Publications by authors named "Garick D Hill"

28 Publications

  • Page 1 of 1

Non-invasive left ventricular myocardial work indices in healthy adolescents at rest.

Int J Cardiovasc Imaging 2021 Mar 15. Epub 2021 Mar 15.

The Heart Institute, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH, 45229, USA.

Global myocardial work (GMW) provides a metric of left ventricular (LV) function and energy consumption. Its non-invasive assessment by echocardiography correlates with invasive measures and normal values have been reported in healthy adults. We aimed to establish normal values in a healthy adolescent population. Fifty-two healthy adolescents (mean age = 14.5 ± 2.0 years, range 11-19 years, 62% male) with normal echocardiograms were included. Brachial cuff blood pressure was obtained immediately following apical imaging in the supine position. Post-processing of echocardiograms for speckle tracking strain measurement and derivation of global myocardial work indices from LV pressure-strain loops was performed. The mean global work index (GWI) was 1802.0 ± 264.4 mmHg% with mean global work efficiency of 95.5 ± 1.1%. The mean global constructive work (GCW) was 2054.5 ± 297.3 mmHg%, and the mean global wasted work 83.8 ± 28.1 mmHg%. On multivariable analysis, there were significant associations between both GWI and GCW with systolic blood pressure (β coefficient = 0.57, p < 0.001; β coefficient = 0.67, p < 0.001 respectively) and LV global longitudinal strain (GLS) (β coefficient = - 0.56, p < 0.001; β coefficient = - 0.52, p < 0.001 respectively). There were no associations with any of the work indices with age, sex, body surface area, heart rate or LV ejection fraction. This study provides echocardiographic reference ranges for non-invasive indices of GMW in normal adolescents.
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http://dx.doi.org/10.1007/s10554-021-02218-yDOI Listing
March 2021

Commentary: The growing problem in congenital heart surgery.

Authors:
Garick D Hill

J Thorac Cardiovasc Surg 2021 Feb 4. Epub 2021 Feb 4.

Division of Cardiology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Electronic address:

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http://dx.doi.org/10.1016/j.jtcvs.2021.01.120DOI Listing
February 2021

Surgical Timing and Outcomes of Unilateral Versus Bilateral Superior Cavopulmonary Anastomosis: An Analysis of Pediatric Heart Network Public Databases.

Pediatr Cardiol 2021 Mar 8;42(3):662-667. Epub 2021 Jan 8.

Cincinnati Children's Hospital, Heart Institute, Cincinnati, OH, USA.

Requiring bilateral superior cavopulmonary anastomosis (bSCPA) instead of unilateral superior cavopulmonary anastomosis (uSCPA) could influence surgical timing and outcomes. We compared surgical timing and outcomes for patients who underwent uSCPA to those who underwent bSCPA through use of the Pediatric Heart Network's public datasets for the Infant Single Ventricle trial and Single Ventricle Reconstruction trial. There was no statistically significant difference in median age at SCPA (158 vs. 150 days, p = 0.68), hospital length of stay (LOS) (7 vs. 7 days, p = 0.74), intensive care unit (ICU) LOS (4 vs. 5 days, p = 0.53), time requiring ventilator support (2 vs. 2 days, p = 0.51), or oxygen saturation at discharge (82 vs. 81%, p = 0.22) between the uSCPA and bSCPA groups, respectively. However, sub-analysis comparing only those who underwent early SCPA, at < 120 days of age, revealed significantly longer hospital LOS (8 vs. 13 days, p = 0.04), ICU LOS (5 vs. 11 days, p = 0.01), and time requiring ventilator support (2 vs. 4 days, p = 0.03) for the early bSCPA group when compared to the early uSCPA group. A multivariable logistic regression revealed bSCPA to be the only significant predictor of prolonged hospital LOS for patients who underwent early SCPA (odds ratio 4.1, 95% CI 1.2-14.2). Overall, there was no difference in surgical timing or outcome measures between uSCPA and bSCPA. However, early bSCPA, performed at < 120 days, had worse outcome measures than early uSCPA. Delaying elective bSCPA until at least 120 days of age could minimize morbidity in infants with bilateral superior venae cavae.
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http://dx.doi.org/10.1007/s00246-020-02527-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7791322PMC
March 2021

Interstage Home Monitoring for Infants With Single Ventricle Heart Disease: Education and Management: A Scientific Statement From the American Heart Association.

J Am Heart Assoc 2020 08 11;9(16):e014548. Epub 2020 Aug 11.

This scientific statement summarizes the current state of knowledge related to interstage home monitoring for infants with shunt-dependent single ventricle heart disease. Historically, the interstage period has been defined as the time of discharge from the initial palliative procedure to the time of second stage palliation. High mortality rates during the interstage period led to the implementation of in-home surveillance strategies to detect physiologic changes that may precede hemodynamic decompensation in interstage infants with single ventricle heart disease. Adoption of interstage home monitoring practices has been associated with significantly improved morbidity and mortality. This statement will review in-hospital readiness for discharge, caregiver support and education, healthcare teams and resources, surveillance strategies and practices, national quality improvement efforts, interstage outcomes, and future areas for research. The statement is directed toward pediatric cardiologists, primary care providers, subspecialists, advanced practice providers, nurses, and those caring for infants undergoing staged surgical palliation for single ventricle heart disease.
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http://dx.doi.org/10.1161/JAHA.119.014548DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7660817PMC
August 2020

Long-Term Kidney Function After the Fontan Operation: JACC Review Topic of the Week.

J Am Coll Cardiol 2020 07;76(3):334-341

Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio. Electronic address:

The Fontan procedure has improved survival and quality of life for patients with single ventricle physiology. Along with extended life expectancy comes a growing population that is experiencing long-term multiorgan adverse effects. Whereas cardiorenal interactions have been extensively studied in patients with a structurally normal heart, these are less well understood in patients with a single ventricle. Several studies have investigated the prevalence of reduced glomerular filtration rate and albuminuria in the Fontan population; however, the long-term implication of renal dysfunction is not well established in this population. This paper provides a concise review of the published reports on the pathophysiology and spectrum of Fontan-associated renal disease. It also identifies gaps in currently available evidence that can guide ongoing and future research.
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http://dx.doi.org/10.1016/j.jacc.2020.05.042DOI Listing
July 2020

Atrioventricular Valve Regurgitation in Single Ventricle Heart Disease: A Common Problem Associated With Progressive Deterioration and Mortality.

