Fundoplication & Gastrostomy ...outcome of single ventricle

Keating JJ et al.

JTCVS 2012;143:891-895

1999=2007

n=155

32 (21%) had fundoplication &/or G-tube (24 had both. 7 had G-tube only).

65 (42%) were HLHS. 24 of 65 had Fundos &/or G-tube.

Need for fundo &/or Gtube was associated with lower transplant-free survival.

CLOSURE trial result (PFO closure for Stroke)

Closure or Medical Therapy for Cryptogenic Stroke with Patent Foramen Ovale

Anthony J. Furlan et al.

NEJM 2012;366:991-9

Results:
A total of 909 patients were enrolled in the trial. The cumulative incidence (Kaplan–Meier estimate) of the primary end point was 5.5% in the closure group (447 patients) as compared with 6.8% in the medical-therapy group (462 patients) (adjusted hazard ratio, 0.78; 95% confidence interval, 0.45 to 1.35; P=0.37). The respective rates were 2.9% and 3.1% for stroke (P=0.79) and 3.1% and 4.1% for TIA (P=0.44). No deaths occurred by 30 days in either group, and there were no deaths from neurologic causes during the 2-year follow-up period. A cause other than paradoxical embolism was usually apparent in patients with recurrent neurologic events.

Conclusions:
In patients with cryptogenic stroke or TIA who had a patent foramen ovale, closure with a device did not offer a greater benefit than medical therapy alone for the prevention of recurrent stroke or TIA. (Funded by NMT Medical).

Procedures: Pericardiocentesis

Emergency pericardiocentesis video from NEJM 2012;366:e17

Realtime 3D in Congenital Heart Disease

Echocardiography 2012;29:232-41

Real time three-dimensional echocardiography (RT3DE) has been increasingly used in the diagnosis and assessment of congenital heart disease. A growing body of literature suggests that this new technology can be used as an integrated approach to assess the morphology of simple and complex congenital heart defects, flow abnormality, and left, right, and single ventricular function both qualitatively and quantitatively. This review summarizes the available evidence for the use of RT3DE in each of these areas. Future technology refinement in RT3DE and development of practice guidelines will increase the utilization of this new technology as a valuable tool to compliment 2D echocardiography/Doppler in clinical care and research to improve the care and outcome of congenital heart disease. (Echocardiography 2012;29:232-241)

How To Define Congenital Heart Disease in Scientific Studies

Congenital Heart Disease 2011;7(1):46-49

Estimates of the prevalence of congenital heart defects (CHD) have been published over many years and from many regions. As they are based on different definitions of which cases to include in the CHD prevalence, published prevalence estimates vary substantially. With the increasing use of echocardiography in neonatal intensive care, a patent ductus arteriosus (PDA) or flow over the atrial septum will often be visible. These findings may be coded as CHD at discharge and in this way falsely increase the CHD prevalence in the population. There are several purposes for which population-based data on CHD may be used: etiology, planning of treatment, or obtain information on outcome, including mortality. For etiology studies, it is important to include terminations of pregnancy as well as all births with CHD. For mortality studies in live births, inclusion of preterm born infants with PDA will increase overall mortality of CHD. The Danish Register of Congenital Heart Disease is based on hospital discharge diagnoses and diagnoses from outpatient visits. To increase the validity of these data, extensive data cleaning has been carried out based on record review and knowledge on the discharge coding practice. We include PDA and atrial septal defects as CHD cases if these defects are still open 2 months after birth. International consensus on how to define CHD would improve the validity and comparability of epidemiological studies on CHD.

Guidelines and Reviews on TAVI

Transcatheter Aortic Valve Implantation.
Resource center at Cardiosource.Org

PDE-5 inhibitors - Cardiac Uses

State-of-the-art Review: JACC 2012;59(1):9-15.

Bryan Schwartz et al.

