Pediatric Cardiology Featured Articles of October 2015

1. Mortality-related resource utilization in the inpatient care of hypoplastic left heart syndrome.

Danford DA, Karels Q, Kulkarni A, Hussain A, Xiao Y, Kutty S.

Orphanet J Rare Dis. 2015 Oct 22;10:137. doi: 10.1186/s13023-015-0355-1.

PMID: 26494006 Free PMC Article

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Abarbanell picture smallComment from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: There have been several advances made in the care/palliation of infants and children with hypoplastic left heart syndrome (HLHS) over the last few decades.  However, the resource utilization for these patients is high compared to other congenital heart defects.  In this study, researchers queried the Pediatric Health Information System (PHIS) administrative database to evaluate inpatient hospital utilization of patients with HLHS and specifically the mortality-related resource utilization.  The PHIS database was searched for patients 21 years old and younger with HLHS.  11,122 HLHS admissions were identified with a total length of stay of 277,027 inpatient-days and $3,928,794,660 in billed charges.  The inpatient mortality rate was 10.3% (1145 inpatient deaths).  A negative correlation in inpatient mortality rate was found with calendar year and institutional volume.  Additionally, inpatient mortality was remarkably higher in patients under 1 year and those 15 years old or older at hospital admission.  See figure 1.  In this article researchers developed a new index termed mortality-related resource utilization fraction (MRRUF).  This index was designed to “measure the investment of inpatient care resources in the care of HLHS, and determine how much of that investment takes place during hospitalizations that have a fatal outcome.”  The MRRUF was calculated as follows using the variables of hospital length of stay (LOS) and billed charges (BC).

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Interestingly, researchers did not find a significant association between MRRUF for calendar year or institutional volume.  This would suggest the amount of resources utilized by patients that did not survive did not change by calendar year or institutional volume despite the improved inpatient mortality rate. Compare Figures 1, 2, and 3.  Researchers did find a spike in MRRUF for HLHS in patients less than 1 year and those in late childhood.  See figure 4.  Researchers did state in limitations – “This does not include analysis of MRRUF as a function of other potentially interesting socioeconomic markers like insurance status, race, and ethnicity.”  Researchers concluded:  “Regardless of the large fraction of resource utilization in HLHS care that does not produce survivors, this analysis must not be construed as an argument against sophisticated invasive surgical palliative management of HLHS. Instead, these findings highlight the need for data-driven critical review of standard practices to identify patterns of care associated with success with HLHS, and to modify approaches objectively.”

Take Home Points:

  1. This article highlights the decreasing inpatient mortality rate for HLHS over time.
  2. There is increased mortality rate in the very young (less than 1 year of age) and older patients (over age 15 years) with HLHS which would account for the associated mortality with the Norwood procedure in infancy and the onset of Fontan failure in older patients with HLHS.
  3. The mortality-related resource utilization fraction (MRRUF) maybe an interesting marker to evaluate resource utilization for those patients that died.
  4. As correctly concluded by the researchers – “this analysis must not be construed as an argument against sophisticated invasive surgical palliative management of HLHS. Instead, these findings highlight the need for data-driven critical review of standard practices to identify patterns of care associated with success with HLHS, and to modify approaches objectively”.

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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. 2015 Oct 17. pii: S0022-3476(15)01054-9. doi: 10.1016/j.jpeds.2015.09.041. [Epub ahead of print]

PMID: 26490132

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Comment from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: Preservation of head growth in the setting of low weight (asymmetric growth restriction) has been shown to be an important factor in outcomes of premature infants verses infants with intrauterine growth restriction.  Researchers in this article used data from the Infant Single Ventricle Trial to evaluate whether asymmetric growth in infants with single ventricle cardiac physiology is a predictor of worse neurodevelopmental outcomes.  The following growth parameters were evaluated in this study:

  • Weight-for-age z-score (WAZ)
  • Length-for-age z-score (LAZ)
  • Head circumference-for-age z-score (HCAZ)
  • Weight-for-length z-score (WLZ) –marker of asymmetric growth
  • WAZ minus HCAZ – asymmetry between standardized weight and head circumference

Infants underwent neurodevelopmental testing using the Bayley Scales of Infant Development (BSID)-II at age 14 months.  For this study researchers used the Psychomotor Developmental Index (PDI) of the BSID-II, which assesses gross motor and fine motor skills, and the Mental Developmental Index (MDI), which measures cognitive functioning through assessment of memory, problem solving, number concepts, vocalization, and language and social interaction skills.

