116 results match your criteria Progress in Pediatric Cardiology [Journal]


Differential Response to Heart Failure Medications in Children.

Prog Pediatr Cardiol 2018 Jun;49:27-30

Division of Cardiology, Department of Medicine, University of Colorado Denver School of Medicine, 12700 E 19 Ave, Aurora, CO USA 80045.

There have been many advances in the treatment of heart failure over the past several years. While these advancements have resulted in improved outcomes in adults with heart failure, these same treatments do not seem to be as efficacious in children with heart failure. Investigations of the failing pediatric heart suggest that there are unique phenotypic, pathologic and molecular differences that could influence how children with heart failure response to adult-based therapies. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2018.01.011DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6023416PMC
June 2018
2 Reads

Noncompaction cardiomyopathy and heterotaxy syndrome.

Prog Pediatr Cardiol 2017 Sep 10;46:23-27. Epub 2017 Jul 10.

Department of Pediatrics, Division of Pediatric Cardiology, Indiana University School of Medicine, Riley Hospital for Children at Indiana University Health, 705 Riley Hospital Drive, Riley Research 127, Indianapolis, IN 46202, United States.

Left ventricular noncompaction cardiomyopathy (LVNC) is characterized by compact and trabecular layers of the left ventricular myocardium. This cardiomyopathy may occur with congenital heart disease (CHD). Single cases document co-occurrence of LVNC and heterotaxy, but no data exist regarding the prevalence of this association. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2017.06.007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5808997PMC
September 2017
5 Reads

Induced Pluripotent Stem Cell-Derived Cardiomyocytes: A Platform for Testing For Drug Cardiotoxicity.

Authors:
Daniel Bernstein

Prog Pediatr Cardiol 2017 Sep 18;46:2-6. Epub 2017 Jul 18.

Department of Pediatrics (Cardiology), Stanford University, Stanford, California USA.

Off-target cardiotoxicity has been a significant impediment to the development of new drugs. Traditional platforms for screening for cardiotoxicity are both overly sensitive and limited in their ability to predict cardiotoxicity that is often only uncovered after years of clinical use. A major impediment has been the lack of a human cardiomyocyte cell line. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2017.07.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5708578PMC
September 2017
8 Reads

Prevention of Sudden Cardiac Death in Children and Young Adults.

Prog Pediatr Cardiol 2017 Jun 21;45:37-42. Epub 2017 Mar 21.

Heart Institute, Cedars-Sinai Medical Center, Los Angeles, CA.

In the present review, we summarize current approaches to the prevention of sudden cardiac death (SCD) in children and young adults, focusing on age less than 35 years. SCD in the young is rare, but devastating from the societal perspective. While coronary artery disease is the main etiology of SCD in the older age groups, conditions such as cardiomyopathies and electrical channelopathies are more likely to be found in the young. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2017.03.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5646418PMC
June 2017
4 Reads

Early extubation in pediatric heart surgery across a spectrum of case complexity: Impact on hospital length of stay and chest tube days.

Prog Pediatr Cardiol 2017 Jun 6;45:63-68. Epub 2016 Dec 6.

Department of Surgery, University of North Carolina School of Medicine; 3041 Burnett-Womack Building; Chapel Hill, NC; 27599; USA.

Early extubation is increasingly common in congenital heart surgery, but there are limited outcomes data across the spectrum of case complexity. We performed a retrospective review of 201 pediatric operations using cardiopulmonary bypass between 2012 and 2014. Patients extubated in the operating room or immediately on arrival to the ICU were compared to those extubated by traditional protocols. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2016.12.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5509209PMC
June 2017
19 Reads

Atlas-Based Ventricular Shape Analysis for Understanding Congenital Heart Disease.

Prog Pediatr Cardiol 2016 Dec 18;43:61-69. Epub 2016 Aug 18.

Department of Bioengineering, University of California San Diego, La Jolla, CA, USA; Department of Medicine, University of California San Diego, La Jolla, CA, USA.

Congenital heart disease is associated with abnormal ventricular shape that can affect wall mechanics and may be predictive of long-term adverse outcomes. Atlas-based parametric shape analysis was used to analyze ventricular geometries of eight adolescent or adult single-ventricle CHD patients with tricuspid atresia and Fontans. These patients were compared with an "atlas" of non-congenital asymptomatic volunteers, resulting in a set of z-scores which quantify deviations from the control population distribution on a patient-by-patient basis. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2016.07.010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5222611PMC
December 2016
10 Reads

PROGRESSION OF CHRONIC KIDNEY DISEASE AFTER ACUTE KIDNEY INJURY.

