Jan Marek

Targeted Neonatal Echocardiography in the Neonatal Intensive Care Unit: Practice Guidelines and Recommendations for Training Writing group of the American Society of Echocardiography (ASE) in collaboration with the European Association of Echocardiography (EAE) and the Association for European Pediatric Cardiologists (AEPC)

Luc Mertens, MD, PhD, FASE, FESC, Istvan Seri, MD, PhD, HonD, Jan Marek, MD, PhD, FESC, Romaine Arlettaz, MD, Piers Barker, MD, FASE, Patrick McNamara, MD, MB, FRCPC, Anita J. Moon-Grady, MD, Patrick D. Coon, RDCS, FASE, Shahab Noori, MD, RDCS, John Simpson, MD, FRCP, FESC, Wyman W. Lai, MD, MPH, FASE, Toronto, Ontario, Canada; Los Angeles and San Francisco, California; London, United Kingdom; Zurich, Switzerland; Durham, North Carolina; Philadelphia, Pennsylvania; New York, New York

Targeted Neonatal Echocardiography in the Neonatal Intensive Care Unit: Practice Guidelines and Recommendations for Training

AAPn: American Academy of PediatricsnAEPCn: Association for European Paediatric CardiologynASEn: American Society of EchocardiographynCDHn: Congenital diaphragmatic hernianCHDn: Congenital heart diseasenEAEn: European Association of EchocardiographynECMOn: Extracorporeal membrane oxygenationnEFn: Ejection fractionnLVn: Left ventricularnMPIn: Myocardial performance indexnmVCFcn: Mean velocity of circumferential fiber shorteningnNICUn: Neonatal intensive care unitnPAn: Pulmonary arterynPDAn: Patent ductus arteriosusnRAn: Right atrialnRVn: Right ventricularnRVSpn: Right ventricular systolic pressurenSFn: Shortening fractionnSVCn: Superior vena cavanTEEn: Transesophageal echocardiographynTNEn: Targeted neonatal echocardiographynTVIn: Time-velocity integraln2Dn: Two-dimensionalnVLBWn: Very low birth weightnnThe role of echocardiography in the neonatal intensive care unit (NICU) has changed over the past few years. Previously, nearly all echocardiographic studies in the NICU were performed by pediatric cardiologists to diagnose or monitor congenital heart disease (CHD) and to screen for patent ductus arteriosus (PDA). More recently, neonatologists have become interested in the echocardiographic assessment of hemodynamic instability in infants. The terms functional echocardiography and point-of-care echocardiography have been introduced to describe the use of echocardiography as an adjunct in the clinical assessment of the hemodynamic status in neonates.1–4 The increasing availability of echocardiography, with miniaturization of the technology, has resulted in more widespread use of echocardiography in NICUs around the world.5 Perhaps the most significant challenge for the application of so-called functional studies is that newborns in the NICU with hemodynamic instability are at a much higher risk for having underlying CHD. In addition, newborns in the NICU are unique in that they are in the process of …

Long term results of percutaneous balloon valvoplasty of congenital aortic stenosis: independent predictors of outcome

