0.0001). CONCLUSION: There are increasing trends in childhood obesity and pre-pregnancy maternal BMI. Higher pre-pregnancy BMI increases the risk for increased BMI z-score and blood pressure z-score in children. Since elevated BMI and blood pressure clearly are known risk factors of future CKD, targeting healthier weights prior to conception is likely to reduce the CKD burden in children.

Safety considerations with mycophenolate sodium.

Following 10 years of clinical use of mycophenolate mofetil (MMF), a prodrug of mycophenolic acid, the FDA has approved enteric-coated mycophenolate sodium (EC-MPS). EC-MPS was developed to reduce the upper-gastrointestinal (GI) effects of MMF. Unlike oral MMF, where absorption starts in the stomach, EC-MPS releases MPA in the small intestine. Along with the pharmacology and pharmacokinetics, three randomized, controlled clinical trials in solid-organ transplantation, comparing MMF and EC-MPS, are reviewed. Disappointingly, EC-MPS was similar to MMF in efficacy and safety and did not significantly improve the GI side effects. Moreover, bioequivalence dosing has only been established with concomitant ciclosporin. The pharmacokinetic characteristics must be studied in greater detail. EC-MPS is a safe and effective immunosuppressive agent approved for use in the prevention of acute rejection after renal transplantation. However, the anticipated improvement of GI side effects has not been forthcoming.