SCOTTSDALE, Ariz., Feb. 24, 2018 /PRNewswire-USNewswire/
-- Experts in pediatric heart disease from multiple centers
worldwide are sharing their findings at a large national conference
sponsored by the Cardiac Center at Children's Hospital of
Philadelphia (CHOP). Cardiology
2018, the 21th Annual Update on Pediatric and Congenital
Cardiovascular Disease, meets Feb. 21 to
Feb. 25 in Scottsdale.
Conference organizers honored eight clinical researchers with the
Outstanding Investigator Award. The following news briefs highlight
their presentations in congenital heart disease.
Frailty Measurements May Offer Clinical Tool to Assess
Pediatric Cardiology Patients
The researchers assessed whether the Fried frailty phenotype,
well-studied in the elderly as a measure of global infirmity, could
be adapted to assess children and adolescents with significant
cardiac disease. This pilot study compared 34 pediatric heart
patients with 22 age- and sex-matched healthy controls in five
domains of frailty. The heart patients performed significantly
worse in all five domains, specifically in the 6-minute walk test,
handgrip strength, body composition (height and weight),
self-reported exhaustion and physical activity. The results support
the relevance of frailty phonotype to assess global infirmity in
this pediatric population. Longitudinal studies should further
investigate whether frailty predicts adverse health outcomes in
these patients.
Chaitanya Panchangam, et al,
Children's Mercy Hospital; Translation of the Frailty Paradigm from
Older Adults to Children with Cardiac Disease: A Pilot
Study (abstract 63)
In Public Reporting for Pediatric Heart Surgery Outcomes,
Higher Rated Programs Do Not Reflect Higher Volume or More Complex
Cases
This team probed the relationship between case volume and
complexity and the Star Rating system established by the Society of
Thoracic Surgeons (STS) to report patient outcomes in congenital
heart surgery. Using data from the STS Congenital Heart Surgery
Public Reporting website, the study team investigated case volume,
mortality, and case complexity for 2013 through 2016. The STS
system assigns a Star Rating to each institution based on the
number of observed deaths divided by the number of expected deaths
for an institution's case mix. A 3 Star Rating implies that a
center performed better than expected for its case-mix; a 1 Star
Rating implies that it performed worse than expected. The study
team assessed case complexity using five categories that group
procedures by risk level. When the team compared overall case
volume and case complexity among 69 institutions, they found that 3
Star programs performed fewer cases, and especially fewer
high-complexity cases, than did the high-volume centers.
Andrea Kennedy, et al,
Children's Hospital of Philadelphia; Relationship of Institutional
Case Volume and Case Complexity to the STS Star Rating
System (abstract 98)
After Fontan Surgery, Taller Patients Have Better Survival
and Quality of Life
This multicenter, longitudinal study
evaluated height, body mass index (BMI) and associated quality of
life and exercise capacity among survivors of Fontan surgery
enrolled in the Pediatric Heart Network Fontan Longitudinal
Studies. Researchers compared height and BMI, taken at 3 time
points in Fontan patients, with gender specific population norms.
The first-time point (F1) included 536 patients, aged 11.9 plus or
minus 3.4 years; the third-time point (F3) included 362 patients
aged 21.1 plus or minus 3.5 years. Fontan survivors are shorter
compared to the normal population, but have similar BMI.
Shorter subjects had worse survival,
while survivors with who were taller had better quality
of life and exercise capacity compared to survivors with shorter
stature. Survivors with higher BMI had lower quality of life and
exercise capacity than those with lower BMI. Among Fontan patients,
height was most affected in those with single right ventricles.
Linda M. Lambert, et al,
University of Utah; Longitudinal Study
of Anthropometry in Fontan Survivors: PHN Fontan Study
(abstract 56)
Thromboelastography Testing May Help Predict Bleeding
Risk in Children on ECMO
Better laboratory tools are needed to predict the risk of
bleeding in children receiving extracorporeal membrane
oxygenation (ECMO) for severe heart failure. Currently, adult
anticoagulation guidelines are extrapolated to children, but there
are age-dependent physiologic differences in hemostasis. This
single-center clinical data collection investigated the
use of thromboelastography (TEG) in 40 infants supported
by ECMO. TEG, currently more commonly used in
adults, measures the physical properties of blood clot
formation. The researchers found that TEG maximum
amplitude and reaction time, along with fibrinogen
concentration, can jointly predict bleeding risk in children on
ECMO support. Further studies in larger samples are needed to
confirm the specific thresholds identified for four risk
strata.
