Periscopic technique in Norwood operation is associated with better preservation of early ventricular function.

OBJECTIVE: Although the right ventricle (RV) to pulmonary artery conduit in stage 1 Norwood operation results in improved interstage survival, the long-term effects of the ventriculotomy used in the traditional technique remain a concern. The periscopic technique (PT) of RV to pulmonary artery conduit placement has been described as an alternative technique to minimize RV injury. A retrospective study was performed to compare the effects of traditional technique and PT on ventricular function following Norwood operation. METHODS: A retrospective study of all patients who underwent Norwood operation from 2012 to 2019 was performed. Patients with baseline RV dysfunction and significant tricuspid valve regurgitation were excluded. Prestage 2 echocardiograms were reviewed by a blinded experienced imager for quantification of RV function (sinus and infundibular RV fractional area change) as well as for regional conduit site wall dysfunction (normal or abnormal, including hypokinesia, akinesia, or dyskinesia). Wilcoxon rank-sum tests were used to assess differences in RV infundibular and RV sinus ejection fraction and the Fisher exact test was used to assess differences in regional wall dysfunction. RESULTS: Twenty-two patients met inclusion criteria. Eight underwent traditional technique and 14 underwent PT. Median infundibular RV fractional area change was 49% and 37% (P = .02) and sinus RV fractional area change was 50% and 41% for PT and traditional technique (P = .007) respectively. Similarly qualitative regional RV wall function was better preserved in PT (P = .002). CONCLUSIONS: The PT for RV to pulmonary artery conduit in Norwood operation results in better preservation of early RV global and regional systolic function. Whether or not this benefit translates to improved clinical outcome still needs to be studied.

Diagnosis of anomalous origin of the right subclavian artery from the right pulmonary artery in a patient with D-transposition of the great arteries utilizing transthoracic echocardiography.

Diagnosis of anomalous origin of the right subclavian artery (AORSA) from the right pulmonary artery (RPA) is usually made using CT, MRI, or invasive angiography. We report a patient diagnosed using transthoracic echocardiography (TTE). A newborn girl prenatally known to have d-TGA presented with cyanosis sparing the right hemithorax and arm. Oxygen saturations on the right hand were persistently higher than on the right ear and other extremities. Repeat TTE using a modified echocardiographic imaging plane allowed for full visualization of the entire subclavian artery course, revealing AORSA from RPA. We discuss further the approach to echocardiographic diagnosis and surgical implications.

Inferior Right Ventricular Wall Thickness by Echocardiogram: A Novel Method of Assessing Hypertrophy in Neonates and Infants.

An established echocardiographic (echo) standard for assessing the newborn right ventricle (RV) for hypertrophy has not been thoroughly developed. This is partially due to the RV's complex architecture, which makes quantification of RV mass by echo difficult. Here, we retrospectively evaluate the thickness of the inferior RV wall (iRVWT) by echo in neonates and infants with normal cardiopulmonary physiology. Inferior RVWT was defined at the medial portion of the inferior wall of the RV at the mid-ventricular level, collected from a subxiphoid, short axis view. iRVWT was indexed to body surface area (BSA) to the 0.5 power and normalized to iLVWT to explore the best normalization method. Ninety-eight neonates and 32 infants were included in the final analysis. Mean age for neonates and infants was 2 days and 59 days, respectively. Mean ± SD for neonate and infant end-diastole iRVWT was 2.17 ± 0.35 mm and 1.79 ± 0.28 mm, respectively. There was no residual relationship between the index iRVWT and BSA (r = 0.03, p = NS). In the infant cohort, the iRVWT was significantly lower and iLVWT was significantly higher compared to neonate, consistent with known physiologic changes of RV and LV mass. Thus, iRVWT may serve as a reliable and accurate proxy for RV mass and the parameter warrants further evaluation.

Left Ventricular Mass Quantification by Two-Dimensional Echocardiography in a Pediatric Population: Correlation with Cardiac Magnetic Resonance Imaging.

