Publications by authors named "Robert W Loar"

24 Publications

  • Page 1 of 1

Dobutamine stress cardiac MRI is safe and feasible in pediatric patients with anomalous aortic origin of a coronary artery (AAOCA).

Int J Cardiol 2021 Apr 20. Epub 2021 Apr 20.

Pediatric Cardiology of Alaska, Seattle Children's Hospital, 3841 Piper St, Suite T345, Anchorage, AK 99508, USA.

Background: Risk stratification in anomalous aortic origin of a coronary artery (AAOCA) is challenged by the lack of a reliable method to detect myocardial ischemia. We prospectively studied the safety and feasibility of Dobutamine stress-cardiac magnetic resonance (DSCMR), a test with excellent performance in adults, in pediatric patients with AAOCA.

Methods: Consecutive DSCMR from 06/2014-12/2019 in patients≤20 years old with AAOCA were included. Hemodynamic response and major/minor events were recorded. Image quality and spatial/temporal resolution were evaluated. Rest and stress first-pass perfusion and wall motion abnormalities (WMA) were assessed. Inter-observer agreement was assessed using kappa coefficient.

Results: A total of 224 DSCMR were performed in 182 patients with AAOCA at a median age of 14 years (IQR 12, 16) and median weight of 58.0 kg (IQR 43.3, 73.0). Examinations were completed in 221/224 (98.9%), all studies were diagnostic. Heart rate and blood pressure increased significantly from baseline (p < 0.001). No patient had major events and 28 (12.5%) had minor events. Inducible hypoperfusion was noted in 31/221 (14%), associated with WMA in 13/31 (42%). Inter-observer agreement for inducible hypoperfusion was very good (Κ = 0.87). Asymptomatic patients with inducible hypoperfusion are considered high-risk and those with a negative test are of standard risk.

Conclusions: DSCMR is feasible in pediatric patients with AAOCA to assess for inducible hypoperfusion and WMA. It can be performed safely with low incidence of major/minor events. Thus, DSCMR is potentially a valuable test for detection of myocardial ischemia and helpful in the management of this patient population.
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http://dx.doi.org/10.1016/j.ijcard.2021.04.031DOI Listing
April 2021

Single systemic right ventricle longitudinal strain: Intravendor reproducibility and intervendor agreement in children.

Echocardiography 2021 Mar 1;38(3):402-409. Epub 2021 Feb 1.

Pediatric Cardiology, Cook Children's Medical Center, Fort Worth, TX, USA.

Purpose: Strain derived from speckle-tracking echocardiography is emerging as a useful tool in the assessment of single ventricle function. The purpose of this study is to compare layer-specific longitudinal strain values in children with single, systemic right ventricles (sRV) using two commercially available software platforms (GE EchoPAC (EP) and TomTec (TT)).

Methods: Two readers analyzed two-dimensional longitudinal strain on EP (v 202) and TT (v 2.21.25) in 40 pediatric sRV patients. Intravendor reproducibility and intervendor agreement between layer-specific measurements were assessed by intraclass correlation coefficient and Bland-Altman analysis. Absolute difference (AbΔ) and relative mean errors (RME) were calculated. Subgroup comparisons (stratified by age, heart rate (HR), and frames per second (FPS): HR ratio) were made.

Results: Median age was 4.4 years. 32 (80%) patients had hypoplastic left heart syndrome; 19 (48%) were post-Fontan. Intravendor reproducibility was excellent with high ICC (0.86-0.97). AbΔ between readers was small (1.2%-1.5%) with interobserver RME slightly higher for TT (11%-12% vs 8%-9% for EP). Layer-specific intervendor agreement was poor (ICC 0.45-0.62). Default layer comparisons (EP mid vs TT endo) showed good agreement (ICC 0.72-0.77) and less variability (AbΔ 2%, RME 15%) than layer-to-layer. There were no differences in ICC for groups dichotomized by age, HR, or FPS:HR ratio. sRV strain values are more negative when using EP.

Conclusion: Intravendor reproducibility for sRV peak longitudinal strain in children is excellent with acceptable variability between experienced users. Intervendor, layer-specific strain agreement is poor. Vendor default layer strain values show better agreement but are not interchangeable.
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http://dx.doi.org/10.1111/echo.14985DOI Listing
March 2021

Assessing the atria in pediatric sickle cell disease: Beyond the dilation.

Echocardiography 2021 Feb 17;38(2):189-196. Epub 2020 Dec 17.

Pediatric Cardiology, Baylor College of Medicine/Texas Children's Hospital, Houston, TX, USA.

