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

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.

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

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.

Association of Wolff-Parkinson-White With Left Ventricular Noncompaction Cardiomyopathy in Children.

BACKGROUND: Wolff-Parkinson-White (WPW) has been associated with left ventricular noncompaction (LVNC) in children. Little is known about the prevalence of this association, clinical outcomes, and treatment options. METHODS: Retrospective review of subjects with LVNC. LVNC was defined by established criteria; those with congenital heart disease were excluded. Electrocardiograms (ECGs) were reviewed for presence of pre-excitation. Outcomes were compared between those with isolated LVNC and those with WPW and LVNC. RESULTS: A total of 348 patients with LVNC were identified. Thirty-eight (11%) were found to have WPW pattern on ECG, and 84% of those with WPW and LVNC had cardiac dysfunction. In Kaplan-Meier analysis, there was significantly lower freedom from significant dysfunction (ejection fraction ≤ 40%) among those with WPW and LVNC (P < .001). Further analysis showed a higher risk of developing significant dysfunction in patients with WPW and LVNC versus LVNC alone (hazard ratio 4.64 [2.79, 9.90]). Twelve patients underwent an ablation procedure with an acute success rate of 83%. Four patients with cardiac dysfunction were successfully ablated, 3 having improvement in function. CONCLUSION: WPW is common among children with LVNC and is associated with cardiac dysfunction. Ablation therapy can be safely and effectively performed and may result in improvement in function.

Imaging of the pulmonary valve in the adults.

PURPOSE OF REVIEW: Pulmonary valve is the least imaged of the cardiac valves in adults. This review will address the strengths and the limitations of various imaging modalities that are commonly used for evaluation of pulmonary valve diseases in the adults. RECENT FINDINGS: Valvular pulmonary stenosis is mostly congenital and pulmonary regurgitation is usually an acquired pulmonary valve disease. Combined pulmonary stenosis and pulmonary regurgitation as sequel to previous surgeries for congenital heart diseases is the most common form of pulmonary valve disease in the adults. Transthoracic echocardiography (TTE) is the first-line imaging for the pulmonary valve, however, TTE pose considerable technical limitations in adults secondary to the body habitus, previous surgeries, and imaging artifacts by the interposed foreign tissue. Transesophageal echocardiography is infrequently used because of farther location of the imaging probe from the pulmonary valve precluding optimal imaging. Cardiac computerized tomography (CT) and cardiac MRI are fast emerging as the most preferred imaging modalities for pulmonary valve diseases in the adults.CT is used to obtain precise anatomic information about the pulmonary valve and the contiguous anatomy such as the right ventricular outflow tract, the distal pulmonary arteries including the coronary arteries. MRI by virtue of its high temporal resolution has the unique advantage of obtaining hemodynamic information related to the pulmonary valve; such as quantification of the pulmonary regurgitation, the right ventricle volumes, mass, and the systolic function. Combined imaging with CT and MRI provide anatomic and hemodynamic information in a variety of pulmonary valve diseases; is useful for understanding of the right ventricle adaptive mechanism and remodeling process, for preprocedure planning during percutaneous pulmonary valve implantation and for surveillance of different pulmonary valve diseases. SUMMARY: Multimodality imaging involving a combination of TTE, CT, and (or) MRI are useful to delineate the anatomic and hemodynamic abnormalities associated with different pulmonary valve diseases in the adults.

Late outcomes in children with Shone's complex: a single-centre, 20-year experience.

OBJECTIVE: Shone's syndrome is a complex consisting of mitral valve stenosis in addition to left ventricle outflow obstruction. There are a few studies evaluating the long-term outcomes in this population. We sought to determine the long-term outcomes in our paediatric population with Shone's syndrome and the factors associated with left heart growth. METHODS: All patients diagnosed with Shone's syndrome with biventricular circulation treated between 1978 and 2010 were reviewed. Baseline echocardiograms and data from catheterisations were also reviewed. Number of interventions (surgical+transcatheter), incidence of mitral valve replacement, and incidence of heart transplantation were tracked. Survival of the population and left heart structural growth were also reviewed. RESULTS: A total of 121 patients with Shone's syndrome presented at a median age of 28 days (0-17.3 years) and were followed-up for 7.2 years (0.01-35.5 years). These patients underwent 258 interventions during the study period, and the presence of coarctation was associated with repeat left heart interventions. The 10-year, transplant-free survival was 86%. Presence of pulmonary hypertension was associated with mortality. Left heart structural growth was seen for mitral and aortic valve annuli and left ventricular end-diastolic dimension over time. CONCLUSIONS: Shone's syndrome patients undergo a number of left heart interventions. Coarctation of the aorta is associated with an increased likelihood for repeat interventions. Survival appears to be more favourable than expected. Significant left heart growth will occur in the population. Pulmonary hypertension is associated with an increased risk of mortality.

