Childhood Heart Research Articles

EHPnet: American Heart Association

Vol. 112, No. 15 EnvironewsOpen AccessEHPnet: American Heart Association Erin E. Dooley Erin E. Dooley Search for more papers by this author Published:1 November 2004https://doi.org/10.1289/ehp.112-a873AboutSectionsPDF ToolsDownload CitationsTrack Citations ShareShare onFacebookTwitterLinked InReddit Many diseases fall under the umbrella of “cardiovascular disease,” including high blood pressure, cardiac arrhythmia, and congenital heart disease. These conditions affect young and old alike, as well as every ethnic group. In the United States, heart disease is the leading cause of death for both men and women. In 2004 alone, it is expected to cost the U.S. economy over $238 billion in health care services, medications, and lost working hours. Since 1924 the American Heart Association (AHA) has been working to raise funds for heart disease research and to generate awareness among the general public about the seriousness of these diseases. The AHA website located at http://www.heart.org/ disseminates information about heart disease and the association’s wide range of public information and continuing education programs.A menu bar down the left-hand column of the homepage includes a pull-down listing of 10 diseases and conditions, which can then be selected for in-depth information. For example, the page on high blood pressure provides facts on the disease, ways to keep it in check, how to sign up for the AHA’s monthly e-mail list service, and more. Visitors can also find information on risk factors, how a condition can affect health, and information geared toward medical professionals.Another option on the menu bar leads to information on heart disease specifically in children. Along with a basic overview of childhood heart disease, this section includes in-depth pages on topics including DiGeorge syndrome, Kawasaki disease, and exercise for children. Also accessible from the children’s section is HeartPower!, a free, downloadable curriculum devised to help teachers in grades pre-kindergarten through 8 teach their students about healthier lifestyles.The Healthy Lifestyle link from the homepage leads to information on topics ranging from diet and nutrition to heart disease in women. Various pages provide tips on creating healthy eating habits, developing and maintaining food plans, reducing cholesterol, and keeping fit. The Health Tools portion of this section has a cardiovascular disease risk assessment tool; a family health history tree; and online logs for tracking blood glucose, blood pressure, cholesterol, and exercise.The Publications section provides links to lists of AHA-produced consumer and patient education materials, many of which can be ordered for free. Visitors will also find a listing of AHA cookbooks (with sample recipes posted online) and other books on heart health. The AHA publishes five journals that also can be accessed online, as can a variety of other scientific publications, including Scientific Statements from the AHA, performance measures, and clinical data standards. The online Heart and Stroke Encyclopedia is available from the homepage as its own section.Visitors to the homepage will find information on current events, and can search by zip code for nearby events and information on local AHA chapters. Many of the site’s pages are available in Spanish, and can be accessed through the En Español link on the homepage.FiguresReferencesRelatedDetails Vol. 112, No. 15 November 2004Metrics About Article Metrics Publication History Originally published1 November 2004Published in print1 November 2004 Financial disclosuresPDF download License information EHP is an open-access journal published with support from the National Institute of Environmental Health Sciences, National Institutes of Health. All content is public domain unless otherwise noted. Note to readers with disabilities EHP strives to ensure that all journal content is accessible to all readers. However, some figures and Supplemental Material published in EHP articles may not conform to 508 standards due to the complexity of the information being presented. If you need assistance accessing journal content, please contact [email protected]. Our staff will work with you to assess and meet your accessibility needs within 3 working days.