Background: Diastolic dysfunction (DD) and pulmonary hypertension (PH) are common causes of mortality for sickle cell disease (SCD) patients in developed countries. We hypothesized that left and right atrial strain (LAS-Ɛ, RAS-rƐ) are decreased in SCD adolescents, and that worsening values correlate with laboratory markers of disease severity.

Methods: Prospective cohort study of patients with HbSS genotype of SCD was compared with healthy controls. LAS and RAS were measured from 4- and 2-chamber views by a blinded reader. Peak strain and strain rate values were obtained for atrial contraction (ac), reservoir (res), and conduit (con) phases. Mitral/tricuspid Doppler velocities, left atrial volume, right atrial area were obtained. Laboratory variables were obtained from the electronic record with the three prior values being averaged. Differences in variables were assessed with Wilcoxon rank sum test, and correlations assessed with Spearman's coefficient.

Results: There were 33 SCD patients compared to 35 healthy controls of similar age, gender, and size. SCD patients had increased left atrial volume and right atrial area. For LAS, Ɛres was significantly lower in SCD patients. For RAS, RƐcon was significantly lower. Neither measurement correlated with clinical markers. The majority of SCD patients had relatively normal atrial strain values. Those with markedly lower values had similar atrial size.

Conclusions: A sub-set of SCD patients have markedly low Ɛres and rƐcon. No correlation with clinical markers was identified. Larger, longitudinal studies may determine utility of atrial strain as a screening tool in this at-risk population.
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http://dx.doi.org/10.1111/echo.14956DOI Listing
February 2021

Assessing the left atrium of childhood cancer survivors.

Int J Cardiovasc Imaging 2021 Jan 16;37(1):155-162. Epub 2020 Aug 16.

Pediatric Cardiology, Baylor College of Medicine/Texas Children's Hospital, Houston, TX, USA.

Survivors of childhood cancer are at risk of cancer therapy-related cardiac dysfunction (CTRCD) characterized by systolic impairment, with little known about diastolic function. Left atrial strain (LAS) is a surrogate measure of left ventricular filling. We hypothesized that survivors (1) have lower LAS versus controls, and (2) survivors exposed to higher anthracycline dosage have even lower LAS. Cross-sectional study of 45 survivors exposed to anthracyclines ≥ 1 year after chemotherapy and 45 healthy controls. Echo variables included mitral spectral and tissue Doppler, left ventricular ejection fraction (LV EF), LV dimension, LA volume, LV global longitudinal strain (GLS), and LAS. Peak strain (Ɛ) and strain rate (SR) at three phases were obtained: atrial contraction (ac), reservoir (res), and conduit (con). Two sub-analyses of cancer survivors were performed: (1) those with anthracycline dosage ≥ 250 mg/m, and (2) those with Ɛres in the lowest quartile. On the whole, survivors had lower Ɛres and Ɛcon values. The majority of survivors had relatively normal LAS, while a subset had very low LAS values and were more likely to be older. Survivors exposed to ≥ 250 mg/m anthracycline also had lower Ɛres than those < 250 mg/m. There were no differences in mitral spectral/tissue Doppler, LV dimension, left atrial volume, or GLS. A subset of childhood cancer survivors have lower LAS than their healthy counterparts, while most are essentially normal. Those exposed to higher anthracycline dosage have even lower Ɛres. Longitudinal study of LAS may prove useful in monitoring for CTRCD.
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http://dx.doi.org/10.1007/s10554-020-01970-xDOI Listing
January 2021

Left Atrial Strain Correlates with Elevated Filling Pressures in Pediatric Heart Transplantation Recipients.

J Am Soc Echocardiogr 2020 04 24;33(4):504-511.e1. Epub 2020 Jan 24.

Department of Pediatric Cardiology, Baylor College of Medicine/Texas Children's Hospital, Houston, Texas.

Background: Noninvasive assessment of diastolic function in pediatric heart transplantation (PHTx) patients is important for monitoring of rejection, cardiac allograft vasculopathy, and nonspecific graft failure. We hypothesized that left atrial strain (LAS) would correlate with pulmonary capillary wedge pressure (PCWP) and that cutoff values to identify elevated left ventricular (LV) filling pressure could be derived for clinical practice and future testing.

Methods: This was a secondary analysis of a prospectively collected cohort of PHTx patients undergoing same-day cardiac catheterization with biopsy and transthoracic echo. There were 70 patients with 85 clinical encounters. Traditional mitral inflow Doppler, LAS, LV diastolic strain and strain rate, and ratios for mitral E to LV diastolic strain and strain rate were assessed. Correlation with PCWP was performed, and receiver operator characteristic curves were generated for an elevated mean PCWP, acute rejection, and cardiac allograft vasculopathy.

