Pediatric Cardiology Featured Articles of February 2016

1. Neurocognitive development and behaviour in school-aged children after surgery for univentricular or biventricular congenital heart disease.

Sarrechia I, Miatton M, De Wolf D, François K, Gewillig M, Meyns B, Vingerhoets G.

Eur J Cardiothorac Surg. 2016 Jan;49(1):167-74. doi: 10.1093/ejcts/ezv029. Epub 2015 Feb 17.

PMID: 25694470

Shaji Menon Portrait 12.15.14Comment from Dr. Shaji Menon (Salt Lake City, UT), section editor of Pediatric Cardiology Journal Watch: This cross-sectional study evaluates the long-term neuropsychological and behavioral profile of school-aged children who were treated for univentricular heart (UVH) conditions or biventricular heart defect (BiVH) in infancy. The study evaluated 63 patients, 17 UVH (13 males, 4 females) and 46 BiVH (19 males, 27 females), were assessed at a mean age of 9.1 years. Authors report that the mean intelligence and neuropsychological scores were within normal ranges for all groups. However, compared to controls, UVH patients had lower auditory sustained and alternating attention, fine motor skills, visuospatial information processing, and to a lesser extent, memory performance. Parents of UVH patients reported more externalizing problems and school problems. After Fontan, UVH patients had intelligence score similar to normal population. However, UVH patients had higher incidence of abnormalities in attention, fine motor skills, visuospatial information processing and externalizing behavioral problems.

Take home points:

  1. School-aged children with univentricular heart undergoing staged palliation in the current era have to a great extent preserved neurodevelopmental outcome.
  2. UVH patients shows subtle abnormalities in attention, fine motor skills and visuospatial information processing. These children were also found at risk for internalizing and especially externalizing problem behavior and more school problems by parental reports.
  3. In UVH patients, comprehensive neurodevelopmental evaluation and early referral will help in early identification and management of neurodevelopmental abnormalities resulting in improved developmental trajectories.

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2. Growth Asymmetry, Head Circumference, and Neurodevelopmental Outcomes in Infants with Single Ventricles.

Miller TA, Zak V, Shrader P, Ravishankar C, Pemberton VL, Newburger JW, Shillingford AJ, Dagincourt N, Cnota JF, Lambert LM, Sananes R, Richmond ME, Hsu DT, Miller SG, Zyblewski SC, Williams RV; Pediatric Heart Network Investigators.

J Pediatr. 2016 Jan;168:220-225.e1. doi: 10.1016/j.jpeds.2015.09.041. Epub 2015 Oct 17.

PMID: 26490132

Comment from Dr. Shaji Menon (Salt Lake City, UT), section editor of Pediatric Cardiology Journal Watch: This study evaluates the variability in asymmetric growth and its association with neurodevelopment in infants with single ventricle (SV). Associations between growth indices and scores on the Psychomotor Developmental Index (PDI) and Mental Developmental Index (MDI) of the Bayley Scales of Infant Development-II (BSID-II) at 14 months were assessed in subjects prospectively enrolled in the Infant Single Ventricle Trial. Of the 230 subjects enrolled in the Infant Single Ventricle trial, complete growth data and BSID-II scores were available in 168 (73%). BSID-II scores were not associated with growth asymmetry indices at 14 months. In multivariable modeling, pre-superior cavopulmonary connection (SCPC) surgery head circumference-for-age z-score (HCAZ) was an independent predictor of PDI (P = .03), but not MDI.

Take-home points:

  1. In infants with SV, growth asymmetry was not associated with neurodevelopment at 14 months.
  2. Pre-superior cavopulmonary connection (SCPC) surgery head circumference-for-age z-score (HCAZ) was an independent predictor of PDI, but not MDI.
  3. Asymmetric growth, important in other high-risk infants, is not a brain-sparing adaptation in infants with SV.

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3. Ischemic Stroke in Children and Young Adults With Congenital Heart Disease.

Mandalenakis Z, Rosengren A, Lappas G, Eriksson P, Hansson PO, Dellborg M.

J Am Heart Assoc. 2016 Feb 23;5(2). pii: e003071. doi: 10.1161/JAHA.115.003071.