Quantification of left ventricular (LV) mass by echocardiography has not been validated against the gold standard of cardiac magnetic resonance imaging (CMR) in the pediatric population. The purpose of this study was to compare LV mass by two-dimensional and conventional M-mode echocardiography versus CMR in children. Consecutive CMR studies were paired with echocardiograms and retrospectively analyzed in children age ≤ 16 years (3 days old to 16 years old). Studies performed > 3 months between modalities and single ventricle anatomy were excluded. Unindexed LV mass was calculated using M-mode, area-length (AL), and truncated ellipsoid (TE) methods via echocardiography, and compared to cine stack CMR images. There were 46 patients included in the study (both MRI and echocardiography). Good correlations were observed for LV mass measured by CMR and all echocardiographic methods: M-mode (R = 0.965), AL (R = 0.975), and TE (R = 0.975). There was a significant overestimation using TE echocardiography, by a mean of 10.5 g (95% confidence interval 5.7-15.2 g, p < 0.05). There was no significant over- or underestimation of LV mass observed by M-mode or AL echocardiographic measurements, with tight limits of agreement when compared to CMR (95% confidence interval - 5.2 to 4.4 g and - 1.5 to 6.7 g, respectively). Interobserver agreement was good for each of the echocardiographic measurements, but inferior with M-mode (ICC, 0.89) compared to two-dimensional methods (ICC, 0.97). Echocardiographic estimates of LV mass have good correlation with CMR in children. Performance comparison showed AL echocardiographic method provides the most accurate measurement of LV mass with the best reproducibility compared to other methods.

3D Printing to Guide Ventricular Assist Device Placement in Adults With Congenital Heart Disease and Heart Failure.

As the population of adults with congenital heart disease continues to grow, so does the number of these patients with heart failure. Ventricular assist devices are underutilized in adults with congenital heart disease due to their complex anatomic arrangements and physiology. Advanced imaging techniques that may increase the utilization of mechanical circulatory support in this population must be explored. Three-dimensional printing offers individualized structural models that would enable pre-surgical planning of cannula and device placement in adults with congenital cardiac disease and heart failure who are candidates for such therapies. We present a review of relevant cardiac anomalies, cases in which such models could be utilized, and some background on the cost and procedure associated with this process.

Blood Pool Segmentation Results in Superior Virtual Cardiac Models than Myocardial Segmentation for 3D Printing.

The method of cardiac magnetic resonance (CMR) three-dimensional (3D) image acquisition and post-processing which should be used to create optimal virtual models for 3D printing has not been studied systematically. Patients (n = 19) who had undergone CMR including both 3D balanced steady-state free precession (bSSFP) imaging and contrast-enhanced magnetic resonance angiography (MRA) were retrospectively identified. Post-processing for the creation of virtual 3D models involved using both myocardial (MS) and blood pool (BP) segmentation, resulting in four groups: Group 1-bSSFP/MS, Group 2-bSSFP/BP, Group 3-MRA/MS and Group 4-MRA/BP. The models created were assessed by two raters for overall quality (1-poor; 2-good; 3-excellent) and ability to identify predefined vessels (1-5: superior vena cava, inferior vena cava, main pulmonary artery, ascending aorta and at least one pulmonary vein). A total of 76 virtual models were created from 19 patient CMR datasets. The mean overall quality scores for Raters 1/2 were 1.63 ± 0.50/1.26 ± 0.45 for Group 1, 2.12 ± 0.50/2.26 ± 0.73 for Group 2, 1.74 ± 0.56/1.53 ± 0.61 for Group 3 and 2.26 ± 0.65/2.68 ± 0.48 for Group 4. The numbers of identified vessels for Raters 1/2 were 4.11 ± 1.32/4.05 ± 1.31 for Group 1, 4.90 ± 0.46/4.95 ± 0.23 for Group 2, 4.32 ± 1.00/4.47 ± 0.84 for Group 3 and 4.74 ± 0.56/4.63 ± 0.49 for Group 4. Models created using BP segmentation (Groups 2 and 4) received significantly higher ratings than those created using MS for both overall quality and number of vessels visualized (p < 0.05), regardless of the acquisition technique. There were no significant differences between Groups 1 and 3. The ratings for Raters 1 and 2 had good correlation for overall quality (ICC = 0.63) and excellent correlation for the total number of vessels visualized (ICC = 0.77). The intra-rater reliability was good for Rater A (ICC = 0.65). Three models were successfully printed on desktop 3D printers with good quality and accurate representation of the virtual 3D models. We recommend using BP segmentation with either MRA or bSSFP source datasets to create virtual 3D models for 3D printing. Desktop 3D printers can offer good quality printed models with accurate representation of anatomic detail.

