Jing Zhou

Characterization of cardiac tumors in children by cardiovascular magnetic resonance imaging: a multicenter experience.

OBJECTIVESnThe aim of this study was to report the results of an international multicenter experience of cardiac magnetic resonance imaging (MRI) evaluation of cardiac tumors in children, each with histology correlation or a diagnosis of tuberous sclerosis, and to determine which characteristics are predictive of tumor type.nnnBACKGROUNDnIndividual centers have relatively little experience with diagnostic imaging of cardiac tumors in children, because of their low prevalence. The accuracy of cardiac MRI diagnosis on the basis of a pre-defined set of criteria has not been tested.nnnMETHODSnAn international group of pediatric cardiac imaging centers was solicited for case contribution. Inclusion criteria comprised: 1) age at diagnosis ≤18 years; 2) cardiac MRI evaluation of cardiac tumor; and 3) histologic diagnosis or diagnosis of tuberous sclerosis. Data from the cardiac MRI images were analyzed for mass characteristics. On the basis of pre-defined cardiac MRI criteria derived from published data, 3 blinded investigators determined tumor type, and their consensus diagnoses were compared with histologic diagnoses.nnnRESULTSnCases (n = 78) submitted from 15 centers in 4 countries had the following diagnoses: fibroma (n = 30), rhabdomyoma (n = 14), malignant tumor (n = 12), hemangioma (n = 9), thrombus (n = 4), myxoma (n = 3), teratoma (n = 2), and paraganglioma, pericardial cyst, Purkinje cell tumor, and papillary fibroelastoma (n = 1, each). Reviewers who were blinded to the histologic diagnoses correctly diagnosed 97% of the cases but included a differential diagnosis in 42%. Better image quality grade and more complete examination were associated with higher diagnostic accuracy.nnnCONCLUSIONSnCardiac MRI can predict the likely tumor type in the majority of children with a cardiac mass. A comprehensive imaging protocol is essential for accurate diagnosis. However, histologic diagnosis remains the gold standard, and in some cases malignancy cannot be definitively excluded on the basis of cardiac MRI images alone.

Regional Dysfunction of the Right Ventricular Outflow Tract Reduces the Accuracy of Doppler Tissue Imaging Assessment of Global Right Ventricular Systolic Function in Patients with Repaired Tetralogy of Fallot

BACKGROUNDnThe influence of regional right ventricular (RV) dysfunction on the accuracy of Doppler tissue imaging (DTI) assessment of global RV function is unknown. The objective of this study was to determine the effect of regional dysfunction of the RV outflow tract (RVOT) on the correlations between RV DTI indices and cardiac magnetic resonance (CMR) measurements of global RV function in patients with repaired tetralogy of Fallot.nnnMETHODSnConsecutive patients with repaired tetralogy of Fallot who underwent echocardiography with DTI of the right ventricle and CMR within 4 weeks of each other were retrospectively analyzed. RV DTI measurements were obtained from the lateral wall at the level of the tricuspid valve annulus. CMR measurements included end-diastolic and end-systolic volumes, stroke volume, and ejection fraction (EF) of the entire right ventricle and measured separately for the RV sinus and RVOT segments.nnnRESULTSnThe median age of the 51 patients included was 19 years (range, 9.7-71.6 years), and the median interval between echocardiography and CMR was 0 days. The mean RV free wall peak S, isovolumic acceleration, and global, sinus, and RVOT EFs were 8.4 ± 2.0 cm/s, 102 ± 37 cm/s(2), and 46.1 ± 9.8%, 47.9 ± 9.9%, and 33 ± 13.1%, respectively. The correlation between peak S and global RV EF was weak (rxa0= 0.23) in patients with RVOT dysfunction (RVOT EF <30%) but higher (rxa0= 0.66) in those with RVOT EFs ≥30%. Peak S ≥8.4 cm/s (area under the receiver operating characteristic curve, 0.77) and isovolumic acceleration ≥95 cm/s(2) (area under the receiver operating characteristic curve, 0.68) best discriminated between patients with global RV EFs >45% and <45%.nnnCONCLUSIONSnIn this group of patients with repaired TOF, RV DTI indices showed reasonable correlation with CMR-derived global RV EF, but this correlation was substantially weaker in those with moderate and severe dysfunction of the RVOT. Peak S <8.4 cm/s and isovolumic acceleration <95cm/s(2) by DTI should prompt an evaluation of RV function by CMR.