Results: Decreased LAS during the atrial reservoir phase (Ɛres) correlated with higher invasively measured PCWP (r = -0.40, P < .001). An Ɛres cutoff of 14.5% had good discriminatory ability for an elevated PCWP (sensitivity 75%, specificity 82%), and Ɛres > 22.0% had 100% negative predictive value; Ɛres was superior to other measures of diastolic function. Subanalyses for recent acute rejection (n = 9) showed good discriminatory ability for Ɛres of 14.5% (sensitivity 89%, specificity 74%).

Conclusions: LAS correlates with invasively measured PCWP and can identify elevated pressures better than traditional and other advanced diastolic function parameters. Use of LAS in PHTx patients may aid in noninvasive monitoring for rejection and nonspecific graft dysfunction.
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http://dx.doi.org/10.1016/j.echo.2019.11.004DOI Listing
April 2020

Myocardial Stress Perfusion MRI Using Regadenoson: A Weight-based Approach in Infants and Young Children.

Radiol Cardiothorac Imaging 2019 Oct 31;1(4):e190061. Epub 2019 Oct 31.

Department of Pediatrics, Division of Pediatric Cardiology (J.C.W., T.T.D., R.W.L., C.V.N.), Department of Radiology (A.S.P., P.M.M.), and Department of Pediatric Anesthesiology (P.M.T.), Texas Children's Hospital, Baylor College of Medicine, 6651 Main St, Legacy Tower, E1920, Houston, TX 77030.

Purpose: To determine the safety and feasibility of stress cardiac MRI by using weight-based dosing of regadenoson in patients less than 40 kg and whether stress cardiac MRI affects patient management.

Materials And Methods: All patients less than 40 kg undergoing stress cardiac MRI by using weight-based dosing (8 μg/kg) of regadenoson were included in this retrospective single-center study. Hemodynamic response, adverse events, and cardiac MRI abnormalities in myocardial perfusion, wall motion, and delayed enhancement were evaluated. Patient management based on the results of the stress cardiac MRI were evaluated.

Results: Forty-six consecutive stress cardiac MRI examinations were performed in 36 patients (median age, 9.0 years; age range, 2 months to 13.9 years) with congenital and acquired heart disease. Thirty-one of 46 (67.4%) studies were performed with the use of sedation. A myocardial perfusion defect was present in 20 of 46 (43.5%) studies, five with inducible defects only, and the remaining 15 with fixed or irreversible defects. In the 46 total studies, there were no major adverse events and nine (19.6%) minor adverse events including emesis ( = 1) and transient hypotension requiring pharmacologic intervention in eight patients who were all sedated. Sedation was an independent predictor for hypotension ( =.040). Twenty-six negative studies had no coronary interventions performed, and of the 20 positive studies, 15 were referred for catheterization, eight of which underwent coronary interventions.

Conclusion: Weight-based dosing of regadenoson for stress cardiac MRI was safe and feasible in infants and young children and played an integral role in the outcome and treatment decisions for children with coronary artery disease.© RSNA, 2019.
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http://dx.doi.org/10.1148/ryct.2019190061DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7977799PMC
October 2019

Regadenoson Stress Perfusion Cardiac Magnetic Resonance Imaging in Children With Kawasaki Disease and Coronary Artery Disease.

Am J Cardiol 2019 10 16;124(7):1125-1132. Epub 2019 Jul 16.

Department of Pediatrics, The Lillie Frank Abercrombie Section of Cardiology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.

