Cardio-renal consequences of low birth weight and preterm birth
In this comprehensive review of cardio-renal syndrome in preterm, low birth weight infants, we present contemporary evidence for alterations in nephrogenesis and vasculogenesis among this vulnerable population. There is substantial evidence that the nephron endowment, the number of nephrons that an individual is allotted for a lifetime, is determined by the length of gestation, the intra-uterine environment and the brief, but significant, postnatal course. Since nephrogenesis is complete by 36weeks' gestation in utero, preterm birth during active nephrogenesis predisposes to an abrupt cessation in development leading to a ...
Source: Progress in Pediatric Cardiology - January 27, 2016 Category: Cardiology Authors: Marissa J. DeFreitas, Chryso P. Katsoufis, Carolyn L. Abitbol Tags: Review Source Type: research

Early detection of acute kidney injury after pediatric cardiac surgery
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. (Source: Progress in Pediatric Cardiology)
Source: Progress in Pediatric Cardiology - January 26, 2016 Category: Cardiology Authors: John Lynn Jefferies, Prasad Devarajan Tags: Review Source Type: research

Cardio-renal consequences of low birth weight and preterm birth
In this comprehensive review of cardio-renal syndrome in preterm, low birth weight infants, we present contemporary evidence for alterations in nephrogenesis and vasculogenesis among this vulnerable population. There is substantial evidence that the nephron endowment, the number of nephrons that an individual is allotted for a lifetime, is determined by the length of gestation, the intra-uterine environment and the brief, but significant, postnatal course. Since nephrogenesis is complete by 36weeks' gestation in utero, preterm birth during active nephrogenesis predisposes to an abrupt cessation in development leading to a ...
Source: Progress in Pediatric Cardiology - January 26, 2016 Category: Cardiology Authors: Marissa J. DeFreitas, Chryso P. Katsoufis, Carolyn L. Abitbol Tags: Review Source Type: research

The role of continuous flow ventricular assist device for destination therapy in children: Can it work or is it a bridge too far?
The field of mechanical circulatory support for children has shown tremendous growth and evolution over the last decade. This is in part due to the worldwide epidemic of heart failure in adults—a prevalence of 23 million and counting [1,2] feeding the burgeoning market for ventricular assist device (VAD) design and development. Device design in children has been markedly more challenging due to the biotechnical demands of device miniaturization and the limited market field in children compared to adults. (Source: Progress in Pediatric Cardiology)
Source: Progress in Pediatric Cardiology - January 18, 2016 Category: Cardiology Authors: Christina J. VanderPluym, Elizabeth D. Blume Tags: Review Source Type: research

Optimizing Noninvasive Approaches to Rejection Surveillance in Cardiac Allograft Recipients
Optimizing immune suppression so as to balance the risk of antibody-mediated and cellular rejection vs. the risk of opportunistic infections is one of the primary management goals in cardiac transplant recipients. Routine surveillance transcatheter endomyocardial biopsy (EMB) remains a key element of achieving this goal for most pediatric cardiac programs despite years of effort pursuing alternative methods. The cost, risk, and clinical burden of this procedure are well documented. The risk of serious adverse events during EMB for pediatric transplant recipients has been evaluated in a multicenter study of 2655 biopsies in...
Source: Progress in Pediatric Cardiology - January 18, 2016 Category: Cardiology Authors: Steven D. Colan, Fatima I. Lunze, Tajinder P. Singh Tags: Review Source Type: research

Titin truncating mutations: A rare cause of dilated cardiomyopathy in the young
Truncating mutations in the TTN gene are the most common genetic cause of dilated cardiomyopathy in adults but their role in young patients is unknown. We studied 82 young dilated cardiomyopathy subjects and found that the prevalence of truncating TTN mutations in adolescents was similar to adults, but surprisingly few truncating TTN mutations were identified in affected children, including one confirmed de novo variant. In several cases, truncating TTN mutations in children with dilated cardiomyopathy had evidence of additional clinical or genetic risk factors. (Source: Progress in Pediatric Cardiology)
Source: Progress in Pediatric Cardiology - January 18, 2016 Category: Cardiology Authors: Diane Fatkin, Lien Lam, Daniel S. Herman, Craig C. Benson, Leanne.E. Felkin, Paul J.R. Barton, Roddy Walsh, Sukru Candan, James S. Ware, Angharad M. Roberts, Wendy K. Chung, Leslie Smoot, Helen Bornaun, Anne M. Keogh, Peter S. Macdonald, Christopher S. Ha Tags: Review Source Type: research

The role of continuous flow ventricular assist device for destination therapy in children: ?
The field of mechanical circulatory support for children has shown tremendous growth and evolution over the last decade. This is in part due to the worldwide epidemic of heart failure in adults – a prevalence of 23 million and counting [1,2] feeding the burgeoning market for ventricular assist device (VAD) design and development. Device design in children has been markedly more challenging due to the biotechnical demands of device miniaturization and the limited market field in children compared to adults. (Source: Progress in Pediatric Cardiology)
Source: Progress in Pediatric Cardiology - January 18, 2016 Category: Cardiology Authors: Christina J. VanderPluym, Elizabeth D. Blume Tags: Review Source Type: research