Prog Pediatr Cardiol 2016 Jun;41:33-40

The Heart Institute, Cincinnati Children's Hospital Medical Center.

The incidence of chronic kidney disease (CKD) in children and adults is increasing. Cardiologists have become indispensable members of the care provider team for children with CKD. This is partly due to the high incidence of CKD in children and adults with congenital heart disease, with current estimates of 30-50%. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2015.12.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4943846PMC
June 2016
5 Reads

Early detection of acute kidney injury after pediatric cardiac surgery.

Prog Pediatr Cardiol 2016 Jun;41:9-16

Division of Nephrology and Hypertension, Cincinnati Children's Hospital Medical Center, United States.

Acute kidney injury (AKI) is increasingly recognized as a common problem in children undergoing cardiac surgery, with well documented increases in morbidity and mortality in both the short and the long term. Traditional approaches to the identification of AKI such as changes in serum creatinine have revealed a large incidence in this population with significant negative impact on clinical outcomes. However, the traditional diagnostic approaches to AKI diagnosis have inherent limitations that may lead to under-diagnosis of this pathologic process. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2016.01.011DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4943672PMC
June 2016
25 Reads

Hypertension in chronic kidney disease: role of ambulatory blood pressure monitoring.

Prog Pediatr Cardiol 2016 Jun 17;41:67-73. Epub 2015 Dec 17.

Division of Pediatric Nephrology and Hypertension, Cincinnati Children's Hospital Medical Center, Cincinnati, OH USA.

Children with chronic kidney disease have a markedly increased risk of cardiovascular morbidity and children with end stage renal disease have an estimated 30 times greater risk of cardiovascular mortality than the general pediatric population. In adults, the link between hypertension and cardiovascular disease is well-documented but that association has not been so readily apparent in children with chronic kidney disease. This may be in part because the early changes in blood pressure that occur in these patients do not necessarily manifest with changes in casual blood pressure measurements. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10589813150009
Publisher Site
http://dx.doi.org/10.1016/j.ppedcard.2015.12.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4915382PMC
June 2016
5 Reads

Integrating Genetics and Medicine: Disease-Modifying Treatment Strategies for Hypertrophic Cardiomyopathy.

Authors:
Carolyn Y Ho

Prog Pediatr Cardiol 2016 Mar;40:21-23

Cardiovascular Division, Brigham and Women's Hospital, 75 Francis Street, Boston, MA 02115, Tel: 617-732-5685.

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http://dx.doi.org/10.1016/j.ppedcard.2016.01.007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4862597PMC
March 2016
7 Reads

Premature Atherosclerotic Cardiovascular Disease in Childhood Cancer Survivors.

Prog Pediatr Cardiol 2015 Nov 17;39(2 Pt A):59-66. Epub 2015 Nov 17.

Department of Pediatrics, University of Minnesota Masonic Children's Hospital, Minneapolis, MN.

Survival rates of childhood cancer have increased over the last 30 years, revealing a population with unique characteristics and risks. The effects of radiation and cardiotoxic chemotherapy predispose these children to both early and late cardiovascular disease. Cranial radiation also increases the likelihood of growth hormone deficiency, which leads to metabolic disturbances. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2015.10.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4725595PMC
November 2015
5 Reads

Mustard baffle obstruction and leak - How successful are percutaneous interventions in adults?

Prog Pediatr Cardiol 2015 Dec 22;39(2 Pt B):157-163. Epub 2015 Oct 22.

Nationwide Children's Hospital Department of Pediatric Medicine & The Ohio State University Department of Internal Medicine, Columbus, OH, USA.

Atrial switch operations for D-Transposition of the great arteries (D-TGA) were performed until the late 20th century. These patients have substantial rates of re-operation, particularly for baffle related complications. This study sought to analyze the efficacy of percutaneous transcatheter intervention (PTI) for baffle leak and/or stenosis in adult atrial switch patients. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2015.10.018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5856449PMC
December 2015
5 Reads

Late Cardiotoxicity in Aging Adult Survivors of Childhood Cancer.

Prog Pediatr Cardiol 2014 Sep;36(1-2):19-26

The University of Tennessee Health Science Center College of Medicine.