Erratum to: Abnormal Myocardial Strain Indices in Children Receiving Anthracycline Chemotherapy.

Erratum to: Pediatr Cardiol DOI 10.1007/s00246-015-1203-8. The original version of this article unfortunately contained a mistake in the author's group. The given name of Payam Ghazi was misspelled and the first and middle names of John Lynn Jefferies were interchanged. The two co-author names are corrected with this erratum.

Abnormal Myocardial Strain Indices in Children Receiving Anthracycline Chemotherapy.

Anthracycline chemotherapy (AC) is associated with impaired left ventricular (LV) systolic function. LV ejection fraction (EF %) obtained by two-dimensional echocardiography is the current gold standard for detection and monitoring of LV systolic function. However, dependence on LVEF has been shown to be unreliable due to its inherent limitations. Speckle tracking echocardiography (STE) measures myocardial strain and is a sensitive method to detect LV systolic dysfunction with demonstrated utility in such detection in adult and pediatric cohort studies. Compare myocardial strain indices derived by STE with LVEF to detect ACT-induced LV systolic dysfunction. Prospective, cross-sectional measurements of LV myocardial strain indices derived from STE with LVEF. Pediatric cohort of 25 patients (pts): 17 females, eight males with a mean age 9.8 ± 5.8 years, who received anthracyclines (AC); median cumulative dose ≥150 ± 124.4 mg/m(2), range 60-450 mg/m(2) showing normal LV end-diastolic diameter (mm) and normal LVEF (≥55 %) underwent STE to obtain LV myocardial strain indices: strain and strain rate. The inter- and intraobserver variability for the strain indices was 5 %. Fifteen of 25 pts (60 %) showed abnormal global longitudinal peak systolic strain (GLPSS) and 19/25 pts (76 %) showed abnormal peak circumferential strain (PCS) compared to age-matched controls (p = 0.005). In contrast, no significant differences was observed in either indices with the dose of AC. Likewise, no significant changes in the systolic or diastolic strain rate were noted with the dose of AC (r (2) = 0.0076 for peak E, r (2) = 0.072 for peak A, p = NS). GLPSS and PCS were diminished and, however, correlated poorly with the cumulative dose of AC. These observations indicate an early onset of LV systolic dysfunction by the strain indices in pts who continue to show a normal LVEF implying presence of occult LV systolic dysfunction. These novel strain indices may assist in early detection of LV systolic dysfunction with implications for monitoring and prevention of AC-induced LV systolic dysfunction.

Mitral Annular Disjunction and Its Progression during Childhood in Marfan Syndrome.

BACKGROUND: Data on mitral annular disjunction (MAD) in children with Marfan syndrome (MFS) are sparse. OBJECTIVES: To investigate the diagnostic yield of MAD by echocardiography and cardiac magnetic resonance imaging (CMR), its prevalence and progression during childhood. METHODS: We included patients <21 years old with MFS, defined by 2010 Ghent criteria and a pathogenic FBN1 variant or ectopia lentis. Two readers measured systolic separation between the mitral valve (MV) posterior hinge point and left ventricular (LV) myocardium on initial and subsequent imaging. MAD was defined as MV-LV separation ≥2 mm, MV prolapse (MVP) as atrial displacement ≥2 mm. Kappa coefficients evaluated echocardiogram-CMR agreement. Bland-Altman and intraclass correlation coefficients (ICC) assessed interrater and intermodality reliability. Univariable mixed-effects linear regression was used to evaluate longitudinal changes of MAD. RESULTS: MAD was detected in 60% (110/185) eligible patients. MVP was present in 48% (53/110) of MAD and MAD in 90% (53/59) of MVP. MAD detection by CMR and echocardiography had 96% overall agreement (Kappa = 0.89, p < 0.001) and a 0.32-mm estimate bias (95%CI 0.00, 0.65). ICC by echocardiography, CMR, and between modalities were 0.97 (95%CI 0.93, 0.98), 0.92 (95%CI 0.79, 0.97), and 0.91 (95%CI 0.85, 0.94), respectively. MAD was associated with aortic root dilation (p < 0.001). MAD was found in children of all ages, increased +0.18 mm/year (95%CI +0.14, + 0.22) during a median duration of 5.5 years (IQR 3.1, 7.5 years). MAD indexed by height yielded a constant value +0.0002 mm/m/year (95%CI -0.0002, + 0.0005 mm/m/year). CONCLUSIONS: MAD was common in pediatric MFS and was associated with aortic root dilation. MAD detection by echocardiography and CMR was highly reliable, suggesting that routine assessment in MFS is feasible. MAD was present in neonates and progressed over time but remained constant when indexing by height. Further studies are needed to evaluate MAD as a biomarker for clinical outcomes in pediatric MFS.