Abarbanell picture smallComment from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: This is a population-based study from Sweden evaluating the incidence of ischemic stroke among children and young adults with congenital heart disease.  25,985 children and young adults (birthdates 1970-1993) with CHD were identified within the Swedish Patient Register with follow up data through 2011 (maximum follow up of 40 years).  A control group matched 10:1 by age, sex and county was randomly selected from the general population.  Within the CHD group 140 (0.5%) developed an ischemic stroke compared to 137 (0.05%) in the control group.  The median age at the time of ischemic stroke diagnosis was 23.1 years in CHD patients versus 30.5 years in controls.  Overall patients with CHD had a nearly 11 fold increased risk [hazards ratio (HR)10.76] of developing an ischemic stroke compared to controls. Patients with the most complex CHD, had a 12 fold increased risk of developing ischemic stroke compared to controls (HR12.22). Patients with an isolated CHD diagnosis of coarctation of the aorta (HR 12.86), isolated double-inlet ventricle (HR 4.49) or atrial shunts (HR 10.0) had significantly increased risks of developing an ischemic stroke.  Using multivariate analysis of CHD patients the following co-morbidities were associated with an increased risk of ischemic stroke:  congestive heart failure (HR 6.9), hypertension (HR 3.89) and atrial fibrillation (HR 2.94).

Take Home Points:

  1. This is a large population-based study to evaluate the incidence of ischemic stroke with a follow up period of nearly 40 years from Sweden.
  2. The incidence of stroke in patients with CHD is 0.5%. The incidence in the control group 10 times lower at 0.05%.
  3. Result from regression models and multivariable analysis reported:
  • A 11 fold overall increased risk of developing ischemic stroke in CHD patients compared to controls.
  • Patients with the most complex CHD had a 12 fold increased risk of developing ischemic stroke compared to controls.
  • The following isolated CHD diagnoses conferred the highest risk of developing ischemic stroke: Coarctation of the aorta, isolated double inlet left ventricle and atrial shunts.
  • Co-morbidities of congestive heart failure, hypertension and atrial fibrillation were independent risk factors for developing ischemic stroke in patients with CHD.
  1. The median age of CHD diagnosis was around age 6 and the age of ischemic stroke was 23 years old.

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4. Elevated Low-Shear Blood Viscosity is Associated with Decreased Pulmonary Blood Flow in Children with Univentricular Heart Defects.

Cheng AL, Takao CM, Wenby RB, Meiselman HJ, Wood JC, Detterich JA.

Pediatr Cardiol. 2016 Feb 18. [Epub ahead of print]

PMID: 26888364

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Comment from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: The Glenn and Fontan circulation in the univentricular heart depends on passive blood flow into the pulmonary arteries.  This passive blood flow state is a low-shear blood flow state.  This article sought to evaluate the effect of low-shear blood flow in Glenn and Fontan patients on blood viscosity and in turn pulmonary vascular resistance (PVR) and pulmonary blood flow (PBF) compared to a biventricular heart control group.  The control group consisted of patients presenting to the cardiac cath lab for atrial septal defect (ASD) closure.  62 children were included in this study (20 with isolated ASD, 22 s/p Glenn procedure and 20 s/p Fontan procedure).  As expected the blood viscosity at all shear rates was higher in Glenn and Fontan patients compared to ASD patients (see Figure).   In Glenn and Fontan patients, low-shear blood viscosity negatively correlated with PBF (R2 =  0.27, p <0.001).  Also in Glenn patients low shear blood viscosity was negatively correlated with pulmonary artery pressure (R2 = 0.15, p = 0.01) and positively correlated with PVR in Fontan (R2 = 0.28, p = 0.02).

Take Home Points:

  1. The low-shear blood flow state in patients after a Glenn and Fontan procedure leads to increased blood viscosity. This study demonstrates an association between increased blood viscosity and pulmonary blood flow, pulmonary artery pressure and pulmonary vascular resistance.
  2. Further studies are needed to further evaluate this relationship with blood viscosity in Glenn and Fontan patients in relation to pulmonary blood flow.