Assessment of Left Ventricular Mass and Hypertrophy by Cardiovascular Magnetic Resonance Imaging in Pediatric Hypertension.

Cardiovascular magnetic resonance (CMR) imaging in adults is considered the gold standard for assessment of left ventricular mass (LVM) and left ventricular hypertrophy (LVH). The authors aimed to evaluate agreement of LVM measurements and LVH determination between echocardiography (ECHO) and CMR imaging in children with hypertension (HTN) confirmed by 24-hour ambulatory blood pressure monitoring (ABPM). The children (n=22) underwent contemporaneous ECHO, CMR imaging, and ABPM. Patients had a mean body mass index of 30.9±7.5 (kg/m2 ), and 81.8% had severe HTN. LVM measured by ECHO was 189.6±62.1 g and by CMR imaging was 164.6±44.7 g (P<.0001). Bland-Altman analysis revealed significant variability between ECHO and CMR imaging in the measurement of LVM. Interobserver error was higher with ECHO than with CMR imaging. ECHO had high sensitivity and low specificity in LVH determination. In conclusion, ECHO overestimates LVM and is less accurate in measuring LVM as compared with CMR imaging in children with HTN. Further prospective study using CMR imaging to assess LVM in children is warranted.

Feature Tracking-Derived Peak Systolic Strain Compared to Late Gadolinium Enhancement in Troponin-Positive Myocarditis: A Case-Control Study.

Cardiac magnetic resonance (CMR) assesses myocardial involvement in myocarditis (MYO). Current techniques are qualitative, subjective, and prone to interpretation error. Feature tracking (FT) analyzes myocardial strain using CMR and has not been examined in MYO. We hypothesize that regional left ventricular (LV) strain is abnormal in MYO. Regional strain by FT was compared to late gadolinium enhancement (LGE) and troponin leak as measures of myocardial involvement. This single-center, retrospective CMR study reviewed patients with clinical MYO and structurally normal hearts who underwent CMR at our institution. Young adults with normal cardiac anatomy, function, and absent LGE served as controls. MYO patients with documented troponin leak and normal global ejection fraction (EF > 50 %) were included in comparison. FT determined regional myocardial peak systolic strain (pkS) in longitudinal and circumferential distributions. T tests compared strain values between cases and controls. Receiver operating characteristic curves determined pkS values with highest sensitivity and specificity for concurrent troponin leak and LGE. FT was performed on 57 patients: 37 MYO and 20 controls. Twenty-eight cases with normal EF, and 20 control patients were included in final analysis. Nearly all cases with normal function demonstrated abnormal regional pkS (27/28, 96 %). Cases had significantly diminished pkS when compared to controls in all regions except the longitudinal 2C distribution. FT-derived longitudinal and circumferential pkS is sensitive and specific in identifying myocardial involvement, namely the presence of troponin leak and LGE. FT may be a useful adjunctive, objective measure of myocardial involvement in patients with MYO and normal LV function.

Application of Virtual Three-Dimensional Models for Simultaneous Visualization of Intracardiac Anatomic Relationships in Double Outlet Right Ventricle.

Our goal was to construct three-dimensional (3D) virtual models to allow simultaneous visualization of the ventricles, ventricular septal defect (VSD) and great arteries in patients with complex intracardiac anatomy to aid in surgical planning. We also sought to correlate measurements from the source cardiac magnetic resonance (CMR) image dataset and the 3D model. Complicated ventriculo-arterial relationships in patients with complex conotruncal malformations make preoperative assessment of possible repair pathways difficult. Patients were chosen with double outlet right ventricle for the complexity of intracardiac anatomy and potential for better delineation of anatomic spatial relationships. Virtual 3D models were generated from CMR 3D datasets. Measurements were made on the source CMR as well as the 3D model for the following structures: aortic diameter in orthogonal planes, VSD diameter in orthogonal planes and long axis of right ventricle. A total of six patients were identified for inclusion. The path from the ventricles to each respective outflow tract and the location of the VSD with respect to each great vessel was visualized clearly in all patients. Measurements on the virtual model showed excellent correlation with the source CMR when all measurements were included by Pearson coefficient, r = 0.99 as well as for each individual structure. Construction of virtual 3D models in patients with complex conotruncal defects from 3D CMR datasets allows for simultaneous visualization of anatomic relationships relevant for surgical repair. The availability of these models may allow for a more informed preoperative evaluation in these patients.