Future research directions in pediatric cardiomyopathy
Cardiomyopathy is a rare but serious disorder of the heart muscle, resulting in some of the worst pediatric cardiology outcomes, and it remains the leading cause of heart transplantation in children over one year of age [1–5]. Nearly 40% of children who present with symptomatic cardiomyopathy receive a heart transplant or die within the first 2years, and the percentage of children receiving transplants has not declined over the past 10years [5,6]. Studies in both the United States and Australia have shown that the incidence of pediatric cardiomyopathy is approximately 1 per 100,000 children and 8–12 times higher in the...
Source: Progress in Pediatric Cardiology - January 18, 2016 Category: Cardiology Authors: Steven E. Lipshultz, Wendy K. Chung, Jeffrey A. Towbin, Elfriede Pahl, Melanie D. Everitt, Charles E. Canter, John L. Jefferies, Joseph W. Rossano, Paul F. Kantor, Steven A. Webber, Daphne T. Hsu, Jason D. Czachor, James D. Wilkinson, for the Pediatric Ca Tags: Review Source Type: research

Pediatric cardiac transplantation for Non-dilated cardiomyopathies
Cardiomyopathy is the leading diagnosis for pediatric heart transplantation in children over 1year of age. The majority of these patients usually have dilated cardiomyopathy. Children with hypertrophic and restrictive types of cardiomyopathy comprise less than 20% of the total children transplanted. Hypertrophic cardiomyopathy is the smallest subgroup to receive a cardiac transplant (6%). The mean age at listing in the Pediatric Heart Transplant Study (PHTS) was 7.6years, however, 39% were infants listed at a mean of 3.2+2.8months of age. (Source: Progress in Pediatric Cardiology)
Source: Progress in Pediatric Cardiology - January 16, 2016 Category: Cardiology Authors: Linda J. Addonizio Tags: Review Source Type: research

From Partnership to Progress in the Field of Pediatric Cardiomyopathy
Founder & Executive Director, Children's Cardiomyopathy Foundation (Source: Progress in Pediatric Cardiology)
Source: Progress in Pediatric Cardiology - January 15, 2016 Category: Cardiology Authors: Lisa Yue Tags: Review Source Type: research

Quality Improvement in Pediatric Heart Failure
Quality improvement science permeates the culture of health care. Infrastructure and systematic changes have been instituted with the ultimate goal of improved care delivery at reduced expenditures. Unfortunately, the scope and status of pediatric heart failure quality improvement cannot be accurately assessed as there is a paucity of publications in the literature. Nonetheless, there are models in health care that exist as is evidenced by the American College of Cardiology and American Heart Association approved process measures for quality improvement in heart failure in the adult population. (Source: Progress in Pediatric Cardiology)
Source: Progress in Pediatric Cardiology - January 14, 2016 Category: Cardiology Authors: Timothy M. Hoffman Tags: Review Source Type: research

Echocardiographic Endpoints in Pediatric Cardiomyopathy and Heart Failure Trials
In clinical research, the desired result of an intervention typically involves improvement in one or more of the following clinically meaningful outcomes: increasing long-term survival, reducing risk of hospitalization/re-hospitalization, improving quality of life, and improving functional status. The very nature of these long-term, clinically meaningful outcomes limits their use in the research setting, however, and many studies rely on surrogate endpoints or composite endpoints which combine the two. (Source: Progress in Pediatric Cardiology)
Source: Progress in Pediatric Cardiology - January 14, 2016 Category: Cardiology Authors: Renee Margossian Tags: Review Source Type: research

Integrating Genetics and Medicine: Disease-Modifying Treatment Strategies for Hypertrophic Cardiomyopathy
Hypertrophic cardiomyopathy (HCM) was the first inherited cardiomyopathy characterized at the molecular level [1,2]. Approximately 25years have elapsed since landmark genetic studies demonstrated that sarcomere mutations cause disease. In that time, genetic testing has evolved from residing strictly in the realm of research performed at a small number of laboratories, to becoming a commercially-available test used in clinical practice. Genetic testing now routinely identifies disease-causing (pathogenic) sarcomere mutations in patients with HCM. (Source: Progress in Pediatric Cardiology)
Source: Progress in Pediatric Cardiology - January 14, 2016 Category: Cardiology Authors: Carolyn Y. Ho Tags: Review Source Type: research

Comparison of pulmonary arterial wall properties between a congenital heart disease patient and a normal subject using pressure-diameter measurements: A feasibility study
Patients with congenital heart disease (CHD) requiring pulmonary valve and right ventricular intervention often suffer from residual lesions including pulmonary regurgitation or pulmonary artery (PA) obstruction after the placement of a patch or artificial conduit during surgery. These residual lesions can result in significant changes in the structure and function of the right ventricle (RV) and PA. Characterization of the material properties such as stress, strain, and compliance of the PA provides a quantitative assessment of RV-PA dysfunction and may aid in the longitudinal assessment of CHD patients. (Source: Progress...
Source: Progress in Pediatric Cardiology - January 12, 2016 Category: Cardiology Authors: Rajit Banerjee, Gavin A. D'Souza, Namheon Lee, Michael D. Taylor Source Type: research

Comparison of pulmonary arterial wall properties between a congenital heart disease patient and a normal subject using in vivo pressure–diameter measurements: A feasibility study
Patients with congenital heart disease (CHD) requiring pulmonary valve and right ventricular intervention often suffer from residual lesions including pulmonary regurgitation or pulmonary artery (PA) obstruction after the placement of a patch or artificial conduit during surgery. These residual lesions can result in significant changes in the structure and function of the right ventricle (RV) and PA. Characterization of the material properties such as stress, strain, and compliance of the PA provides a quantitative assessment of RV–PA dysfunction and may aid in the longitudinal assessment of CHD patients. (Source: Progre...
Source: Progress in Pediatric Cardiology - January 11, 2016 Category: Cardiology Authors: Rajit Banerjee, Gavin A. D'Souza, Namheon Lee, Michael D. Taylor Source Type: research