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5. Executive Functioning and School Performance among Pediatric Survivors of Complex Congenital Heart Disease.

Gerstle M, Beebe DW, Drotar D, Cassedy A, Marino BS.

J Pediatr. 2016 Feb 11. pii: S0022-3476(16)00030-5. doi: 10.1016/j.jpeds.2016.01.028. [Epub ahead of print]

PMID: 26875011

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Comment from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: There have been several recent studies evaluating the neurodevelopment of children with congenital heart disease (CHD).  This study is unique in that the focus was to evaluate how deficits in executive function in children with CHD manifest in real-world settings.  143 children age 8-16 years were recruited from a regional registry of children with CHD.  Children included in the study were divided into two groups: (1) complex 2-ventricle CHD consisting of d-transposition of the great arteries or tetralogy of Fallot  and (2) single-ventricle CHD requiring Fontan palliation.  The following neurodevelopmental tests were used and compared to published norms (no control group).

  1. Parent-report version of the Behavior Rating Inventory of Executive Function (BRIEF). BRIEF is designed to evaluate executive functioning in an everyday environment in school-aged children.  BRIEF has 2 summary indices: metacognition and behavioral regulation. Metacognition includes working memory, initiating, planning and organization.  Behavior regulation is a child’s ability to regulate and control emotions and behavior.  BRIEF often includes a parent report and a teacher report.  Only the parent-report was utilized in this study.
  2. Child Behavior Checklist (CBCL). CBCL is completed by the parent and inquires about a child’s typical grades and the need for educational support.
  3. Pediatric Quality of Life Inventory (Peds-QL) version 4.0, school function subscale. This portion is completed by the child and the parent and inquires about day-to-day classroom skills, for example keeping up with schoolwork.
  4. Wechsler Intelligence Scale for Children-Fourth Edition, (8 subset short form). This test was used to evaluate IQ.

Researchers in this study found the following:

  • 27% of the children in this study were diagnosed with a learning disability and had an individualized education program (IEP). There was no statistical difference between the complex 2 ventricle vs. single ventricle group.
  • 39% of children with single ventricle palliation had a history of grade retention compared to 11% of the complex 2 ventricle CHD group (p <0.001).
  • Overall, all children with CHD scored worse compared to published norms on all the above tests except for on the BRIEF behavior regulation index. See table
  • Metacognition was more affected than behavior regulation (measured on the BRIEF) and correlated with age with worse scores seen in adolescent children. See Figure.
  • Parents of children with single ventricle palliation reported lower school competency and school QOL (CBCL and parent portion of school subscale of Peds-QL) compared to parents of children with 2 ventricle CHD.
  • Children with single ventricle palliation reported lower school QOL than children with complex 2 ventricle CHD. (Child portion of school subscale of Peds-QL).
  • Metacognition (measured on the parent BRIEF) was the strongest single predictor of school performance above IQ, race, sex, or heart lesion complexity.

Take Home Points:

  1. Several studies have demonstrated that children with CHD have difficulty with executive function. This study in particular highlights that children with deficits in metacognition (working memory, initiating, planning and organization) are  more likely to have difficulty in school.
  2. Children with CHD also were reported to have worse metacognition compared to norms that was more prevalent with increasing age.
  3. These results highlight the need to continually evaluate children with CHD for school difficulties and help families and schools to obtain/provide interventions when needed.

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6. Long-Term Nationwide Follow-Up Study of Simple Congenital Heart Disease Diagnosed in Otherwise Healthy Children.

Videbæk J, Laursen HB, Olsen M, Høfsten DE, Johnsen SP.

Circulation. 2016 Feb 2;133(5):474-83. doi: 10.1161/CIRCULATIONAHA.115.017226. Epub 2015 Dec 18.

