ACC
: American College of Cardiology
ACE
: angiotensin-converting enzyme
ACS
: acute coronary syndrome
AF
: atrial fibrillation
AGNES
: Arrhythmia Genetics in the Netherlands
AHA
: American Heart Association
AMIOVIRT
: AMIOdarone Versus Implantable cardioverter-defibrillator:

/pdf/2015-esc-guidelines-for-the-management-of-patients-with-3hzn8vw0c4.pdf

2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: The Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC)

A scientific consensus is emerging that the origins of adult disease are often found among developmental and biological disruptions occurring during the early years of life. These early experiences can affect adult health in 2 ways—either by cumulative damage over time or by the biological embedding of adversities during sensitive developmental periods. In both cases, there can be a lag of many years, even decades, before early adverse experiences are expressed in the form of disease. From both basic research and policy perspectives, confronting the origins of disparities in physical and mental health early in life may produce greater effects than attempting to modify health-related behaviors or improve access to health care in adulthood.

https://jamanetwork.com/HttpHandlers/ArticlePdfHandler.ashx?journal=JAMA&articleId=184019&pdfFileName=jsc90003_2252_2259.pdf

Neuroscience, Molecular Biology, and the Childhood Roots of Health Disparities: Building a New Framework for Health Promotion and Disease Prevention

2015 ESC Guidelines for the Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death

/pdf/2015-esc-guidelines-for-the-management-of-patients-with-4lhfmy4xir.pdf

2015 ESC Guidelines for the Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death.

It is important that the medical profession play a central role in critically evaluating the use of diagnostic procedures and therapies introduced and tested for detection, management, or prevention of disease. Rigorous, expert analysis of the available data documenting absolute and relative

/pdf/acc-aha-2008-guidelines-for-the-management-of-adults-with-4oqjti8ewr.pdf

ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: Executive summary - A report of the American College of Cardiology/American Heart Association Task Force on practice guidelines (writing committee to develop guidelines for the management of adults with congenital heart disease)

Diabetic autonomic neuropathy (DAN) is a serious and common complication of diabetes. Despite its relationship to an increased risk of cardiovascular mortality and its association with multiple symptoms and impairments, the significance of DAN has not been fully appreciated. The reported prevalence of DAN varies widely depending on the cohort studied and the methods of assessment. In randomly selected cohorts of asymptomatic individuals with diabetes, 20% had abnormal cardiovascular autonomic function. DAN frequently coexists with other peripheral neuropathies and other diabetic complications, but DAN may be isolated, frequently preceding the detection of other complications. Major clinical manifestations of DAN include resting tachycardia, exercise intolerance, orthostatic hypotension, constipation, gastro- paresis, erectile dysfunction, sudomotor dysfunction, impaired neurovascular function, "brit- tle diabetes," and hypoglycemic autonomic failure. DAN may affect many organ systems throughout the body (e.g., gastrointestinal (GI), genitourinary, and cardiovascular). GI distur- bances (e.g., esophageal enteropathy, gastroparesis, constipation, diarrhea, and fecal inconti- nence) are common, and any section of the GI tract may be affected. Gastroparesis should be suspected in individuals with erratic glucose control. Upper-GI symptoms should lead to con- sideration of all possible causes, including autonomic dysfunction. Whereas a radiographic gastric emptying study can definitively establish the diagnosis of gastroparesis, a reasonable approach is to exclude autonomic dysfunction and other known causes of these upper-GI symptoms. Constipation is the most common lower-GI symptom but can alternate with episodes of diarrhea. Diagnostic approaches should rule out autonomic dysfunction and the well-known causes such as neoplasia. Occasionally, anorectal manometry and other specialized tests typically performed by the gastroenterologist may be helpful. DAN is also associated with genitourinary tract disturbances including bladder and/or sexual dysfunction. Evaluation of bladder dysfunc- tion should be performed for individuals with diabetes who have recurrent urinary tract infec- tions, pyelonephritis, incontinence, or a palpable bladder. Specialized assessment of bladder dysfunction will typically be performed by a urologist. In men, DAN may cause loss of penile erection and/or retrograde ejaculation. A complete workup for erectile dysfunction in men should include history (medical and sexual); psychological evaluation; hormone levels; mea- surement of nocturnal penile tumescence; tests to assess penile, pelvic, and spinal nerve func- tion; cardiovascular autonomic function tests; and measurement of penile and brachial blood pressure. Neurovascular dysfunction resulting from DAN contributes to a wide spectrum of clinical disorders including erectile dysfunction, loss of skin integrity, and abnormal vascular reflexes. Disruption of microvascular skin blood flow and sudomotor function may be among the earliest manifestations of DAN and lead to dry skin, loss of sweating, and the development of fissures and cracks that allow microorganisms to enter. These changes ultimately contribute to the development of ulcers, gangrene, and limb loss. Various aspects of neurovascular function can be evaluated with specialized tests, but generally these have not been well standardized and have limited clinical utility. Cardiovascular autonomic neuropathy (CAN) is the most studied and clinically important form of DAN. Meta-analyses of published data demonstrate that reduced cardiovascular autonomic function as measured by heart rate variability (HRV) is strongly (i.e., relative risk is doubled) associated with an in- creased risk of silent myocardial ischemia and mortality. The determination of the presence of CAN is usually based on a battery of auto- nomic function tests rather than just on one test. Proceedings from a consensus conference in 1992 recommended that three tests (R-R variation, Valsalva maneuver, and postural blood pressure testing) be used for longitudi- nal testing of the cardiovascular autonomic system. Other forms of autonomic neuropathy can be evaluated with specialized tests, but these are less standardized and less available than commonly used tests of cardiovascular autonomic function, which quantify loss of HRV. Interpretability of serial HRV testing re- quires accurate, precise, and reproducible procedures that use established physiological maneuvers. The battery of three recom- mended tests for assessing CAN is readily per- formed in the average clinic, hospital, or diagnostic center with the use of available technology. Measurement of HRV at the time of diagnosis of type 2 diabetes and within 5 years after diagnosis of type 1 diabetes (unless an individual has symptoms suggestive of au- tonomic dysfunction earlier) serves to estab- lish a baseline, with which 1-year interval tests can be compared. Regular HRV testing pro- vides early detection and thereby promotes timely diagnostic and therapeutic interven- tions. HRV testing may also facilitate differen- tial diagnosis and the attribution of symptoms (e.g., erectile dysfunction, dyspepsia, and diz- ziness) to autonomic dysfunction. Finally, knowledge of early autonomic dysfunction can encourage patient and physician to im- prove metabolic control and to use therapies such as ACE inhibitors and -blockers, proven to be effective for patients with CAN. Diabetes Care 26:1553-1579, 2003

