Pediatric Cardiology Featured Articles of March 2016

1. Genetic link between renal birth defects and congenital heart disease.

San Agustin JT, Klena N, Granath K, Panigrahy A, Stewart E, Devine W, Strittmatter L, Jonassen JA, Liu X, Lo CW, Pazour GJ.

Nat Commun. 2016 Mar 22;7:11103. doi: 10.1038/ncomms11103.

PMID: 27002738 Free PMC Article

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Select item 27006349

Take Home Points:

  1. This study on mutant mice with CHD suggests that renal abnormalities are associated with CHD.
  2. This study also confirmed similar findings in human subjects retrospectively with 30% of patients with CHD having documented renal abnormalities.
  3. Kidney evaluation may be beneficial in patients with CHD.

Abarbanell picture smallCommentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: This study evaluated renal abnormalities in mouse mutants identified to have congenital heart disease (CHD).  Researchers screened more than 80,000 fetal mice with in utero ultrasound imaging (4200 mutant lines) which revealed a wide spectrum of CHD.  Causative mutations for CHD could be identified in 135 mutant lines by whole exome sequencing and genotyping.  Non-cardiac abnormalities within the CHD mutants were characterized and there was a high prevalence of renal abnormalities in the CHD mutants.  39 of the 135 CHD mutant lines were observed to have kidney defects (See table 1).  The most common renal abnormality seen in the CHD mutants was a duplex kidney.  Researchers also conducted a retrospective clinical review of human patients (n=77) with CHD requiring surgical intervention.  30% of the human patients with CHD had renal defects.  See figure 5.  Researchers did not find a correlation between cardiac lesion type and the different renal abnormalities.  Researchers conclude  that the findings “probably reflects the conservation of developmental pathways and cell signaling mechanisms that regulate cardiovascular and renal development…In addition this work indicates that CHD patients would benefit from routine evaluation for renal anomalies to reduce potential renal complications and improve outcome in this high-risk patient population.”

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2. Clinical and Financial Impact of Ordering an Echocardiogram in Children with Left Axis Deviation on Their Electrocardiogram.

Ravi P, Ashwath R, Strainic J, Li H, Steinberg J, Snyder C.

Congenit Heart Dis. 2016 Mar;11(2):110-4. doi: 10.1111/chd.12294. Epub 2015 Aug 24.

PMID: 26303069

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Select item 26915011

Take Home Points:

  1. Left axis deviation on ECG in infancy is associated with significant CHD. However, in older patients with a normal physical examination the finding of LAD is most likely a benign finding.
  2. In the setting of an ECG with LAD only in older patients, a normal physical examination would suggest that no further work-up is needed.

Commentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: How valid is left axis deviation (LAD) on ECG beyond infancy in identifying structural congenital heart disease (CHD)?  This retrospective study would suggest that beyond infancy the physical examination by a cardiologist can be the used to determine whether an echocardiogram is warranted in the presence of LAD on an ECG.  It is well known that in early infancy, LAD on the ECG can be associated with atrioventricular canal defects, Ebstein anomaly of the tricuspid valve, tricuspid atresia, hypoplastic left heart syndrome, and Wolff–Parkinson–White syndrome.  However beyond infancy the sensitivity and specificity of LAD on ECG to predict CHD is unknown.  In this study, 146 patients (age 4 months to 18 years) with an abnormal ECG with LAD that were seen by cardiology and received an echocardiogram were reviewed.  Results found that 46.5% (68) had a normal physical examination (PE) and ECHO, 1.4% (2) had a normal PE and abnormal ECHO, 47.3% (69) had an abnormal PE and ECHO, and 4.8% (7) had an abnormal PE and normal ECHO. The sensitivity and specificity of PE for detecting abnormalities in this population was 97% and 90%. Positive and negative predictive value of PE was 91% and 97.5%. In patients with normal PE, the cost to identify an ECHO abnormality was $8365, and $263 for those with abnormal PE.  The 2 patients that had LAD on ECG with a normal PE and abnormal echocardiogram were found to have incidental CHD (bicuspid aortic valve and anomalous right coronary artery from left sinus of Valsalva).  The researchers conclude “The study provides evidence that in pediatric patients with LAD on their resting ECG, one should rely on the PE performed by a pediatric cardiologist as a highly sensitive and specific marker with regard to the presence of CHD”.

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3. Paracetamol for the treatment of patent ductus arteriosus in preterm neonates: a systematic review and meta-analysis.

Terrin G, Conte F, Oncel MY, Scipione A, McNamara PJ, Simons S, Sinha R, Erdeve O, Tekgunduz KS, Dogan M, Kessel I, Hammerman C, Nadir E, Yurttutan S, Jasani B, Alan S, Manguso F, De Curtis M.

Arch Dis Child Fetal Neonatal Ed. 2016 Mar;101(2):F127-36. doi: 10.1136/archdischild-2014-307312. Epub 2015 Aug 17.