Percutaneous atrial septal defect closure in infants and toddlers: predictors of success.

Device closure of atrial septal defect (ASD) is commonly performed in older children and adults. Infants and toddlers (age <4 years) are seldom referred for ASD closure due to size constraints. However, in many cases device ASD closure can be performed in this population. Between 2002 and 2012, 61 infants and toddlers were taken to the catheterization laboratory at our institution for ASD closure. Precatheterization transthoracic echocardiograms, intracatheterization transesophageal echocardiograms, and catheterization reports were reviewed. Fifty-three infants and toddlers presented for percutaneous ASD occlusion. Forty-eight (79 %) underwent successful closure, and 13 were referred for surgery without device attempt (n = 8) or after unsuccessful device occlusion (n = 4). Median age and weight at time of ASD closure were 2.99 years (range 0.3-3.8) and 11.7 kg (range 3.7-16.5). The device-to-septal length ratio was 0.81 (range 0.44-1.03). The 12 unsuccessful cases occurred in patients with larger defects (ASD diameter 17.5 ± 6.1 vs. 12.1 ± 4.2, p < 0.01). Deficient rims (absent or ≤ 4 mm) were seen in 9 of 12 (75 %) unsuccessful cases and in 19 of 41 (46 %) successful cases (p = 0.12). Multivariate analysis showed that patient size and ASD size were not independently associated with procedural success but that ASD size-to-patient weight ratio <1.2 (hazard ratio 9.5 [range1.7-17]) was associated with successful ASD closure. ASD device occlusion can be safely achieved in small children. An ASD size-to-patient weight ratio >1.2, not absolute patient weight or age, is associated with failure of the percutaneous approach. The midterm outcomes in these young patients are excellent.

Imaging and guiding intervention for tricuspid valve disorders using 3-dimensional transesophageal echocardiography in pediatric and congenital heart disease.

In the pediatric and congenital heart disease (CHD) population, tricuspid valve (TV) disorders are complex due to the variable TV morphology, its sophisticated interaction with the right ventricle as well as associated congenital and acquired lesions. While surgery is the standard of care for TV dysfunction in this patient population, transcatheter treatment for bioprosthetic TV dysfunction has been performed successfully. Detailed and accurate anatomic assessment of the abnormal TV is essential in the preoperative/preprocedural planning. Three-dimensional transthoracic and 3D transesophageal echocardiography (3DTEE) provides added value to 2-dimensional imaging in the characterization of the TV to guide therapy and 3DTEE serves as an excellent tool for intraoperative assessment and procedural guidance of transcatheter treatment. Notwithstanding advances in imaging and therapy, the timing and indication for intervention for TV disorders in this population are not well defined. In this manuscript, we aim to review the available literature, provide our institutional experience with 3DTEE, and briefly discuss the perceived challenges and future directions in the assessment, surgical planning, and procedural guidance of (1) congenital TV malformations, (2) acquired TV dysfunction from transvenous pacing leads, or following cardiac surgeries, and (3) bioprosthetic TV dysfunction.

Single tertiary center experience using Gore Cardioform Atrial Septal Defect Occluder for secundum atrial septal defect closure with a focus on deficient rims.