/pdf/diabetic-autonomic-neuropathy-4kabtayo3d.pdf

Diabetic Autonomic Neuropathy

A 15-year-old girl suffered recurrent syncopal episodes during 7 years. Events were precipitated by exercise or emotional stress, leading to the diagnosis of reflex syncope. Exercise testing induced recurrent salvos of nonsustained right ventricular outflow tract tachycardia. This arrhythmia is often asymptomatic, reflex syncope is very frequent and both causes are related to the same triggering situations. It was therefore essential to obtain recordings during syncopal events and to observe the clinical evolution under effective treatment in order to make the right diagnosis.

/pdf/repetitive-ventricular-tachycardia-in-a-syncopal-child-cause-38cemj1fns.pdf

Repetitive ventricular tachycardia in a syncopal child: Cause or incidental finding ?

rate monitoring among diabetic children Patterns of physical activity determined by heart

A 38-week gestational age boy was admitted after birth by Caesarean section to a neonatal unit. At 6 hours of life, a dextrose infusion was started because of hypoglycaemia. At 36 hours he developed cyanosis and tachypnoea. Physical examination revealed tachycardia, 2/6 systolic murmur, weakly palpable limb pulses, hepatomegaly and pulmonary rales. Laboratory tests were within the normal range, except blood gas analysis which showed respiratory and metabolic acidosis. Management entailed fluid resuscitation, mechanical ventilation and antibiotic administration. Because of recurrent metabolic acidosis and unstable haemodynamics, the infant was transferred at 48 hours to our neonatal intensive care unit with a diagnosis of probable sepsis. On admission all limb pulses were absent but carotid pulses were weakly palpable and became strong after establishing stable haemodynamics with inotropic support. Chest X-ray showed cardiomegaly and pulmonary oedema. Echocardiography confirmed the clinical diagnosis. The clinical condition of the patient improved quickly after initiation of treatment. Twenty-four hours later he was catheterized because of associated lesions. Left ventricular angiography is shown …

/pdf/a-neonate-with-an-unusual-pulse-discrepancy-2qxyj8dciu.pdf

A neonate with an unusual pulse discrepancy

BACKGROUND—Measurements of heart rate variability (HRV) are increasingly used as markers of cardiac autonomic activity.
AIM—To examine circadian variation in heart rate and HRV in children.
SUBJECTS—A total of 57 healthy infants and children, aged 2 months to 15 years, underwent ambulatory 24 hour Holter recording. Monitoring was also performed on five teenagers with diabetes mellitus and subclinical vagal neuropathy in order to identify the origin of the circadian variation in HRV.
METHODS—The following variables were determined hourly: mean RR interval, four time domain (SDNN, SDNNi, rMSSD, and pNN50) and four frequency domain indices (very low, low and high frequency indices, low to high frequency ratio). A chronobiological analysis was made by cosinor method for each variable.
RESULTS—A significant circadian variation in heart rate and HRV was present from late infancy or early childhood, characterised by a rise during sleep, except for the low to high frequency ratio that increased during daytime. The appearance of these circadian rhythms was associated with sleep maturation. Time of peak variability did not depend on age. Circadian variation was normal in patients with diabetes mellitus.
CONCLUSION—We have identified a circadian rhythm of heart rate and HRV in infants and children. Our data confirm a progressive maturation of the autonomic nervous system and support the hypothesis that the organisation of sleep, associated with sympathetic withdrawal, is responsible for these rhythms.

https://adc.bmj.com/content/archdischild/83/2/179.full.pdf

Circadian rhythm of heart rate and heart rate variability

Objective: Measurements of the variability in heart rate are increasingly used as markers of cardiac autonomic activity. We sought to establish the development this variability in healthy young infants while sleeping. Patients: We carried out polygraphic studies with electrocardiographic recording in 587 healthy infants aged from 5 to 26 weeks. Methods: We determined several variables over a period of 400 minutes sleeping: mean RR interval, 5 time-domain (SDNN, SDNNi, SDANNi, RMSSD, and pNN50) and 5 frequency-domain indexes (spectral power over 3 regions of interest, total power and low-to-high frequency ratio). Frequency-domain indexes were also assessed separately for the periods of quiet sleep and those of rapid eye movement sleep. Results: Our data showed a significant correlation between the indexes of heart rate variability and the mean RR interval, the breathing rate, and the corrected age of the infants. We also demonstrated the importance of the maturation of the sleeping patterns. Conclusion: These data in a large cohort of healthy infants confirm a progressive maturation of the autonomic nervous system during sleep, and may be used to examine the influence of physiological and pathophysiological factors on autonomic control during polygraphic studies.

Normal ranges for the variability in heart rate in young infants while sleeping.

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