Appropriate Use Criteria for Pediatric Transthoracic Echo

J Am Coll Cardiol. 2014 Nov 11;64(19):2039-60. doi: 10.1016/j.jacc.2014.08.003. Epub 2014 Sep 29.

ACC/AAP/AHA/ASE/HRS/SCAI/SCCT/SCMR/SOPE 2014 

Appropriate Use Criteria for Initial Transthoracic Echocardiography in Outpatient Pediatric Cardiology

Campbell RM, Douglas PS, Eidem BW, Lai WW, Lopez L, Sachdeva R.

Also, Pediatrics 2014;134:e1774

Likelihood Ratio: The AAP's Don'ts and Don'ts of Bronchiolitis, Expl...

Likelihood Ratio: The AAP's Don'ts and Don'ts of Bronchiolitis, Expl...: A lot of clinicians are confused about the new American Academy of Pediatrics guidelines on treating bronchiolitis , released just in tim...



Hilarious posting!

Rupture of sinus of Valsalva

Catheterization and Cardiovascular Interventions 2014;84:1184-89.

Li Zhong et al.

Transcatheter closure of ruptured sinus of Valsalva aneurysm.

n=22

From...To:

Non-coronary sinus to RA 11

Non-coronary sinus to RVinflow 1

Right coronary sinus to RVOT 8

Right coronary sinus to RA 2

Devices used: 

Amplatzer duct occluder 19

Amplatzer muscular VSD device 1

Outcome:

Successful in 20.

No residual shunt in 18, Small residual shunt in 2.

Complications:

Two patient needed urgent AVR because of procedure-related aortic valve regurgitation.

PDA closure: Ductal Spasm

Catheter Cardiovasc Interv. 2014 Apr 1;83(5):762-7. doi: 10.1002/ccd.25120. Epub 2013 Nov 9.

Ductal spasm during performance of transcatheter ductal occlusion.

Batlivala SP1, Glatz AC, Gillespie MJ, Dori Y, Rome JJ.

Author information

Abstract

OBJECTIVES:

Transcatheter patent ductus arteriosus (PDA) occlusion is a staple of pediatric catheterization laboratories. We present the phenomenon of significant PDA spasm to prevent failure to occlude a hemodynamically significant duct.

BACKGROUND:

Transcatheter techniques have evolved, allowing safe and effective occlusion of PDAs in younger and smaller patients. Neonatal care is evolving with increasing survival at younger gestational ages. Premature infants often have PDAs, so the proportion of formerly premature children referred for transcatheter ductal occlusion will likely rise.

METHODS:

We reviewed all transcatheter PDA occlusions performed at our institution since 2001 (N = 331). Retrospective data included: gestational age, age at catheterization, precatheterization echocardiographic parameters, PDA size (after spasm relief), device specifications, and most recent follow-up data.

RESULTS:

Seven cases were identified. Median age was 12 months, median gestational age was 28 weeks. All were born prematurely. All PDAs were restrictive and six had left-heart volume overload. All patients were examined by the interventional cardiologist and had ductal murmurs. When reauscultated (three of seven), murmurs were absent during spasm. Once spasm relieved, PDA diameters ranged from 1.5 to 8 (median 2) mm. All patients accommodated a 6-mm-or larger-Amplatzer device. No significant complications occurred and all patients were well at follow-up.

CONCLUSIONS:

Ductal spasm occurs during transcatheter occlusion and may be an unrecognized cause of procedural failure. The phenomenon seems to occur in children born prematurely, and can occur after infancy. Loss of a continuous murmur confirms the diagnosis. Care should be taken to avoid device under-sizing when spasm occurs.

Marfan syndrome: Losartan vs. Atenolol

NEJM 2014 - Online first (Presented at AHA 2014)

Atenolol versus Losartan in Children and Young Adults with Marfan's Syndrome

Lacro RV, et al.

Among children and young adults with Marfan's syndrome who were randomly assigned to losartan or atenolol, we found no significant difference in the rate of aortic-root dilatation between the two treatment groups over a 3-year period.

Protein losing enteropathy after Fontan operation - Improved survival

JACC 2014;64(1):54-62.

Clinical outcomes and improved survival in patients with protein-losing enteropathy after the Fontan Operation.
Anitha S. John, et al.

From Mayo clinic.