Use of a 1.0 Tesla open scanner for evaluation of pediatric and congenital heart disease: a retrospective cohort study.

BACKGROUND: Open cardiovascular magnetic resonance (CMR) scanners offer the potential for imaging patients with claustrophobia or large body size, but at a lower 1.0 Tesla magnetic field. This study aimed to evaluate the efficacy of open CMR for evaluation of pediatric and congenital heart disease. METHODS: This retrospective, cross-sectional study included all patients ≤18 years old or with congenital heart disease who underwent CMR on an open 1.0 Tesla scanner at two centers from 2012-2014. Indications for CMR and clinical questions were extracted from the medical record. Studies were qualitatively graded for image quality and diagnostic utility. In a subset of 25 patients, signal-to-noise (SNR) and contrast-to-noise (CNR) ratios were compared to size- and diagnosis-matched patients with CMR on a 1.5 Tesla scanner. RESULTS: A total of 65 patients (median 17.3 years old, 60% male) were included. Congenital heart disease was present in 32 (50%), with tetralogy of Fallot and bicuspid aortic valve the most common diagnoses. Open CMR was used due to scheduling/equipment issues in 51 (80%), claustrophobia in 7 (11%), and patient size in 3 (5%); 4 patients with claustrophobia had failed CMR on a different scanner, but completed the study on open CMR without sedation. All patients had good or excellent image quality on black blood, phase contrast, magnetic resonance angiography, and late gadolinium enhancement imaging. There was below average image quality in 3/63 (5%) patients with cine images, and 4/15 (27%) patients with coronary artery imaging. SNR and CNR were decreased in cine and magnetic resonance angiography images compared to 1.5 Tesla. The clinical question was answered adequately in all but 2 patients; 1 patient with a Fontan had artifact from an embolization coil limiting RV volume analysis, and in 1 patient the right coronary artery origin was not well seen. CONCLUSIONS: Open 1.0 Tesla scanners can effectively evaluate pediatric and congenital heart disease, including patients with claustrophobia and larger body size. Despite minor artifacts and differences in SNR and CNR, the majority of clinical questions can be answered adequately, with some limitations with coronary artery imaging. Further evaluation is necessary to optimize protocols and image quality.

Two-dimensional speckle-tracking-derived segmental peak systolic longitudinal strain identifies regional myocardial involvement in patients with myocarditis and normal global left ventricular systolic function.

The presence of myocardial late gadolinium enhancement (LGE) by cardiac magnetic resonance (CMR) imaging in concert with electrocardiography and elevated biomarkers helps support the diagnosis of acute myocarditis. Two-dimensional echocardiography is limited to global and qualitative regional function assessment and may not contribute to the diagnosis, especially in the presence of normal LV systolic function. Two-dimensional speckle-tracking (2D-STE)-derived segmental peak systolic (pkS) longitudinal strain (LS) may identify segmental myocardial involvement in myocarditis. We sought to identify an association between segmental pkS, LGE, and troponin levels in patients with myocarditis. Retrospective analysis of myocardial segmental function by 2D-STE segmental strain was compared to the presence of LGE and admission peak troponin levels in patients with acute myocarditis and preserved global LV systolic function. American Heart Association 17-segment model was used for comparison between imaging modalities. Global function was assessed by m-mode-derived shortening fraction (SF). Descriptive statistics and regression analysis were utilized. Forty-four CMRs performed to evaluate for myocarditis were identified. Of the 44, 10 patients, median age 17.5 years (14-18.5 years) and median SF 35 % (28-44 %), had paired CMR and 2D-STE data for analysis, and 161/170 segments could be analyzed by both methods for comparison. PkS LS was decreased in 51 % of segments that were positive for LGE with average pkS of -14.7 %. Segmental pkS LS abnormalities were present in all but one patient who had abnormal pkS circumferential strain. Global pkS LS was decreased in patients with myocarditis. There is a moderate correlation between decreased pkS LS and the presence of LGE by CMR, 2D-STE for myocardial involvement in acute myocarditis can serve as an useful noninvasive adjunct to the existing tests used for the diagnosis of acute myocarditis and might have a role in prognostication.