The survival rate for childhood cancer is steadily improving, and the current estimate for the prevalence of childhood cancer survivors in the United States is 420,000. With this encouraging trend and the aging of this population, there is an ever-increasing responsibility to identify adult survivors of childhood cancer with adverse health outcomes related to cancer treatment across the span of their lives. To accomplish this, large cohort studies have been developed to follow survivors longitudinally. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2014.09.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4580976PMC
September 2014
4 Reads

Toward Personalized Medicine: Does Genetic Diagnosis of Pediatric Cardiomyopathy Influence Patient Management?

Prog Pediatr Cardiol 2015 Jul;39(1):43-47

Department of Pediatrics and Medical and Molecular Genetics, Indiana University School of Medicine, Indianapolis, IN 46202.

A goal of personalized medicine is to provide increasingly sophisticated, individualized approaches to management and therapy for disease. Genetics is the engine that drives personalized medicine, holding the promise of therapeutics directed toward the unique needs of each patient. The 3 International Conference on Cardiomyopathy in Children provided a forum to discuss the current status of personalized approaches to diagnosis, management, and therapy in the pediatric cardiomyopathy population. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10589813150000
Publisher Site
http://dx.doi.org/10.1016/j.ppedcard.2015.01.008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4568845PMC
July 2015
9 Reads

Cardiomyopathies in Noonan syndrome and the other RASopathies.

Prog Pediatr Cardiol 2015 Jul;39(1):13-19

Dipartimento di Ematologia, Oncologia e Medicina Molecolare, Istituto Superiore di Sanità, Rome, Italy.

Noonan syndrome and related disorders (Noonan syndrome with multiple lentigines, Costello syndrome, cardiofaciocutaneous syndrome, Noonan syndrome with loose anagen hair, and other related traits) are autosomal dominant traits. Mutations causing these disorders alter proteins relevant for signaling through RAS. Thus, these traits are now collectively called the RASopathies. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2015.01.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4568836PMC
July 2015
10 Reads

GENETIC CAUSES OF DILATED CARDIOMYOPATHY.

Prog Pediatr Cardiol 2014 Dec;37(1-2):13-18

Cardiovascular Institute and Adult Medical Genetics, University of Colorado Denver, Aurora, Colorado.

Dilated cardiomyopathy is a disease of the myocardium characterized by left ventricular dilatation and/or dysfunction, affecting both adult and pediatric populations. Almost half of cases are genetically determined with an autosomal pattern of inheritance. Up to 40 genes have been identified affecting proteins of a wide variety of cellular structures such as the sarcomere, the nuclear envelope, the cytoskeleton, the sarcolemma and the intercellular junction. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2014.10.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4288017PMC
December 2014
5 Reads

Patient-Specific Pluripotent Stem Cells in Doxorubicin Cardiotoxicity: A New Window Into Personalized Medicine.

Prog Pediatr Cardiol 2014 Dec;37(1-2):23-27

Departments of Pediatrics and Medicine, Stanford Cardiovascular Institute, Stanford University.

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http://dx.doi.org/10.1016/j.ppedcard.2014.10.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4267060PMC
December 2014
14 Reads

Arrhythmogenic Cardiomyopathy - New Insights into Disease Mechanisms and Drug Discovery.

Prog Pediatr Cardiol 2014 Dec;37(1-2):3-7

Department of Pathology, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA.

Arrhythmogenic cardiomyopathy (ACM) is a primary myocardial disorder characterized by the early appearance of ventricular arrhythmias often out of proportion to the degree of ventricular remodeling and dysfunction. ACM typically presents in adolescence or early adulthood. It accounts for 10% of sudden cardiac deaths in individuals under the age of 18 years. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2014.10.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4260412PMC
December 2014
12 Reads

Transesophageal Echocardiography in Healthy Young Adult Male Baboons (): Normal Cardiac Anatomy and Function in Subhuman Primates Compared to Humans.

Prog Pediatr Cardiol 2013 Aug;35(2):109-120

Cardiac Regenerative Surgery Research Laboratories, Cardiology and Cardiac Surgery of The Ward Family Heart Center, Children's Mercy Hospital, Kansas City, Missouri.

Implantable, viable tissue engineered cardiovascular constructs are rapidly approaching clinical translation. Species typically utilized as preclinical large animal models are food stock ungulates for which cross species biological and genomic differences with humans are great. Multiple authorities have recommended developing subhuman primate models for testing regenerative surgical strategies to mitigate xenotransplant inflammation. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2013.09.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3974204PMC
August 2013
12 Reads

The Role of the Geneticist and Genetic Counselor in an ACHD Clinic.