What’s New In Pediatric Cardiology

1. Phat Pham, MD* 2. Michael Silberbach, MD† 1. *Fellow in Pediatric Cardiology 2. †Associate Professor of Pediatrics, Doernbecher Children’s Hospital, Portland, Ore After completing this article, readers should be able to: 1. Recognize the role of genes in the development of cardiac malformations. 2. Describe advances in noninvasive imaging of congenital heart disease. 3. Characterize the role of interventional catheterization in the treatment of congenital heart disease. 4. Describe emerging concepts for pacemakers and implantable cardioverter defibrillators for use in children. Pediatric cardiology was transformed during the latter half of the 20th century from a field that engendered intense curiosity about the heart in infants and children who had uniformly fatal conditions to an established clinical discipline that routinely offered effective medical and surgical therapies. However, cardiac malformations continue to be the most common birth defects and the leading causes of infant mortality, killing twice the number of children who die from childhood cancer. According to the American Heart Association, more than 2 million Americans are living with congenital heart defects, and more than 91 thousand life-years are lost each year to congenital heart disease (CHD). The Association estimates that the cost of hospital care alone for patients who have CHD exceeds $3 billion per year. Today’s researchers are using the tools of molecular genetics to dissect the circuitry of cardiac embryogenesis and to understand the causes of cardiac malformation. In the 21st century, as the fields of computer technology, information management, and biology become integrated, exciting new approaches to the treatment of childhood heart diseases will become a reality. The following is a brief summary of the recent advances in the understanding of cardiac development, the pathogenesis of heart disease, and the current state of diagnostic modalities and therapies aimed at improving the care of children who have heart disease. Although the “nature versus nurture” debate regarding the origin of CHD continues, it generally is believed that many, if not most, cardiac malformations begin as …

Adult Patent Ductus Arteriosus: Successful Surgical Therapy in a Rare Presentation of a Missed Finding

Delayed clinical presentation of patent ductus arteriosus (PDA) in adults is very rare. The clinical presentation in adults consists of either aortic or pulmonary valve endocarditis. We report the case of 34-year-old patient with a known history of rheumatic heart disease in childhood and chronic heart failure for 5 years who presented with acute heart failure and no evidence of PDA at prior echocardiography. Blood cultures grew Staphylococcus epidermidis, and echocardiography showed infective endocarditis of both aortic and pulmonary valves in the context of a large and severely inflamed PDA. Cardiopulmonary bypass and deep hypothermic circulatory arrest were used to interrupt the PDA from within the pulmonary artery because of inability to ligate the severely inflamed and calcified PDA prior to bypass. Combined pulmonary and aortic valve replacement and high-dose inotropic support were used. Dialysis was used for renal failure. The patient recovered and was discharged 10 days postoperatively. Six months later the patient was asymptomatic, and renal failure had resolved. The history and surgical management are discussed with an updated review of the literature.

Influence of CD36 Deficiency on Heart Disease in Children

The physiological role of the CD36 molecule in pediatric heart disease has not been fully investigated. The CD36 antigen in platelets and monocytes was measured by flow cytometry in 189 patients with various heart diseases; 15 (7.9%) had a diagnosis of CD36 deficiency (type I: 2[1 boy, 1 girl], type II: 13 [6 boys, 7 girls]). The prevalence in each heart disease was as follows: group A (congenital heart disease) 7.6% (9/118, type II: 9 [6 boys, 3 girls]); group B (myocardial disease) 20.0% (3/15, I: 1 girl, II: 2[1 boy, 1 girl]), group C (Kawasaki disease) 4.9% (2/41, II: 2 [1 boy, 1 girl]), group D (arrhythmia): 6.7% (1/15, I: 1 boy). Three patients in group B had transient myocardial damage, which was thought to be related to abnormal myocardial long-chain fatty acid metabolism. The frequency of CD36 deficiency in childhood heart disease was almost identical to that of healthy individuals. Some patients with CD36 deficiency may be susceptible to myocardial damage in the presence of disadvantageous conditions, such as serious infections or massive steroid therapy.

Pediatric heart transplantation.

Heart transplantation is now a treatment option with good outcome for infants and children with end-stage heart failure or complex, inoperable congenital cardiac defects. One-year and 5-year actuarial survival rates are high, approximately 75% and 65%, respectively, with overall patient survival half-life greater than 10 years. To date, survival has been improving as a result of reducing early mortality. Further reductions in late mortality, in part because of graft coronary artery disease and rejection, will allow achievement of the goal of decades-long survival. Quality of life in surviving children, as judged by activity, is usually "normal." Somatic growth is usually at the low normal range but linear growth can be reduced. Of infant recipients, 85% evaluated at 6 years of age or older were in an age-appropriate grade level. Long-term management of childhood heart recipients requires the collaboration of transplant physicians, given the increasing number of immunosuppressive agents and the balance between rejection and infection. Currently, recipients are maintained on immunosuppressive medications that target calcineurin (eg, cyclosporine, tacrolimus), lymphocyte proliferation (eg, azathioprine, mycophenolate mofetil [MMF], sirolimus) and, in some instances antiinflammatory corticosteroids. Emerging evidence now suggests a favorable immunologic opportunity for transplantation in childhood and, conversely, a higher mortality rate in children who have had prior cardiac surgery. Further studies are needed to define age-dependent factors that are likely to play a role in graft survival and possible graft-specific tolerance (eg, optimal conditions for tolerance induction and how immunosuppressive regimens should be changed with maturation of the immune system). As late outcomes continue to improve, the need for donor organs likely will increase, as transplantation affords a better quality and duration of life for children with complex congenital heart disease, otherwise facing a future of multiple palliative operations and chronic heart failure.