J Am Heart Assoc 2020 06 16;9(11):e015737. Epub 2020 May 16.

Department of Pediatrics The Heart Institute Cincinnati Children's Hospital Medical Center University of Cincinnati College of Medicine Cincinnati OH.

The Fontan procedure has provided patients with single ventricle physiology extended survival into adulthood and in many cases has improved their quality of life. Atrioventricular valve regurgitation (AVVR) is common in single ventricle patients and is associated with increased risk of mortality. AVVR is more common in patients with a systemic tricuspid or common atrioventricular valve but is generally progressive irrespective of underlying valve morphology. AVVR can be attributable to diverse structural and functional abnormalities at multiple levels of the valvar apparatus, as well as ventricular dysfunction and dilation. Multiple imaging modalities including recent advances in 3-dimensional echocardiography and cross-sectional imaging have been used to further understand AVVR. Surgery to address AVVR must be tailored to the underlying mechanism and the timing of surgical repair should be chosen carefully. In this review, we discuss the etiologies, treatment options, surgical timing, and outcomes of valve repair or replacement for AVVR in patients with single ventricle congenital heart disease, with a focus on those with a Fontan circulation as AVVR is associated with increased risk for Fontan failure and mortality. In-depth understanding of the current literature will help guide clinicians in their approach and management of AVVR in this population.
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http://dx.doi.org/10.1161/JAHA.119.015737DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7429008PMC
June 2020

Improved National Outcomes Achieved in a Cardiac Learning Health Collaborative Based on Early Performance Level.

J Pediatr 2020 07 13;222:186-192.e1. Epub 2020 May 13.

Division of Pediatric Cardiology, Congenital Heart Center, C.S. Mott Children's Hospital, University of Michigan Medical School, Ann Arbor, MI; and.

Objective: Within the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), a learning health network developed to improve outcomes for patients with hypoplastic left heart syndrome and variants, we assessed which centers contributed to reductions in mortality and growth failure.

Study Design: Centers within the NPC-QIC were divided into tertiles based on early performance for mortality and separately for growth failure. These groups were evaluated for improvement from the early to late time period and compared with the other groups in the late time period.

Results: Mortality was 3.8% for the high-performing, 7.6% for the medium-performing, and 14.4% for the low-performing groups in the early time period. Only the low-performing group had a significant change (P < .001) from the early to late period. In the late period, there was no difference in mortality between the high- (5.7%), medium- (7%), and low- (4.6%) performing centers (P = .5). Growth failure occurred in 13.9% for the high-performing, 21.9% for the medium-performing, and 32.8% for the low-performing groups in the early time period. Only the low-performing group had a significant change (P < .001) over time. In the late period, there was no significant difference in growth failure between the high- (19.8%), medium- (21.5%), and low- (13.5%) performing groups (P = .054).

Conclusions: Improvements in the NPC-QIC mortality and growth measures are primarily driven by improvement in those performing the worst in these areas initially without compromising the success of high-performing centers. Focus for improvement may vary by center based on performance.
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http://dx.doi.org/10.1016/j.jpeds.2020.03.014DOI Listing
July 2020

Variation in care for children undergoing the Fontan operation for hypoplastic left heart syndrome.

Cardiol Young 2019 Dec 26;29(12):1510-1516. Epub 2019 Nov 26.

Divisions of Cardiothoracic Surgery and Pediatric Cardiology, University of Utah, Primary Children's Hospital, Salt Lake City, UT, USA.

Background: The Single Ventricle Reconstruction Trial randomised neonates with hypoplastic left heart syndrome to a shunt strategy but otherwise retained standard of care. We aimed to describe centre-level practice variation at Fontan completion.

Methods: Centre-level data are reported as median or median frequency across all centres and range of medians or frequencies across centres. Classification and regression tree analysis assessed the association of centre-level factors with length of stay and percentage of patients with prolonged pleural effusion (>7 days).

Results: The median Fontan age (14 centres, 320 patients) was 3.1 years (range from 1.7 to 3.9), and the weight-for-age z-score was -0.56 (-1.35 + 0.44). Extra-cardiac Fontans were performed in 79% (4-100%) of patients at the 13 centres performing this procedure; lateral tunnels were performed in 32% (3-100%) at the 11 centres performing it. Deep hypothermic circulatory arrest (nine centres) ranged from 6 to 100%. Major complications occurred in 17% (7-33%). The length of stay was 9.5 days (9-12); 15% (6-33%) had prolonged pleural effusion. Centres with fewer patients (<6%) with prolonged pleural effusion and fewer (<41%) complications had a shorter length of stay (<10 days; sensitivity 1.0; specificity 0.71; area under the curve 0.96). Avoiding deep hypothermic circulatory arrest and higher weight-for-age z-score were associated with a lower percentage of patients with prolonged effusions (<9.5%; sensitivity 1.0; specificity = 0.86; area under the curve 0.98).

Conclusions: Fontan perioperative practices varied widely among study centres. Strategies to decrease the duration of pleural effusion and minimise complications may decrease the length of stay. Further research regarding deep hypothermic circulatory arrest is needed to understand its association with prolonged pleural effusion.
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http://dx.doi.org/10.1017/S1047951119002658DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7207034PMC
December 2019

Survival After Norwood Procedure in High-Risk Patients.

Ann Thorac Surg 2020 03 11;109(3):828-833. Epub 2019 Sep 11.

Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin; and the Division of Cardiology, Children's Hospital of Wisconsin, Milwaukee, Wisconsin.

Background: Multiple single-ventricle populations are noted to be at increased risk for mortality after the Norwood procedure. Preoperative risk factors include low birth weight, restrictive/intact atrial septum, obstructed pulmonary veins, ventricular dysfunction, and atrioventricular valve regurgitation. We report outcomes of the Norwood procedure in standard- and high-risk patients in the recent era.