Coronary artery (CA) stenosis and occlusion in convalescent Kawasaki disease (KD) is progressive and may result in myocardial infarction. The use of regadenoson, a strong selective CA vasodilator with low side effect profile, for stress cardiac magnetic resonance (CMR) imaging has not been studied in children with KD. The safety, feasibility, and diagnostic utility of regadenoson stress CMR was assessed in children with KD and CA abnormalities. A retrospective review of regadenoson stress CMR in children with convalescent KD was performed. Hemodynamics changes after regadenoson administration and adverse effects were recorded. First-pass perfusion was evaluated at rest and during pharmacologic stress. The results were compared with anatomic CA imaging. Forty-one stress CMR (18 sedated examinations, 44%) were performed successfully in 32 patients. Median age was 11.2 years (range 2.2 to 18.6) and weight 41 kg (range 13 to 93.4). Heart rate increased 66 ± 25% (p <0.005) after regadenoson. Minor adverse events occurred in 6 sedated and 1 unsedated patients. Hypoperfusion during stress occurred in 16 of 41 (39%), including 5 inducible, 9 inducible and fixed, and 2 fixed lesions. Late gadolinium enhancement was present in 10 of 16 with hypoperfusion and in 1 without hypoperfusion. Stress CMR had 100% positive agreement and >90% negative and overall agreement with moderate-to-severe CA stenoses. Four patients with hypoperfusion underwent revascularization for severe CA stenoses. In conclusion, regadenoson stress CMR is hemodynamically safe and feasible in children with KD and CA disease. It has excellent agreement with CA angiography and aided decision-making to proceed with revascularization.
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http://dx.doi.org/10.1016/j.amjcard.2019.06.033DOI Listing
October 2019

Serial Strain Analysis Identifies Hypoplastic Left Heart Syndrome Infants at Risk for Cardiac Morbidity and Mortality: A Pilot Study.

J Am Soc Echocardiogr 2019 05 22;32(5):643-650. Epub 2019 Feb 22.

Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.

Background: Validated, objective measures of right ventricular (RV) function assessment in hypoplastic left heart syndrome (HLHS) are needed. In other populations, speckle-tracking echocardiography-derived strain is a sensitive measure that outperforms conventional parameters of RV function. We hypothesized that speckle-tracking echocardiography-derived measures of RV function would be worse in patients with HLHS who have a poor cardiac outcome.

Methods: Prospective serial echocardiography was performed in 35 infants with HLHS during the first 6 months of life. Patients not undergoing staged palliation or with other variants of single RV were excluded. Traditional RV measurements and strain analysis were performed from standard apical and basal views. The primary outcome of cardiac death, heart transplantation, or persistent ≥ moderate RV dysfunction was examined using Cox regression analysis, and receiver operating characteristic curve analyses were performed to derive cutoff values.

Results: At median follow-up of 10.9 months (interquartile range 5.6, 15.2), eight patients reached the outcome and demonstrated worse RV strain measures compared with those without the outcome. A post-Norwood global longitudinal strain (GLS) of > -16% (area under the curve [AUC] = 0.76; P = .04) and pre-Glenn GLS > -13% (AUC, 0.98; P ≤ .01) were highly sensitive and specific for poor outcome. Other thresholds included post-Norwood GLS rate (GLSr) > -1.15 %/s (AUC, 0.78; P = .03), pre-Glenn GLSr = -0.85%/sec (AUC, 0.89; P < .01), post-Glenn circumferential strain rate > -0.85%/sec (AUC, 0.92; P < .01), and GLSr > -0.85%/sec (AUC, 0.84; P = .02).

Conclusions: Strain analysis may help identify at-risk HLHS infants. In this pilot study, interstage strain indices were worse in infants with HLHS who had a poor cardiac outcome.
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http://dx.doi.org/10.1016/j.echo.2019.01.006DOI Listing
May 2019

The impact of fetal endoscopic tracheal occlusion in isolated left-sided congenital diaphragmatic hernia on left-sided cardiac dimensions.

Prenat Diagn 2018 10 29;38(11):812-820. Epub 2018 Aug 29.

Lillie Frank Abercrombie Section of Pediatric Cardiology, Baylor College of Medicine, Houston, TX, USA.

Objectives: Fetal endoscopic tracheal occlusion (FETO) is offered to fetuses with congenital diaphragmatic hernia (CDH) and severe lung hypoplasia to promote lung growth and may secondarily affect left heart growth. The effects of FETO on left heart hypoplasia (LHH) are not described post-CDH repair.

Methods: A retrospective analysis was performed for fetuses with left-sided CDH who underwent FETO and severity-matched controls from 2007 to 2016 at our institution. Echocardiographic, ultrasound, and MRI data were reviewed. Left heart dimensions were assessed prenatally and postnatally. Primary clinical outcome evaluated was death.

Results: Twelve FETO patients and 18 controls were identified. Fetal LHH was noted in both groups and worsened after FETO. Postnatal mitral valve dimensions were larger in the FETO group pre-CDH repair (P = .03). Post-CDH repair, mitral valve and left ventricular dimensions were not significantly different between groups (P = .79 and P = .63 respectively) while FETO aortic valve dimensions were smaller (P = .04). Extracorporeal membrane oxygenation use was lower in the FETO group. No associations were found between left heart dimensions and outcomes.