The cohort was noted to be small at time of enrollment (mean age 21 days) with lower WAZ, LA and HCAZ.   The cohort demonstrated improved head size but drop in weight at time of pre-superior cavopulmonary connection (Glenn/hemi-Fontan).  This cohort scored slightly below normal on  BSID-II testing for both the PDI  and MDI. However, growth asymmetry indices were not associated with BSID-II score.  On multivariate analysis independent factors associated with PDI were: HCAZ at pre-superior cavopulmonary connection,  LAZ at 14 months, ECMO and neonatal hospital length of stay.  On multivariate analysis independent factors associated with MDI were: LAZ at pre-superior cavopulmonary connection, ECMO, number of serious adverse events and clinical site.  Authors conclude the following:  “Although variability in asymmetric growth was considerable in our population of infants with single ventricle, the degree of asymmetry was not associated with neurodevelopmental testing outcomes. Regardless of the proportionality, head circumference and length, but not weight, were associated with outcomes.  These findings reinforce the fact that neurodevelopment is affected by multiple factors, and  that targeting weight gain alone in this population is not adequate to improve outcomes.”

Take Home Points:

  1. All infants in this single ventricle cohort scored slightly below average on the BSID-II neurodevelopmental testing.
  2. Asymmetric growth in infants with single ventricle physiology was not associated with neurodevelopmental outcomes.
  3. However, head circumference and length (NOT WEIGHT) were associated with improved outcomes on the BSID-II neurodevelopmental testing.
  4. Following weight gain alone may not be adequate for improving neurodevelopmental outcomes.


 3. A Patent Ductus Arteriosus Severity Score Predicts Chronic Lung Disease or Death before Discharge.

El-Khuffash A, James AT, Corcoran JD, Dicker P, Franklin O, Elsayed YN, Ting JY, Sehgal A, Malikiwi A, Harabor A, Soraisham AS, McNamara PJ.

J Pediatr. 2015 Oct 13. pii: S0022-3476(15)01041-0. doi: 10.1016/j.jpeds.2015.09.028. [Epub ahead of print]

PMID: 26474706

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Comment from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: This multicenter prospective observational cohort study from tertiary neonatal intensive care units in Ireland, Canada, and Australia sought to develop a patent ductus arteriosus (PDA) severity score (PDAsc) to predict chronic lung disease and/or death before discharge.  Several different echocardiographic parameters and clinical parameters were studied to find the best predictors of chronic lung disease and/or death in premature infants (less than 29 weeks gestation) with the presence of a PDA.  The following PDAsc was found to be the most predictive of chronic lung disease or death.  All echocardiographic measurements were taken at 2 days of life.


PDAsc = (gestation in weeks x -1.304) + (PDA diameter in mm x 0.781) + (LVO* in mL/kg/min x 0.008) + (maximum PDA velocity in m/s x -1.065) + (LV a’ wave in cm/s x -0.470) + 41

*LVO = LV output [(aortic cross-sectional area x  VTI (ascending aorta) x heart rate) ÷ weight]

LV a’ wave = tissue Doppler imaging (TDI) from apical 4 chamber

This score ranges between 0 (low risk) and 13 (high risk). The PDAsc was compared to the predictive valve of gestational age alone or PDA diameter (measured on day 2) alone, and another score derived from a combination of the following 5 clinical characteristics: gestation, use of antenatal steroids, days on invasive ventilation, late onset sepsis; and necrotizing enterocolitis.  See Figure 3 for ROC for each model.  The PDAsc had the highest area under the curve. A cut-off of 5 using the PDAsc had a sensitivity of 92%, a specificity of 87%, a positive predictive value of 92%, and a negative predictive value of 82%.  The PDAsc maybe a helpful scoring system to predict outcomes of a PDA and a tool for decision making in whether treatment of a PDA is needed.  Further testing of this scoring system was suggested by the authors.

Take Home Points:

  1. Researchers from Ireland, Canada and Australia have developed a scoring system to predict chronic lung disease and/or death in premature infants with a PDA. This scoring system is based on gestational age and echocardiographic parameters.
  2. This scoring system maybe helpful to the clinician with decision making regarding treatment for a PDA. A score over 5 maybe indicate a higher risk for development of chronic lung disease and/or death.