Phosphodiesterase-5 inhibitors (PDE5Is) improve erectile function by enhancing nitric oxide availability in the penis and its supplying vasculature, resulting in vasodilation and increased blood flow. PDE5Is might benefit cardiovascular diseases because phosphodiesterase-5 is also located elsewhere in the body, including the pulmonary and systemic vasculature and in hypertrophied myocardium. PDE5Is are approved for pulmonary arterial hypertension, given that they improved several hemodynamic and clinical parameters in large randomized trials. Initial evidence suggests that PDE5Is benefit patients with congestive heart failure and secondary pulmonary hypertension. PDE5Is seem to improve hemodynamic and clinical parameters in patients with high-altitude pulmonary edema (HAPE) and high-altitude pulmonary hypertension. In climbers with prior episodes of HAPE, PDE5Is prevented HAPE in 2 small randomized trials. In small randomized trials of PDE5Is, patients with Raynaud's phenomenon demonstrated improved blood flow, fewer symptoms and frequency of attacks, and resolution of digital ulcers. In addition to enhancing vasodilation, PDE5Is seem to protect the myocardium through complex pathways that involve nitric oxide, cyclic guanosine monophosphate, protein kinase G, extracellular-signal-regulated kinase, B-cell lymphoma protein-2, and Rho kinase inhibition. In animal models of acute myocardial infarction, PDE5Is consistently reduced infarct size indicating cardioprotection and PDE5Is also promote reverse remodeling and reduce myocardial apoptosis, fibrosis, and hypertrophy. PDE5Is might also benefit patients with treatment-resistant hypertension, preeclampsia, or peripheral arterial disease. This review presents the pathophysiology and trial data with regard to the use of PDE5Is for cardiac diseases.

CLARINET trial

Wessel, D. et al.
(Presented at AHA 2010)

Background: Infants with cyanotic congenital heart disease (CCHD) palliated with a systemic-to-pulmonary artery (PA) shunt are at increased risk for shunt thrombosis and mortality.

Methods: We conducted a multi-center, randomized, double-blind, placebo-controlled trial to determine whether the addition of clopidogrel, 0.2mg/kg/day, to conventional therapy reduces all-cause mortality and shunt-related morbidity in infants with CCHD palliated with a PA shunt. The clopidogrel dose was selected to inhibit ADP-induced platelet aggregation by 30-50%, similar to the 75 mg adult dose. The primary efficacy outcome event was the first occurrence of any component of the composite endpoint of death, shunt thrombosis or a cardiac procedure before 120 days of age following an event considered of thrombotic nature. This event-driven trial conducted in 32 countries had 80% power to detect a 30% relative reduction in the primary event rate with 172 events and .05 overall type I error rate.

Results: 906 infants <3 months of age who had undergone a PA shunt were randomly assigned to receive clopidogrel (467, 51.5%) or placebo (439, 48.5%) in addition to conventional therapy. Median duration of treatment was 162 days. Concomitant aspirin was administered in 88% of subjects.

The primary composite outcome rate did not differ significantly between the clopidogrel vs. placebo groups: 19.1% vs. 20.5%, respectively, with relative risk reduction = 11.1% (95%CI: –19.2, 33.6; p=.43).

Components of the composite primary outcome, analyzed separately, also did not differ significantly between clopidogrel and placebo groups: mortality rate was 11.8% vs 13.9%; shunt thrombosis was 5.8% vs. 4.8%, and cardiac-related interventions occurred in 4.5% vs. 3.2%, respectively.
Clopidogrel treatment was not significantly beneficial within any subgroups, specific cardiac defect groups or shunt types. The percentage of subjects in the clopidogrel vs. placebo groups with any bleeding event (18.8% vs. 20.2%) and with severe bleeding events (4.1% vs. 3.4%) was similar.

Conclusion: Clopidogrel treatment of infants < 3 months of age with CCHD palliated with a PA shunt and predominantly receiving concomitant aspirin therapy does not reduce all-cause mortality or shunt-related morbidity.

PICOLO trial results

Dosing of Clopidogrel for Platelet Inhibition in Infants and Young Children
Primary Results of the Platelet Inhibition in Children On cLOpidogrel (PICOLO) Trial
Jennifer S, Li et al. for the PICOLO Investigators

Circulation 2008;117:553-9

Background— Infants and young children with certain types of heart disease are at increased risk for thromboses. Clopidogrel 75 mg/d is used in adults to prevent thrombotic events. The dose to achieve similar platelet inhibition in children is unknown. The objectives of the present study were (1) to determine the dose of clopidogrel needed in infants and young children to achieve a mean 30% to 50% inhibition of 5-μmol/L ADP–induced platelet aggregation (ie, inhibition similar to that observed with 75 mg in adults) and (2) to assess the safety and tolerability of clopidogrel in infants and young children.