Conclusion: Although increased lung growth was seen after FETO, fetal LHH persisted with relative normalization seen post-repair. Persistent LHH post-FETO could be secondary to a small contribution of pulmonary venous return to the fetal left heart and increased intrathoracic pressures post-FETO.
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http://dx.doi.org/10.1002/pd.5333DOI Listing
October 2018

State of the art review: Chemotherapy-induced cardiotoxicity in children.

Congenit Heart Dis 2018 Jan 11;13(1):5-15. Epub 2017 Dec 11.

Pediatric Cardiology, Texas Children's Hospital/Baylor College of Medicine, Houston, Texas, USA.

Chemotherapy-induced cardiotoxicity in adults and children is a topic with a growing interest in the cardiology literature. The ability to detect cardiac dysfunction in a timely manner is essential in order to begin adequate treatment and prevent further deterioration. This article aims to provide a review on the myocardial injury process, chemotherapeutic agents that lead to cardiotoxicity, the definition of cardiotoxicity, and the methods of timely detection and treatment.
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http://dx.doi.org/10.1111/chd.12564DOI Listing
January 2018

Fatal cardiac arrest in pediatric heart transplant recipients: Query of the UNOS database.

Pediatr Transplant 2018 02 10;22(1). Epub 2017 Dec 10.

Pediatric Cardiology, The Hospital for Sick Children, Toronto, ON, Canada.

The incidence of death by CA after PHTx is unknown. We aimed to determine the incidence and factors for fatal CA after PHTx, and whether a PM affects survival. Retrospective cohort study utilizing the United Network of Organ Sharing registry of patients transplanted ≤18 years. Multivariable analyses in hazard-function domain and Kaplan-Meier analyses were performed for an outcome of death due to CA. There were 7719 PHTx patients queried. CA was the reported cause of death in 11%. Age ≥13 years at time of transplant, presence of a PM, and depressed EF were identified as significant factors for fatal CA. Death due to CA beyond 10 years post-transplant was associated with depressed EF, CAV, and presence of a PM. Kaplan-Meier analysis demonstrated higher likelihood of fatal CA in patients with CAV and in those with a PM vs those without. In total, 15% of patients with a PM died from CA. CA is a relatively common cause of death after PHTx. The benefit of a PM remains unclear, but its presence does not confer complete protection. Patients with associated factors warrant vigilant surveillance and consideration for retransplantation.
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http://dx.doi.org/10.1111/petr.13094DOI Listing
February 2018

Visual Diagnosis: A Baby with a Scalp Lesion, Rash, and Left-Foot Deformity.

Pediatr Rev 2017 Jun;38(6):e20-e23

Pediatric Cardiology, Department of Pediatrics, Texas Children's Hospital/Baylor College of Medicine, Houston, TX.

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http://dx.doi.org/10.1542/pir.2016-0078DOI Listing
June 2017

Percutaneous Pulmonary Valve Implantation-Associated Ventricular Tachycardia in Congenital Heart Disease.

J Interv Cardiol 2016 Dec 26;29(6):639-645. Epub 2016 Sep 26.

The Lillie Frank Abercrombie Section of Pediatric Cardiology Texas Children's Hospital/Baylor College of Medicine, Houston, Texas.

Background: Percutaneous pulmonary valve implantation (PPVI) is utilized for dysfunctional right ventricular outflow tracts (RVOT) to relieve obstruction, regurgitation, and RV dysfunction. PPVI has not been reported to induce arrhythmias. This study is the first to report the incidence of ventricular tachycardia (VT) after PPVI.

Methods: This was a retrospective study of all patients who had PPVI at a single institution. All patients were admitted after PPVI for overnight telemetry monitoring. Patients with no prior history of VT and newly detected VT within 24 hours post-PPVI were considered to have VT related to PPVI.

Results: In total, 79 patients had PPVI (age 17 ± 9 years, 66% tetralogy of Fallot/pulmonary atresia). PPVI-related VT was detected in 6 patients (7.5%). These patients had a lower BMI (17.5 ± 2.0 vs. 23.1 ± 6.6, P = 0.04). There was no difference in age, native conduit or percutaneous valve size, or change in the minimum diameter of the RVOT from pre- to post-PPVI. In the 6 patients, VT was non-sustained and monomorphic at rates between 120 and 170. Five started on β-blockers. No patient required surgical explantation of the valve. Over a median follow-up of 2 years, 4 have weaned off medications and all are free of recurrence of VT with normal Holters.