Frequency of aortic dilation in Noonan syndrome.

Noonan syndrome (NS) is a genetic disorder caused by mutations altering proteins relevant to RAS/mitogen-activated protein kinase signal transduction. Cardiac involvement is common, most prevalently pulmonary valve stenosis and hypertrophic cardiomyopathy. Because abnormal mitogen-activated protein kinase signaling contributes to the aortopathy in Marfan syndrome and with rare reports of aortic aneurysm in NS, we undertook a retrospective study of ascending aortic anatomy in 37 patients with NS and without confounding medical conditions. Age ranged from 0.6 to 32 years. Based on the most recent echocardiogram, the aortic annulus and root were dilated in the cohort (mean z scores of 1.14 and 0.98, respectively, p <0.005) but the sinotubular junction and ascending aorta were not (mean z scores of 0.05 and 0.19, respectively). The aortic root was aneurysmal (>2 z scores) in 8 subjects (21.6%). PTPN11 mutations were present in 14 subjects, whose aortic status was similar to the cohort overall. Comparison of age and z scores revealed a modest tendency for the aortic annulus and root to dilate over time. Among 13 subjects with multiple imaging studies over an average of 6.8 years, the average z score increased to 0.78 and 0.39 for the aortic annulus and root, respectively. Multivariate analysis revealed that age accounted for 7.0% and 11.0% of the variance in the aortic annular and root diameters, respectively. In conclusion, we found that aortic annular dilation and aortic root aneurysm are prevalent in NS, often presenting during childhood and progressing over time. Further study is needed to identify potential risks associated with these abnormalities.

Subpulmonary stenosis assessed in midtrimester fetuses with tetralogy of Fallot: a novel method for predicting postnatal clinical outcome.

This study aimed to determine whether quantification of subpulmonary stenosis (SPS) in tetralogy of Fallot (TOF) in the second-trimester fetus can predict postnatal clinical outcome measured by pulmonary valve size and/or timing or type of intervention. The study retrospectively identified fetuses with TOF from 1998 to 2010 diagnosed at 26 weeks gestation or earlier. The data evaluated included pre- and postnatal pulmonary valve z-scores (PVZ). To quantify fetal SPS, the authors created a novel index, the SPS/DAO ratio, a ratio of the minimum infundibular diameter to the descending aorta diameter (DAO). Multiple linear regression was used to predict postnatal PVZ from prenatally determined parameters, including SPS/DAO. Fetal parameters were analyzed by logistic regression for association with postnatal outcomes, namely, timing of surgery (<1 month), used as a surrogate for severity, and type of surgery [transannular patch (TAP) vs valve sparing surgery]. A total of 23 fetuses met the inclusion criteria. The mean gestational age was 21.8 ± 1.9 weeks (range, 16.6-25.4 weeks). There was excellent correlation between predicted and measured PVZ (r = 0.82; p < 0.0001) using the following derived equation: -3.68 + (0.91 × prenatal PVZ) - (4.44 × SPS/DAO) - 3.19 (prenatal PVZ × SPS/DAO). An SPS/DAO value lower than 0.5 had 100 % sensitivity and 56 % specificity for repair before the age of 1 month, and a value lower than 0.47 had 100 % sensitivity and 75 % specificity for TAP repair. Prenatal PVZ and the SPS/DAO ratio at 26 weeks gestation or earlier can reliably predict postnatal PVZ in fetuses with TOF. Quantification of SPS with the SPS/DAO ratio identifies patients who may require early intervention secondary to disease severity and may predict the type of repair, thereby influencing prenatal counseling.

Echocardiographic myocardial deformation evaluation of right ventricular function in comparison with CMRI in repaired tetralogy of Fallot: a cross-sectional and longitudinal validation study.

