Heart broken kids and all causes of heart failure

Although Kawasaki disease (KD) is a self-limiting disease, it may cause sudden cardiac death. Diagnosis of KD is principally based on clinical signs; however, some infant cases do not meet the criteria. Such cases are identified as incomplete KD. The sudden death risk in incomplete KD cases is similar to conventional KD. In our 5-month-old case, he had been admitted to a hospital for a fever and suppuration at the site of Bacille de Calmette et Guerin (BCG) vaccination. However, after discharge from the hospital, his C-reactive protein (CRP) levels declined, he got indisposed and died suddenly...

Cardiomyopathy is a rare disorder of the heart muscle, affecting 1.13 cases per 100,000 children, from birth to 18 years of age. Cardiomyopathy is the leading cause of heart transplantation in children over the age of 1. The Pediatric Cardiomyopathy Registry funded in 1994 by the National Heart, Lung, and Blood Institute was established to examine the epidemiology of the disease in children below 18 years of age. More than 3500 children across the United States and Canada have been enrolled in the Pediatric Cardiomyopathy Registry, which has followed-up these patients until death, heart transplantation, or loss to follow-up...

BACKGROUND: The natural history of acute myocarditis (AM) remains highly variable and predictors of outcome are largely unknown. The objectives were to determine the potential value of various cardiovascular magnetic resonance (CMR) parameters for the prediction of adverse long-term outcome in patients presenting with suspected AM. METHODS: In a single-centre longitudinal prospective study, 203 routine consecutive patients with an initial CMR-based diagnosis of AM (typical Late Gadolinium Enhancement, LGE) were followed over a mean period of 18...

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BACKGROUND: Implantable cardioverter-defibrillators (ICDs) reduce the incidence of sudden cardiac death (SCD) in adults with end-stage heart failure; however, their efficacy in pediatric patients awaiting heart transplantation is not well established. OBJECTIVES: This study sought to investigate the role of ICDs in preventing SCD and waiting list mortality as well as to determine risk factors for SCD in pediatric patients listed for heart transplantation. METHODS: We queried the United Network for Organ Sharing database for all pediatric patients (age ≤18 years) listed for heart transplantation (2005-2014)...

Acute worsening heart failure (WHF) is seen in a sizable portion of patients hospitalized for heart failure, and is increasingly being recognized as an entity that is associated with an adverse in-hospital course. WHF is generally defined as worsening heart failure symptoms and signs requiring an intensification of therapy, and is reported to be seen in anywhere from 5% to 42% of heart failure admissions. It is difficult to ascertain the exact epidemiology of WHF due to varying definitions used in the literature...

An exaggerated increase in circulatory blood volume is linked to congestive heart failure. Despite this increase, reduction of the "effective circulatory blood volume" in congestive heart failure is associated with decreased cardiac output, and can weaken the sensitivity of baroreceptors. Thereafter, tonic inhibition of the baroreceptor-mediated afferent pathway of vagal nerves is removed, providing an increase in non-osmotic release of arginine vasopressin (AVP). In the renal collecting duct, the aquaporin-2 (AQP2) water channel is regulated by sustained elevation of AVP release, and this leads to augmented hydroosmotic action of AVP, that results in exaggerated water retention and dilutional hyponatremia...

BACKGROUND: Left ventricular noncompaction (LVNC) is a distinct form of cardiomyopathy characterized by hypertrabeculation of the left ventricle. The LVNC phenotype may occur in isolation or with other cardiomyopathy phenotypes. Prognosis is incompletely characterized in children. METHODS AND RESULTS: According to diagnoses from the National Heart, Lung, and Blood Institute-funded Pediatric Cardiomyopathy Registry from 1990 to 2008, 155 of 3,219 children (4.8%) had LVNC...

Systolic and diastolic myocardial dysfunction has been demonstrated to be associated with an activation of the circulating and local renin-angiotensin-aldosterone system (RAAS), and with a subsequent inappropriately increased production of reactive oxygen species (ROS). While, at low concentrations, ROS modulate important physiological functions through changes in cellular signalling and gene expression, overproduction of ROS may adversely alter cardiac mechanics, leading to further worsening of systolic and diastolic function...

BACKGROUND: Life-threatening ventricular arrhythmias (VAs) and sudden cardiac death (SCD) are common in patients awaiting heart transplantation (HT), and the implantable cardioverter-defibrillator (ICD) is often used for primary prevention in this setting. Use of ICDs in these patients is not without risks and is sometimes contraindicated. The wearable cardioverter-defibrillator (WCD) may be a reasonable alternative to bridge the period of risk leading up to HT. METHODS: We obtained a convenience sample of patients prescribed an WCD as a bridge therapy to HT...

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