MRI - Delayed enhancement (Congenital Aortic Stenosis)

Myocardial extracellular remodeling is associated with ventricular diastolic dysfunction in children and young adults with congenital aortic stenosis.

Dusenbery, SM, et al. JACC 2014;63(17):1778-85.

Objectives

This study sought to analyze cardiac magnetic resonance (CMR) measurements of myocardial extracellular volume fraction (ECV) and late gadolinium enhancement (LGE) in children and young adults with congenital aortic stenosis (AS) to determine the extent of fibrosis and examine their association with aortic valve and ventricular function.

Background

Patients with congenital AS frequently have impaired diastolic ventricular function and exercise capacity that may be related to myocardial fibrosis.

Methods

A total of 35 patients with congenital AS (median age 16 years) and 27 normal control subjects (median age 16 years) were evaluated by CMR. ECV was calculated from pre- and post-gadolinium contrast T1 measurements of blood and myocardium, and the hematocrit.

Results

ECV was significantly higher in AS patients than in normal subjects (median 0.27 [range 0.22 to 0.42] vs. 0.25 [range 0.18 to 0.27], p = 0.001). LGE was present in 8 (24%) of the AS patients. A higher ECV was correlated with echocardiographic indexes of diastolic dysfunction including a higher mitral E-wave z-score (r = 0.58, p = 0.002), E/septal E′ z-score (r = 0.56, p = 0.003), E/mean E′ z-score (r = 0.55, p = 0.003), and indexed left atrial volume (r = 0.56, p = 0.001). Other factors associated with an elevated ECV (>0.28) included a greater number of aortic valve interventions (p = 0.004) and a greater number of aortic valve balloon valvuloplasties (p = 0.003). ECV was not significantly associated with AS gradient, left ventricular mass, mass/volume ratio, or ejection fraction.

Conclusions

In young patients with AS, myocardial ECV is significantly elevated compared with control subjects and is associated with echocardiographic indexes of diastolic dysfunction. ECV measured by CMR may be a useful method for risk stratification and monitoring therapies targeting fibrosis.

Pediatric Dilated Cardiomyopathy - Recovery

Recovery of echocardiographic function in children with idiopathic cardiomyopathy. Results from Pediatric Cardiomyopathy Registry.

Melanie D. Everitt, et al. JACC 2014; 63(14): 1405-13.

Editorial: Alan Lewis. JACC 2014; 63(14): 1414-15.

Droxidopa (Northera) approved (Feb 2014)

For orthostatic hypotension.
Similar warnings like Midodrine.
Link to this news

Age at surgery: For D-TGA

JACC 2014:63:407-16
Objectives  This study sought to examine the impact of surgical timing on major morbidity and hospital reimbursement for late preterm and term infants with dextrotransposition of the great arteries (d-TGA).

Background  Neonatal arterial switch operation is the standard of care for d-TGA. Little is known about the effects of age at operation on clinical outcomes or costs for these neonates.

Methods  We conducted a retrospective cohort study of infants at ≥36 weeks' gestation, with d-TGA, with or without ventricular septal defects, admitted to our institution at 5 days of age or younger, between January 1, 2003 and October 1, 2012. Children with other cardiac abnormalities or other major comorbid conditions were excluded. Univariable and multivariable analyses were performed to determine the effects of age at operation on major morbidity and hospital reimbursement.

Results  A total of 140 infants met inclusion criteria. Reimbursement data were available for them through January 1, 2012 (n = 128). The mortality rate was 1.4% (n = 2). Twenty percent (n = 28) experienced a major morbidity. The median costs were $60,000, in 2012 dollars (range: $25,000 to $549,000). The median age at operation was 5 days (range: 1 to 12 days). For every day later that surgery was performed, beyond day of life 3, the odds of major morbidity increased by 47% (range: 23% to 66%, p < 0.001) and costs increased by 8% (range: 5% to 11%, p < 0.001), after considering the effects of sex, birth weight, gestational age, year at which surgery was performed, transfer, weekend admission, insurance, surgeon, septostomy, bypass and cross-clamp times, and the presence of ventricular septal defects or abnormal coronary anatomy.