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4. Determinants of Resource Utilization in a Tertiary Pediatric and Congenital Echocardiographic Laboratory.

Banka P, Schaetzle B, Gauvreau K, Geva T.

Am J Cardiol. 2015 Oct 1;116(7):1139-43. doi: 10.1016/j.amjcard.2015.07.013. Epub 2015 Jul 16.

PMID: 26275580

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Comment from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: This study from Boston sought to evaluate resource utilization of performing transthoracic echocardiograms (TTE).  Total time to perform a TTE study was used as a surrogate for resource utilization.  The researchers identified risk factors predictive of increased total time per TTE study and thus higher resource utilization.

Data on all TTE performed over 3 months was collected and total time to perform each study recorded.  The medial time to perform TTE  was 65 minutes.  A quarter of the studies lasted at least 85 minutes.  This study found that on average, scanning comprised 47% of the total time with the remaining 53% spent on prescan- and postscan-related activities.  A scoring system for predicting high scanning times was developed.  See Table 3 and Figure 4.  As the researchers conclude, these study results may be informative in discussions about resource allocation and reimbursement for pediatric/congenital echocardiography.

Take Home Points:

  1. As many cardiologists can attest, there are many factors that can influence the time and resources needed in performing a pediatric echocardiogram.  This study has identified several factors, which can lead to increased time needed to complete a pediatric TTE study.
  2. This study has developed a scoring system to help evaluate which echocardiograms will take longer to complete and may require more hospital resources. These findings maybe helpful for resource allocation and re-imbursement in the future.

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5. Prevalence of atrial tachyarrhythmia in adults after Fontan operation.

Quinton E, Nightingale P, Hudsmith L, Thorne S, Marshall H, Clift P, de Bono J.

Heart. 2015 Oct 15; 101(20):1672-7. doi: 10.1136/heartjnl-2015-307514. Epub 2015 Aug 19.

PMID: 26289423

Shaji Menon Portrait 12.15.14Comment from Dr. Shaji Menon (Salt Lake City, UT), section editor of Pediatric Cardiology Journal Watch:  Arrhythmias are an inevitable and common long-term complication following a Fontan operation. Multiple surgeries and surgical lesions in the atria resulting from staged single ventricle palliation creates a proarrhythmogenic environment.  This retrospective cohort study from University Hospitals Birmingham (2001-2013) evaluates incidence and outcomes of arrhythmias in adult Fontan patients. The study included 166 patients (mean age 29.1 years). The types of Fontan included in this study: 63% atriopulmonary (AP) Fontan, 13% lateral tunnel (LT), 24% extracardiac total cavopulmonary connection (TCPC)). The mean follow-up since Fontan operation was 18.6 years. The most common arrhythmia was intra-atrial re-entrant tachycardia (66%). Tachyarrhythmia was observed in 42% of patients (the overall incidence of arrhythmia 2.23 per 100 patient-years). The incidence of arrhythmia increased with age. The average age at arrhythmia onset was 26.7 years (±9.3). The mean length of time from surgery to the development of arrhythmia was 14.2 years (±6.4, median 14 years, IQR 11–19). There was 100% incidence of arrhythmia by 25.5 years post-surgery. There was also a significant incidence of arrhythmia in the LT and TCPC groups (23% at mean follow-up of 19.6 years and 13.6 years, respectively). Survival analysis showed only age at the time of Fontan completion as a significant predictor for arrhythmia onset (p<0.001) irrespective of the surgical approach. Arrhythmia was a very strong predictor of mortality. Once patients have developed an arrhythmia, they were at a significantly higher risk of death (HR 23.0, 95% CI 6.4 to 82.5).

Take-home points:

#1. Arrhythmias, especially atrial arrhythmias are an inevitable long-term complication following a Fontan operation.

#2. The advantage of different types of Fontan surgeries in reducing the long-term atrial arrhythmia burden remains unclear. Irrespective of type of Fontan surgery, if these patients are followed long enough, most of them will develop atrial arrhythmias.

#3. The onset of arrhythmia in Fontan survivors is associated with increased mortality.

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6. Stress echocardiography in paediatric cardiology.