Objective: To evaluate long term results and independent predictors of outcome of aortic valvoplasty. Design: Retrospective follow up study. Independent predictors of outcome identified by multiple logistic regression. Setting: Tertiary referral centre. Patients: 269 consecutive patients treated at the median age of 8 months (0–23 years): 80 (30%) under 4 weeks, 59 (22%) between 4 weeks and 1 year, and 130 (48%) over 1 year. The follow up period was up to 14.8 years (median 5.3, in survivors 6.4 years). Interventions: Percutaneous balloon valvoplasty with mean (SD) balloon to annulus ratio 0.97 (0.08). Main outcome measures: Restenosis ⩾ 70 mm Hg, grade 3 aortic insufficiency, cusps disruption, surgery, death, and valvoplasty failure (significant restenosis or insufficiency or surgery or death). Results: The mortality rate was 10.4% (n u200a=u200a 28), the restenosis rate was 16.7% (nu200a=u200a45), significant insufficiency developed in 22.3% (nu200a=u200a60), surgery was needed in 20.1% (nu200a=u200a54), and “valvoplasty failure” occurred in 41.6% (n u200a=u200a 112) patients. Mean (SEM) survival probability 14.4 years after the procedure was 0.89 (0.02) and mean (SEM) probability of surgery-free survival was 0.50 (0.08). The independent predictors were as follows. For restenosis: small aortic annulus; for cusp disruption: large aortic annulus; for insufficiency: bicuspid aortic valve; for need for surgery: bicuspid aortic valve; for death: small aortic annulus, low left ventricular shortening fraction, and low sequential number of the valvoplasty; and for valvoplasty failure: small aortic annulus, bicuspid aortic valve, and high grade of mitral insufficiency. Conclusion: Independent predictors of unfavourable outcome are small aortic annulus, bicuspid aortic valve, poor function of left ventricle or mitral valve, and limited operator experience.

Left ventricular hypertrophy and diastolic dysfunction in children with sickle cell disease are related to asleep and waking oxygen desaturation

Premature death and cardiac abnormalities are described in individuals with sickle cell disease (SCD), but the mechanisms are not well characterized. We tested the hypothesis that cardiac abnormalities in children with SCD are related to sleep-disordered breathing. We enrolled 44 children with SCD (mean age, 10.1 years; range, 4-18 years) in an observational study. Standard and tissue Doppler echocardiography, waking oxygen saturation averaged over 5 minutes, and overnight polysomnography were obtained in participants, each within 7 days. Eccentric left ventricular (LV) hypertrophy was present in 46% of our cohort. After multivariable adjustment, LV mass index was inversely related to average asleep and waking oxygen saturation. For every 1% drop in the average asleep oxygen saturation, there was a 2.1 g/m(2.7) increase in LV mass index. LV diastolic dysfunction, as measured by the E/E ratio, was present in our subjects and was also associated with low oxygen saturation (sleep or waking). Elevated tricuspid regurgitant velocity (> or = 2.5 m/sec), a measure of pulmonary hypertension, was not predicted by either oxygen saturation or sleep variables with multivariable logistic regression analysis. These data provide evidence that low asleep and waking oxygen saturations are associated with LV abnormalities in children with SCD.

Predictors of left ventricular remodelling and failure in right ventricular pacing in the young.

Aims To identify risk factors for left ventricular (LV) dysfunction in right ventricular (RV) pacing in the young. Methods and results Left ventricular function was evaluated in 82 paediatric patients with either non-surgical (n = 41) or surgical (n= 41) complete atrioventricular block who have been 100% RV paced for a mean period of 7.4 years. Left ventricular shortening fraction (SF) decreased from a median (range) of 39 (24–62)% prior to implantation to 32 (8–49)% at last follow-up (P < 0.05). Prevalence of a combination of LV dilatation (LV end-diastolic diameter >+2z-values) and dysfunction (SF < 0.26) was found to increase from 1.3% prior to pacemaker implantation to 13.4% (11/82 patients) at last follow-up (P = 0.01). Ten of these 11 patients had progressive LV remodelling and 8 of 11 were symptomatic. The only significant risk factor for the development of LV dilatation and dysfunction was the presence of epicardial RV free wall pacing (OR = 14.3, P < 0.001). Other pre-implantation demographic, diagnostic, and haemodynamic factors including block aetiology, pacing variables, and pacing duration did not show independent significance. Conclusion Right ventricular pacing leads to pathologic LV remodelling in a significant proportion of paediatric patients. The major independent risk factor is the presence of epicardial RV free wall pacing, which should be avoided whenever possible.