Conclusions  Delay of neonatal arterial switch operation beyond 3 days is significantly associated with increased morbidity and healthcare costs.

Monitoring for brain injury during ECMO

Go to:

Abstract

Objective

To determine if, in children, plasma glial fibrillary acidic protein (GFAP) is associated with brain injury during extracorporeal membrane oxygenation (ECMO) and with mortality.

Design

Prospective, observational study.

Setting

Pediatric intensive care unit in an urban tertiary care academic center.

Patients

Neonatal and pediatric ECMO patients (n=22).

Interventions

Serial blood sampling for GFAP measurements.

Measurements and Main Results

Prospective patients age 1 day-18 years who required ECMO from April 2008 to August 2009 were studied. GFAP was measured using an electrochemiluminescent immunoassay developed at Johns Hopkins. Control samples were collected from 99 healthy children (0.5-16 years) and 59 NICU infants without neurologic injury. In controls, the median GFAP concentration was 0.055 ng/mL (IQR: 0-0.092 ng/mL) and the 95th percentile of GFAP was 0.436ng/mL. In ECMO patients, plasma GFAP was measured at 6, 12 and every 24 hours after cannulation. We enrolled 22 children who underwent ECMO. Median age was 7 days (IQR, 2 days-9 years), and primary ECMO indication was: cardiac failure, 6/22 (27.3%), respiratory failure, 12/22 (54.5%), ECPR, 3/22 (13.6%), and sepsis, 1/22 (4.6%). Seven of 22 (32%) patients developed acute neurologic injury (intracranial hemorrhage, brain death or cerebral edema diagnosed by imaging). Fifteen of 22 (68%) survived to hospital discharge. In the ECMO group, peak GFAP levels were higher in children with brain injury than those without (median, 5.9 vs 0.09ng/mL, p=0.04) and in non-survivors compared to survivors to discharge (median, 5.9 vs 0.09ng/mL, p=0.04). The odds ratio (OR) for brain injury for GFAP >0.436ng/mL vs normal was 11.5 (95%CI: 1.3-98.3) and the OR for mortality was 13.6 (95%CI: 1.7-108.5).

Conclusions

Pediatr Crit Care Med. 2011 September; 12(5): 572–579.

doi:  10.1097/PCC.0b013e3181fe3ec7

PMCID: PMC3686089

Glial fibrillary acidic protein as a brain injury biomarker in children undergoing extracorporeal membrane oxygenation

Bembea, MM, Savage W, Strouse JJ, et al.

Patients age 1 day-18 years who required ECMO from April 2008 to August 2009 were studied. 

GFAP was measured using an electrochemiluminescent immunoassay developed at Johns Hopkins. Control samples were collected from 99 healthy children (0.5-16 years) and 59 NICU infants without neurologic injury. 

In controls, the median GFAP concentration was 0.055 ng/mL (IQR: 0-0.092 ng/mL) and the 95th percentile of GFAP was 0.436ng/mL. 

In ECMO patients, plasma GFAP was measured at 6, 12 and every 24 hours after cannulation. 

We enrolled 22 children who underwent ECMO. Median age was 7 days (IQR, 2 days-9 years), and primary ECMO indication was: cardiac failure, 6/22 (27.3%), respiratory failure, 12/22 (54.5%), ECPR, 3/22 (13.6%), and sepsis, 1/22 (4.6%). Seven of 22 (32%) patients developed acute neurologic injury (intracranial hemorrhage, brain death or cerebral edema diagnosed by imaging). Fifteen of 22 (68%) survived to hospital discharge. 

In the ECMO group, peak GFAP levels were higher in children with brain injury than those without (median, 5.9 vs 0.09ng/mL, p=0.04) and in non-survivors compared to survivors to discharge (median, 5.9 vs 0.09ng/mL, p=0.04). 

The odds ratio (OR) for brain injury for GFAP >0.436ng/mL vs normal was 11.5 (95%CI: 1.3-98.3) and the OR for mortality was 13.6 (95%CI: 1.7-108.5).

Conclusions

High GFAP during ECMO is significantly associated with acute brain injury and death. Brain injury biomarkers may aid in outcome prediction and neurologic monitoring of ECMO patients to improve outcomes and benchmark new therapies.