BACKGROUND: Decreased right ventricular (RV) ejection fraction (EF) increases morbidity in repaired tetralogy of Fallot (rToF). Cardiac magnetic resonance (CMR) is the accepted reference standard for RV EF measurement. There are no established color Doppler tissue imaging (cDTI) values that identify decreased RV EF in rToF. We sought to assess accuracy of cDTI myocardial deformation and velocity indices in predicting CMR-derived RV EF. METHODS: Retrospective evaluation of CMR and echocardiography studies performed on the same day. cDTI-derived peak systolic velocity (pkS), isovolumic acceleration (IVA) of the tricuspid valve annulus, strain (ε) and strain rate (SR) of the RV free wall, adjusted for age, volume and pressure overload were used to compute RV EF. The probability of correctly classifying CMR measured RV EF <45% was estimated by logistic regression analysis. The accuracy of the model was tested on 10 prospective patients. RESULTS: Sixty matched echocardiographic and CMR studies were analyzed. None of the individual cDTI parameters could reliably identify patients with CMR-derived decreased RV EF. However, when adjusted for age in a logistic model: Y = -0.5 - 0.07 × age + 0.016 × Îµ + 0.46 × pkS, where Y is the logit (log odds), patients with RV EF <45% were identified with 86% sensitivity, 93.5% specificity. Prospective application of this model correctly identified all subjects with RV EF <45%. CONCLUSION: The age-adjusted logistic model and not individual parameters, derived from cDTI-derived pkS, and ε can reliably identify patients with decreased RV EF <45%.

Cardiovascular magnetic resonance as an alternate method for serial evaluation of proximal aorta: comparison with echocardiography.

Thoracic aortic disease is a known cause of aortic dilatation and poses significant risk of aortic dissection and rupture. Serial assessment of aortic root dimensions is traditionally performed using echocardiography, which is limited with older age and following surgery, due to poor acoustic windows. Although diastolic measurements are utilized as standard practice in decision making of adult aortopathy, systolic diameters are utilized in pediatric practice. Three-dimensional steady-state free precision (3D-SSFP) has shown promise as an alternate method for providing accurate and reproducible aortic measurements. The agreement between proximal aorta measurements by diastolic 3D-SSFP and echocardiography (both systole and diastole) was examined in 40 subjects. The maximum inner diameters at aortic annulus, root and sinotubular junction demonstrated excellent agreement between 3D-SSFP and echocardiography for all the 3 levels. The best agreement was observed for diastolic root dimensions with a mean difference of +0.01 cm, limits of agreement being -0.26 to +0.28 cm. Three D-SSFP can be used interchangeably with echocardiography in the serial assessment of the aortic root size. Careful attention to obtain an imaging plane utilizing 3D multiplanar reformatting is critical to maximize the agreement between the two imaging modalities.

Comparison between proximal thoracic vascular measurements obtained by contrast-enhanced magnetic resonance angiography and by transthoracic echocardiography in infants and children with congenital heart disease.

Accurate assessment of the proximal thoracic vasculature in infants and children with congenital heart disease (CHD) is vital for deciding the appropriate surgical or interventional procedure and predicting outcomes. This information usually is obtained by transthoracic echocardiography (TTE). Contrast-enhanced magnetic resonance angiography (CE-MRA) frequently is used to obtain diagnostic data when the image quality by TTE is limited. Calculation of z-scores for measurements obtained by CE-MRA in this population currently is not possible due to the lack of normative data. A reasonable agreement between vessel dimensions by CE-MRA and TTE will allow the use of TTE-based z-scores on measurements from CE-MRA. This study examines the accuracy and agreement of proximal thoracic vascular measurements obtained by CE-MRA versus TTE. Infants and children younger than 3 years with CHD who had a CE-MRA between August 2006 and May 2011 were retrospectively identified. Main and branch pulmonary arteries, ascending aorta, distal transverse arch, and aortic isthmus were measured from CE-MRA and TTE in analogous imaging planes and locations by two investigators blinded to each other. The study enrolled 35 subjects with CHD. The median age was 129 days (range, 0-1077 days), and the median weight was 5.8 kg (range, 2.16-17 kg). The median interval between the two imaging methods was 9 days (range, 0-60 days). Data analysis was performed with 129 of the 210 possible paired measurements. The remaining 81 paired measurements could not be performed due to inaccurate visualization of vessel borders or an unavailable imaging plane from TTE, CE-MRA, or both. The range of vessel sizes measured from 2.8 to 23.4 mm. There was excellent correlation between CE-MRA and TTE (r = 0.94, p < 0.001). The mean difference between the measurements was -0.1 ± 1.2 mm, and the limits of agreement were -2.5 to 2.3 mm. Proximal thoracic vascular measurements obtained by CE-MRA and TTE in infants and children with CHD have a strong correlation. The agreement between these two imaging methods is adequate. Until normative data for vessel size measurements obtained from CE-MRA are available for this population, TTE-based z-scores can be applied to the measurements obtained by CE-MRA.