Methods and Results— We performed a prospective, multicenter, randomized, placebo-controlled trial evaluating the pharmacodynamics of clopidogrel in children (0 to 24 months) with a cardiac condition at risk for arterial thrombosis. Patients were randomized to clopidogrel versus placebo in a 3:1 ratio in 4 sequential groups (0.01, 0.10, 0.20, and 0.15 mg/kg) for 7 abd 28 days. Platelet aggregation was assessed at baseline and steady state by light-transmission aggregometry. Of 116 patients enrolled, 92 (50% neonates, 50% infants/toddlers) were randomized, and 73 completed the study. A total of 79% of the randomized and treated patients were taking aspirin.

Compared with placebo, clopidogrel 0.20 mg · kg−1 · d−1 resulted in a mean 49.3% (95% confidence interval 25.7% to 72.8%) inhibition of the maximum extent of platelet aggregation and a mean 43.9% (95% confidence interval 18.6% to 69.2%) inhibition of the rate of platelet aggregation. There was marked interpatient variability in the degree of platelet aggregation inhibition within each treatment-dose group and age group. No serious bleeding events occurred.

Conclusions— Clopidogrel 0.20 mg/kg/day in children 0 to 24 months of age achieves a platelet inhibition level similar to that in adults taking 75 mg/d. Clopidogrel is well tolerated in infants and young children at this dose.

Risk Stratification in Brugada Syndrome

Results of PRELUDE (PRogrammed ELectrical stimUllation preDictive valuE) Registry.

SG Priori et al.

JACC 2012;59:37-45.

Study of 247 men (Median age 44 yrs, Range 18-72 yrs)

Results: During a median follow-up of 34 months, 14 arrhythmic events (4.5%) occurred (13 appropriate shocks of the implantable defibrillator, and 1 cardiac arrest). Programmed electrical stimulation performed with a uniform and pre-specified protocol induced ventricular tachyarrhythmias in 40% of patients: arrhythmia inducibility was not a predictor of events at follow-up (9 of 14 events occurred in noninducible patients). History of syncope and spontaneous type I ECG (hazard ratio [HR]: 4.20), ventricular refractory period <200 ms (HR: 3.91), and QRS fragmentation (HR: 4.94) were significant predictors of arrhythmias.

Conclusions: Our data show that VT/VF inducibility is unable to identify high-risk patients, whereas the presence of a spontaneous type I ECG, history of syncope, ventricular effective refractory period <200 ms, and QRS fragmentation seem useful to identify candidates for prophylactic implantable cardioverter defibrillator.

Radiation Dose

Use of dose-dependent follow-up protocol and mechanism to reduce patient and staff radiation exposure in congenital and structural interventions.
Sawdy JM, et al. Cath Cardiovasc Interv 2011;78:136-42
Editorial: Cath Cardiovasc Interv 2011;78:143-4

Cath: Balloon Pulmonary Valvuloplasty

Predictors of reintervention in neonates with critical PS or PA-IVS.

Ghassan Shaath et al.

Cath Cardiovasc Interv 2012;79:659-664.

n=43 babies.

Mean f-up 19 mo.

36% required reintervention after at mean age of 7.4 mo.

Predictors of reintervention were the following:

1) Diagnosis of PA-IVS

2) Hospital stay > 7.5 days

3) TR gradient > 43 mmHg on the day after intervention

All were statistically-derived parameters. No good clinical explanation in the paper.

From discussion secton:

Humpl et al.Circ 2003;108:826-32 reported reintervention was more likely if TV z-score was < -5.

Fedderly et al. JACC 1995;25:460-5 reintervention was less likely if TV annulus > 11 mm and PV annulus > 7 mm.

Alwi et al. Cardiol Young 2005;15:141-7. PDA stenting is suggested if if small RV and TV z-score between -2.5 and -4.5.