Comparison of cardiac MRI tissue tracking and myocardial tagging for assessment of regional ventricular strain

This study sought to compare regional measures of ventricular strain by tissue tracking (TT) to those derived from myocardial tagging (MT) within cardiac MR (CMR), in normal subjects and patients with hypertrophic cardiomyopathy. CMR images from 13 normal subjects and 11 subjects with hypertrophic cardiomyopathy were retrospectively analyzed. For each subject, equivalent mid-papillary level short-axis cine steady-state free precession and MT slices from the same examination were evaluated. The time to peak circumferential strain and magnitude of the peak strain were calculated for 6 matched left ventricular segments. Data from 24 slices (nxa0=xa0144 segments) were compared. The mean difference between techniques in magnitude of peak strain and time to peak strain was 1xa0±xa09% and 1xa0±xa058xa0ms, respectively. The mean difference in the standard deviation of time to peak strain within a slice was 0xa0±xa019xa0ms (mean cardiac cycle duration 1,013xa0±xa0204xa0ms). Bland–Altman analysis showed closer agreement in time to peak strain than peak strain magnitude. Measurements of segmental time to peak strain by TT and MT were in close agreement; agreement for the magnitude of peak segmental strain was more modest. The TT approach does not add to CMR examination time and may be a useful tool for the assessment of ventricular synchrony.

Needles in Hay: Chest Pain as the Presenting Symptom in Children with Serious Underlying Cardiac Pathology

INTRODUCTIONnChest pain in children is common, but rarely heralds serious underlying cardiac pathology. Despite this, the anxiety of missing a potentially life threatening condition creates a large burden of referrals and diagnostic testing. We evaluated patients diagnosed with 1 of 9 serious cardiac diseases and detailed the clinical signs and symptoms of the patients presenting with chest pain.nnnMETHODSnPatients diagnosed between the ages of 7 and 21 years from January 2000 to December 2009 at Childrens Hospital Boston (CHB) were identified from a database using diagnostic and billing codes for aortic dissection, coronary anomalies, dilated cardiomyopathy, hypertrophic cardiomyopathy, myocarditis, pericarditis, pulmonary embolus, pulmonary hypertension, and Takayasu arteritis. Patients with previously diagnosed congenital or acquired heart disease were excluded.nnnRESULTSnFour hundred eighty-four patients were included and 35% presented with chest pain. Forty-one (24%) of these patients with chest pain were diagnosed in the outpatient cardiology clinic, while the remaining 130 patients (76%) were diagnosed in the emergency department (ED) or inpatient setting. Coronary artery anomalies were the most common diagnosis made in cardiology clinic, and 16 of the 23 (70%) patients with serious coronary anomalies had exercise-induced chest pain. Patients presenting to the ED or inpatient units tended to have other important nonspecific symptoms (35-44%), high-risk past medical histories (12%), physical examination findings (32%), and electrocardiogram (ECG) abnormalities (78%) that heighten clinical suspicion of cardiac disease.nnnCONCLUSIONSnIdentifying underlying cardiac pathology in the CHB outpatient cardiology department in patients presenting with chest pain is rare, with only 41 cases over a 10-year period. The presence of exertional chest pain was important in identifying patients with coronary artery anomalies. A detailed history and physical examination, along with a critical review of an ECG, seem to identify those patients with rare diseases who need further diagnostic testing.

Interstudy variability in cardiac magnetic resonance imaging measurements of ventricular volume, mass, and ejection fraction in repaired tetralogy of fallot: A prospective observational study

To assess the interstudy variability of cardiac magnetic resonance imaging (CMR) parameters of ventricular size and function in repaired tetralogy of Fallot (TOF).