Prog Pediatr Cardiol 2012 Aug 12;34(1):15-20. Epub 2012 Jul 12.

The Heart Institute, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, 240 Albert Sabin Way, MLC 7020, Cincinnati, OH 45229-3039, USA.

There is a growing population of adults with congenital heart disease due to the advancements in surgical repair and medical management. At the same time, the understanding of the genetic basis of both syndromic and isolated congenital heart disease has grown tremendously and is being rapidly translated into changes in clinical care, resulting in an increasing need for incorporation of genetic expertise into the care of adult congenital heart disease patients. Here we review the importance of delivery of genetic information to the adult congenital heart disease population and highlight the shared and distinct roles of clinical geneticists and genetic counselors in provision of services. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2012.05.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3462440PMC
August 2012
5 Reads

The FDA Review Process for Cardiac Medical Devices in Children: A Review for the Clinician.

Prog Pediatr Cardiol 2012 May 27;33(2):105-109. Epub 2012 Mar 27.

Department of Cardiology, Children's Hospital Boston; Department of Pediatrics, Harvard Medical School, Boston, MA.

Pediatric medical devices play a vital role in the treatment of children with cardiovascular disease. Most cardiac medical devices used in children today are used off-label where the risk-benefit of devices has not been well characterized. Pediatric medical devices face a variety of challenges to FDA approval related in large part to the small target population, heterogeneity of the patient population and ethical considerations of device testing in children. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2012.02.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3363973PMC
May 2012
4 Reads

Opportunities and challenges in linking information across databases in pediatric cardiovascular medicine.

Prog Pediatr Cardiol 2012 Jan;33(1):21-24

Department of Pediatrics, Duke University School of Medicine, and the Duke Clinical Research Institute, Duke University Medical Center, Durham, NC.

Multicenter databases are increasingly utilized in pediatric cardiovascular research. In this review, we discuss the rational for using these types of data sources, provide several examples of how large datasets have been utilized in clinical research, and describe different mechanisms for linking databases to enable studies not possible with individual datasets alone. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2011.12.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3651671PMC
January 2012
4 Reads

Current applications and Future Needs for Biomarkers in Pediatric Cardiomyopathy and Heart Failure: Summary From The Second International Conference On Pediatric Cardiomyopathy.

Prog Pediatr Cardiol 2011 Aug;32(1):11-14

University of Toronto, Labatt Family Heart Centre, Hospital for Sick Children, Toronto, CANADA.

Biomarkers have established an important role in the diagnosis and prognosis of heart failure in adults, with early indications being that their sensitivity and specificity will be similar in the pediatric population. Since early detection of remodeling is vital to preemptive management in cardiomyopathy and in heart disease arising from congenital lesions, biomarkers may offer a means of identifying high risk patients before they develop symptoms. Although in current use, natriuretic peptides high sensitivity troponins, and C-reactive protein need to have their applications more clearly defined in pediatrics, by evidence based guidelines. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2011.06.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3167166PMC
August 2011
5 Reads

Recent clinical and translational research on pediatric myocarditis.

Prog Pediatr Cardiol 2011 Aug;32(1):15-18

Division of Pediatric Cardiology, Department of Pediatrics, Washington University St. Louis, MO, One Children's Place, Department of Pediatrics St. Louis MO, 63110, Campus Box 8116-NWT, United States.

Myocarditis is one of the most common causes of a pediatric dilated cardiomyopathy phenotype. While pediatric myocarditis is generally associated with resolution of myocardial dysfunction, approximately 30% of pediatric myocarditis patients will die or undergo heart transplantation. Cardiac magnetic resonance imaging is increasingly being utilized as the primary diagnositic modality in adult myocarditis. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2011.06.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3649092PMC
August 2011
4 Reads

THE ROLE OF β-ADRENERGIC RECEPTORS IN HEART FAILURE: DIFFERENTIAL REGULATION OF CARDIOTOXICITY AND CARDIOPROTECTION.

Prog Pediatr Cardiol 2011 Jan;31(1):35-38

Division of Pediatric Cardiology, Department of Pediatrics, Stanford University.

