Tricuspid Valve Size Relationship to Right Ventricular Volume in Post-operative Tetralogy of Fallot Patients.

In post-operative Tetralogy of Fallot (TOF) patients cMRI data are used for determining right ventricle (RV) dilation to justify the pulmonary valve placement. Echocardiogram analysis of RV volumes has inherent accuracy issues. There are no data exploring the relationship of transthoracic echocardiography tricuspid valve (TV) annulus size and cMRI RV dilation. Retrospective study in post-operative TOF patients. Patients less than 21 years old and with high-quality transthoracic echocardiogram and MRI within 6 months of each other were included. We collected demographic data, type of initial repair, echocardiographic, and cMRI data. A single reviewer measured echocardiogram TV size. Boston z-scores were used. 77 echocardiogram and cMRI pairs were analyzed. Average age was 14 years old. There is not a strong linear relationship between TV z-score and indexed right ventricular end diastolic volume (RVEDVi) (R2 = 0.31). Twenty patients had RVEDVi's of greater than 150 ml/m2. From those 20 patients, 9 patients had a TV z-score of > 2. No patient had a TV z-score > 2 and a normal RVEDVi. For a TV z-score < 2, there was a positive predictive value of 100%, a negative predictive value of 83%, specificity of 100%, and sensitivity of 45%. In our study population, the TV z-score is not sufficient to predict RV dilation. However, TV z-score > 2 has a high positive predictive value for an RVEDVi of > 150 ml/m2. This would suggest that a cMRI should be done if the TV z-score is > 2 on echocardiography.

Use of C-Reactive Protein and Ferritin Biomarkers in Daily Pediatric Practice.

Recent pediatric clinical research has begun to focus on risk stratification tools using multibiomarker models. C-reactive protein (CRP) and ferriti biomarkers are widely available and used to varying degrees in daily practice, but there is no single source examining the evidence behind their use.We set out to summarize the evidence behind the use of CRP and ferritin biomarkers in pediatric practice and to begin development of a consensus for their future use for pediatricians.All the literature involving CRP and ferritin in pediatrics available on PubMed was surveyed. Research applicable to daily pediatric practice was summarized in the body of the article. Pediatric clinicians of various subspecialties contributed to the summary of the use of CRP and ferritin biomarkers in clinical practice in various disease processes. A clinical decision pathway is described, and evidence is summarized.CRP and ferritin biomarkers have diverse uses with various cutoff values in the literature, making their use in daily practice difficult. Elevation of these markers coincides with their significant elevation in uncontrolled inflammation.CRP and ferritin biomarkers are widely used in pediatrics. This review provides a resource summarizing evidence into a single source. There is sufficient evidence to indicate that these biomarkers of inflammation can be useful in guiding clinical decision making in specific clinical scenarios; however, further work is needed to improve their use in clinical practice.

Cardiovascular risk reduction in high-risk pediatric patients: a scientific statement from the American Heart Association Expert Panel on Population and Prevention Science; the Councils on Cardiovascular Disease in the Young, Epidemiology and Prevention, Nutrition, Physical Activity and Metabolism, High Blood Pressure Research, Cardiovascular Nursing, and the Kidney in Heart Disease; and the Interdisciplinary Working Group on Quality of Care and Outcomes Research: endorsed by the American Academy of Pediatrics.

Although for most children the process of atherosclerosis is subclinical, dramatically accelerated atherosclerosis occurs in some pediatric disease states, with clinical coronary events occurring in childhood and very early adult life. As with most scientific statements about children and the future risk for cardiovascular disease, there are no randomized trials documenting the effects of risk reduction on hard clinical outcomes. A growing body of literature, however, identifies the importance of premature cardiovascular disease in the course of certain pediatric diagnoses and addresses the response to risk factor reduction. For this scientific statement, a panel of experts reviewed what is known about very premature cardiovascular disease in 8 high-risk pediatric diagnoses and, from the science base, developed practical recommendations for management of cardiovascular risk.

Cardiovascular risk reduction in high-risk pediatric patients: a scientific statement from the American Heart Association Expert Panel on Population and Prevention Science; the Councils on Cardiovascular Disease in the Young, Epidemiology and Prevention, Nutrition, Physical Activity and Metabolism, High Blood Pressure Research, Cardiovascular Nursing, and the Kidney in Heart Disease; and the Interdisciplinary Working Group on Quality of Care and Outcomes Research.