Fetal aortic valvuloplasty for evolving HLHS

Circulation 2014;130:638-45

Fetal Aortic Valvuloplasty for Evolving Hypoplastic Left Heart Syndrome

Postnatal Outcomes of the First 100 Patients

Linday R. Freud et al. from Boston

Background—Fetal aortic valvuloplasty can be performed for severe midgestation aortic stenosis in an attempt to prevent progression to hypoplastic left heart syndrome (HLHS). A subset of patients has achieved a biventricular (BV) circulation after fetal aortic valvuloplasty. The postnatal outcomes and survival of the BV patients, in comparison with those managed as HLHS, have not been reported.

Methods and Results—We included 100 patients who underwent fetal aortic valvuloplasty for severe midgestation aortic stenosis with evolving HLHS from March 2000 to January 2013. Patients were categorized based on postnatal management as BV or HLHS. Clinical records were reviewed. Eighty-eight fetuses were live-born, and 38 had a BV circulation (31 from birth, 7 converted after initial univentricular palliation). Left-sided structures, namely aortic and mitral valve sizes and left ventricular volume, were significantly larger in the BV group at the time of birth (P<0 .01="" 10="" 5.4="" 5="" 84="" 96="" a="" after="" all="" among="" and="" at="" better="" bv="" cardiac="" death="" em="" follow-up="" freedom="" from="" hlhs="" log-rank="" median="" of="" patients="" than="" was="" which="" years="">P

=0.04). There was no cardiac mortality in patients with a BV circulation from birth. All but 1 of the BV patients required postnatal intervention; 42% underwent aortic or mitral valve replacement. On the most recent echocardiogram, the median left ventricular end-diastolic volume z score was +1.7 (range, −1.3 to +8.2), and 80% had normal ejection fraction.

Conclusions—Short- and intermediate-term survival among patients who underwent fetal aortic valvuloplasty and achieved a BV circulation postnatally is encouraging. However, morbidity still exists, and ongoing assessment is warranted. 

• • Valvular Heart Disease

Heart Valve Prostheses in Pregnancy: Outcomes for Women and Their Infants

1. Claire M. Lawley, et al.
2. 1. JAHA 2014;3:e000953
   2. 1. 
         

1. ¹Clinical Population Perinatal Health Research, Kolling Institute, University of Sydney, Sydney, Australia (C.M.L., C.S.A., J.B.F., C.L.R.)
2. ²Department of Cardiology, Royal North Shore Hospital, St Leonards, Australia (C.M.L., G.A.F.)
3. ³Department of Obstetrics and Gynaecology, Royal North Shore Hospital, St Leonards, Australia (T.A.N.)

1. Correspondence to:
   Claire M. Lawley, BSc(Med)Hons, MBBS(Hons), Clinical Population Perinatal Health Research Group, The Kolling Institute, University of Sydney at Royal North Shore Hospital, Sydney, Australia. E‐mail: claw2317@uni.sydney.edu.au

Abstract

Background As the prognosis of women with prosthetic heart valves improves, an increasing number are contemplating and undertaking pregnancy. Accurate knowledge of perinatal outcomes is essential, assisting counseling and guiding care. The aims of this study were to assess outcomes in a contemporary population of women with heart valve prostheses undertaking pregnancy and to compare outcomes for women with mechanical and bioprosthetic prostheses.

Methods and Results Longitudinally linked population health data sets containing birth and hospital admissions data were obtained for all women giving birth in New South Wales, Australia, 2000–2011. This included information identifying presence of maternal prosthetic heart valve. Cardiovascular and birth outcomes were evaluated. Among 1 144 156 pregnancies, 136 involved women with a heart valve prosthesis (1 per 10 000). No maternal mortality was seen among these women, although the relative risk for an adverse event was higher than the general population, including severe maternal morbidity (139 versus 14 per 1000 births, rate ratio [RR]=9.96, 95% CI 6.32 to 15.7), major maternal cardiovascular event (44 versus 1 per 1000, RR 34.6, 95% CI 14.6 to 81.6), preterm birth (183 versus 66 per 1000, RR=2.77, 95% CI 1.88 to 4.07), and small‐for‐gestational‐age infants (193 versus 95 per 1000, RR=2.03, 95% CI 1.40 to 2.96). There was a trend toward increased maternal and perinatal morbidity in women with a mechanical valve compared with those with a bioprosthetic valve.

Conclusions Pregnancies in women with a prosthetic heart valve demonstrate an increased risk of an adverse outcome, for both mothers and infants, compared with pregnancies in the absence of heart valve prostheses. In this contemporary population, the risk was lower than previously reported.the

Congenital Heart Surgery Risk Scores

RACHS
RACHS-1
Aristotle Comprehensive Complexity Score (ACC)
STAT score (STS-EACTS)

Paper comparing RACHS-1 and ACC: Ann Thoracic Surgery 2011;92(3):949-56.
The ACC was a better predictor of operative mortality and length of intensive care unit stay than RACHS-1. In order to achieve similar performance, regression models including RACHS-1 need to be further adjusted on age, prematurity, and major extracardiac abnormalities.