β-adrenergic receptor blockers have demonstrated significant survival benefit and have become standard therapy for adults with dilated cardiomyopathy, although their efficacy in pediatric patients is still unproven. Recent data suggests that the two major cardiac β-adrenergic receptor subtypes (β1 and β2) couple differentially to intracellular signaling pathways regulating contractility and remodeling. This has led some to suggest that the β1 receptor is the "cardiotoxic subtype" whereas the β2 receptor is "cardioprotective. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2010.11.007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3135901PMC
January 2011
6 Reads

Genetic diagnosis in pediatric cardiomyopathy: clinical application and research perspectives.

Authors:
Stephanie M Ware

Prog Pediatr Cardiol 2011 May;31(2):99-102

Department of Pediatrics, the Heart Institute, Cincinnati Children's Hospital Medical Center and University of Cincinnati College of Medicine, Cincinnati, OH, 45229-3039.

There has been tremendous growth in the application of genetics to the clinical practice of pediatric cardiomyopathy. The identification of the genetic basis for cardiomyopathies is important for establishing a causal diagnosis, providing definitive identification of at risk family members, and providing cost-effective screening and surveillance. Additional research is needed to better understand the genetic heterogeneity of cardiomyopathy in children, the implications of specific genotypes, the best approach to cardiac surveillance and genetic testing, and the utility of genotyping for individual risk stratification. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2011.02.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3129816PMC
May 2011
4 Reads

The Heart in Friedreich's Ataxia: Basic Findings and Clinical Implications.

Authors:
R Mark Payne

Prog Pediatr Cardiol 2011 May;31(2):103-109

Riley Heart Research Center, Wells Center for Pediatric Research, Indiana University School of Medicine, Indianapolis, IN, 46202.

Friedreich's Ataxia is the most common inherited ataxia in man. It is a mitochondrial disease caused by severely reduced expression of the iron binding protein, frataxin. A large GAA triplet expansion in the human FRDA gene encoding this protein inhibits expression of this gene. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2011.02.007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3117664PMC
May 2011
3 Reads

New Paradigms in Hypertrophic Cardiomyopathy: Insights from Genetics.

Authors:
Carolyn Y Ho

Prog Pediatr Cardiol 2011 May;31(2):93-98

Understanding the genetic basis of hypertrophic cardiomyopathy (HCM) provides a remarkable opportunity to predict and prevent disease. HCM is caused by mutations in sarcomere genes and is the most common monogenic cardiovascular disorder. Although unexplained left ventricular hypertrophy (LVH) is considered diagnostic, LVH is not always present. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2011.02.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3115723PMC
May 2011
6 Reads

Bioenergetics, mitochondria, and cardiac myocyte differentiation.

Prog Pediatr Cardiol 2011 May;31(2):75-81

Department of Pediatrics, University of Rochester Medical Center, 601 Elmwood Ave., Rochester, NY, USA.

Cardiac metabolism is finely tuned, and disruption of myocardial bioenergetics can be clinically devastating. Many cardiomyopathies that present early in life are due to disruption of the maturation of these metabolic pathways. However, this bioenergetic maturation begins well before birth, when the embryonic heart is first beginning to beat, and continues into the mature animal. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2011.02.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3096664PMC
May 2011
11 Reads

The costamere bridges sarcomeres to the sarcolemma in striated muscle.

Prog Pediatr Cardiol 2011 May;31(2):83-88

Department of Medicine, University of California-San Diego, 9500 Gilman Drive, La Jolla, California, 92093, USA.

Costameres are sub-membranous, Z-line associated structures found in striated muscle. They have been shown to have important roles in transmission of force from the sarcomere to the sarcolemma and extracellular matrix, maintaining mechanical integrity of the sarcolemma, and orchestrating mechanically related signaling. The costamere is akin to the more well-known focal adhesion complex present in most cells. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2011.02.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3770312PMC
May 2011
12 Reads

Computational Simulation of the Pulmonary Arteries and its Role in the Study of Pediatric Pulmonary Hypertension.

Prog Pediatr Cardiol 2010 Dec;30(1-2):63-69

Department of Bioengineering, University of Colorado at Denver Anschutz Medical Campus (UCD-AMC), Aurora, CO, USA.