Methods: All patients born with hypoplastic left heart syndrome between 2006 and 2016 who underwent a Norwood procedure at our institution were included. Patient data were retrospectively reviewed, and Kaplan-Meier analysis was used to evaluate survival between groups.

Results: The cohort included 177 patients. Fifty patients were determined high-risk preoperatively: low birth weight (n = 18), ventricular dysfunction/atrioventricular valve regurgitation (n = 13), intact or restrictive atrial septum/obstructed anomalous pulmonary venous return (n = 14), and multiple factors (n = 5). There were 2 (1.6%) deaths before Glenn in the standard-risk group, with a total of 10 (20%) from the high-risk groups (P < .0001). Survival at 1 year differed greatly between groups, with highest being standard risk at 89% and lowest in the intact septum/obstructed veins group at 54%. The difference between groups in long-term survival was significant (P < .001).

Conclusions: Outcomes after the Norwood procedure have improved for standard-risk patients. Those with preoperative risk factors account for most of the early deaths after the Norwood procedure. This high-risk status does not resolve after Glenn, because longer-term survival continues to diverge from the standard-risk group.
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http://dx.doi.org/10.1016/j.athoracsur.2019.07.070DOI Listing
March 2020

Selective Use of Inpatient Interstage Management After Norwood Procedure.

Ann Thorac Surg 2020 01 10;109(1):139-147. Epub 2019 Sep 10.

Department of Pediatric Cardiology, Children's Hospital of Wisconsin, Milwaukee, Wisconsin.

Background: We report our intermediate-term results after Norwood procedure, including use of an interstage inpatient management strategy for high-risk patients, and seek to create a predictive model for probability of discharge.

Methods: A single-site retrospective review was conducted for all patients undergoing Norwood procedure from 2006 to 2016 (N = 177). We compared those discharged home with those who either remained hospitalized until Glenn procedure or died before Norwood procedure discharge. Multivariable logistic regression was used to develop a predictive model for discharge.

Results: During the study period, 120 (68%) patients were discharged home, 45 (25%) remained hospitalized, and 12 (7%) died before Glenn procedure (median age: 71 days). Interstage survival for those discharged after Norwood procedure was 100%. Longitudinal survival for the cohort was 86%, 81%, and 77% at 1, 5, and 10 years, resepectively. Ten-year survival was significantly greater for the discharged group compared with the interstage inpatients (86% vs 56%, P < .001). A reduced predictive model of discharge included lower gestational age (odds ratio [OR]: 0.95), lower median income for ZIP code (OR: 0.4), lower birth-weight-for-age z-score (OR: 0.56), longer cardiopulmonary bypass time (OR: 0.45), and Blalock-Taussig shunt (OR: 0.32).

Conclusions: Survival up to 10 years after Norwood procedure is good using a strategy of inpatient care for a subset of high-risk patients to mitigate home interstage mortality. A probabilistic model used after Norwood procedure was able to predict interstage discharge with good accuracy, but will require external validation to ensure generalizability. Further work is also needed to determine optimal palliative pathways for the high-risk patients because of the notable attrition beyond successful bidirectional Glenn procedure.
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http://dx.doi.org/10.1016/j.athoracsur.2019.07.062DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6917862PMC
January 2020

Outcomes Associated With Unplanned Interstage Cardiac Interventions After Norwood Palliation.

Ann Thorac Surg 2019 11 7;108(5):1423-1429. Epub 2019 Aug 7.

Division of Critical Care, Department of Pediatrics, Michael E. DeBakey Department of Surgery, Texas Children's Hospital and Baylor College of Medicine, Houston, Texas.

Background: Anatomic lesions are a common cause of decompensation during the interstage period after Norwood stage 1 palliation (S1P). This study describes the risk factors for and outcomes after unplanned surgical and catheter-based interstage cardiac interventions.

Methods: Participants in the National Pediatric Cardiology Quality Improvement Collaborative registry discharged from the hospital after S1P between 2008 and 2016 were studied. Variables at S1P, interstage, and at stage 2 palliation (S2P) hospitalizations were examined. Multivariable logistic regression was used to compare those who had an unplanned interstage intervention to those who did not.

Results: Of 1994 participants from 60 programs, 343 (17.1%) had at least 1 unplanned interstage intervention. Aortic valve dilation before S1P, longer S1P cardiopulmonary bypass time, pulmonary artery stent placement between S1P and discharge, aortic arch obstruction on the S1P discharge echocardiogram, and lower weight at S1P discharge were independently associated with receiving an unplanned interstage intervention. Interstage mortality between groups was similar at 6%, as was interstage duration. Participants undergoing unplanned interstage interventions were more likely to undergo heart transplant before S2P or deemed to be unsuitable for S2P (7.3% vs 2.7%, P < .001).

Conclusions: Unplanned interstage interventions after S1P did not increase interstage mortality, but participants with an unplanned intervention were less likely to progress to S2P. Residual anatomic lesions are risk factors for unplanned interstage interventions. For those with progressive ventricular dysfunction in the presence of arch obstruction by echocardiogram, aortic arch reintervention is warranted.
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http://dx.doi.org/10.1016/j.athoracsur.2019.06.041DOI Listing
November 2019

Echocardiographic assessment of single-ventricle diastolic function and its correlation to short-term outcomes after the Fontan operation.

Congenit Heart Dis 2019 Sep 24;14(5):720-725. Epub 2019 Jun 24.

Department of Pediatrics, University of Cincinnati, Cincinnati, Ohio.

Background/hypothesis/objectives: Postoperative complications after the Fontan operation for single ventricle heart disease are common and include persistent pleural drainage and prolonged length of hospital stay (LOS). Diastolic ventricular dysfunction may increase risk for postoperative complications by raising central venous pressures. We sought to determine the relationship between preoperative echocardiographic measurements of diastolic function, including myocardial deformation imaging, on (a) preoperative invasive catheterization measurements and (b) postoperative outcomes after the Fontan procedure.

Design/methods: All patients that underwent Fontan procedure from 2011 to 2017 were included. Echocardiograms performed within 6 months prior to Fontan operation were evaluated. Measurements of ventricular global and diastolic strain and strain rate were performed offline with TomTec speckle tracking software. Other diastolic function measurements included atrioventricular valve inflow and annular tissue Doppler imaging. Diastolic function measurements were correlated with pre-Fontan catheterization measurements and postoperative Fontan outcomes using Spearman's rho. Multivariable logistic regression for a prolonged LOS (>75%ile for postoperative LOS) was performed to adjust for preoperative risk factors.