Outcome predictors and implications for management of scimitar syndrome.

BACKGROUNDnScimitar syndrome is a rare congenital anomaly. We evaluated risk factors for postoperative pulmonary vein stenosis or death and predictive factors for survival without scimitar vein surgery in patients with scimitar syndrome.nnnMETHODSnThe records of patients with scimitar syndrome evaluated at our medical center between 1964 and 2011 were reviewed.nnnRESULTSnScimitar syndrome was identified in 80 patients, with a median follow-up of 4.5 years. Patients presenting less than 1 year of age had a higher incidence of symptoms, aortopulmonary collaterals, coexisting congenital heart disease (CHD), extracardiac anomalies, and pulmonary hypertension. Of 36 patients having scimitar vein surgery, 18 had postoperative pulmonary vein obstruction that occurred with similar frequency after baffle or reimplantation procedures, early or late in the study period, and tended to be more common in infants (P = .10). Overall, 19 (24%) of 80 died. Multivariate risk factors for death included systolic pulmonary pressure >0.5 systemic level (P = .007) and left pulmonary vein stenosis (P = .009). Pulmonary artery systolic pressure <0.5 systemic level (P = .01) and absence of CHD excluding atrial septal defect (P = .01) were predictive factors in 28 patients who survived and did not have scimitar vein surgery; these patients had no or mild right ventricular dilation and a ratio of pulmonary-to-systemic flow <1.6 either at baseline, after coiling aortopulmonary collaterals or nonscimitar vein intervention.nnnCONCLUSIONSnPostoperative pulmonary vein obstruction is common after scimitar vein surgery regardless of redirection technique. Pulmonary hypertension and left pulmonary vein stenosis are risk factors for death, whereas patients without significant pulmonary hypertension or associated CHD did well without scimitar vein surgery. These observations may guide management decisions in patients with scimitar syndrome.

Feasibility and Reproducibility of Three-Dimensional Echocardiographic Assessment of Right Ventricular Size and Function in Pediatric Patients

BACKGROUNDnQuantitative assessment of right ventricular (RV) size and function is important in congenital heart disease. Although three-dimensional (3D) echocardiography overcomes geometric challenges that limit two-dimensional echocardiography, its feasibility and reproducibility in children have not been systematically evaluated. The goal of this study was to determine the feasibility and reproducibility of 3D echocardiographic RV measurements in children.nnnMETHODSnPatients aged 0 to 20 years were prospectively enrolled. Full-volume 3D echocardiographic RV data sets were acquired in each from the subxiphoid and apical four-chamber views by a trained sonographer. Another sonographer then acquired a second image data set from the same patient. RV volumes and ejection fraction were then measured offline. Outcomes included successful acquisition, measurable data set, and observer and interstudy variability.nnnRESULTSnThree-dimensional echocardiographic RV data sets were obtainable in 67 of 70 patients (96%) and were measurable from at least one view in 39 (58%). Factors associated with nonmeasurable data sets by univariate analysis included older age, larger body surface area and body mass index, no sedation, and female gender. Multivariate analysis identified older age (odds ratio, 1.34; 95% confidence interval, 1.17-1.55, P < .0001) and female gender (odds ratio, 6.06; 95% confidence interval, 1.45-25.3, P = .0135) as independently associated with nonmeasurable RV data sets. Intraobserver, interobserver, and interstudy agreement was excellent for analyzable RV volumes and modest for RV ejection fraction.nnnCONCLUSIONSnTransthoracic 3D echocardiographic RV volumes were measurable in 58% of children with and without congenital heart disease. Older age and female gender were independently associated with nonanalyzable data sets. When feasible, measurements of RV volumes are highly reproducible.