The hemodynamic state of the pulmonary arteries is challenging to routinely measure in children due to the vascular circuit's position in the lungs. The resulting relative scarcity of quantitative clinical diagnostic and prognostic information impairs management of diseases such as pulmonary hypertension, or high blood pressure of the pulmonary circuit, and invites new techniques of measurement. Here we examine recent applications of macro-scale computational mechanics methods for fluids and solids - traditionally used by engineers in the design and virtual testing of complex metal and composite structures - applied to study the pulmonary vasculature, both in healthy and diseased states. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2010.09.008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3076725PMC
December 2010
4 Reads

Rare variant mutations identified in pediatric patients with dilated cardiomyopathy.

Prog Pediatr Cardiol 2011 Jan;31(1):39-47

Miami Institute for Human Genomics, Department of Human Genetics, University of Miami Miller School of Medicine, Miami, FL, USA.

Dilated cardiomyopathy (DCM) in infants and children can be partially explained by genetic cause but the catalogue of known genes is limited. We reviewed our database of 41 cases diagnosed with DCM before 18 years of age who underwent detailed clinical and genetic evaluation, and summarize here the evidence for mutations causing DCM in these cases from 15 genes (PSEN1, PSEN2, CSRP3, LBD3, MYH7, SCN5A, TCAP, TNNT2, LMNA, MYBPC3, MYH6, TNNC1, TNNI3, TPM1, and RBM20). Thirty-five of the 41 pediatric cases had relatives with adult-onset DCM. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2010.11.008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3072577PMC
January 2011
3 Reads

Image-Based Patient-Specific Ventricle Models with Fluid-Structure Interaction for Cardiac Function Assessment and Surgical Design Optimization.

Prog Pediatr Cardiol 2010 Dec;30(1-2):51-62

Mathematical Sciences Department, Worcester Polytechnic Institute, Worcester, MA 01609.

Recent advances in medical imaging technology and computational modeling techniques are making it possible that patient-specific computational ventricle models be constructed and used to test surgical hypotheses and replace empirical and often risky clinical experimentation to examine the efficiency and suitability of various reconstructive procedures in diseased hearts. In this paper, we provide a brief review on recent development in ventricle modeling and its potential application in surgical planning and management of tetralogy of Fallot (ToF) patients. Aspects of data acquisition, model selection and construction, tissue material properties, ventricle layer structure and tissue fiber orientations, pressure condition, model validation and virtual surgery procedures (changing patient-specific ventricle data and perform computer simulation) were reviewed. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2010.09.007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3041970PMC
December 2010
7 Reads

Potential latent effects of prenatal cocaine exposure on growth and the risk of cardiovascular and metabolic disease in childhood.

Prog Pediatr Cardiol 2011 Jan;31(1):59-65

Division of Pediatric Clinical Research, Department of Pediatrics, University of Miami Leonard M. Miller School of Medicine, Miami, FL, USA.

The literature strongly suggests that prenatal exposure to certain medications and substances does not cause major malformations in early childhood. However, these exposures may have far-reaching latent health effects, such as restricted growth, hypertension, and cardiovascular events in adulthood. We reviewed the literature to identify the effects of prenatal cocaine exposure on growth and the risk of cardiovascular and metabolic disease in late adolescence and early adulthood by examining studies that were published in peer-reviewed English-language journals from 1990 through 2009 and indexed in MEDLINE. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2010.11.010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3037026PMC
January 2011
8 Reads

Molecular mechanisms of sarcomere dysfunction in dilated and hypertrophic cardiomyopathy.

Prog Pediatr Cardiol 2011 Jan;31(1):29-33

Departments of Pediatrics, Division of Cardiology, Johns Hopkins University School of Medicine.

The sarcomeres form the molecular motor of the cardiomyocyte and consist of a complex multi-protein of thick and thin filaments which are anchored to the cytoskeleton. The thick filament, composed of myosin and associated proteins, and the thin filament composed of actin, tropomyosin and the troponins develop actinmyosin crossbridges which cycle in response to calcium resulting in sliding of the filaments and contraction. The thin filament in fixed to the cardiomyocyte cytoskeleton at the Z-disc, a complex of structural and regulatory proteins. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2010.11.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3032173PMC
January 2011
6 Reads

In Vitro Simulation and Validation of the Circulation with Congenital Heart Defects.

Prog Pediatr Cardiol 2010 Dec;30(1-2):71-80

Departments of Mechanical Engineering and Bioengineering, Clemson University, Clemson, SC, USA.