Results: A total of 141 patients were included in the study. Majority had single morphologic right ventricle (58.9%). Median age at time of Fontan was 3.4 years (IQR 2.9-4). Median hospital LOS was 9 days (IQR 7-11). Circumferential diastolic strain rate weakly correlated with LOS (rho = -0.21, P = .01). There was no correlation between any other diastolic strain measurements and pre-Fontan end-diastolic pressure or postoperative LOS. In multivariable analysis, E/E' was the only echo measurement that predicted prolonged hospital LOS (OR 1.4, 95%CI: 1.1-1.8, P = .003).

Conclusion: Preoperative diastolic strain measurements did not have a strong association with postoperative Fontan outcomes. Increased E/E' ratio, however, did predict greater LOS after Fontan procedure, and may be useful in preoperative risk stratification. Future studies are needed to further assess the utility of diastolic strain imaging in the single-ventricle population.
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http://dx.doi.org/10.1111/chd.12814DOI Listing
September 2019

Transplacental treatment of fetal tachycardia: A systematic review and meta-analysis.

Prenat Diagn 2017 Nov;37(11):1076-1083

Division of Cardiology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, USA.

Objective: Multiple transplacental medications can be used to treat fetal tachycardia. We sought to perform a systematic review and meta-analysis to determine whether digoxin, flecainide, or sotalol was the most efficacious therapy for converting fetal tachycardia to sinus rhythm.

Method: We performed a systematic review and meta-analysis to compare digoxin, flecainide, or sotalol as first-line therapy for fetal tachycardia. Studies were identified by a search of PubMed (Medline), Web of Science, and Scopus.

Results: There were 21 studies included. Flecainide (OR: 1.4, 95% CI: 1.1-2.0, I  = 60%, P = 0.03) and sotalol (OR:1.4, 95% CI:1.1-2.0, I  = 30%, P = 0.02) were superior to digoxin for conversion of fetal tachycardia to sinus rhythm. In those with hydrops, the benefit over digoxin was more notable for both flecainide (OR: 5.0, 95% CI: 2.5-10.0, I  = 0%, P < 0.001) and sotalol (OR: 2.5, 95% CI: 1.7-5.0, I  = 0%, P < 0.001). When limited to atrioventricular reentrant tachycardia, flecainide was superior to digoxin (OR:1.7, 95% CI:1.1-3.3, I  = 62%, P = 0.03) and sotalol (OR:1.3, 95% CI:1.1-1.7, I  = 0%, P = 0.01).

Conclusion: Digoxin should not be first-line therapy for fetal tachycardia, particularly in the presence of hydrops fetalis. Flecainide should be the first-line therapy of choice in atrioventricular reentrant tachycardia. Further study may identify further sub-populations responding differently.
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http://dx.doi.org/10.1002/pd.5144DOI Listing
November 2017

Earlier Pulmonary Valve Replacement in Down Syndrome Patients Following Tetralogy of Fallot Repair.

Pediatr Cardiol 2017 Aug 14;38(6):1251-1256. Epub 2017 Jun 14.

Pediatric Cardiology, Children's Hospital of Wisconsin, 9000 W Wisconsin Ave, Milwaukee, WI, 53226, USA.

The association between Down syndrome and pulmonary hypertension could contribute to more severe pulmonary regurgitation after tetralogy of Fallot repair and possibly earlier pulmonary valve replacement. We compared cardiac magnetic resonance measures of pulmonary regurgitation and right ventricular dilation as well as timing of pulmonary valve replacement between those with and without Down syndrome after tetralogy of Fallot repair. Review of our surgical database from 2000 to 2015 identified patients with tetralogy of Fallot with pulmonary stenosis. Those with Down syndrome were compared to those without. The primary outcome of interest was time from repair to pulmonary valve replacement. Secondary outcomes included pulmonary regurgitation and indexed right ventricular volume on cardiac magnetic resonance imaging. The cohort of 284 patients included 35 (12%) with Down syndrome. Transannular patch repair was performed in 210 (74%). Down syndrome showed greater degree of pulmonary regurgitation (55 ± 14 vs. 37 ± 16%, p = 0.01) without a significantly greater rate of right ventricular dilation (p = 0.09). In multivariable analysis, Down syndrome (HR 2.3, 95% CI 1.2-4.5, p = 0.02) and transannular patch repair (HR 5.5, 95% CI 1.7-17.6, p = 0.004) were significant risk factors for valve replacement. Those with Down syndrome had significantly lower freedom from valve replacement (p = 0.03). Down syndrome is associated with an increased degree of pulmonary regurgitation and earlier pulmonary valve replacement after tetralogy of Fallot repair. These patients require earlier assessment by cardiac magnetic resonance imaging to determine timing of pulmonary valve replacement and evaluation for and treatment of preventable causes of pulmonary hypertension.
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http://dx.doi.org/10.1007/s00246-017-1653-2DOI Listing
August 2017

Assessment of Growth 6 Years after the Norwood Procedure.

J Pediatr 2017 01 14;180:270-274.e6. Epub 2016 Nov 14.

Departments of Pediatrics, Primary Children's Hospital, University of Utah, Salt Lake City, UT.

At 6 years of age, patients with hypoplastic left heart syndrome had mean age-adjusted z-scores for weight and height below the normative population, and body mass index was similar to the normative population. Males had the greatest increase in z-scores for body mass index.

Trial Registration: ClinicalTrials.gov: NCT00115934.
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http://dx.doi.org/10.1016/j.jpeds.2016.09.048DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5183480PMC
January 2017

Center Variability in Timing of Stage 2 Palliation and Association with Interstage Mortality: A Report from the National Pediatric Cardiology Quality Improvement Collaborative.

Pediatr Cardiol 2016 Dec 24;37(8):1516-1524. Epub 2016 Aug 24.

Divison of Cardiology, Department of Pediatrics, Medical College of Wisconsin, 9000 W Wisconsin Ave, Milwaukee, WI, 53226, USA.