Early Childhood Heart Rate Does Not Predict Externalizing Behavior Problems at Age 7 Years

In previous research, low resting heart rate in childhood and adolescence has been shown to predict aggressive and/or delinquent behavior at subsequent ages. It has been found that heart rate recorded as early as age 3 years could predict externalizing behavior at age 11 years. This study explored the possibility of a similar relationship between heart rate and externalizing behavior problems. Heart rate recorded at ages 14, 20, 24, 36 months and 7 years was used to predict combined parental ratings on the Aggressive and Delinquent Behavior scales of the Child Behavior Checklist (CBCL/4-18) as well as the Externalizing composite scale measured at age 7 years. Subjects consisted of same-sex twin pairs, treated as singletons in the present study, participating in the MacArthur Longitudinal Twin Study. Subjects were grouped into high and low heart rate groups and also into high CBCL/4-18 scoring and low CBCL/4-18 scoring groups. Heart rate was not significantly related to scores on either of the 2 subscales or the Externalizing composite scale at any age. Heart rate group membership did not predict CBCL/4-18 scores. Conversely, CBCL/4-18 group membership did not predict heart rate at any age. In this sample, heart rate does not predict externalizing behavior at age 7.

Polyploidy in cardiac myocytes of normal and hypertrophic human hearts; range of values.

Two-wavelength scanning DNA cytophotometry was used for DNA and protein estimation in human ventricular myocytes. In many hypertrophic hearts weighing more than 500 g the DNA content assessed by ploidy of myocytes, was within the range of normal adult variation (4-10c, where c is the haploid DNA content). A correlation was found between the protein content of myocytes and the weight of the hypertrophic ventricle. In congenital heart disease, the excessive polyploidy (up to 15-20c) developed through the normal route of myocyte polyploidization in childhood. Excessive polyploidization was revealed only in overloaded hypertrophied ventricles. A correlation was identified between the ploidy level, the ventricular weight and age of the child. Excessive polyploidy was also detected in adults with congenital or acquired in childhood diseases. There was no correlation between the myocyte ploidy and age. We propose that childhood polyploidy excess persists in these adults. The ranges of polyploidy are compared with the recent data on genome: protein ratio in cardiac myocytes and the interrelationships allow us to discuss the significance of childhood heart polyploidy as a reserve utilised under pathological conditions in adults.

Cost Assessment of the Evaluation of Heart Murmurs in Children

Echocardiography available directly to primary care physicians represents an alternative strategy to pediatric cardiology consultation for childhood heart murmur evaluation. A decision analysis model compared the costs of two diagnostic strategies: (1) echocardiography first, referral to the cardiologist if appropriate; and (2) cardiologist evaluates murmur, echocardiography if appropriate. The model incorporated probability of heart disease, frequency of cardiologist-ordered echocardiography, and echocardiography results established by review of 388 records of consecutive patients evaluated for murmurs in a pediatric cardiology clinic. Echocardiography-first strategy costs were $257 greater than referral-strategy costs. Referral-strategy advantages were not highly sensitive to changes in model assumptions. Pediatric cardiology consultation is the preferred approach provided (1) consultation costs are moderate, (2) echocardiography costs are moderate to high, and (3) the rate at which the cardiologist orders echocardiography for patients with innocent murmurs is low.