Although for most children the process of atherosclerosis is subclinical, dramatically accelerated atherosclerosis occurs in some pediatric disease states, with clinical coronary events occurring in childhood and very early adult life. As with most scientific statements about children and the future risk for cardiovascular disease, there are no randomized trials documenting the effects of risk reduction on hard clinical outcomes. A growing body of literature, however, identifies the importance of premature cardiovascular disease in the course of certain pediatric diagnoses and addresses the response to risk factor reduction. For this scientific statement, a panel of experts reviewed what is known about very premature cardiovascular disease in 8 high-risk pediatric diagnoses and, from the science base, developed practical recommendations for management of cardiovascular risk.

Limited efficacy of growth hormone (GH) during transition of GH-deficient patients from adolescence to adulthood: a phase III multicenter, double-blind, randomized two-year trial.

CONTEXT: Treatment of GH-deficient adolescents in transition to adulthood remains challenging. OBJECTIVE: The objective was to assess the safety and efficacy of GH in GH-deficient adolescents in transition. PATIENTS: Fifty-eight GH-deficient adolescents (mean age, 15.8 +/- 1.8 yr; 33 males) at near completion of their linear growth participated in the study. INTERVENTION: Baseline studies were done while subjects were on GH. Subjects were retested (insulin-induced hypoglycemia) 4 wk after GH discontinuation and reclassified as persistently GH-deficient or controls (n = 18). GH-deficient subjects were randomized to GH (n = 25, approximately 20 microg/kg.d) or placebo (n = 15). SETTING: The multicenter study was conducted over a 2-yr period. MAIN OUTCOMES: Changes in body composition, bone mineral density (BMD), quality of life (QOL), cardiovascular and metabolic markers were measured. RESULTS: All groups had normal measures of lipid and carbohydrate metabolism, body composition, BMD, cardiac function, muscle strength, and QOL at baseline and after 2 yr. IGF-I concentrations decreased in all, but less so in the GH-group (P = 0.013). There was a greater increase in lean body mass (lesser adiposity) in the GH group than placebo at 12 months, but not at 24 months. CONCLUSIONS: 1) GH-deficient patients properly treated in childhood can have normal BMD, body composition, cardiac function, muscle strength, carbohydrate and lipid metabolism, and QOL when reaching adult height; and 2) continuation of GH therapy for 2 yr did not change these measures as compared to placebo-treated or control subjects. GH-deficient adolescents in good metabolic status at the time of epiphyseal fusion may safely discontinue GH for at least 2 yr. Follow-up is needed to determine whether GH therapy is eventually warranted in subjects treated with GH during childhood.

Outcome of staged surgical approach to neonates with single left ventricle and moderate size bulboventricular foramen.

Neonates with double-inlet left ventricle or tricuspid atresia with transposed great arteries and a bulboventricular foramen (BVF) area <2 cm(2)/m(2) develop BVF obstruction. This study examined the outcome of neonates with BVF area between 1 and 2 cm(2)/m(2) whose BVF was bypassed after the neonatal period. We reviewed 29 neonates with double-inlet left ventricles (n = 18) or tricuspid atresia (n = 11) and transposed great arteries. The study group consisted of 9 patients with neonatal BVF areas of 1 to 2 cm(2)/m(2) who did not undergo repair of the BVF obstruction as a neonate. The comparison group consisted of 8 "ideal" patients without BVF obstruction. Precavopulmonary shunt data from cardiac catheterization and echocardiogram and outcomes of the cavopulmonary shunt were compared. Study group patients developed a mild BVF gradient (18 +/- 10 mm Hg by cardiac catheterization) by a mean of 7 months. Left ventricular wall thickness, however, remained in the normal range (4.2 +/- 0.3 mm) and was not statistically different from the comparison group (4.1 +/- 0.4 mm). No difference was found in the precavopulmonary mean pulmonary artery pressure (15 +/- 5 vs 15 +/- 6 mm Hg), transpulmonary gradient (8 +/- 4 vs 8 +/- 5 mm Hg), and left ventricular end-diastolic pressure (7 +/- 2 vs 8 +/- 3 mm Hg). One patient in the study group died from respiratory syncytial virus pneumonia while awaiting cavopulmonary shunt. Neither group had mortality from the cavopulmonary shunt. The lengths of hospital stay were comparable (8.3 +/- 3.7 vs 8.9 +/- 6.0 days). Thus, neonates with BVF area between 1 and 2 cm(2)/m(2) develop mild but hemodynamically insignificant BVF gradient by 7 months of age. This group of patients can be managed safely with relief of BVF obstruction later in infancy.