For infants with single-ventricle lesions with aortic arch hypoplasia, the interstage period from discharge following stage 1 palliation (S1P) until stage 2 palliation (S2P) remains high risk. Significant variability among institutions exists around the timing of S2P. We sought to describe institutional variation in timing of S2P, determine the association between timing of S2P and interstage mortality, and determine the impact of earlier S2P on hospital morbidity and mortality. The National Pediatric Cardiology Quality Improvement Collaborative registry was queried. Centers were divided based on median age at S2P into early (n = 15) and late (n = 16) centers using a cutoff of 153 days. Groups were compared using Chi-squared or Wilcoxon rank-sum test. Multivariable logistic regression was used to determine risk factors for interstage mortality. The final cohort included 789 patients from 31 centers. There was intra- and inter-center variability in timing of S2P, with the median age by center ranging from 109 to 214 days. Late centers had a higher mortality (9.9 vs. 5.7 %, p = 0.03) than early centers. However, the event rate (late: 8.2 vs. early: 5.8 deaths per 10,000 interstage days) was not different by group (p = 0.26). Survival to hospital discharge and hospital length of stay following S2P were similar between groups. In conclusion, in a large multi-institution collaborative, the median age at S2P varies among centers. Although optimal timing of S2P remains unclear, centers performing early S2P did not experience worse S2P outcomes and experienced less interstage mortality.
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http://dx.doi.org/10.1007/s00246-016-1465-9DOI Listing
December 2016

Variable Myocardial Response to Load Stresses in Infants with Single Left Ventricular Anatomy: Influence of Initial Physiology and Surgical Palliative Strategy.

Pediatr Cardiol 2016 Dec 23;37(8):1569-1574. Epub 2016 Aug 23.

Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, 8701 Watertown Plank Road, Milwaukee, WI, 53226, USA.

Initial surgical strategies in neonates with single left ventricular (LV) anatomy vary based on adequacy of pulmonary and systemic blood flow. Differing myocardial responses to these strategies, as reflected in indices of systolic function, ventricular size, and mass have not been well defined. We sought to evaluate single LV myocardial response to varied physiology and initial palliation and determine whether the response is consistent and predictable. Infants with single LV physiology were divided based on neonatal palliation: no palliation/PA band (NO); BT shunt only (BT); or Norwood procedure (NP). Echo measures were obtained at presentation, early post-bidirectional Glenn (BDG), late post-BDG follow-up, and post-Fontan procedure. Measures included ejection fraction, LV mass indexed to height and end diastolic volume indexed to body surface area, and mass/volume ratio. The cohort included 38 children (13 NO, 13 BT, 12 NP). Ejection fraction was similar but depressed in all groups at all stages. LV mass was higher in the NP group than the BT group at early post-BDG (p = 0.03) and higher than both BT and NO groups (p < 0.01) at late post-BDG, but the difference was resolved by post-Fontan follow-up. The NP group had the most remarkable remodeling in LV size from BDG to Fontan, suggesting that volume unloading is most valuable in this subgroup. Ventricular remodeling can be identified by echocardiography in children with single LV physiology, despite variable initial surgical palliative strategies. Importantly, these initial surgical strategies do not result in significant differences after Fontan palliation during early childhood.
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http://dx.doi.org/10.1007/s00246-016-1471-yDOI Listing
December 2016

Surgical Valvotomy Versus Balloon Valvuloplasty for Congenital Aortic Valve Stenosis: A Systematic Review and Meta-Analysis.

J Am Heart Assoc 2016 08 8;5(8). Epub 2016 Aug 8.

Division of Cardiology, Department of Pediatrics, Duke University, Durham, NC.

Background: Optimal initial treatment for congenital aortic valve stenosis in children remains unclear between balloon aortic valvuloplasty (BAV) and surgical aortic valvotomy (SAV).

Methods And Results: We performed a contemporary systematic review and meta-analysis to compare survival in children with congenital aortic valve stenosis. Secondary outcomes included frequency of at least moderate regurgitation at hospital discharge as well as rates of aortic valve replacement and reintervention. Single- and dual-arm studies were identified by a search of PubMed (Medline), Embase, and the Cochrane database. Overall 2368 patients from 20 studies were included in the analysis, including 1835 (77%) in the BAV group and 533 (23%) in the SAV group. There was no difference between SAV and BAV in hospital mortality (OR=0.98, 95% CI 0.5-2.0, P=0.27, I(2)=22%) or frequency of at least moderate aortic regurgitation at discharge (OR=0.58, 95% CI 0.3-1.3, P=0.09, I(2)=54%). Kaplan-Meier analysis showed no difference in long-term survival or freedom from aortic valve replacement but significantly more reintervention in the BAV group (10-year freedom from reintervention of 46% [95% CI 40-52] for BAV versus 73% [95% CI 68-77] for SAV, P<0.001). Results were unchanged in a sensitivity analysis restricted to infants (<1 year of age).

Conclusions: Although higher rates of reintervention suggest improved outcomes with SAV, indications for reintervention may vary depending on initial intervention. When considering the benefits of a less-invasive approach, and clinical equipoise with respect to more clinically relevant outcomes, these findings support the need for a randomized controlled trial.
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http://dx.doi.org/10.1161/JAHA.116.003931DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5015309PMC
August 2016

Efficacy of Reconstituted Oral Chloral Hydrate from Crystals for Echocardiography Sedation.

J Am Soc Echocardiogr 2016 Apr 9;29(4):337-40. Epub 2016 Jan 9.

Medical College of Wisconsin, Milwaukee, Wisconsin.

Background: Chloral hydrate has been the drug of choice for uncooperative infants and children requiring sedation for echocardiography. Recently, the commercially available liquid formulation was discontinued by the manufacturer, and the only oral form of chloral hydrate available was made using reconstituted crystals. The aim of this study was to compare sedation efficacy before and after this change in chloral hydrate formulas.

Methods: Consecutive patients presenting for echocardiography sedation during the transition from the manufacturer-derived old formulation to the locally reconstituted new formulation were retrospectively reviewed for time to onset of level 3 sedation, duration of level ≤3 sedation, requirement for additional sedative medications, sedation failure, ability to complete the echocardiographic examination, and adverse events related to the sedatives.