Conclusions: PPVI may be associated with transient VT in the acute peri-procedural period. Patients of smaller size may be more susceptible. All patients were managed conservatively and none of the patients had a recurrence, which is suggestive of a transient phenomenon.
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http://dx.doi.org/10.1111/joic.12344DOI Listing
December 2016

Complex Surgical Repair of a Flail Tricuspid Valve After Chest Wall Trauma in a Pediatric Patient.

Ann Thorac Surg 2016 Mar;101(3):e65-7

Department of Congenital Heart Surgery, Texas Children's Hospital and Baylor College of Medicine, Houston, Texas.

Traumatic injury to the tricuspid valve can result from severe nonpenetrating chest wall trauma. We present the case of an initially asymptomatic 11-year-old girl who was kicked in the chest by a horse. The trauma resulted in avulsed papillary muscles, ruptured chordae, and right heart failure. She underwent early tricuspid valve reconstruction and annuloplasty. We advocate for routine use of echocardiography to assess for traumatic injury to intracardiac structures. In addition, we believe that early surgical intervention may prevent right-sided heart dysfunction, atrial arrhythmias, and the need for valve replacement.
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http://dx.doi.org/10.1016/j.athoracsur.2015.09.041DOI Listing
March 2016

Genotype-phenotype Correlations of Hypertrophic Cardiomyopathy When Diagnosed in Children, Adolescents, and Young Adults.

Congenit Heart Dis 2015 Nov-Dec;10(6):529-36. Epub 2015 Jun 10.

Children's Medical Center, Department of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minn, USA.

Background: Hypertrophic cardiomyopathy (HCM) is the most common heritable cardiovascular disease and a leading cause of identifiable sudden cardiac death (SCD) in the young. Herein, we sought to determine the genotype-phenotype correlations in a cohort of unrelated, genotyped patients diagnosed with HCM at a young age, as well as to characterize the differences between HCM diagnosed in adulthood and HCM diagnosed at a young age.

Methods And Results: From 1999 to 2011, 1053 unrelated patients diagnosed with HCM were enrolled in research-based genetic testing. The electronic medical record was reviewed to identify those with HCM diagnosed at ≤21 years (N = 137, mean age at diagnosis 13.2 ± 6 years, 64% male). From this cohort of patients recruited from a tertiary care referral center, the genetic test was positive in 71 (52%), which was significantly higher than patients diagnosed >21 years (31%; P < .001). Genotype-positive patients had increased maximum left ventricular wall thickness (24.9 ± 8.0 vs. 21.6 ± 7.4 mm, P = .01) and higher incidence of reverse-curve ventricular septal morphology (71% vs. 40%, P < .001). Unrelated to genotype status, 26/137 patients (19%) experienced significant HCM-related morbidity/mortality including progressive heart failure symptoms in 12, transplantation in 4, and death in 10.

Conclusions: Among patients diagnosed with HCM during the first two decades of life, the yield of genetic testing is significantly higher than when diagnosed at later age. While the phenotype of young HCM patients is worse than patients whose HCM is diagnosed at later age, the phenotypes of genotype-positive and genotype-negative young patients were similar. Independent of genotype, nearly 30% of the patients with follow-up in this study had symptom progression, transplant, or death.
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http://dx.doi.org/10.1111/chd.12280DOI Listing
October 2016

Lymphadenopathy With Ipsilateral Eye Involvement.

Clin Pediatr (Phila) 2015 Aug 27;54(9):910-2. Epub 2015 May 27.

Mayo Clinic, Rochester, MN, USA

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http://dx.doi.org/10.1177/0009922815588824DOI Listing
August 2015

Sudden cardiac arrest during sex in patients with either catecholaminergic polymorphic ventricular tachycardia or long-QT syndrome: a rare but shocking experience.

J Cardiovasc Electrophysiol 2015 Mar 15;26(3):300-4. Epub 2015 Jan 15.

Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota, USA.

Background: Patients with catecholaminergic polymorphic ventricular tachycardia (CPVT) and long-QT syndrome (LQTS) are susceptible to cardiac events during sympathetic nervous system activation. Herein, we sought to determine the risk of cardiac events associated with sex in CPVT and LQTS patients.