Cardiovascular Effects of Extreme Physical Training

After a short historical remark the development of athlete's heart in childhood is described. Within 2 years significant differences were observed between endurance-trained (swimming) and untrained girls and boys determined by X-ray and echocardiographical examinations. The limits of the physiological size in relation to body weight were not exceeded within 10 years of longitudinal studies. A second point deals with athlete's heart from physiological and clinical viewpoints. The largest healthy heart ever found in our examinations of athletes had a size of 1,700 ml. Sixteen years after stopping the active career it was reduced to 950 ml without a pathological finding. Questionable and pathological cases are described. A third chapter covers the blood supply of internal organs during exercise combined with air or oxygen breathing. In this connection liver, kidneys, heart, lungs, and brain have been investigated. The reduced blood supply of the liver and kidneys during intense exercise on the cycle ergometer was not influenced significantly by inspiration of oxygen. A significant blood volume increase of the lungs was noticed during incremental rates of work. Exercise augmented also blood flow of the brain in relation to the work rate (at 100 W a 27% increase in grey matter flow of the right hemisphere). A fourth chapter deals with new hormonal and neurohormonal aspects related to the cardiovascular system. Beta-endorphines remained unchanged at work rates below the anaerobic threshold but increased significantly during maximal rate of work. The opiate antagonist naloxone abolished the rise in body temperature seen during ergometer exercise. The serotonin antagonist ketanserin lowered the blood pressure and the arterial lactic acid level during an incremental exercise test, similar to the results with the dopamine agonist pergolide. The hormone cardiodilatin is produced in the atrial appendages, and it is a potent substance in the regulation of the cardiovascular system. The adaptive reaction of the sympathetic nerve fibres in the myocard revealed different directions: activation, degeneration, and regeneration. These findings correlated highly significantly with the total amount of catecholamines in the heart muscle.

Changes in the cardiovascular system in diseases of the biliary tract in childhood

The question of the influence of lesions of the hepatobiliary system on the activity of the heart in childhood is not sufficiently covered in the literature.

Form and structure of cell nuclei in growing and hypertrophied human hearts

Tissue portions were taken from eight different places in the right and left ventricle from 17 autopsied human hearts (fetuses, children, adults, hypertrophied and atrophic hearts), and sectioned and smear preparations were obtained and stained after Feulgen. Twenty-eight light microscopic features of heart muscle nuclei were identified, and their percent frequency in individual hearts was obtained. The studies showed: 1. Heart muscle nuclei in fetal hearts and in hearts soon after birth show a compact, rounded form in cross section . During childhood heart growth, the nuclei become more and more angular and develop pointed or bizarre projections. In marked heart hypertrophy, almost all heart muscle nuclei assume a bizarre shape and have pointed projections. Compact nuclei are encountered only very occasionally. 2. In longitudinal section , 80% of heart muscle nuclei in fetal hearts have an oval shape, 20%) are rectangular with rounded corners. During childhood heart growth, the nuclei are angular, and the corners frequently form a point. The smaller edges of the nuclei are cleft. Hypertrophied hearts again show more rounded nuclei. 3. The external nuclear membrane during childhood heart growth is either smooth or mildly wavy, in contrast to adult heart nuclei which are grooved. Heart hypertrophy again shows predominantly smooth nuclear membranes. 4. The internal structure of myocardial nuclei (heterochromatin) is granular in the fetal and perinatal periods, whereas it is predominantly netlike in structure during childhood growth phase. Heterochromatin is exclusively coarsely granular in normal, adult hearts, but again finely distributed in a net-like fashion in hypertrophied hearts. Myocardial nuclei from fetuses and children typically show large, round bodies , representing compact DNA aggregates (chromocenters). These are encountered only as isolated findings in normal, adult hearts and in hypertrophied hearts. 5. The length of heart muscle nuclei remains constant at 9.8 μm during physiologic heart growth, and increases during heart hypertrophy to a mean of 19.2 μm. 6. Approximately 10% of heart muscle cells from all hearts contain double nuclei and nuclear sequences . The morphological findings of these multinucleate heart muscle cells suggest amitotic nuclear division and nuclear increase. Shape and structural alterations in heart muscle nuclei permit conclusions to be drawn on the one hand regarding the functional status of the heart muscle, and on the other hand regarding the growth process of the myocardium in physiological heart growth and in pathologic heart hypertrophy. Morphologic evidence is given for amitotic nuclear division as the cause of numerical nuclear hyperplasia in hypertrophied hearts.