Results: The cohort included 124 patients (63 old, 61 new). Although the mean age at sedation was younger for the new group, the weight and average dose of chloral hydrate used were not significantly different. There were no adverse events in either group. Time to onset of sedation was the same between the two formulations, but the duration of sedation was significantly shorter for the new group (42.4 ± 24.5 vs 55.3 ± 26.2 min, P = .01). In addition, the need for secondary sedating agents because of inadequate sedation and sedation failure were significantly greater using the new compared with the old formulation.

Conclusions: Chloral hydrate reformulation using reconstituted crystals results in a shorter duration of sedation, more frequent requirement for a secondary sedative agent, more frequent sedation failure, and occasional inability to complete the echocardiographic examination compared with the manufacturer's formulation.
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http://dx.doi.org/10.1016/j.echo.2015.11.014DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4818731PMC
April 2016

The Externally Supported Ross Operation: Early Outcomes and Intermediate Follow-Up.

Ann Thorac Surg 2015 Aug 2;100(2):631-8. Epub 2015 Jul 2.

Department of Cardiothoracic Surgery, Medical College of Wisconsin, Milwaukee, Wisconsin.

Background: The externally supported Ross (supported Ross), consisting of a Dacron (DuPont, Wilmington, DE) graft to support the neoaortic root, has been used in adolescent and adult patients to prevent neoaortic dilatation. Outcomes after the supported Ross technique were compared with the Ross procedure using the standard aortic root replacement technique (standard Ross).

Methods: This was a retrospective analysis of 36 adolescent and young adult patients who underwent the Ross procedure between 1992 and 2013. The outcomes of supported Ross procedures in 26 patients were compared with the Ross procedure in 10 patients. End points included survival, neoaortic root dilatation, development of neoaortic regurgitation, and the need for reintervention.

Results: The median age at operation was 14 years (range, 11 to 31 years), and indications for the operation were mixed lesions (47%), followed by aortic regurgitation (42%) and stenosis (11%). There were no early deaths. The mean follow-up was 2.2 years (range, 1 to 11 years). At the 1-year (p = 0.01) and 3-year (p < 0.05) follow-up, patients in the supported Ross cohort had a smaller neoaortic root z-score. Neither cohort had a large number of patients with significant neoaortic regurgitation, with 1 patient in the supported cohort compared with 3 patients in the standard cohort. Overall, 4 patients (40%) in the standard Ross cohort had required reintervention, including 3 directed at the neoaortic root. One patient in the supported Ross cohort required early reintervention for revision of the right coronary artery.

Conclusions: At intermediate follow-up, patients who underwent the supported Ross technique were less likely to have neoaortic root dilatation compared with patients who underwent a standard Ross procedure. Further studies are needed to evaluate the long-term durability of this technique, particularly in regards to the development of significant aortic regurgitation, the rate of reintervention, and application to younger and smaller patients.
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http://dx.doi.org/10.1016/j.athoracsur.2015.04.123DOI Listing
August 2015

Long-term outcomes after surgical repair of complete atrioventricular septal defect.

J Thorac Cardiovasc Surg 2015 Aug 7;150(2):369-74. Epub 2015 May 7.

Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wis; Department of Internal Medicine, Medical College of Wisconsin, Milwaukee, Wis.

Objective: Survival after surgical repair for complete atrioventricular septal defect (CAVSD) has improved, but patients are at risk for reoperation to address left atrioventricular valve regurgitation and left ventricular outflow tract obstruction. We examined the long-term survival, need for reoperation, and associated risk factors after CAVSD repair at our institution.

Methods: Between 1974 and 2000, a total of 198 patients underwent surgical repair for CAVSD. Of these, 178 patients survived to hospital discharge, of whom 153 (86%) had available follow-up data at a median postoperative time point of 17.2 years (range: 2 months to 38.1 years).

Results: Overall perioperative mortality was 10.1%, with a significant decrease to 2.9% in the late surgical era: 1991 to 2000 (P < .001). The overall estimated survival for the entire cohort was 85% at 10 years, 82% at 20 years, and 71% at 30 years after initial CAVSD repair. Requiring a reoperation after initial CAVSD repair was a risk factor for late mortality (P = .04). The estimated freedom from reoperation was 88% at 10 years, 83% at 20 years, and 78% at 30 years after initial CAVSD repair. Indications for reoperation included left atrioventricular valve regurgitation in 14 patients (7.1%) and left ventricular outflow obstruction in 7 patients (3.5%).

Conclusions: Long-term survival after repair of CAVSD remains good. However, the need for reoperation is common and affects long-term survival after CAVSD repair.
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http://dx.doi.org/10.1016/j.jtcvs.2015.05.011DOI Listing
August 2015

The Impact of Obesity on Postoperative Outcomes in Adults with Congenital Heart Disease Undergoing Pulmonary Valve Replacement.

Congenit Heart Dis 2015 Sep-Oct;10(5):E197-202. Epub 2015 Apr 27.

Department of Pediatrics, Division of Pediatric Cardiology, Medical College of Wisconsin, Milwaukee, Wis, USA.

Objective: The impact of obesity on surgical morbidity in adults with congenital heart disease is currently unknown. The aim of our study was to investigate the impact of obesity on postoperative outcomes in adults with congenital heart disease undergoing reoperation for pulmonary valve replacement.

Methods: A retrospective analysis was performed assessing the influence of obesity on surgical outcomes. Obesity was defined as a body mass index ≥30 kg/m2.

Results: The mean body mass index of the cohort was 25.9 ± 6.9 kg/m2 . The cohort included 71 patients with 17 patients (24%) being obese. There was no postoperative mortality. Obese patients had a longer hospital length of stay (6.6 vs. 4.7 days; P < .001) and increased incidence of postoperative arrhythmias (29% vs. 5.6%; P = .003) compared with nonobese patients. Multivariable analysis performed using logistic regression with backwards elimination demonstrated obesity was independently associated with hospital length of stay >5 days (odds ratio [OR] = 5.2; 95% confidence interval [CI]: 1.5-18.2, P = .01) and with increased postoperative arrhythmias (OR = 4.2; 95% CI: 1.7-40, P < .01).