Lynn Sleeper, et al, Boston
Children's Hospital; Thermoelastography Testing Provides Effective
Risk
Stratification for Bleeding in Patients on
Extracorporeal Membrane Oxygenation Support (abstract
74)
Inflammatory Cytokine Levels Are High in Pleural Fluid after
Fontan Surgery
Prolonged pleural drainage from chest tubes
is a common complication after the Fontan procedure, and is linked
to short- and long-term morbidities. This single-center cohort
study was the first to measure inflammatory cytokines in pleural
fluid. The study team compared 25 Fontan patients to 15 age-matched
controls (bi-ventricular patients undergoing cardiopulmonary
bypass). Researchers found that inflammatory cytokines in Fontan
patients' pleural fluid increased out of proportion compared to the
controls. The increased cytokine levels may perpetuate prolonged
pleural drainage. Further study is needed to investigate the
mechanisms of the pleural inflammatory response, and to determine
whether pleural inflammation is the cause or the result of the
prolonged chest tube drainage.
Stephanie Goldstein, et al,
University of Michigan; Analysis of
Inflammatory Cytokines in Postoperative Fontan Pleural
Drainage (abstract 22)
Thoracic Duct Pressure May Be Elevated in Fontan
Patients
A subset of children who undergo Fontan surgery for
single-ventricle disease have rare complications in the lymphatic
system that are associated with altered lymphatic circulation. This
retrospective, single-center study investigated thoracic duct (TD)
physiology in the setting of chronically-elevated central venous
pressure. The research team analyzed direct TD pressure
measurements in 29 Fontan patients who presented for lymphatic
interventions. Overall, there was a significant positive
correlation between higher Fontan pressures and higher TD
pressures. Individually, about half of the patients had a TD
pressure that was higher than their own Fontan pressure at
baseline. In addition, there was a significant increase in TD
pressure and evoked pulsatility with acute TD outlet occlusion,
which may have implications for outcomes in patients who undergo
lymphatic interventions. Further research should focus on the
relationship between TD pressure and the severity and/or chronicity
of clinical lymphatic complications. The authors recommend that
follow-up studies investigate the TD pressure in Fontan patients
without clinically-apparent lymphatic abnormalities.
Jill Savla, et al, Children's
Hospital of Philadelphia; Direct
Measurement of Thoracic Duct Pressure in Patients with Fontan
Physiology (abstract 64)
Multicenter Study Reveals Patient Outcomes, Risk Factors in
Truncus Arteriosus Repair
This retrospective study performed the first known multicenter
analysis of intermediate-term outcomes in children who underwent
truncus arteriosus (TA) repair. The analysis included 216 children
at 15 U.S. centers who had TA repair without concomitant arch
obstruction from 2009-2016. Median follow-up was 2.9 years. Overall
mortality was 13 percent, with the majority of deaths occurring in
the first year of life. Risk factors for overall mortality were
DiGeorge syndrome, preoperative mechanical ventilation,
postoperative ECMO, delayed sternal closure and postoperative
infection. In this cohort, 49 percent of patients required at least
one right ventricle to pulmonary artery (RV-PA) conduit
intervention during the study period, frequently in early
childhood. The probability of RV-PA conduit intervention was
significantly higher (approximately two-fold) for aortic and
pulmonary homografts than for Contegra conduits.
Jason Buckley, et al,
Medical University of South Carolina;
Early Childhood Outcomes Following Repair of Truncus Arteriosus: A
Contemporary Multicenter Analysis (abstract 12)
About Children's Hospital of Philadelphia: Children's Hospital of
Philadelphia was founded in 1855
as the nation's first pediatric hospital. Through its long-standing
commitment to providing exceptional patient care, training new
generations of pediatric healthcare professionals and pioneering
major research initiatives, Children's Hospital has fostered many
discoveries that have benefited children worldwide. Its pediatric
research program is among the largest in the country. In addition,
its unique family-centered care and public service programs have
brought the 535-bed hospital recognition as a leading advocate for
children and adolescents. For more information, visit
http://www.chop.edu.
Contact: Joey McCool Ryan
The Children's Hospital of Philadelphia
Phone: (267) 258-6735
McCool@email.chop.edu
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SOURCE Children's Hospital of Philadelphia