The role of right ventricular function in paediatric idiopathic dilated cardiomyopathy.

INTRODUCTION: The prevalence of right ventricular dysfunction in idiopathic dilated cardiomyopathy is incompletely studied in children. Furthermore, right ventricular function may signal worse outcomes. We evaluated recently published right ventricular function echocardiographic indices in identifying dysfunction in children with idiopathic dilated cardiomyopathy and the impact of right ventricular dysfunction on long-term prognosis. METHODS: A retrospective database review of right ventricular function indices in 30 patients with idiopathic dilated cardiomyopathy was compared with 60 age- and sex-matched controls from January, 2001 until December, 2010. Right ventricular function was assessed by Doppler tissue peak systolic S', early and late diastolic E' and A' waves and isovolumic acceleration at the tricuspid valve annulus; pulsed wave Doppler tricuspid valve inflow E and A waves; right ventricular myocardial performance index; tricuspid annular plane systolic excursion; right ventricular fractional area change. RESULTS: Right ventricular systolic and diastolic function in idiopathic dilated cardiomyopathy was significantly impaired. All measured indices except for isovolumic acceleration and fractional area change were significantly reduced, with a p-value less than 0.05. There was no right ventricular index predictive of death or transplantation. Patients with poor outcome were significantly more likely to need inotropic support (p-value equal to 0.018), be placed on a ventricular assist device (p equal to 0.005), and have a worse left ventricular ejection fraction z-score (p-value equal to 0.002). CONCLUSION: Right ventricular dysfunction is under-recognised in children presenting with idiopathic dilated cardiomyopathy. The need for clinical circulatory support and left ventricular ejection fraction z-score less than minus 8 were primary determinants of outcome, independent of the degree of derangement in right ventricular function.

Normal values of left atrial volume in pediatric age group using a validated allometric model.

BACKGROUND: Left atrial volume (LAV) increase is an indicator of diastolic dysfunction and a surrogate marker of significant left to right shunts. Normalization of LAV is currently performed by indexing to body surface area(1) (BSA(1)). The indexed LAV thus derived does not account for the nonlinear relationship of physiologic variables to BSA and has not been tested for independence to body size. Our objective was to identify a valid allometric model for indexing LAV and use it to develop Z-scores in children. METHODS AND RESULTS: LAV was measured in 300 normal subjects by echocardiography using the biplane area length method. LAV/BSA(1) had a residual relationship to BSA (r=0.52, P<0.0001). The allometric exponent (AE) derived for the entire cohort (1.27) using the least squares regression analysis also failed to eliminate the residual relationship to BSA (r=-0.15, P=0.01). Dividing the cohort in two groups with a BSA cut-off of 1 m(2) provided the best-fit allometric model. The AE for each group was 1.48 and 1.08 for BSA≤1 m(2) and >1 m(2), respectively, and was validated against an independent sample. The mean indexed LAV±SD for BSA≤1 m(2) and >1 m(2) is 31.5±5.5 mL and 26.0±4.2 mL, respectively, and was used to derive Z-scores. CONCLUSIONS: This study demonstrates the fallacy of using "per-BSA(1) standards" for normalization of LAV in pediatrics. LAV/BSA(1.48) for children with BSA≤1 m(2) and LAV/BSA(1.08) for those with BSA>1 m(2) is accurate and can be used to derive Z-scores.

Imaging of congenital coronary artery anomalies.

Imaging of the coronary arteries is an important part of the evaluation of children with congenital heart disease and isolated congenital coronary artery anomalies. Echocardiography remains the main imaging modality and is complemented by MRI and CT angiography in the older or difficult-to-image child. We review echocardiography, MRI, and CT angiography for coronary artery imaging, with emphasis on techniques. The clinical implications of isolated congenital coronary artery anomalies are also addressed, along with a discussion about the current consensus on optimal management of these anomalies.