Paper using STAT score: JTCVS 2014;147:666-71. Adult congenital heart surgery.

RACHS categories - partial list

Clinical outcome and improved survival in patients with protein-losing enteropathy after Fontan operation

Anitha John, et al.
From Mayo Clinic
JACC 2014;64(1):54-62.

Also see, Editorial JACC 2014;64(1):63-64.

Spectrum and outcome of primary cardiomyopathies diagnosed during fetal life

R Weber, ..., E. Jaeggi.
From Toronto group
JACC Heart Failure 2014;2(4):403-411

Incidence and causes of sudden death in college athletes.

Barry Maron et al.
JACC 2014;63:1636-43.

Drug induced Brugada syndrome in Children

Clinical features, device-based management and long-term follow-up.

Conte, G., et al.
JACC 2014;63:2272-9.

Elevation of cardiac troponin-T, but not cardiac troponin-I in patients with neuromuscular diseases

Implications for the diagnosis of myocardial infarction.
Rittoo D, et al.
JACC  2014;63:2411-20

Emergency Pericardiocentesis — NEJM

Emergency Pericardiocentesis — NEJM



Adult. Quite useful. But, this does not show placement of a catheter for continued drainage.

Digoxin in worsening heart failure

The use of digoxin in patients with worsening chronic heart failure.
Reconsidering an old drug to reduce hospital admissions.

Andrew Ambrosy, Javed Butler, Ali Ahmed, Muthiah Vaduganathan, Dirk J. van Veldhuisen, Wilson Colucci & Mihai Gheorghiade.
JACC 2014;63:1823-32.

Digoxin is the oldest cardiac drug still in contemporary use, yet its role in the management of patients with heart failure (HF) remains controversial. A purified cardiac glycoside derived from the foxglove plant, digoxin increases ejection fraction, augments cardiac output, and reduces pulmonary capillary wedge pressure without causing deleterious increases in heart rate or decreases in blood pressure. Moreover, it is also a neurohormonal modulator at low doses. In the pivotal DIG (Digitalis Investigation Group) trial, digoxin therapy was shown to reduce all-cause and HF-specific hospitalizations but had no effect on survival. With the discovery of neurohormonal blockers capable of reducing mortality in HF with reduced ejection fraction, the results of the DIG trial were viewed as neutral, and the use of digoxin declined precipitously. Although modern drug and device-based therapies have dramatically improved the survival of ambulatory patients with HF, outcomes for patients with worsening chronic HF, defined as deteriorating signs and symptoms on standard therapy often leading to unscheduled clinic or emergency department visits or hospitalization, have largely remained unchanged over the past 2 decades. The available data suggest that a therapeutic trial of digoxin may be appropriate in patients with worsening chronic heart failure who remain symptomatic.

Short QT syndrome

J Am Coll Cardiol. 2014 Apr 8;63(13):1300-8. doi: 10.1016/j.jacc.2013.09.078. Epub 2013 Nov 28.

Novel insight into the natural history of short QT syndrome.

Mazzanti A1, Kanthan A1, Monteforte N1, Memmi M1, Bloise R1, Novelli V1, Miceli C1, O'Rourke S2, Borio G1, Zienciuk-Krajka A3, Curcio A1, Surducan AE4,Colombo M4, Napolitano C5, Priori SG6.

Author information

Abstract

OBJECTIVES:

This study intends to gain further insights into the natural history, the yield of familial and genetic screening, and the arrhythmogenic mechanisms in the largest cohort of short QT syndrome (SQTS) patients described so far.

BACKGROUND:

SQTS is a rare genetic disorder associated with life-threatening arrhythmias, and its natural history is incompletely ascertained.

METHODS:

Seventy-three SQTS patients (84% male; age, 26 ± 15 years; corrected QT interval, 329 ± 22 ms) were studied, and 62 were followed for 60 ± 41 months (median, 56 months).