BACKGROUND: The data on the use of Gore Cardioform Septal Occluder (GCA; W. L. Gore and Associates, Inc.) for atrial septal defect (ASD) with deficient rims is limited. METHODS: All patients evaluated by transesophageal echocardiogram (TEE) for ASD occlusion were included. TEE planes at 35°, 0°, and 90° were assessed for anterior-superior (AS) and posterior (P), anterior-inferior (AI) and posterior-superior (PS), as well as superior (S) and inferior (I) rims. ASD size >20 mm, and rims less than 5 mm were defined as large and deficient, respectively. We included patients who had a procedural failure along with the patients in whom the procedure was not attempted after echocardiogram in the unsuccessful group. RESULTS: In 148 patients, the median weight, age, and ASD size were 36 kg (range, 8-60 kg), 11.8 years (range, 1-60 years), and 14.2 ± 8.28 mm, respectively. One or more deficient rims were noted in 112 of 148 (75.7%): 99 (67%) AS, 36 (24%) P, 17 (11%) AI, 30 (20%) PS, 26 (18%) S, and 33 (22%) I. ASD closure was performed in 115 (78%) patients. The procedure was successful in 111 (96.5%) patients with procedural failure in 4 (3.4%) patients. Multiple deficient rims were associated with reduced procedural success (OR 0.36, 95% CI, 0.25-0.56). On multivariate analysis deficient P, PS, and I rims were associated with an unsuccessful group (P = .001, .046, and .005, respectively). Complications included 1 device embolization, 1 vascular injury, and 5 arrhythmias. CONCLUSIONS: Transcatheter closure of ASDs with deficient rims is feasible using GCA. Large ASDs with deficient P, PS, and I rims were associated with unsuccessful closure. Risk stratification and comprehensive evaluation of ASD rims is vital for the use of GCA.

Excessive Trabeculation of the Left Ventricle: JACC: Cardiovascular Imaging Expert Panel Paper.

Excessive trabeculation, often referred to as "noncompacted" myocardium, has been described at all ages, from the fetus to the adult. Current evidence for myocardial development, however, does not support the formation of compact myocardium from noncompacted myocardium, nor the arrest of this process to result in so-called noncompaction. Excessive trabeculation is frequently observed by imaging studies in healthy individuals, as well as in association with pregnancy, athletic activity, and with cardiac diseases of inherited, acquired, developmental, or congenital origins. Adults with incidentally noted excessive trabeculation frequently require no further follow-up based on trabecular pattern alone. Patients with cardiomyopathy and excessive trabeculation are managed by cardiovascular symptoms rather than the trabecular pattern. To date, the prognostic role of excessive trabeculation in adults has not been shown to be independent of other myocardial disease. In neonates and children with excessive trabeculation and normal or abnormal function, clinical caution seems warranted because of the reported association with genetic and neuromuscular disorders. This report summarizes the evidence concerning the etiology, pathophysiology, and clinical relevance of excessive trabeculation. Gaps in current knowledge of the clinical relevance of excessive trabeculation are indicated, with priorities suggested for future research and improved diagnosis in adults and children.

Correlating decline in left ventricular ejection fraction and longitudinal strain in pediatric cancer patients.

PURPOSE: Left ventricular ejection fraction (LVEF) is routinely used to monitor cardiac function in cancer patients. Global longitudinal strain (GLS) detects subclinical myocardial dysfunction. There is no consensus on what constitutes a significant change in GLS in pediatric cancer patients. We aim to determine the change in GLS associated with a simultaneous decline in LVEF in pediatric cancer patients. METHODS: This is a retrospective longitudinal study of pediatric cancer patients treated with anthracyclines between October 2017 and November 2019. GLS was measured by 2-dimensional speckle tracking. The study outcome was a decline in LVEF, defined as a decrease in LVEF of ≥ 10% points from baseline or LVEF < 55%. We evaluated two echocardiograms per patient, one baseline, and one follow-up. The follow-up echocardiogram was either (1) the first study that met the outcome or (2) the last echocardiogram available in patients without the outcome. Statistical analyses included receiver operator characteristic curves and univariable and multivariable Cox proportional hazards regression. RESULTS: Out of 161 patients, 33 (20.5%) had a decline in LVEF within one year of follow-up. GLS reduction by ≥ 15% from baseline and follow-up GLS >-18% had sensitivities of 85% and 78%, respectively, and specificities of 86% and 83%, respectively, to detect LVEF decline. GLS reduction by ≥ 15% from baseline and follow-up GLS >-18% were independently associated with simultaneous LVEF decline [hazard ratio (95% confidence intervals): 16.71 (5.47-51.06), and 12.83 (4.62-35.63), respectively]. CONCLUSION: Monitoring GLS validates the decline in LVEF in pediatric cancer patients.

Cardiovascular findings in duplication 17p11.2 syndrome.