Pediatric echocardiography laboratory organization and clinical productivity.

BACKGROUND: The American Society of Echocardiography's Committee on Pediatric Echocardiography Laboratory Productivity (C-PELP) was formed to study the organizational and productivity issues particular to academic pediatric echocardiography laboratories. After much deliberation, the committee chose studies per physician full-time equivalent per day --the average number of studies interpreted per day by a full-time echocardiography physician dedicated to the laboratory -as the primary measure of physician productivity. METHODS: A survey was sent to 74 North American pediatric echocardiography laboratory directors. The aims of the survey were to (1) determine the annual laboratory volume and types of echocardiographic studies performed, (2) define the average number of studies performed by a pediatric cardiac sonographer in a year, (3) assess the productivity of echocardiography physicians, and (4) identify factors (programmatic or laboratory related) that affect clinical productivity. RESULTS: There were 54 responses to the C-PELP 2011 survey. The average number of studies per physician full-time equivalent per day was 15.0 + 4.5 (median, 13.8; range, 6.2 -27.1), and the average number of studies performed per year by a sonographer was 1,297 + 326 (median, 1,279; range 717 -2,475). These figures were not adjusted for case complexity, time requirement for transesophageal echocardiography, level of expertise, or availability of sonographer assistance. Moreover, the issues of study quality and accuracy were not addressed. CONCLUSIONS: The C-PELP 2011 survey gathered important information on the current organization and staffing of academic pediatric echocardiography laboratories, but the committee did not attempt to craft guidelines or recommendations on staffing requirements. The results of the survey, however, should provide a framework for additional investigation into the optimal structure and staffing of pediatric echocardiography laboratories.

Results of aortic valve-sparing and restoration with autologous pericardial leaflet extensions in congenital heart disease.

BACKGROUND: The purpose of this study is to evaluate the efficacy of aortic valve-sparing repair with glutaraldehyde-treated autologous pericardium in congenital valvular pathology. METHODS: Sixty-two patients underwent reparative aortic valve surgery from January 1997 through December 2003. The mean age was 25 +/- 20 years (+/- standard deviation) (range, 10 days to 81 years). Fifty percent (31 of 62) were less than 19 years old at operation. The diagnoses included bicuspid aortic valve (39 patients), ventricular septal defect (14 patients), severe aortic stenosis (6 patients), subaortic stenosis (7 patients), bacterial endocarditis (7 patients), neonatal truncus arteriosus (2 patients), Shone's complex (2 patients), transposition complex (1 patient), double-chambered right ventricle (1 patient), and Marfan's syndrome (1 patient). Twelve patients (19 %) had prior sternotomy and cardiac operations. Valve-sparing techniques included pericardial leaflet extensions in 62 patients, creation of one or more pericardial neoaortic sinuses in 8, subcommissuroplasty in 8, pericardial patch of perforated leaflets in 9, Dacron mesh wrap (Boston Scientific, Wayne, NJ) of dilated ascending aorta in 12, and concomitant tricuspid and mitral valve repairs in 3 and 4 patients, respectively. RESULTS: There was one early death (1.6%). There were no late deaths at a mean follow-up of 25 +/- 16 (range, 0.1 to 72.5 months). Six patients required reoperation and prosthetic or homograft replacement for aortic valve incompetence. One out of 6 reoperations required re-repair. The remaining patients are well with a mean aortic regurgitation grade by echocardiography of 1.3 +/- 0.9 (scale, 0 to 4). CONCLUSIONS: Aortic valve repair with pericardial leaflet extension is a promising technique for the growing child.