Pediatric Cardiology Articles of July 2015

1. Is Aortic Valve Leaflet Morphology Predictive of Outcome in Pediatric Aortic Valve Stenosis?

Loomba RS, Bowman JL, Cao Y, Tweddell J, Dearani JA, Simpson PM, Cetta F, Pelech AN.

Congenit Heart Dis. 2015 Jul 29. doi: 10.1111/chd.12278. [Epub ahead of print]

PMID: 26219421

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Select item 26233604

Abarbanell picture smallComment from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: Is aortic valve leaflet morphology predictive of outcome in pediatric aortic valve stenosis?  The answer to this question based on this study is NO.  This is a retrospective review of pre-procedural echocardiograms of patients who presented with aortic stenosis (critical and noncritical) prior to either a primary surgical or balloon intervention.  Echocardiographic parameters evaluated included:  (1) annular dimension, (2) leaflet number, (3) leaflet excursion, and (4) degree of leaflet coaptation.  102 patients were included (31 critical, 71 non-critical aortic stenosis).  Of the patients with critical aortic stenosis 9 (29%) had a surgical procedure and 22 (71%) had balloon valvuloplasty for initial palliation.  The reintervention rate in the critical aortic stenosis group was 53.1% with the reason for reintervention being residual obstruction or early recurrence of valve obstruction.  Those with noncritical aortic stenosis, 43 (61%) had a surgical procedure, whereas 28 (39%) had balloon valvuloplasty for initial palliation.  In the noncritical aortic stenosis group, the reintervention rate was 18.4%.  There was no significant difference in time to reintervention or death whether initial palliation consisted of surgical commissurotomy or balloon valvuloplasty with respect to any of the morphologic characteristics studied using Kaplan–Meier analysis. Valve morphologic features did not impact time to reintervention or death in patients with critical or noncritical aortic stenosis.

Take home points: 

  1. The choice of initial procedure (surgical vs. balloon intervention) did not impact the time to reintervention or death.
  2. There appears to be a higher reintervention rate in those with critical aortic stenosis compared to non-critical aortic stenosis.
  3. Valve morphology did not differ in the time to reintervention or death in this patient population.


2. Severe Congenital Heart Defects Are Associated with Global Reduction of Neonatal Brain Volumes.

von Rhein M, Buchmann A, Hagmann C, Dave H, Bernet V, Scheer I, Knirsch W, Latal B; Heart and Brain Research Group.

J Pediatr. 2015 Jul 29. pii: S0022-3476(15)00728-3. doi: 10.1016/j.jpeds.2015.07.006. [Epub ahead of print]

PMID: 26233604

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Select item 26223202

Comment from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: In the May 2015 edition of Journal Watch (Fetal Cardiology) an article by Schellen et al1 was reviewed which found that the total brain volume, gray matter volume and subcortical brain volume were decreased in fetuses with Tetralogy of Fallot.  This current article by von Rhein et al finds similar findings in neonates with congenital heart disease (CHD) prior to surgical repair.  19 infants with significant congenital heart defects and 19 health newborn infants (control group) underwent brain MRIs.  All MRIs were performed prior to surgical correction of CHD.  The cardiac diagnoses included d-transposition of the great arteries, univentricular heart malformation, interrupted aortic arch, and aortic coarctation with severe aortic arch hypoplasia.  Volumetric analysis demonstrated that infants with severe CHD had smaller global brain volumes on presurgery MRI compared to control infants. All areas of the brain were affected, including cortical and deep grey matter, white matter, and cerebellar volumes. See table

Take home points: 

  1. This study is similar to the fetal study by Schellen et al. Both suggest that brain development in fetuses with a congenital heart defect is affected.
  2. These findings may in the future help in our understanding of the neurodevelopmental deficits in children with congenital heart defects.

1.Schellen C, Schwartz E, Gruber GM, Mlczoch E, Weber M, Brugger PC, et al. Fetal MRI detects early alterations of brain development in Tetralogy of Fallot. Am J Obstet Gynecol. 2015 May 23.

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3. Evaluating cost and resource use associated with pulse oximetry screening for critical congenital heart disease: Empiric estimates and sources of variation.

Reeder MR, Kim J, Nance A, Krikov S, Feldkamp ML, Randall H, Botto LD.

Birth Defects Res A Clin Mol Teratol. 2015 Jul 28. doi: 10.1002/bdra.23414. [Epub ahead of print]

PMID: 26215888

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Select item 26220813

Comment from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch:  In 2011 the United States Secretary of the Department of Health and Human Services (HHS) recommended that all newborns be screened for critical congenital heart disease (CCHD) using pulse oximetry. Several states have or are considering legislation to mandate CCHD testing.  This paper is a pilot study from Utah to evaluate the cost of CCHD screening.  Two newborn nurseries in urban Utah were included in this study.  Each hospital was similar in the number of deliveries per year, 4000 births per year per site.  At site A, medical assistants using disposable probes did the majority of the CCHD screens.  At site B, mostly nursing assistants using reusable probes performed the CCHD screen.  The median screening time was 8.6 minutes with no difference between sites.  The cost at site A was $24.52 per baby compared to $2.60 at site B.  See table for further details.   Almost all the difference in cost was due to the cost of the disposable probes.

Take home points: 

  1. CCHD screening with pulse oximetry can be done quickly in less than 10 minutes.
  2. The cost of CCHD screening ranges from $2.60 to $24.52 with the majority of the cost from disposable probes.

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4. Distribution of Hypertrophy and Late Gadolinium Enhancement in Children and Adolescents with Hypertrophic Cardiomyopathy.

Windram JD, Benson LN, Dragelescu A, Yoo SJ, Mertens L, Wong D, Grosse-Wortmann L.

Congenit Heart Dis. 2015 Jul 20. doi: 10.1111/chd.12286. [Epub ahead of print]

PMID: 26193909

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Select item 26194592

Comment from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch:  The hypertrophy pattern, prevalence and clinical significance of scar in the hearts of younger patients with hypertrophic cardiomyopathy (HCM) have not been well studied.  This study from Toronto evaluated 38 children (age 12.83 ± 2 years) with HCM who underwent cardiac magnetic resonance imaging (CMR).  50% (19) had diffuse septal, 34% (13) diffuse concentric and 16% (6) had isolated basal hypertrophy.  There were no cases of midventricular hypertrophy or apical hypertrophy observed in this cohort. (See figure) Late gadolinium enhancement (LGE) involving the interventricular septum occurred in 7 children (18%).  These 7 children had a larger left ventricular mass index.  2/7 children with LGE presented with malignant arrhythmias. LGE was observed in 18% of the cohort, which is lower than the reported prevalence in adults of 30-80%.  HCM genotypes did not demonstrate an association with specific patterns of hypertrophy (see figure).

Take home points: (Summary taken directly from paper)

  1. The most common left ventricular phenotype of pediatric sarcomeric HCM is diffuse septal hypertrophy.
  2. The prevalence of LGE is much lower in a pediatric population as compared with that reported in adult patients.
  3. The presence of LGE appears to be associated with a greater risk for malignant ventricular arrhythmias in children.

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