PMID: 26283668

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Select item 26411865

Take Home Points:

  1. Systematic review and meta-analysis of the literature would suggest that paracetamol may have a clinical role in the management of PDA.
  2. The effectiveness of paracetamol compared to ibuprofen in this review was similar with no increased safety risk.
  3. Further well-designed studies are needed to support the use of paracetamol for treatment of PDA.

Commentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: There has been increasing research suggesting that paracetamol (acetaminophen) is an effective treatment for patent ductus arteriosus (PDA) closure in premature infants similar to ibuprofen and indomethacin.  This systematic review and meta-analysis from Italy found that paracetamol is as effective as ibuprofen with no increase in side effects.  This systematic review of the literature identified 16 studies (2 randomized control trials (RCT) and 14 uncontrolled studies) using their inclusion criteria.  Results of the meta- analysis are as follows:

  1. Meta-analysis of RCT (See tables 2 and 3)
  • No difference in PDA closure with paracetamol compared to ibuprofen, after 3 and 6 days of treatment
  • No significant difference between the paracetamol and ibuprofen groups in terms of mortality, morbidity or ductal reopening
  • Risk of hyperbilirubinaemia was higher for ibuprofen compared to paracetamol
  1. Uncontrolled studies (See table 4 and 5)
  • A pooled ductal closure rate of 49% and 76% after 3 and 6 days of treatment with paracetamol
  • Safety profiles of paracetamol and ibuprofen are similar
  • A significant improvement in efficacy was observed when paracetamol was used in subjects with GA 28 weeks, postnatal age <7 days and when it was used as first-line therapy
  • There was a trend to greater benefit when paracetamol was used by oral route and at lower dose
  • A transient increase in aspartate and alanine aminotransferases or γ-glutamyl transpeptidase was reported only in six patients enrolled in three of the 14 uncontrolled studies

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4. Changes in Ventricular Geometry Predict Severity of Right Ventricular Hypertension.

Averin K, Michelfelder E, Sticka J, Cash M, Hirsch R.

Pediatr Cardiol. 2016 Mar;37(3):575-81. doi: 10.1007/s00246-015-1317-z. Epub 2015 Dec 14.

PMID: 26667960

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Select item 26667958

Take Home Points:

  1. Left ventricular end-systolic eccentricity index (LVEI) can be easily measured from the parasternal short axis images.
  2. LVEI demonstrated a significant correlation with the right ventricular pressure/peak systolic aortic pressure obtained by cardiac catheterization.
  3. A LVEI >48 demonstrated a sensitivity of 76 % and specificity of 100 % in predicting RVp/pAo >0.50, while a LVEI >1.24 had a sensitivity of 88 % and specificity of 83 %.
  4. LVEI seems to be helpful echocardiographic measurement in evaluating the severity of pulmonary hypertension.

Commentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: This retrospective study from Cincinnati Children’s Hospital Medical Center correlated echocardiographic findings of pulmonary hypertension with cardiac catheterization finding with particular interest to the left ventricular end-systolic eccentricity index (LVEI) and cardiac catheterization measurements of the ratio of peak systolic right ventricular pressure (RVp) to peak systolic aortic pressure (pAo).  The LVEI is easily measured from the echocardiographic parasternal short axis images.  See figure 1.  46 studies in 29 subjects (median age 3.8 years), with a median time from echocardiogram to catheterization of 1.0 days, were analyzed.  Results demonstrated a statistically significant correlation between the LVEI and RVp/pAo (r =  0.76, p <0.001), mean PA pressure (r =  0.73, p<0.001), transpulmonary gradient (r =  0.74, p< 0.001) and indexed pulmonary vascular resistance (r = 0.49, p =  0.001).  See figure 2.  An LVEI >1.48 was found to have a sensitivity of 76 % and specificity of 100 % in predicting RVp/pAo >0.50, while a LVEI >1.24 had a sensitivity of 88% and specificity of 83 %. LVEI seems to be helpful echocardiographic measurement in evaluating the severity of pulmonary hypertension.

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5. Pediatric Cardiac Intensive Care Society 10th International Conference 2014 Consensus Statement: Pharmacotherapies in Cardiac Critical Care.

Salvin JW, Bronicki R, Costello JM, Moffett B, Procaccini D.

Pediatr Crit Care Med. 2016 Mar;17(3 Suppl 1):S1-2. doi: 10.1097/PCC.0000000000000634. No abstract available.

PMID: 26945323

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Select item 26928105

Take Home Point:

  1. These consensus statements on pharmacotherapies in cardiac critical are succinct and will most likely be very useful for those practicing in pediatric cardiac intensive care.

Commentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch:  Supplement 1 of the Pediatric Critical Care Journal has several consensus statements from the Pediatric Cardiac Intensive Care Society 10th international conference 2014.  Consensus statements included in the supplement include the following:

  1. Pharmacotherapies in cardiac critical care sedation, analgesia and muscle relaxants
  2. Pharmacotherapies in cardiac critical care treatment of acute heart failure
  3. Pharmacotherapies in cardiac critical care chronic heart failure
  4. Pharmacotherapies in cardiac critical care fluid management
  5. Pharmacotherapies in cardiac critical care antiarrhythmics
  6. Pharmacotherapies in cardiac critical care hormone replacement therapy
  7. Pharmacotherapies in cardiac critical care immune therapy
  8. Pharmacotherapies in cardiac critical care anticoagulation and thrombosis
  9. Pharmacotherapies in cardiac critical care pulmonary hypertension
  10. Pharmacotherapies in cardiac critical care antihypertensives

These consensus statements on pharmacotherapies in cardiac critical are succinct and will most likely be very useful for those practicing in pediatric cardiac intensive care.

 

 

6. Resource Utilization for Noncardiac Admissions in Pediatric Patients with Single Ventricle Disease.

Thomas ID, Seckeler MD.

Am J Cardiol. 2016 Mar 2. pii: S0002-9149(16)30303-4. doi: 10.1016/j.amjcard.2016.02.043. [Epub ahead of print]

PMID: 27018934

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Select item 26932151

Take Home Points: 

  1. Health care resource utilization for noncardiac hospital admissions in patients with SV CHD was significantly higher than non CHD patients.
  2. Infants with SV CHD admitted with noncardiac issues are at highest risk of mortality.
  3. Hospitalization for noncardiac diagnoses may be preventable with good outpatient management and preventive care.

Shaji Menon Portrait 12.15.14Commentary from Dr. Shaji Menon (Salt Lake City, UT), section editor of Pediatric Cardiology Journal Watch: This study compare the costs and outcomes for common noncardiac hospitalizations between patients (<18 years) with single ventricle (SV) and patients without congenital heart disease (CHD). The data for this study was obtained from University Health System Consortium discharge data from January 2011 to December 2013. The University Health System Consortium (UHC) is an alliance of 115 academic medical centers and 165 affiliated hospitals. There were a total of 893,264 admissions for patients without CHD (median age 8.1 years, range 1 month to 18 years) and 2,515 noncardiac admissions for patients with SV CHD (median age 1.8 years, range 1 month to 18 years). The SV CHD admissions were more in the younger ages compared to a bimodal distribution in the non CHD cohort.  AKI had the highest ICU admission rate and mortality in patients with SV CHD. Fifteen (88%) of the deaths in patients with SV CHD were in children aged <1 year. Total length of stay (LOS) was significantly longer and costs higher for almost all diagnoses for patients with SV CHD. AKI had the longest LOS for both SV CHD and patients without SV CHD.

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7. Congenital Heart Disease and Primary Ciliary Dyskinesia.

Harrison MJ, Shapiro AJ, Kennedy MP.

Paediatr Respir Rev. 2016 Mar;18:25-32. doi: 10.1016/j.prrv.2015.09.003. Epub 2015 Sep 26. Review.

PMID: 26545972

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Select item 26739006

Take Home Points: 

  1. There is increasing evidence of PCD in patients with complex CHD.
  2. Screening for PCD is recommended in all children with complex congenital heart disease, especially those with disorders of laterality, transposition of the great vessels, double-outlet right ventricle, anomalous venous return, interrupted inferior vena cava and bilateral superior vena cava.
  3. PCD should be considered in patients with above mentioned heart diseases and recurrent oto-sino-pulmonary symptoms.
  4. PCD in patients with CHD may increase morbidity and mortality.

Commentary from Dr. Shaji Menon (Salt Lake City, UT), section editor of Pediatric Cardiology Journal Watch: Primary Ciliary Dyskinesia (PCD) is an autosomal recessive disorder of ciliary motility resulting in defective mucociliary clearance. There is an increases incidence of ciliary dyskinesia in patients with congenital heart disease (CHD). There is increasing evidence of PCD in patients with complex CHD. Recent studies have shown that approximately 50% of patients with PCD have organ laterality defects and approximately 3-6% having cardiovascular malformations and at least 2.6% having a complex cardiovascular defect. This review explores this relationship between PCD and CHD. There is lack of a comprehensive genetic test for PCD. The PCD ERS Consensus Statement on Diagnostic and Treatment Approaches in Children recommends screening for PCD in all children with complex congenital heart disease, especially those with disorders of laterality, transposition of the great vessels, double-outlet right ventricle, anomalous venous return, interrupted inferior vena cava and bilateral superior vena cava. The test for PCD include nasal nitric oxide measurement, ciliary electron microscopy, or functional ciliary analysis, thoraco-abdominal imaging to evaluate for organ laterality. PCD should be considered in patients with above mentioned heart diseases and recurrent oto-sino-pulmonary symptoms. Patients with PCD have a significantly increased risk of postop respiratory complications, need for tracheostomy, and use of beta-agonists.  CHD patient with heterotaxy, in general experience higher morbidity and mortality and this may be partly secondary to PCD.

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