PURPOSE: Cardiovascular abnormalities are newly recognized features of duplication 17p11.2 syndrome. In a single-center study, we evaluated subjects with duplication 17p11.2 syndrome for cardiovascular abnormalities. METHODS: Twenty-five subjects with 17p11.2 duplication identified by chromosome analysis and/or array-based comparative genomic hybridization were enrolled in a multidisciplinary protocol. In our clinical evaluation of these subjects, we performed physical examinations, echocardiography, and electrocardiography. Three of these subjects were followed up longitudinally at our institution. RESULTS: Cardiovascular anomalies, including structural and conduction abnormalities, were identified in 10 of 25 (40%) of subjects with duplication 17p11.2 syndrome. The most frequent abnormality was dilated aortic root (20% of total cohort). Bicommissural aortic valve (2/25), atrial (3/25) and ventricular (2/25) septal defects, and patent foramen ovale (4/25) were also observed. CONCLUSION: Duplication 17p11.2 syndrome is associated with structural heart disease, aortopathy, and electrocardiographic abnormalities. Individuals with duplication 17p11.2 syndrome should be evaluated by electrocardiography and echocardiography at the time of diagnosis and monitored for cardiovascular disease over time. Further clinical investigation including longitudinal analysis would likely determine the age of onset and characterize the progression (if any) of vasculopathy in subjects with duplication 17p11.2 syndrome, so that specific guidelines can be established for cardiovascular management.

Diminished left ventricular function is associated with poor mid-term outcomes in neonates after balloon aortic valvuloplasty.

OBJECTIVES: We studied outcomes of neonatal aortic valvuloplasty to determine the risk factors for poor outcomes. BACKGROUND: Balloon aortic valvuloplasty (BAV) is the primary therapy for neonates with severe aortic stenosis (AS). Limited data are available on the mid-term and long-term outcomes in this population, and reported risk factors for poor outcomes vary among studies. METHODS: We reviewed all cases of BAV in neonates in our institution between 1998 and 2009. We reviewed patient characteristics, preintervention echocardiographic, and procedural details. We tracked repeat BAV, aortic valve replacement (AVR), and death/transplant. Kaplan-Meier and Cox regression analyses were performed. Changes in dimensions of left heart structures post-BAV were also studied. RESULTS: Forty-eight neonates were included-of these, 30 (62%) had critical AS. There was one procedural death. The remaining 47 neonates were followed for 4.8 ± 4.4 years. Repeat BAV was performed in 19 (40%) neonates; AVR was performed in 9 (19%), and death/transplant occurred in 4 (8%) neonates. Multivariate analysis revealed that lower LV shortening fraction pre-BAV, higher pre-BAV and final valve gradient, and need for inotropes were associated with poor long-term outcomes. LV diastolic and systolic dimensions normalized over time, and left heart structures improved in size early and late after BAV. CONCLUSIONS: Neonatal BAV is associated with low mortality. Lower LV shortening fraction pre-BAV is associated with risk of future interventions, while repeat valvuloplasty is safe and effective for recurrent AS. There is significant improvement in dimensions of the LV and aortic valve annulus following BAV.

Relation of Right Atrial Strain to Mortality in Infants With Single Right Ventricles.

We explored associations of surveillance testing in infants with single right ventricle (sRV) physiology with clinical outcomes. This prospective, single-center study included patients with sRV who had initial palliative surgery (September 2019 to December 2020). Echocardiograms and B-type naturetic peptide (BNP) obtained as a pair within 24 hours as part of clinical care were included. The primary outcome was death/heart transplant. Secondary outcomes included interstage duration of milrinone use, hospital length of stay, and no digoxin use. sRV functional assessment (subjective grade, fractional area change, tricuspid annular plane systolic excursion, global longitudinal strain, right atrial strain [RAS]) was performed offline. Associations between echocardiography, BNP, and clinical outcomes were determined. Of 26 subjects (47 encounters), 20 had hypoplastic left heart syndrome (77%). Median age at data collection was 50 days (interquartile range 26 to 90). In most encounters (73%), sRV function was subjectively normal. Median BNP was 332 pg/ml (interquartile range 160 to 1,085). A total of 5 patients (19%) met the primary outcome and had lower RAS (14.1 vs 21.3, p = 0.038), but all other parameters were similar to transplant-free survivors. RAS (16.1%, 0.83) had the highest area under curve, followed by global longitudinal strain (-14.4%, 0.77). Higher RAS was associated with fewer days on milrinone (coefficient -1.37, 95% confidence interval [CI] -2.54 to -0.20, p = 0.02) and higher odds of digoxin use (odds ratio 1.09, 95% CI 1.01 to 1.18, p = 0.047). Higher BNP was only associated with a lower odds of digoxin use (odds ratio 0.69, 95% CI 0.5 to 0.96, p = 0.03). In conclusion, RAS is a potentially important imaging marker in infants with sRV and merits further investigation in larger studies.

