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

Recent studies have reported a strong association between increased red cell distribution width (RDW) and the risk of adverse outcomes for adults with heart failure. This study investigated the association between preoperative RDW and postoperative clinical outcomes for children with cardiac disease. The relation between preoperative RDW and the length of postoperative stay was tested with 688 consecutive children undergoing surgery for congenital heart disease (CHD). The RDW was significantly higher in patients who died during the postoperative hospital stay (mean, 18.34 ± 4.69 vs 16.12 ± 2.84; p = 0.004). The risk of postoperative death was five times higher for patients with an RDW of 16% or more. In the general study population, RDW correlated with the intensive care unit (ICU) stay (p < 0.0001) and with the total hospital stay in the local population (p < 0.0001). The correlation between RDW and ICU stay was stronger for patients with acyanotic CHD (p < 0.0001) than for those with cyanotic CHD (p = 0.0007), and for the subpopulation of patients with acyanotic CHD and normal hemoglobin level (p < 0.0001) than for anemic patients with acyanotic CHD (p = 0.025). Preoperative RDW is a strong predictor of an adverse outcome in children undergoing surgery for CHD, especially in nonanemic patients, for whom it reflects an underlying inflammatory stress.

Relation Between Red Cell Distribution Width and Clinical Outcome After Surgery for Congenital Heart Disease in Children

Objective: The aim of this prospective study was to evaluate the proportion of children with delayed recognition of congenital heart disease (CHD). Methods: Of the 744 children with CHD primarily diagnosed during a 10 year period in one hospital, the patients were identified where the diagnosis of CHD was established with a significant delay. Results: Sixty six patients (8.9%) had delayed diagnosis of CHD. Among patients with cyanotic CHD, 10.4% (7 of 67 cases) were referred after they had initially been discharged home from the birth clinic. Among patients with acyanotic CHD, 8.7% (59 of 677) of all children and 35.1% (59 of 168) of the children who required surgery or interventional catheterisation were referred at an age where elective repair should have already been performed or needed immediate treatment because of their haemodynamic status. Of the 66 patients with delayed diagnosis, one infant with cor triatriatum died at admission because of delayed referral and 10 children had severe complications: preoperative cardiogenic shock in seven cases of aortic coarctation and one case of endocardial fibroelastosis, pulmonary hypertensive crisis in one child after delayed repair of a ventricular septal defect, and infectious endocarditis after dental care in a teenager with undiagnosed moderate aortic stenosis, who required Ross operation a few months later. Discussion and conclusion: A substantial proportion of CHD was detected with relevant delay. In all cases of late diagnosis, clinical cardiac findings were present that should have alerted the physician on the possible presence of underlying CHD.

/pdf/delayed-recognition-of-congenital-heart-disease-4ko7mq5n58.pdf

Delayed recognition of congenital heart disease

Objective - We sought to document the prevalence of Internet access and usage patterns among families with children diagnosed with congenital heart disease. Material and methods - A questionnaire was administered to the parents of 509 unselected children with congenital heart disease attending the outpatient cardiac clinic. Results - 389 questionnaires were completed. The most common source for acquiring medical information was via the paediatric cardiologist (83%), the family doctor (53%), the general paediatrician (40%) and the Internet (15%).Among the 238 families with Internet access (62%), the most common locations for Internet access were home (74%) and work (33%), and 84 used the Internet to obtain information related to their child's cardiac diagnosis, mostly at the time of diagnosis. Two-thirds of these families considered finding information related to the cardiac diagnosis as easy or very easy, and found this information, helpful or very helpful. Conclusion - A quarter of the families uses the Internet to find information about their child's congenital heart disease. Internet use in this patient population is expected to increase and our vigilance in providing accurate references and in identifying inaccurate information is of paramount importance.

Use of the Internet by parents of children with congenital heart disease.

Children who underwent arterial switch operation for simple transposition of the great arteries in the neonatal period are now reaching an age when exercise testing becomes feasible. This study was conducted to assess exercise tolerance and electrocardiographic response to exercise stress in 50 asymptomatic children, aged 4 to 9 years, using the Bruce walking treadmill protocol to voluntary exhaustion. Heart rate and blood pressure response to exercise stress, endurance time, and electrocardiographic changes were analyzed and compared with those of age-matched normal children. Forty-seven patients had normal exercise capacity and parameters. One patient, whose coronary angiogram showed occlusion of the left main coronary artery, developed electrocardiographic signs of myocardial ischemia during exercise. In 1 patient with a single right coronary artery ostium and in another, who underwent a neonatal internal mammary bypass graft for obstruction of the right coronary artery, the resting electrocardiogram showed ventricular premature complexes and exercise stress-induced salvos of ventricular tachycardia. We conclude that most of the children who underwent the neonatal arterial switch operation for simple transposition of the great arteries have a normal exercise capacity. Exercise testing appears to be useful in detecting ischemic damage or exercise-induced arrhythmias possibly secondary to reduced coronary flow reserve.

Results of the Bruce Treadmill Test in Children After Arterial Switch Operation for Simple Transposition of the Great Arteries

The aim of this study was to assess heart rate variability, a noninvasive parameter for studying the neurohumoral control of heart rate, in children with atrial septal defect. We examined five time-domain and three frequency-domain indices of heart rate variability determined from 24-hour Holter recordings in 20 children, aged 3-14 years, with secundum atrial septal defect who were about to undergo operation. The measures were compared to normal ranges and to the hemodynamic data of the preoperative cardiac catheterization. Most indices of heart rate variability were moderately depressed in children with atrial septal defect. Our data revealed negative correlations between indices and the mean right atrial pressure and the enddiastolic right ventricular pressure, respectively. The study demonstrates the importance of the right ventricular filling pressures for the autonomic control of the heart in children with atrial septal defect.

Heart Rate Behavior in Children with Atrial Septal Defect

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