Despite the recent advances in computational modeling, experimental simulation of the circulation with congenital heart defect using mock flow circuits remains an important tool for device testing, and for detailing the probable flow consequences resulting from surgical and interventional corrections. Validated mock circuits can be applied to qualify the results from novel computational models. New mathematical tools, coupled with advanced clinical imaging methods, allow for improved assessment of experimental circuit performance relative to human function, as well as the potential for patient-specific adaptation. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2010.09.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3014583PMC
December 2010
4 Reads
4 Citations

AORTIC COARCTATION: RECENT DEVELOPMENTS IN EXPERIMENTAL AND COMPUTATIONAL METHODS TO ASSESS TREATMENTS FOR THIS SIMPLE CONDITION.

Prog Pediatr Cardiol 2010 Dec;30(1):45-49

Department of Biomedical Engineering, Marquette University.

Coarctation of the aorta (CoA) is often considered a relatively simple disease, but long-term outcomes suggest otherwise as life expectancies are decades less than in the average population and substantial morbidity often exists. What follows is an expanded version of collective work conducted by the authors' and numerous collaborators that was presented at the 1st International Conference on Computational Simulation in Congenital Heart Disease pertaining to recent advances for CoA. The work begins by focusing on what is known about blood flow, pressure and indices of wall shear stress (WSS) in patients with normal vascular anatomy from both clinical imaging and the use of computational fluid dynamics (CFD) techniques. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2010.09.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2997687PMC
December 2010
9 Reads

Neuroprotection in Pediatric Cardiac Surgery: What is On the Horizon?

Prog Pediatr Cardiol 2010 Aug;29(2):113-122

Divisions of Pediatric Cardiovascular Anesthesiology and Congenital Heart Surgery, Texas Children's Hospital, and the Departments of Anesthesiology, Pediatrics, and Surgery, Baylor College of Medicine, Houston, Texas.

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http://dx.doi.org/10.1016/j.ppedcard.2010.06.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2928483PMC
August 2010
15 Reads

Effects of congenital heart disease on brain development.

Prog Pediatr Cardiol 2010 Aug;29(2):79-85

Department of Pediatrics, Division of Critical Care, University of California, San Francisco, CA, United States.

Brain and heart development occurs simultaneously in the fetus with congenital heart disease. Early morphogenetic programs in each organ share common genetic pathways. Brain development occurs across a more protracted time-course with striking brain growth and activity-dependent formation and refinement of connections in the third trimester. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2010.06.011DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2927012PMC
August 2010
19 Reads

Clinical Issues in the Pediatric Hypertrophic Cardiomyopathies.

Authors:
Steven D Colan

Prog Pediatr Cardiol 2009 Apr;25(1):27-29

Children's Hospital, Harvard Medical School, Boston, MA.

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http://dx.doi.org/10.1016/j.ppedcard.2007.11.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2723819PMC
April 2009
3 Reads

Towards improving the care of children with pulmonary hypertension: The rationale for developing a Pediatric Pulmonary Hypertension Network.

Prog Pediatr Cardiol 2009 Dec;27(1-2):3-6

Department of Pediatrics, University of Illinois at Chicago, Chicago, IL, United States.

Pulmonary hypertension (PH) and related pulmonary vascular diseases contribute to high morbidity and mortality and treatment options remain limited. Despite the availability of new drug therapies, the long-term outcomes of patients with severe PH remain poor. This may be especially true for many children with PH. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2009.09.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4048728PMC
December 2009
4 Reads

Non-congenital heart disease associated pediatric pulmonary arterial hypertension.

Prog Pediatr Cardiol 2009 Dec;27(1-2):13-23

University of Colorado Denver School of Medicine and The Children's Hospital, United States.

Recognition of causes of pulmonary hypertension other than congenital heart disease is increasing in children. Diagnosis and treatment of any underlying cause of pulmonary hypertension is crucial for optimal management of pulmonary hypertension. This article discusses the available knowledge regarding several disorders associated with pulmonary hypertension in children: idiopathic pulmonary arterial hypertension (IPAH), pulmonary capillary hemangiomatosis, pulmonary veno-occlusive disease, hemoglobinopathies, hepatopulmonary syndrome, portopulmonary hypertension and HIV. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2009.09.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3156479PMC
December 2009
3 Reads

Pulmonary arterial hypertension in children: Diagnostic work-up and challenges.

Prog Pediatr Cardiol 2009 Dec;27(1):4-11

Columbia University, College of Physicians and Surgeons, 3959 Broadway, BH-2 North, New York, NY 10032, United States.