Global Longitudinal Strain Analysis of the Single Right Ventricle: Leveling the Playing Field.

BACKGROUND: All available echocardiographic methods to assess single systemic right ventricular systolic function have limitations. Subjective grading is prone to bias and varies among readers. Quantitative methods that require significant manual input, such as fractional area change (FAC), are often not reproducible. The aim of this study was to determine whether global longitudinal strain (GLS) is more reproducible than FAC and subjective grading in patients with systemic right ventricle among individual readers and across different levels of experience. METHODS: Clinically indicated echocardiograms from 40 patients with functional systemic right ventricles were assessed by five readers with varying reading experience: one sonographer, one cardiology fellow, and three attending cardiologists at different career stages. All readers were blinded to patient data and other reader responses. Each reader reviewed the same images for subjective grade (on a scale ranging from 1 [normal] to 8 [severely depressed]), right ventricular end-diastolic and end-systolic area measurements, and longitudinal strain analysis. A repeat analysis was performed under identical conditions after ≥2 weeks on all 40 patients. Inter- and intrareader reproducibility was assessed using intraclass correlation coefficients (ICCs). Correlations between responses were assessed using Spearman's correlation coefficient. RESULTS: The subjective method had fair to good reproducibility (ICC = 0.7; interquartile range [IQR], 0.60-0.72), while the FAC method was poor (ICC = 0.46; IQR, 0.39-0.51) among readers. Reproducibility for GLS was excellent (ICC = 0.88; IQR, 0.88-0.89). Intrareader reproducibility was excellent by subjective grading (ICC = 0.85; IQR, 0.73-0.88), poor by FAC (ICC = 0.63; IQR, 0.35-0.66), and excellent by GLS (ICC = 0.93; IQR, 0.88-0.96). Attending-level readers were more consistent with their subjective grading, while all readers were excellent with GLS. CONCLUSIONS: GLS is more reproducible than conventional methods at assessing systemic right ventricular systolic function among readers with different levels of experience. For most readers it was more consistent than their own subjective grades of right ventricular function. Laboratories staffed by multiple readers are likely to be more consistent in grading systemic right ventricular systolic function using GLS.

Noncompaction of the left ventricular myocardium in a boy with a novel chromosome 8p23.1 deletion.

Interstitial deletion of chromosome 8p23.1 has been reported in patients with congenital heart defects, including atrial and ventricular septal defects, pulmonary stenosis, and complex cyanotic heart defects. GATA4, a zinc-finger transcription factor gene, has been localized to this region. GATA4 interacts with additional transcription factors in the embryogenesis of the primitive heart tube. Mutations in GATA4 are thought to be responsible for the congenital heart defects reported in association with this chromosomal deletion, and several familial point mutations leading to amino acid substitutions have also been identified. Left ventricular noncompaction (LVNC) is a clinically heterogeneous disorder characterized by LV myocardial trabeculations and intertrabecular recesses that communicate with the LV cavity. Patients may be asymptomatic or may present with evidence of severely depressed LV systolic and diastolic function. The LV may be dilated or hypertrophied, and clinical expression may be undulating. Several genetic causes of LVNC have been reported, with variable modes of inheritance, including autosomal dominant and X-linked inheritance, but relatively few responsible genes have been identified. A 12-year-old boy with a history of acute lymphoblastic leukemia, dysmorphic features, and LVNC with preserved LV systolic function was referred to the Cardiovascular Genetics Clinic at our institution. The patient was asymptomatic in terms of cardiovascular function. Chromosome microarray testing revealed an interstitial deletion in the region of 8p23.1 containing GATA4. LVNC has not been reported previously in association with this chromosome deletion. Further investigation into the role of GATA4 in patients with LVNC is warranted.

Percutaneous atrial septal defect closure in a child with interrupted inferior vena cava: successful femoral venous approach.

Interrupted inferior vena cava (IVC) with azygous continuation to the superior vena cava (SVC) is a relatively common systemic venous anomaly. This anomaly can occasionally complicate transcatheter intervention by rendering more difficult the usual direct route to the systemic venous atrium afforded by femoral venous access. We report our experience with successful transcatheter closure of a large residual atrial septal defect (ASD) using the femoral venous route in a 3-year-old patient with heterotaxy syndrome of left isomerism type, dextrocardia, partial atrioventricular canal defect, and interrupted IVC with azygous continuation to the SVC.

Assessing the left atrium of childhood cancer survivors.

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/m2, 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/m2 anthracycline also had lower Ɛres than those < 250 mg/m2. 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.