Permanent Cardiac Pacing in Children: Choosing the Optimal Pacing Site A Multicenter Study

Background— We evaluated the effects of the site of ventricular pacing on left ventricular (LV) synchrony and function in children requiring permanent pacing. Methods and Results— One hundred seventy-eight children (aged <18 years) from 21 centers with atrioventricular block and a structurally normal heart undergoing permanent pacing were studied cross-sectionally. Median age at evaluation was 11.2 (interquartile range, 6.3–15.0) years. Median pacing duration was 5.4 (interquartile range, 3.1–8.8) years. Pacing sites were the free wall of the right ventricular (RV) outflow tract (n=8), lateral RV (n=44), RV apex (n=61), RV septum (n=29), LV apex (n=12), LV midlateral wall (n=17), and LV base (n=7). LV synchrony, pump function, and contraction efficiency were significantly affected by pacing site and were superior in children paced at the LV apex/LV midlateral wall. LV dyssynchrony correlated inversely with LV ejection fraction (R=0.80, P=0.031). Pacing from the RV outflow tract/lateral RV predicted significantly decreased LV function (LV ejection fraction <45%; odds ratio, 10.72; confidence interval, 2.07–55.60; P=0.005), whereas LV apex/LV midlateral wall pacing was associated with preserved LV function (LV ejection fraction ≥55%; odds ratio, 8.26; confidence interval, 1.46–47.62; P=0.018). Presence of maternal autoantibodies, gender, age at implantation, duration of pacing, DDD mode, and QRS duration had no significant impact on LV ejection fraction. Conclusions— The site of ventricular pacing has a major impact on LV mechanical synchrony, efficiency, and pump function in children who require lifelong pacing. Of the sites studied, LV apex/LV midlateral wall pacing has the greatest potential to prevent pacing-induced reduction of cardiac pump function.

Impact of the permanent ventricular pacing site on left ventricular function in children: a retrospective multicentre survey.

Background Chronic right ventricular (RV) pacing is associated with deleterious effects on cardiac function. Objective In an observational multicentre study in children with isolated atrioventricular (AV) block receiving chronic ventricular pacing, the importance of the ventricular pacing site on left ventricular (LV) function was investigated. Methods Demographics, maternal autoantibody status and echocardiographic measurements on LV end-diastolic and end-systolic dimensions and volumes at age <18u2005years were retrospectively collected from patients undergoing chronic ventricular pacing (>1u2005year) for isolated AV block. LV fractional shortening (LVFS) and, if possible LV ejection fraction (LVEF) were calculated. Linear regression analyses were adjusted for patient characteristics. Results From 27 centres, 297 children were included, in whom pacing was applied at the RV epicardium (RVepi, n=147), RV endocardium (RVendo, n=113) or LV epicardium (LVepi, n=37). LVFS was significantly affected by pacing site (p=0.001), and not by maternal autoantibody status (p=0.266). LVFS in LVepi (39±5%) was significantly higher than in RVendo (33±7%, p<0.001) and RVepi (35±8%, p=0.001; no significant difference between RV-paced groups, p=0.275). Subnormal LVFS (LVFS<28%) was seen in 16/113 (14%) RVendo-paced and 21/147 (14%) RVepi-paced children, while LVFS was normal (LVFS≥28%) in all LVepi-paced children (p=0.049). These results are supported by the findings for LVEF (n=122): LVEF was <50% in 17/69 (25%) RVendo- and in 10/35 (29%) RVepi-paced patients, while LVEF was ≥50% in 17/18 (94%) LVepi-paced patients. Conclusion In children with isolated AV block, permanent ventricular pacing site is an important determinant of LV function, with LVFS being significantly higher with LV pacing than with RV pacing.

