Hypertrophic cardiomyopathy (HCM) is the second commonest form of heart muscle disease affecting children and adolescents and is a leading cause of sudden death in young athletes. The aetiology of HCM is heterogeneous in the paediatric population, and includes inborn errors of metabolism, neuromuscular disorders and malformation syndromes. However, most cases of apparently idiopathic HCM in childhood are caused by mutations in cardiac sarcomere protein genes. Patients with metabolic or syndromic HCM usually present in infancy or early childhood, whereas those with neuromuscular disorders are more frequently diagnosed in adolescence. The diagnosis of HCM in infants is often made during evaluation for a heart murmur or congestive heart failure. Older children are usually referred for evaluation of symptoms, electrocardiographic abnormalities or heart murmur, or for family screening following the diagnosis of HCM in a relative. Risk stratification in the paediatric population remains a challenge. As most cases of HCM are familial, evaluation of first-degree relatives and other family members at risk of inheriting the disease should be a routine component of clinical management.

https://heart.bmj.com/content/heartjnl/early/2012/05/15/heartjnl-2011-300531.full.pdf

Hypertrophic cardiomyopathy in children

We studied the experiences of children identified by family screening who were found to be a mutation carrier for a genetic cardiovascular disease (Long QT Syndrome (LQTS), Hypertrophic Cardiomyopathy (HCM), Familial Hypercholesterolemia (FH)). We addressed the (a) manner in which they perceive their carrier status, (b) impact on their daily lives, and (c) strategy used to cope with these consequences. Children (aged 8-18) who tested positive for LQTS (n = 11), HCM (n = 6) or FH (n = 16), and their parents participated in semi-structured audiotaped interviews. Interview topics included illness perception, use of medication, lifestyle modifications, worries, and coping. Each interview was coded by two researchers. The qualitative analysis was guided by Leventhal's model of self-regulation. The children were overall quite articulate about the disease they were tested for, including its mode of inheritance. They expressed positive future health perceptions, but feelings of controllability varied. Adherence and side-effects were significant themes with regard to medication-use. Refraining from activities and maintaining a non-fat diet were themes concerning lifestyle modifications. Some children spontaneously reported worries about the possibility of dying and frustration about being different from peers. Children coped with these worries by expressing faith in the effectiveness of medication, trying to be similar to peers or, in contrast, emphasizing their "being different." Children generally appeared effective in the way they coped with their carrier status and its implications. Nevertheless, dealing with the daily implications of their condition remains difficult in some situations, warranting continued availability of psychosocial support. © 2008 Wiley-Liss, Inc.

Predictive genetic testing for cardiovascular diseases: Impact on carrier children

Esmolol is often used in the acute management of children with arrhythmias and/or hypertension; however, pharmacokinetic studies of the drug in children have been limited. The objective of this study was to determine the pharmacokinetics of esmolol in children with a history of supraventricular arrhythmias (SVT) who were scheduled for diagnostic electrophysiology study or a catheter ablation procedure. Subjects were stratified into two age groups: 2–11 and 12–16 years. After an episode of stimulated or spontaneous SVT, esmolol was administered intravenously as a 1,000 μg/kg bolus followed by continuous infusion at 300 μg/kg/min. Blood samples were collected before, at 5, 10 and 15 min after the loading dose, and 3, 6, 9, 12, 15 and 20 min after the end of the infusion. Plasma concentration of esmolol was quantitated by a specific LC/MS assay. Pharmacokinetic data were available for 25 subjects. Arterial esmolol concentrations were approximately five times greater than venous concentrations. Esmolol had an extremely short distribution half-life (0.6 min), a rapid terminal elimination half-life (6.9 min), and a rapid clearance (119 ± 51 mL/min/kg) which was not related to subject age or weight. Seventeen of the subjects (63%) converted to normal sinus rhythm in an average of 2 min (range 0–5 min). The pharmacokinetics of esmolol and its efficacy in terminating SVT in children is similar to that observed in adults.

The Pharmacokinetics of Esmolol in Pediatric Subjects with Supraventricular Arrhythmias

Background: Children’s health programs in Sub-Saharan Africa have always been oriented primarily to infectious diseases and malnutrition. We are witnessing in the early 21 st century an epidemiological transition marked by the decline of old diseases and the identification of new diseases including heart disease. Therefore, it is necessary to describe the spectrum of these diseases in order to better prepare health workers to these new challenges.
 Methods: We conducted a cross-sectional study focused on heart disease diagnosed by echocardiography in children seen from January 2006 to December 2014 in a pediatric hospital of Yaounde. We collected sociodemographic data and the types of heart disease from registers, patients files as well as the electronic database of echocardiographic records.
 Results: A total of 2,235 patients underwent echocardiographic examination during the study period including 1,666 subjects with heart disease. Congenital cardiopathies were found in 1,230 (73.8%) patients and acquired abnormalities in 429 (25.8%). Seven children (0.4%) had a combination of both types. Congenital heart defects (CHD) were dominated by ventricular septal defect (VSD). Acquired heart disease was mostly rheumatic valvulopathies. Dyspnea on exertion was the most frequent presenting complaint (87.6%). Discovery of a heart murmur was the principal clinical finding on physical examination (81.4%). The median age was 9 months for congenital heart disease and 132 months for acquired heart disease.
 Conclusions: As infectious diseases recede and the diagnostic facilities are improving, pediatric heart diseases occupy a more important position in the spectrum of pediatric diseases in our context. However, the ability to evoke the diagnosis remains unsatisfactory by the majority of health personnel and therefore needs to be improved. Apart from congenital heart diseases, the impact of acquired heart diseases, rheumatic valvulopathy being the highest ranking, is remarkable in pediatrics. Awareness of health personnel for better management of child tonsillitis is more than ever a necessity. This preventive attitude of rheumatic heart disease is the main attitude available in our disadvantaged economic environment.

/pdf/spectrum-of-heart-diseases-in-children-an-echocardiographic-rfot0j0m8g.pdf

Spectrum of heart diseases in children: an echocardiographic study of 1,666 subjects in a pediatric hospital, Yaounde, Cameroon.

The present invention is directed to an inhalable formulation for the treatment of pulmonary hypertension in a mammal (e.g., humans), wherein the formulation comprises at least one hypertension reducing agent, including but not limited to an angiotensin converting enzyme inhibitor, angiotensin receptor blocker, beta-blocker, calcium-channel blocker or vasodilator, or any combination thereof. The formulations of the present invention may be a solution or suspension, and preferably are suitable for administration via nebulization. The present invention is also directed to a method and kit for treating a mammal suffering from pulmonary hypertension.

Inhalable formulations for treating pulmonary hypertension and methods of using same

Hypertrophic cardiomyopathy (HCM) is an inherited cardiac disease characterized by unexplained left ventricular hypertrophy, typically involving the interventricular septum Hypertrophy may be present in infants, but commonly develops during childhood and adolescence Management of children with HCM aims to provide symptomatic relief and prevention of sudden death, which is the primary cause of death

Management of Hypertrophic Cardiomyopathy in Children

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Management of Hypertrophic Cardiomyopathy in Children