Methods And Results: We reviewed the electronic medical record of patients seen in the Genetic Heart Rhythm Clinic. There were 445 patients ≥ 18 years diagnosed with LQTS (N = 402, age at diagnosis 30 ± 16 years) or CPVT (N = 43, age at diagnosis 25 ± 15 years). No sex-induced cardiac events occurred in the LQTS population, and 2 occurred in the CPVT population. Sex-induced events were more likely in CPVT (2/43, 4.7%) than LQTS (0/402, 0%, P = 0.008). One case involved a 22-year-old CPVT1 female with prior cardiac arrest, who experienced several appropriate implantable cardioverter defibrillator shocks during intercourse while taking β-blockers. The second case was a 52-year-old CPVT1 male with history of recurrent exercise-triggered syncope, who had syncope during sex in the setting of β-blocker noncompliance. Extrapolating from published estimates of intercourse frequency by age, the overall event rate was only 0.0004%, and 0.005% among the CPVT cohort.

Conclusions: Potentially life-threatening cardiac events during sex in patients with CPVT are rare and even rarer in LQTS. Overall, the cardiac event per intercourse rate is extremely low. Patients and their partners should be reassured that sex is a low-risk activity from a cardiac standpoint.
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http://dx.doi.org/10.1111/jce.12600DOI Listing
March 2015

Septum primum atrial septal defect in an infant with hypoplastic left heart syndrome.

Heart Surg Forum 2014 Aug;17(4):E232-3

Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota, USA.

Hypoplastic left heart syndrome (HLHS) is a form of congenital heart disease characterized by severe underdevelopment of the left heart, leading to inadequate systemic blood flow. Several different atrial septal morphologies are observed in HLHS, most commonly a secundum atrial septal defect, patent foramen ovale, intact septum, and leftward displacement of the superior attachment of the septum primum. It has been postulated that atrial septal development is associated with the development of the left heart. We present a case of a newborn infant with HLHS and the unusual finding of a primum ASD.
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http://dx.doi.org/10.1532/HSF98.2014358DOI Listing
August 2014

Anasarca, hypoalbuminemia, and anemia: what is the correlation?

Clin Pediatr (Phila) 2014 Jun 18;53(7):710-2. Epub 2014 Mar 18.

Mayo Clinic, Rochester, MN, USA

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http://dx.doi.org/10.1177/0009922814526990DOI Listing
June 2014

Empiric switch from calcineurin inhibitor to sirolimus-based immunosuppression in pediatric heart transplantation recipients.

Pediatr Transplant 2013 Dec 6;17(8):794-9. Epub 2013 Oct 6.

Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, MN, USA.

Sirolimus is used in heart transplant patients with CAV and CNI-induced nephropathy. However, little is known regarding the tolerability, rejection rate, and effect on renal function when used empirically in children. We describe our experience with the empiric use of a sirolimus-based immunosuppressive regimen in pediatric heart transplantation recipients. We reviewed records of patients in whom conversion was attempted to a CNI-free sirolimus-based regimen. Rejection episodes and measures of renal function were recorded. We attempted to convert 20 patients, of which 16 were successful. In total, six of 20 patients (30%) experienced adverse effects. Of the 16 converted, four patients converted to sirolimus due to CNI-induced disease (three nephropathy, one CAV), while 12 patients (mean age 5.5 yr, range 0.1-21 yr; 33% female; 33% with a history of congenital heart disease) were empirically switched to sirolimus at a mean of 2.3 yr after transplant. Follow-up was available for a mean of 2.5 yr after conversion (range 0.5-8.3 yr). The rate of rejection while taking CNIs was 0.18 rejection episodes per patient-year (total of five episodes), compared with 0.03 rejection episodes per patient-year (total of one episode) while on sirolimus. Renal function, in terms of GFR, significantly improved after sirolimus conversion at latest follow-up (from 86 ± 37 mL/min to 130 ± 49 mL/min, p = 0.02). Here, we demonstrate the potential benefit of empiric use of sirolimus in pediatric heart transplant patients in a CNI-free regimen. Larger and longer studies are needed to further clarify risks of rejection and adverse effect profiles.
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http://dx.doi.org/10.1111/petr.12163DOI Listing
December 2013

Rebuttal: Aortic regurgitation and device closure of ASD or PFO.

Catheter Cardiovasc Interv 2015 Jan 8;85(1):179. Epub 2013 Mar 8.

Division of Pediatric Cardiology, Department of Pediatrics, Mayo Clinic, Rochester, Minnesota.

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http://dx.doi.org/10.1002/ccd.24876DOI Listing
January 2015

Posterior reversible encephalopathy syndrome and hemorrhage associated with tacrolimus in a pediatric heart transplantation recipient.

Pediatr Transplant 2013 Mar 17;17(2):E67-70. Epub 2013 Jan 17.

Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, MN, USA.