Echocardiographic Predictors of Left Ventricular Dysfunction after Aortic Valve Surgery in Children with Chronic Aortic Regurgitation

OBJECTIVEnPostoperative left ventricular dysfunction is associated with poor prognosis in adults with severe chronic aortic regurgitation and published practice guidelines aim to minimize this risk. However, only limited information exists in pediatrics. The goal of this study was to define preoperative risk factors for postoperative left ventricular dysfunction in children with chronic aortic regurgitation.nnnMETHODSnPatients fulfilling the following criteria were included in this study: (1) age at preoperative echocardiogram ≤18 years; (2) ≥moderate aortic regurgitation; (3) ≤mild aortic valve stenosis; (4) no additional valve disease/shunt; (5) underwent aortic valve surgery for aortic regurgitation; and (6) available preoperative and ≥6-month postoperative echocardiograms with adequate information. Primary outcome was postoperative left ventricular dysfunction defined as ejection fraction z-score < -2.nnnRESULTSnMedian ages at diagnosis and surgery of the 53 eligible patients were 6.9 (0.04-17.2) and 13 years (1.2-22.4), respectively. Compared with patients whose postoperative left ventricular ejection fraction was normal, those with left ventricular ejection fraction z-score < -2 (n = 10) had significantly higher preoperative left ventricular end-diastolic and systolic volumes and dimensions and lower indices of systolic function. Preoperative left ventricular ejection fraction z-score < -1 was the most sensitive (89%; confidence interval [CI] 52, 100) but least specific (58%; CI 41, 73), whereas left ventricular end-systolic diameter z-score ≥ 5 was the most specific (95%; CI 84, 99) but least sensitive (60%; CI 26, 88) outcome identifier. A combination of shortening fraction z-score < -1 or end-systolic diameter z-score ≥ 5 best identified postoperative left ventricular dysfunction with an area of 0.819 under the receiver-operator characteristic curve.nnnCONCLUSIONnLower indices of left ventricular systolic function and severity of dilation identify children at risk for postoperative left ventricular dysfunction after aortic valve surgery. These identifiers are similar to predictors defined in adult patients albeit with different threshold values.

Blood pressure is associated with occult cardiovascular disease in prospectively studied Hodgkin lymphoma survivors after chest radiation

Abstract The objectives of this study were to prospectively screen a cohort of asymptomatic long-term survivors of Hodgkin lymphoma (HL) treated with chest irradiation for occult cardiovascular disease (CVD), and correlate screen-detected disease with prospectively measured cardiovascular risk factors (CRFs). A total of 182 HL survivors treated with chest irradiation (median follow-up time 14.8 years) were enrolled and underwent prospective CRF measurement and resting and stress echocardiography to assess coronary artery disease (CAD)/valve disease and left ventricular systolic dysfunction (LVSD). Forty-seven (26%) patients had occult CAD/valve disease and/or LVSD. LVSD was not correlated with CRFs. Controlling for treatment factors, hypertension (odds ratio [OR] = 3.0) and elevated high-sensitivity C-reactive protein (hs-CRP) (OR = 2.7) increased the likelihood of occult CAD/valve disease. Risk of CAD/valve disease rose exponentially with increasing blood pressure (BP) values, even in the normal range. Our findings suggest that BP screening may be useful in determining those survivors at greatest risk for occult CVD.

Inter-Study Variability in CMR Measurements of Right Ventricular Volume, Mass and Ejection Fraction in Tetralogy of Fallot: A Prospective Observational Study

Background Cardiac MRI (CMR) is commonly used for serial monitoring of right ventricular (RV) size and function in patients with repaired tetralogy of Fallot (TOF). However, the inter-study variability of these measurements is not known, which hinders accurate interpretation of serial changes. In this study we prospectively assessed the inter-study variability of CMR measurements of RV size and function in TOF patients. Methods Patients with repaired TOF referred for a clinically indicated CMR examination at our institution were prospectively enrolled. ECG-gated steady-state free precession cine imaging of ventricular long- and short-axis planes was obtained on a 1.5 T scanner. Immediately after the conclusion of the examination, patients returned for a second study performed by a different technologist. Ventricular size and function data from both short-axis image sets were analyzed by a single observer and compared using Bland-Altman analysis with calculation of a repeatability coefficient (2SD of difference between studies), and intra-class correlation coefficient (ICC). Results