Conclusions: Obesity is associated with increased morbidity in adults with congenital heart disease undergoing pulmonary valve replacement, including longer hospitalization and higher risk for postoperative arrhythmias.
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http://dx.doi.org/10.1111/chd.12266DOI Listing
August 2016

Impact of initial norwood shunt type on right ventricular deformation: the single ventricle reconstruction trial.

J Am Soc Echocardiogr 2015 May 15;28(5):517-21. Epub 2015 Feb 15.

Medical College of Wisconsin, Milwaukee, Wisconsin.

Background: The Single Ventricle Reconstruction trial demonstrated a transplantation-free survival advantage at 12-month follow-up for patients with right ventricle-pulmonary artery shunts (RVPAS) with the Norwood procedure compared with modified Blalock-Taussig shunts but similar survival and decreased global right ventricular (RV) function on longer term follow-up. The impact of the required ventriculotomy for the RVPAS remains unknown. The aim of this study was to compare echocardiography-derived RV deformation indices after stage 2 procedures in survivors with single RV anomalies enrolled in the Single Ventricle Reconstruction trial.

Methods: Global and regional RV systolic longitudinal and circumferential strain and strain rate, ejection fraction, and short-axis percentage fractional area change were all derived by speckle-tracking echocardiography from protocol echocardiograms obtained at 14.3 ± 1.2 months. Student t tests or Wilcoxon rank sum tests were used to compare groups.

Results: The cohort included 275 subjects (129 in the modified Blalock-Taussig shunt group and 146 in the RVPAS group). Longitudinal deformation could be quantified in 214 subjects (78%) and circumferential measures in 182 subjects (66%). RV ejection fraction and percentage fractional area change did not differ between groups. There were no significant differences between groups for global or regional longitudinal deformation. Circumferential indices showed abnormalities in deformation in the RVPAS group, with decreased global circumferential strain (P = .05), strain rate (P = .09), and anterior regional strain rate (P = .07) that approached statistical significance.

Conclusions: RV myocardial deformation at 14 months, after stage 2 procedures, was not significantly altered by the type of initial shunt placed. However, abnormal trends were appreciated in circumferential deformation for the RVPAS group in the area of ventriculotomy that may represent early myocardial dysfunction. These data provide a basis for longer term RV deformation assessment in survivors after Norwood procedures.
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http://dx.doi.org/10.1016/j.echo.2015.01.018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4426007PMC
May 2015

Tricuspid repair at pulmonary valve replacement does not alter outcomes in tetralogy of Fallot.

Ann Thorac Surg 2015 Mar 14;99(3):899-904. Epub 2015 Jan 14.

Department of Pediatrics, Division of Cardiology, Medical College of Wisconsin, Milwaukee, Wisconsin; Department of Internal Medicine, Division of Adult Cardiovascular Medicine, Medical College of Wisconsin, Milwaukee, Wisconsin. Electronic address:

Background: Chronic pulmonary regurgitation after tetralogy of Fallot repair often leads to progressive right ventricle dilation, dysfunction, and frequently, pulmonary valve replacement. For those with significant tricuspid regurgitation at the time of pulmonary valve replacement, it is unknown whether concomitant tricuspid valve repair improves postoperative outcomes.

Methods: This is a retrospective review of patients after tetralogy of Fallot repair who underwent pulmonary valve replacement between 1999 and 2012. Preoperative and postoperative echocardiograms were assessed for tricuspid regurgitation (vena contracta) and right ventricular size and function (Tomtec software).

Results: Sixty-two patients underwent pulmonary valve replacement. Thirty-six (58%) had greater than or equal to moderate tricuspid regurgitation on preoperative echocardiogram. Significant predictors were not identified. Of the 36, 18 (50%) underwent concomitant tricuspid valve repair at the time of pulmonary valve replacement. After surgery, there was a significant reduction in the degree of tricuspid regurgitation (p < 0.001) and measures of right ventricular size (p < 0.05) in both cohorts. Between surgical groups, there was no statistical difference in the grade of tricuspid regurgitation (p = 0.47) or measures of right ventricular size (p > 0.4) at 6-month follow-up.

Conclusions: Tricuspid regurgitation is a common finding in repaired tetralogy of Fallot, although risk factors for its development remain unclear. After pulmonary valve replacement with or without tricuspid valve repair there is significant improvement in the degree of tricuspid regurgitation and right ventricular size. Finally, 6 months after pulmonary valve replacement there were no statistical differences between those patients undergoing concomitant tricuspid valve repair and those undergoing pulmonary valve replacements alone.
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http://dx.doi.org/10.1016/j.athoracsur.2014.09.086DOI Listing
March 2015

Effect of feeding modality on interstage growth after stage I palliation: a report from the National Pediatric Cardiology Quality Improvement Collaborative.

J Thorac Cardiovasc Surg 2014 Oct 8;148(4):1534-9. Epub 2014 Feb 8.

Division of Critical Care, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wis.

Objectives: Achieving adequate growth after stage 1 palliation for children with single-ventricle heart defects often requires supplemental nutrition through enteral tubes. Significant practice variability exists between centers in the choice of feeding tube. The impact of feeding modality on the growth of patients with a single ventricle after stage 1 palliation was examined using the multiinstitutional National Pediatric Cardiology Quality Improvement Collaborative data registry.

Methods: Characteristics of patients were compared by feeding modality, defined as oral only, nasogastric tube only, oral and nasogastric tube, gastrostomy tube only, and oral and gastrostomy tube. The impact of feeding modality on change in weight for age z-score during the interstage period, from stage 1 palliation discharge to stage 2 palliation, was evaluated by multivariable linear regression, adjusting for important patient characteristics and postoperative morbidities.

Results: In this cohort of 465 patients, all groups demonstrated improved weight for age z-score during the interstage period with a mean increase of 0.3±0.8. In multivariable analysis, feeding modality was not associated with differences in the change in weight for age z-score during the interstage period (P=.72). Risk factors for poor growth were a diagnosis of hypoplastic left heart syndrome (P=.003), vocal cord injury (P=.007), and lower target caloric goal at discharge (P=.001).