Clinical presentation of rhabdomyoma of the heart in infancy and childhood

Four infants with cardiac rhabdomyomas were seen during the past 2 years at Albany Medical Center Hospital; 2 were siblings. The presenting cardiac problems in the 4 infants were congestive heart failure, cyanosis secondary to shunt reversal at atrial level, paroxysmal atrial tachycardia and significant subaortic stenosis, respectively. All 4 patients had tuberous sclerosis. Surgical removal of the tumor was performed in 2. The signs and symptoms produced by myocardial dysfunction secondary to the tumor are reviewed. It is pointed out that the cardiac symptoms produced by rhabdomyoma are not specific and can be produced by other types of cardiac tumors. The possibility of having a second child with rhabdomyoma should not be dismissed. Surgical removal of the tumor should be considered when it is producing significant obstruction of the blood flow on either side of the heart.

Asymmetrical hypertrophy of the heart in childhood: Report of 3 cases

Three children under 6 years of age with the diagnosis of asymmetrical hypertrophy of the heart are described. In 2 patients the diagnosis was confirmed at autopsy, and available clinical data for the third patient indicate this diagnosis. Dysfunction of the myocardium has been the predominant feature. The experience with these cases suggests that the diagnosis may be established clinically with the aid of specific electrocardiographic, angiocardiographic, and radiographic findings.

Characteristics of heart disease seen in a case-finding program.

Introduction The purpose of this paper is to report the characteristics of heart disease observed in the course of a large-scale case-finding program for childhood heart disease in Colorado school children. A secondary purpose is the estimation of the accuracy of diagnosis resulting from a screening type of auscultatory examination of the heart in children. The methodology, 1 preliminary findings as to prevalence, 2 and the results of follow-up studies 3 have been reported. A paper dealing with the relationship of heart disease prevalance to population characteristics 4 is in preparation. Two groups of children were included in the study. The sample was made up of 11,236 children enrolled in the sixth grade of schools in 41 of Colorado's 63 counties. This group of children represents a random sample of sixth graders. The extra sample was composed of 1705 children who did not fall into the regular sample but

Primary tumor of the heart in infancy and early childhood.

Primary tumor of the heart in infancy and early childhood.

Roentgenologic studies of the size of the heart in childhood: I. Three different types of teleroentgenographic changes which occur in acute rheumatic fever

Roentgenologic studies of the size of the heart in childhood: I. Three different types of teleroentgenographic changes which occur in acute rheumatic fever

MEASUREMENTS OF THE SIZE OF THE HEART IN NORMAL CHILDREN

An effort to establish roentgenologic standards for the size of the normal heart in childhood has been made in this investigation by means of a statistical study of the relationship between various measurements of the heart and body. To this end, data have been collected from the literature and combined with some original material. The statistical relationship has been determined between the size of the heart, as measured by both the cardiac surface area and the transverse diameter, and the measurements of body surface area, height, weight and age. MATERIAL FROM THE LITERATURE The material used in this study consists only of those published works on the roentgenologic estimation of the size of the heart which include all of the measurements mentioned. Not all of these publications 1 were suitable for comparison with the original material; therefore, use has been made of the work of only three authors: von Bernuth,

PATHOLOGY OF ACQUIRED HEART DISEASE IN CHILDREN.

When asked to write upon this subject for the Symposium of the Pediatric Society I realized how hard it is to make such a paper more than a mere catalogue of lesions. To avoid this it has seemed better and more profitable to consider the subject in a comparative light, and to take the pathologic changes in the adult as a standard by which to develop the difference in the pathology of the child heart, together with some inquiry as to the causes of these disturbances. With such an object in view it seems best to take up first the conditions of nutrition and disease particularly liable to affect the heart in childhood. NUTRITION OF THE HEART IN CHILDHOOD. It is a general law that the tissues respond more quickly and more readily to stimulation and grow more vigorously in early life than after puberty. A corollary to this is