Posterior reversible encephalopathy syndrome (PRES) is a disorder characterized by gray and white matter abnormalities in the temporal, parietal, and occipital lobes of the brain. Its etiology has been attributed to renal failure, immunosuppressive drugs such as cyclosporine and tacrolimus, and other potential entities leading to acute hypertension. Clinical findings include headaches, altered mental status, seizures, visual changes, and focal neurologic deficits. We report the case of a child who developed PRES with intracerebral and subarachnoid hemorrhages associated with tacrolimus exposure 10 days after heart transplantation for restrictive cardiomyopathy. The patient initially presented with complex partial seizures, headache, agitation, and hypertension. Head MRI was suggestive of PRES along with intracerebral and subarachnoid hemorrhages. Tacrolimus was discontinued and blood pressure was controlled. The patient's encephalopathy resolved, but he has had ongoing neurologic symptoms secondary to hemorrhage. Generally, PRES is less common in children than in the adult population and is a rare complication of calcineurin inhibitors (CNI). Presentation with secondary hemorrhage also can occur. In children receiving CNIs presenting with new neurologic symptoms, PRES should be considered as prompt discontinuation of the offending agent can induce resolution of symptoms. Children can develop hemorrhage in the context of PRES, leading to increased morbidity.
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http://dx.doi.org/10.1111/petr.12039DOI Listing
March 2013

Effect of percutaneous atrial septal defect and patent foramen ovale device closure on degree of aortic regurgitation.

Catheter Cardiovasc Interv 2013 Jun 21;81(7):1234-7. Epub 2013 Feb 21.

Department of Pediatrics, Mayo Clinic, Rochester, Minnesota 55905, USA.

Background: In recent years, percutaneous device closure has become commonplace for treatment of atrial septal defect (ASD) and patent foramen ovale (PFO). However, the effect of device closure on aortic valve regurgitation (AR) has been questioned.

Objective: The purpose of this study was to define changes in AR using echocardiography, before and after device closure of ASD or PFO.

Methods: We retrospectively reviewed all patients who had percutaneous device closure of ASD or PFO at our institution between 2005 and 2009. We compared the preprocedure echocardiogram to the most recent follow-up study. All investigators were blinded to referral diagnosis, patient demographics, and size and type of the device placed. Aortic regurgitation was graded as none/trivial, mild, moderate, or severe.

Results: Overall, 322 patients qualified for inclusion; 204 with PFO and 118 with ASD. Mean age at time of closure was 48 ± 20 years (range 3-85 years). Mean duration of follow-up was 1.2 years (range 2 months to 5 years). Only two patients (0.6%) demonstrated any appreciable increase in AR severity. However, both of these patients manifested a clinically insignificant change from a trivial to mild grade of aortic regurgitation.

Conclusions: Percutaneous device closure of PFO or ASD does not increase the incidence or severity of AR over medium-term follow-up.
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http://dx.doi.org/10.1002/ccd.24779DOI Listing
June 2013

Hyperglycemia and diabetes mellitus in children with pancreatitis.

J Pediatr 2011 Apr 20;158(4):612-616.e1. Epub 2010 Nov 20.

Department of Pediatrics, Section of Endocrinology and Metabolism, Baylor College of Medicine, Houston, TX, USA.

Objective: To assess the risk factors for developing hyperglycemia and diabetes mellitus (DM) in children with pancreatitis.

Study Design: Patients (from infants to age 21 years) hospitalized with acute pancreatitis (AP), acute recurrent pancreatitis (ARP), and chronic pancreatitis were studied retrospectively. Subjects with known DM or cystic fibrosis before presentation with pancreatitis were excluded.

Results: A total of 176 patients met the study criteria. Of these, 140 had AP, 29 had ARP, and 7 had chronic pancreatitis. Severe pancreatitis was associated with hyperglycemia; 41% of the patients with hyperglycemia required insulin, and 8 patients (4.5%) developed DM requiring insulin by the time of discharge. These 8 patients with postpancreatitis DM were more likely to be overweight. Five of the 8 patients had a seizure disorder, and 4 had another comorbidity, such as mental retardation or cerebral palsy. Seven of the 8 patients who developed DM had a single episode of AP, and one patient had ARP.

Conclusions: Our findings indicate that hyperglycemia and DM can occur with pancreatitis. In some cases, postpancreatitis DM was associated with mental retardation, seizure disorder, and use of antiseizure medication. As opposed to adults who develop DM after chronic pancreatitis, children can develop DM due to a single episode of AP.
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http://dx.doi.org/10.1016/j.jpeds.2010.09.066DOI Listing
April 2011