Conclusions: In this large multicenter cohort, interstage growth improved for all groups and did not differ by feeding modality. With appropriate caloric goals and interstage monitoring, adequate growth may be achieved regardless of feeding modality and therefore local comfort and complication risk should dictate feeding modality.
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http://dx.doi.org/10.1016/j.jtcvs.2014.02.025DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4126898PMC
October 2014

Dexamethasone for acute asthma exacerbations in children: a meta-analysis.

Pediatrics 2014 Mar 10;133(3):493-9. Epub 2014 Feb 10.

Departments of Pediatrics, and.

Background And Objective: Dexamethasone has been proposed as an equivalent therapy to prednisone/prednisolone for acute asthma exacerbations in pediatric patients. Although multiple small trials exist, clear consensus data are lacking. This systematic review and meta-analysis aimed to determine whether intramuscular or oral dexamethasone is equivalent or superior to a 5-day course of oral prednisone or prednisolone. The primary outcome of interest was return visits or hospital readmissions.

Methods: A search of PubMed (Medline) through October 19, 2013, by using the keywords dexamethasone or decadron and asthma or status asthmaticus identified potential studies. Six randomized controlled trials in the emergency department of children ≤18 years of age comparing dexamethasone with prednisone/prednisolone for the treatment of acute asthma exacerbations were included. Data were abstracted by 4 authors and verified by a second author. Two reviewers evaluated study quality independently and interrater agreement was assessed.

Results: There was no difference in relative risk (RR) of relapse between the 2 groups at any time point (5 days RR 0.90, 95% confidence interval [CI] 0.46-1.78, Q = 1.86, df = 3, I2 = 0.0%, 10-14 days RR 1.14, 95% CI 0.77-1.67, Q = 0.84, df = 2, I2 = 0.0%, or 30 days RR 1.20, 95% CI 0.03-56.93). Patients who received dexamethasone were less likely to experience vomiting in either the emergency department (RR 0.29, 95% CI 0.12-0.69, Q = 3.78, df = 3, I2 = 20.7%) or at home (RR 0.32, 95% CI 0.14-0.74, Q = 2.09, df = 2, I2 = 4.2%).

Conclusions: Practitioners should consider single or 2-dose regimens of dexamethasone as a viable alternative to a 5-day course of prednisone/prednisolone.
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http://dx.doi.org/10.1542/peds.2013-2273DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3934336PMC
March 2014

Feeding dysfunction in children with single ventricle following staged palliation.

J Pediatr 2014 Feb 22;164(2):243-6.e1. Epub 2013 Oct 22.

Division of Cardiology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI; Division of Adult Cardiovascular Medicine, Department of Internal Medicine, Medical College of Wisconsin, Milwaukee, WI.

Objective: To determine the prevalence of feeding dysfunction in children with single ventricle defects and identify associated risk factors.

Study Design: Patients aged 2-6 years with single ventricle physiology presenting for routine cardiology follow-up at the Children's Hospital of Wisconsin were prospectively identified. Parents of the patients completed 2 validated instruments for assessment of feeding dysfunction. Chart review was performed to retrospectively obtain demographic and diagnostic data.

Results: Instruments were completed for 56 patients; median age was 39 months. Overall, 28 (50%) patients had some form of feeding dysfunction. Compared with a normal reference population, patients with single ventricle had statistically significant differences in dysfunctional food manipulation (P < .001), mealtime aggression (P = .002), choking/gagging/vomiting (P < .001), resistance to eating (P < .001), and parental aversion to mealtime (P < .001). Weight and height for age z-scores were significantly lower in subjects with feeding dysfunction (-0.84 vs -0.33; P < .05 and -1.46 vs -0.56; P = .001, respectively). Multivariable analysis identified current gastrostomy tube use (P = .02) and a single parent household (P = .01) as risk factors for feeding dysfunction.

Conclusion: Feeding dysfunction is common in children with single ventricle defects, occurring in 50% of our cohort. Feeding dysfunction is associated with worse growth measures. Current gastrostomy tube use and a single parent household were identified as independent risk factors for feeding dysfunction.
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http://dx.doi.org/10.1016/j.jpeds.2013.09.030DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3946833PMC
February 2014

Restrictive lung disease is an independent predictor of exercise intolerance in the adult with congenital heart disease.

Congenit Heart Dis 2013 May-Jun;8(3):246-54. Epub 2012 Oct 18.

Division of Cardiology, Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI 53226, USA.

Background/objectives: Following repair of congenital heart disease (CHD), adult patients are at risk for reduced exercise capacity. Restrictive lung disease (RLD) may contribute to reduced exercise capacity in this population. The aim of this study was to determine the prevalence of RLD and its impact on exercise tolerance in the adult with CHD.

Methods: One hundred consecutive adult patients with CHD, who underwent routine cardiopulmonary exercise testing with spirometry, were evaluated. Clinical data were obtained by retrospective chart review.

Results: Patients from 10 major diagnostic groups were identified. The median age for the cohort was 31 years (range 18-63) and included 43 males and 57 females. Most patients, 79%, had at least one previous surgical procedure. Based on spirometry and flow/volume loops, 50 patients were classified as normal pulmonary function, 44 patients had patterns suggestive of RLD, 4 suggestive of mixed (obstructive and restrictive), and 2 indeterminate. Risk factors associated with RLD include history of multiple thoracotomies (odds ratio = 9.01, P =.05) and history of atrial arrhythmias (odd ratio = 4.25, P =.05). Overall, 56% of the patients had abnormal exercise capacity. Spirometry suggestive of RLD was a significant risk factor for decreased exercise capacity (odds ratio = 3.65, P =.03). Patients with spirometry suggesting RLD also had lower exercise duration (P =.004) and a higher New York Heart Association Functional Class (P =.02). History of previous surgery and decreased heart rate reserve were also significant risk factors for decreased exercise capacity.

Conclusion: Abnormal spirometry suggestive of RLD is common in the adult with CHD and is a significant risk factor for decreased exercise tolerance in this population. Further studies are needed to evaluate the relationship between RLD and exercise intolerance and its relationship to mortality in the adult with CHD.
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http://dx.doi.org/10.1111/chd.12010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4832600PMC
January 2014