PMID: 26683488

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Comment from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: This is a report of the long-term mortality and morbidity of children and adults with simple congenital heart disease (CHD).   Simple CHD in this study was defined as an isolated and uncomplicated secundum atrial septal defect, patent ductus arteriosus, ventricular septal defect with normal pulmonary vascular resistance or mild pulmonary stenosis.   This study from Denmark identified 1231 children with simple CHD diagnosed between 1963-1973 and using the Danish National Registry of Patients and Danish Registry of Causes of Death were followed for mortality and morbidity over a 40 year time period.  A control cohort from the general Danish population was matched 10 to 1 for year of birth and sex.   Results of this analysis found that mortality was increased compared to the general population [adjusted hazard ratio [aHR],1.9; 95% confidence interval [CI], 1.5–2.4)].  In these adults with simple CHD there was a 4 to 6 fold increased risk of sudden and unexpected death and cardiac death.  Additionally there was a 4 to 5 fold increased risk of critical cardiac mortality.  Within this population of 1231 adults with simple CHD there were 86 deaths (34 died suddenly), 15 cases of pulmonary hypertension, 15 cases of endocarditis and 6 cases of aortic dissection.  These adults with simple CHD were also twice as likely to have a stroke compared to the control population [aHR of 2.6 (95% CI, 1.8 to 3.7)].  See Figures.

Take Home Points:

  1. This population cohort study from Denmark demonstrated increased long term mortality and morbidity among adults with simple CHD.
  2. Among the adults in this study there was a 4 to 6 fold increased risk of sudden and unexpected death and cardiac death and a 4 to 5 fold increased risk of critical cardiac mortality compared to control sample of the general Danish population.

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7. The Association among Feeding Mode, Growth, and Developmental Outcomes in Infants with Complex Congenital Heart Disease at 6 and 12 Months of Age.

Medoff-Cooper B, Irving SY, Hanlon AL, Golfenshtein N, Radcliffe J, Stallings VA, Marino BS, Ravishankar C.

J Pediatr. 2016 Feb;169:154-159.e1. doi: 10.1016/j.jpeds.2015.10.017. Epub 2015 Nov 13.

PMID: 26585995

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Select item 26547402

Comment from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: This is a prospective cohort study from Children’s Hospital Philadelphia evaluating the effect of early growth and device assisted feeding on neurodevelopmental outcomes.  Of the 72 infants included in the study, almost half (47%) had single ventricle physiology.  The Bayley Scales of Infant Development (version II) was administrated at 6 and 12 months of age.  The Bayley Scales of Infant Development provides 2 scores, the Psychomotor Developmental Index (PDI) and the Mental Developmental Index (MDI).  The PDI assesses gross and fine motor skills while the MDI assesses cognitive, memory, problem solving, generalization, vocalizations and social skills. The PDI and MDI were lower than the normal population at 6 and 12 months of age.  On multivariate regression models the following were independently associated with a lower PDI or MDI.

Factors associated with lower Psychomotor Developmental Index (PDI)

-At 6 months of age:  Device-assisted feeding at 3 months (P  = .04)

Lower length-for-age z score at 3 months (P  =.04)

-At 12 months of age: Lower weight-for-age z score at 3 months (P  =.04)

Lower length-for-age z score at 3 months (P  = .04)

Factors associated with lower Mental Developmental Index (MDI)

-At 6 months of age:  Lower length-for-age z score at 3 months (P  < .01)

Head circumference-for-age z score at 3 months (P  < .05)

-At 12 months of age: Lower weight-for-age z score at 3 months (P  =0.04)

Lower length-for-age z score at 3 months (P  = 0.04)

Increased hospital length of stay (P  < .01)

Lower length-for-age z score at 3 months (P  = .04)

There were no significant differences in PDI or MDI scores at 6 months when the single ventricle and 2 ventricle groups were compared. However at 12 months the infants with single ventricle physiology had significantly lower PDI and MDI scores compared to the 2 ventricle group.   The results of this study demonstrate an association with poor early growth and need for device-assisted feeding with increased risk for poorer neurodevelopmental outcomes.

Take Home Points:

  1. This prospective cohort study of infants with CHD demonstrated an association between poor early growth at 3 months and need for device assisted feeding at 3 months with increased risk for poor neurodevelopmental outcomes.
  2. Infants with CHD in this cohort performed below the published norms on the Bayley Scales of Infant Development (version II) on both the Psychomotor Developmental Index (PDI) and the Mental Developmental Index (MDI).
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