Physiological and Phenotypic Characteristics of Late Survivors of Tetralogy of Fallot Repair Who Are Free From Pulmonary Valve Replacement

Background— Pulmonary valve replacement (PVR) after repair of tetralogy of Fallot is commonly required and is burdensome. Detailed anatomic and physiologic characteristics of survivors free from late PVR and with good exercise capacity are not well described in a literature focusing on the indications for PVR. Methods and Results— Survival and freedom from PVR were tracked in 1085 consecutive patients receiving standard tetralogy of Fallot repair in a single institution from 1964 to 2009. Of 152 total deaths, 100 occurred within the first postoperative year. Surviving patients between 10 and 50 years of age had an annual risk of death of 4 (confidence limit, 2.8–5.4) times that of normal contemporaries. To date, 189 patients have undergone secondary PVR at mean age of 20±13 years (36% of those alive at 40 years of age). A random sample of 50 survivors (age, 4–57 years) free from PVR underwent cardiovascular magnetic resonance, echocardiography, and exercise testing. These patients had mildly dilated right ventricles (right ventricular end-diastolic volume=101±26 mL/m2) with good systolic function (right ventricular ejection fraction=59±7%). Most had exercise capacity within normal range (z peak O2=−0.91±1.3; z E/ CO2=0.20±1.5). In patients >35 years of age with normal exercise capacity, there was mild residual right ventricular outflow tract obstruction (mean gradient, 24±13 mmu2009Hg), pulmonary annulus diameters <0.5z, and unobstructed branch pulmonary arteries. Conclusions— An important proportion of patients require PVR late after tetralogy of Fallot repair. Patients surviving to 35 years of age without PVR and with a normal exercise capacity may have had a definitive primary repair; their right ventricular outflow tracts are characterized by mild residual obstruction and pulmonary annulus diameter <0.5z.

Value of Tissue Doppler Echocardiography in Children with Pulmonary Hypertension

BACKGROUNDnThe impact of pulmonary hypertension (PHT) on right ventricular and left ventricular (LV) function in children with PHT is unknown, and echocardiographic data combining conventional and Doppler tissue imaging (DTI) on PHT in children are sparse.nnnMETHODSnForty-one children (18 male; mean age, 7.9 ± 5.6 years) with PHT and structurally normal hearts (27 with idiopathic PHT, 14 with associated PHT) and 44 age-matched healthy controls were assessed using conventional echocardiography and DTI.nnnRESULTSnChildren with PHT had enlarged tricuspid valve diameters, right atrial areas, pulmonary artery dimensions, and LV eccentricity indices. In addition, pulmonary acceleration time and tricuspid annular plane systolic excursion were significantly reduced in patients compared with controls. DTI revealed that children with PHT had significantly lower systolic (S) and early diastolic (E) velocities at the tricuspid and septal levels. Despite preserved LV ejection fractions, left lateral free wall systolic velocities were significantly reduced in patients with PHT. Significantly reduced LV rapid filling velocities (E) suggested an underloaded left ventricle or LV diastolic dysfunction in children with PHT compared with controls. Pulmonary acceleration time and tricuspid annular plane systolic excursion correlated best with DTI systolic tricuspid and septal velocities.nnnCONCLUSIONSnDespite not being evident on conventional two-dimensional echocardiography, LV systolic performance appears to be impaired in children with PHT. Quantitative DTI assessment of ventricular function and ventricular-ventricular interactions in this setting might provide further insights into the mechanisms leading to end-stage PHT and may guide clinicians to optimize antifailure treatment.

Three-dimensional Echocardiography in Congenital Heart Disease: An Expert Consensus Document from the European Association of Cardiovascular Imaging and the American Society of Echocardiography

Three-dimensional echocardiography (3DE) has become important in the management of patients with congenitalxa0heart disease (CHD), particularly with pre-surgical planning, guidance of catheter intervention, and functional assessment of the heart. 3DE is increasingly used in children because of good acoustic windowsxa0and the non-invasive nature of the technique. The aim of this paper is to provide a review of the optimal application of 3DE in CHD including technical considerations, image orientation, application to different lesions, procedural guidance, and functional assessment.