The diagnostic evaluation of a pediatric patient with suspected pulmonary arterial hypertension (PAH) is extensive but essential, given the rapid progression of the disease if left undiagnosed and untreated. The major goals of performing a complete diagnostic work-up are to confirm the diagnosis of PAH, assess disease severity, rule out associated diseases, and begin to formulate an individualized treatment plan for the pediatric patient with pulmonary hypertension. This article will provide a comprehensive review of the diagnostic work-up of the child with suspected PAH as well as a review of some of the challenges faced when assessing a child for PAH. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2009.09.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3117303PMC
December 2009
3 Reads

The Pediatric Cardiomyopathy Registry: 1995-2007.

Prog Pediatr Cardiol 2008 Apr;25(1):31-36

Division of Pediatric Clinical Research, Department of Pediatrics, Miller School of Medicine, University of Miami, Miami, FL.

Cardiomyopathy is a serious disorder of the heart muscle and, although rare, it is potentially devastating in children. Funded by the National Heart Lung and Blood Institute since 1994, the Pediatric Cardiomyopathy Registry (PCMR) was designed to describe the epidemiology and clinical course of selected CMs in patients 18 years old or younger and to promote the development of etiology-specific prevention and treatment strategies. Currently, data from more than 3,000 children with cardiomyopathy have been entered in the PCMR database with annual follow-up continuing until death, heart transplant, or loss-to-follow up. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2007.11.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2408445PMC
April 2008
4 Reads

Epidemiological and Outcomes Research in Children with Pediatric Cardiomyopathy: Discussions from the International Workshop on Primary and Idiopathic Cardiomyopathies in Children.

Prog Pediatr Cardiol 2008 Apr;25(1):23-25

Department of Pediatrics, Miller School of Medicine, University of Miami, Miami, FL.

This report summarizes the roundtable discussion held at the first International Workshop on Primary and Idiopathic Cardiomyopathies in Children which focused on future directions for research on the epidemiology, etiology and outcomes for children with cardiomyopathy. Areas identified as important for future research included: 1)developing a standardized approach to the assessment and follow-up of children with myocarditis; 2) investigating the epidemiology of sudden death in children with dilated cardiomyopathy; 3) identification of biomarkers to serve as surrogate endpoints for important clinical outcomes; 4) the continuation of observational studies like the National Heart, Lung, and Blood Institute-sponsored Pediatric Cardiomyopathy Registry; and 5) conducting randomized clinical trials of pharmacological and behavioral interventions. It was concluded that optimal research strategies should employ a multidisciplinary research team including pediatric cardiologists, epidemiologists, biostatisticians, geneticists, patient care staff and advocacy groups. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2007.11.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2408443PMC
April 2008
5 Reads

Signalosomes as Therapeutic Targets.

Prog Pediatr Cardiol 2008 Apr;25(1):51-56

Cardiac Signal Transduction and Cellular Biology Laboratory, Department of Medicine and Pediatrics, University of Miami Miller School of Medicine, R198, P.O. Box 016960, Miami, FL 33101.

Cardiac hypertrophy is the predominant compensatory response of the heart to a wide variety of biomechanical stressors, including exercise, hypertension, myocardial infarction, intrinsic cardiomyopathy or congenital heart disease. Although cardiac hypertrophy can maintain cardiac output in response to elevated wall stress, sustained cardiac hypertrophy is often accompanied by maladaptive remodeling which can ultimately lead to heart failure. Cultured cardiac myocytes, transgenic and knock-out animal models, and pharmacological studies have not only revealed key molecules involved in hypertrophic signaling, but have also highlighted the redundancy in the hypertrophic signaling cascade. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2007.11.012DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2390861PMC
April 2008
9 Reads

Pediatric Cardiomyopathy as a Chronic Disease: A Perspective on Comprehensive Care Programs.

Prog Pediatr Cardiol 2008 ;25(1):103-111

Department of Pediatrics, Leonard M. Miller School of Medicine, University of Miami.

Substantial numbers of children with cardiomyopathy are now surviving into adulthood, making it essentially a chronic disease. As a chronic condition, it may be best treated through comprehensive, multidisciplinary treatment programs. Such programs have improved health outcomes and reduced costs in managing other pediatric chronic diseases and heart failure in adults, but the treatment and cost implications of programs for managing pediatric cardiomyopathy are unknown. Read More

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http://dx.doi.org/10.1016/j.ppedcard.2007.11.011DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2515555PMC
January 2008
3 Reads