Cifra B, Dragulescu A, Border WL, Mertens L.

Eur Heart J Cardiovasc Imaging. 2015 Oct;16(10):1051-9. doi: 10.1093/ehjci/jev159. Epub 2015 Jun 29. Review.

PMID: 26130262

Comment from Dr. Shaji Menon (Salt Lake City, UT), section editor of Pediatric Cardiology Journal Watch
: Stress echocardiography is a well-established and commonly used imaging technique in adult cardiology with guidelines published by the American Society of Echocardiography as well as by the European Association for Echocardiography. However, there are no specific guidelines or recommendations for use of stress echocardiography in pediatric cardiology. This review article provides a comprehensive insight in to the techniques and potential applications of stress echocardiography in children.

Take-home points:

#1. The potential uses of stress echocardiography in children include the following:

  1. Pediatric ischemic heart disease: Kawasaki disease (KD), after heart transplantation, anomalous origin of the coronary arteries, or children after coronary reimplantation (arterial switch procedure and Ross operation).
  2. Dynamic assessment of blood pressure response and gradients: For valvular heart disease like mitral stenosis, evaluating left ventricular outflow tract obstruction in hypertrophic obstructive cardiomyopathy, recoarctation of aorta and tricuspid regurgitation gradient in evaluation of pulmonary hypertension.
  3. Early detection of myocardial damage: Stress echocardiography can be used to study early changes in myocardial function by evaluating the contractile and diastolic response to exercise. For altered response to exercise in strain or diastolic function indices during stress test maybe an early marker of anthracycline-induced myocardial dysfunction.

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7. Survival of Children With Hypoplastic Left Heart Syndrome.

Siffel C, Riehle-Colarusso T, Oster ME, Correa A.

Pediatrics. 2015 Oct;136(4):e864-70. doi: 10.1542/peds.2014-1427. Epub 2015 Sep 21.

PMID: 26391936

Comment from Dr. Shaji Menon (Salt Lake City, UT), section editor of Pediatric Cardiology Journal Watch: This study reports the survival of Infants with nonsyndromic hypoplastic left heart syndrome (HLHS) using population-based data. Nonsyndromic HLHS infants (n = 212) born between 1979 and 2005 were identified through the Metropolitan Atlanta Congenital Defects Program and vital status was ascertained through 2009 based on linkage with vital records. The overall survival probability for the study cohort to 2009 was 24%. Not surprisingly, the survival probability improved significantly over time: from 0% in 1979–1984 to 42% in 1999–2005. Very low and low birth weight or preterm infants and those born in high-poverty neighborhoods had poorer survival. For children surviving to 1 year of age, survival to adolescence was ∼90%.

Take-home points:

#1.  This population based study shows the tremendous progress made in the care and outcomes of infants born with HLHS.

#2. Once the HLHS patients survive the high risk infancy, the majority (90%) survive to adolescence (18 years).

#3. Prematurity, low birth weight and poor socioeconomic status key determinants of increased mortality.

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8. Association of TGFBR2 rs6785358 Polymorphism with Increased Risk of Congenital Ventricular Septal Defect in a Chinese Population.

Li XT, Shen CQ, Zhang R, Shi JK, Li ZH, Liu HY, Sun B, Wang K, Yan LR.

Pediatr Cardiol. 2015 Oct;36(7):1476-82. doi: 10.1007/s00246-015-1189-2. Epub 2015 May 30.

PMID: 26022443

Comment from Dr. Shaji Menon (Salt Lake City, UT), section editor of Pediatric Cardiology Journal Watch: Transforming growth factor beta receptor 2 (TGFBR2) plays a key role in development of normal heart. This case–control study from China evaluated the role of TGFBR2 polymorphism as a risk factor for ventricular septal defect (VSD). The study included 115 children with VSD and 188 healthy children. The study found that the genotype and allele frequency of TGFBR2 rs6785358 were significantly higher in the VSD cohort compared to controls (P<0.05).  In the VSD cohort, the G allele carriers were associated with increased risk of VSD compared with the A allele carriers (OR 3.503, 95 % CI 2.670–4.596).

Take-home points:

#1.  TGFBR2 rs6785358 polymorphism is associated with increased risk of VSD in a Chinese population.

#2. The G allele carriers were associated with increased risk of VSD compared with the A allele carriers.

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