Adult guidelines (Released online on 11/12/13)

Beware, this is for adults. Pediatric guidelines came out in late 2011.

1) Blood cholesterol therapy

2) Life style management to reduce CV risk

3) Management of overweight adult

3) Assessment of CV risk

Practice Guidelines

2013 ACC/AHA Guideline on the Treatment of Blood Cholesterol to Reduce Atherosclerotic Cardiovascular Risk in Adults: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines

Neil J. Stone, et al. J Am Coll Cardiol. Published online November 12, 2013. doi:10.1016/j.jacc.2013.11.002

2013 AHA/ACC Guideline on Lifestyle Management to Reduce Cardiovascular Risk: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines

Robert H. Eckel, et al. J Am Coll Cardiol. Published online November 12, 2013. doi:10.1016/j.jacc.2013.11.003

2013 AHA/ACC/TOS Guideline for the Management of Overweight and Obesity in Adults: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines and The Obesity Society

Michael D. Jensen, et al. J Am Coll Cardiol. Published online November 12, 2013. doi:10.1016/j.jacc.2013.11.004

2013 ACC/AHA Guideline on the Assessment of Cardiovascular Risk: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines

David C. Goff, et al. J Am Coll Cardiol. Published online November 12, 2013. doi:10.1016/j.jacc.2013.11.005

Post-Fontan thrombotic complications

J Am Coll Cardiol. 2013 Jan 22;61(3):346-53. doi: 10.1016/j.jacc.2012.08.1023. Epub 2012 Dec 12.

Factors associated with thrombotic complications after the Fontan procedure: a secondary analysis of a multicenter, randomized trial of primary thromboprophylaxis for 2 years after the Fontan procedure.

McCrindle BW, Manlhiot C, Cochrane A, Roberts R, Hughes M, Szechtman B, Weintraub R, Andrew M, Monagle P; Fontan Anticoagulation Study Group.

Source

Labatt Family Heart Centre, Department of Paediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada. brian.mccrindle@sickkids.ca

Abstract

OBJECTIVES:

The study sought to identify factors associated with increased risk of thrombosis after Fontan.

BACKGROUND:

The Fontan procedure is the culmination of staged palliation for patients with univentricular physiology. Thrombosis is an important complication after this procedure.

METHODS:

An international multicenter randomized controlled trial of acetylsalicylic acid versus warfarin for thromboprophylaxis after the Fontan procedure was conducted in 111 patients, and did not show a significant difference regarding thrombotic complications. We performed a secondary analysis of this previously published manuscript to identify factors associated with thrombosis in this population. Standardized prospective data collection included independent adjudication of all events.

RESULTS:

At 2.5 years after randomization, time-related freedom from thrombosis was 69% (all venous, no arterial events), with 28% of thrombosis presenting with clinical signs or events. Hazard of thrombosis was highest immediately after Fontan with a gradual increase in risk during late follow-up. In multivariable models, factors associated with higher risk of thrombosis were pulmonary atresia with intact ventricular septum (hazard ratio [HR]: 3.64, 95% confidence interval [CI]: 1.04 to 12.70, p = 0.04), pulmonary artery distortion (HR: 2.35, 95% CI: 0.96 to 5.73, p = 0.06), lower pre-operative unconjugated bilirubin (HR: 0.84 μmol/l, 95% CI: 0.72 to 0.99, p = 0.04), use of central venous lines for >10 days or until hospital discharge (HR: 17.8, 95% CI: 3.97 to 79.30, p < 0.001), and lower FiO(2) 24 h after the procedure (HR: 0.67/10%, 95% CI: 0.45 to 1.00, p = 0.06). Patients on warfarin who consistently achieved minimum target international normalized ratio levels or those on acetylsalicylic acid had a decrease in risk of thrombosis compared with patients who often failed to meet target international normalized ratio level (HR: 3.53, 95% CI: 1.35 to 9.20, p = 0.01).

CONCLUSIONS:

More favorable thromboprophylaxis strategies are needed in light of the difficulties in controlling warfarin therapy and the high prevalence of thrombosis in this population.

Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

Comment in

• An aspirin a day... [J Am Coll Cardiol. 2013]