RESULTS:

Cardiac arrest (CA) was the most frequent presenting symptom (40% of probands; range, <1 0.0000001="" 1.3="" 20="" 40="" 41="" 44="" 4="" a="" age="" and="" arrhythmias="" at="" being="" between="" by="" ca.="" ca="" despite="" disease="" familial="" female="" fibrillation="" first="" follow-up="" genetic="" had="" history="" in="" kindreds.="" life="" low="" mainly="" male="" men="" month="" multiple="" observed="" occurred="" occurrence="" occurrences="" of="" only="" onset="" p="" patients="" patterns="" per="" predictor="" predominance="" present="" probability="" profile="" rate="" recurrences="" reproducible="" rest.="" risk="" screening="" superimposable="" that="" the="" to="" two="" ventricular="" was="" were="" with="" year="" years="" yield="">

CONCLUSIONS:

SQTS is highly lethal; CA is often the first manifestation of the disease with a peak incidence in the first year of life. Survivors of CA have a high CA recurrence rate; therefore, implantation of a defibrillator is strongly recommended in this group of patients.

Fontan - Hemodynamics

JACC Cardiovasc Imaging. 2014 Mar;7(3):215-24. doi: 10.1016/j.jcmg.2013.12.010. Epub 2014 Feb 13.

Geometric characterization of patient-specific total cavopulmonary connections and its relationship to hemodynamics.

Tang E1, Restrepo M2, Haggerty CM2, Mirabella L2, Bethel J3, Whitehead KK4, Fogel MA4, Yoganathan AP5.

Author information

• 1School of Chemical and Biomolecular Engineering, Georgia Institute of Technology, Atlanta, Georgia.
• 2Wallace H. Coulter Department of Biomedical Engineering, Georgia Institute of Technology and Emory University, Atlanta, Georgia.
• 3Westat, Rockville, Maryland.
• 4Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
• 5Wallace H. Coulter Department of Biomedical Engineering, Georgia Institute of Technology and Emory University, Atlanta, Georgia. Electronic address: ajit.yoganathan@bme.gatech.edu.

Abstract

Total cavopulmonary connection (TCPC) geometries have great variability. Geometric features, such as diameter, connection angle, and distance between vessels, are hypothesized to affect the energetics and flow dynamics within the connection. This study aimed to identify important geometric characteristics that can influence TCPC hemodynamics. Anatomies from 108 consecutive patients were reconstructed from cardiac magnetic resonance (CMR) images and analyzed for their geometric features. Vessel flow rates were computed from phase contrast CMR. Computational fluid dynamics simulations were carried out to quantify the indexed power loss and hepatic flow distribution. TCPC indexed power loss correlated inversely with minimum Fontan pathway (FP), left pulmonary artery, and right pulmonary artery diameters. Cardiac index correlated with minimum FP diameter and superior vena cava (SVC) minimum/maximum diameter ratio. Hepatic flow distribution correlated with caval offset, pulmonary flow distribution, and the angle between FP and SVC. These correlations can have important implications for future connection design and patient follow-up.

Troponin T: Diurnal Variation

Circulating Cardiac Troponin T Exhibits a Diurnal Rhythm

Klinkenberg LJJ. et al. JACC 2014:63(17):1788-95.

Objectives

The goal of this study was to test the unverified assumption that chronically elevated cardiac troponin T (cTnT) levels fluctuate randomly around a homeostatic set point.

Background

The introduction of high-sensitivity cardiac troponin (cTn) assays has improved sensitivity for acute myocardial infarction (AMI). However, many patients with a single positive cTn test result do not have AMI. Therefore, the diagnosis of AMI relies strongly on serial testing and interpretation of cTn kinetics. Essential in this regard is a profound understanding of the biological variation of cTn.

Methods

Two studies were conducted to assess biological cTnT variation and to investigate the presence of a diurnal rhythm of cTnT. Study 1 comprised 23 male subjects with type 2 diabetes, with no acute cardiovascular disease. Serial venous blood samples were drawn over an 11-h period (8:30 am to 7:30 pm). In study 2, the presence of a diurnal cTnT rhythm was investigated by hourly sampling of 7 subjects from study 1 over 25 h.

Results

In study 1, we observed a gradual decrease in cTnT concentrations during the day (24 ± 2%). This decrease was present in all participants and was most prominent in subjects with the highest baseline cTnT values (Pearson’s R 0.93). Diurnal variation of cTnT, as assessed in study 2, was characterized by peak concentrations during morning hours (8:30 am, 17.1 ± 2.9 ng/l), gradually decreasing values during daytime (8:30 pm, 11.9 ± 1.6 ng/l), and rising concentrations during nighttime (8:30 am the next day, 16.9 ± 2.8 ng/l).

Conclusions

A diurnal cTnT rhythm substantiates the recommendation that all dynamic changes in cTnT should be interpreted in relation to the clinical presentation. Epidemiological studies and risk-stratification protocols with the use of cTnT may benefit from standardized sampling times. (Exercise and Glycemic Control in Type 2 Diabetes; NCT00945165)

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.