Fraser Golding

Improved Classification of Coronary Artery Abnormalities Based Only on Coronary Artery z-Scores After Kawasaki Disease

Competing definitions and classifications of coronary artery abnormalities (CAAs) after Kawasaki disease (KD) have been arbitrarily defined based on clinical experience. We sought to propose a classification system for CAAs based only on coronary artery z-scores. All echocardiograms performed between 1990 and 2007 on patients with a previous history of KD were reviewed. Coronary artery luminal dimensions were converted to body-surface-area-adjusted z-scores and compared to current classification systems. A total of 1356 patients with a previous history of KD underwent 4379 echocardiograms. There was important overlap in the distributions of coronary artery z-scores between the different CAA classes as defined by the American Heart Association (AHA). The AHA classification underestimated the severity of CAAs in 19–32% of small CAAs and 35–78% of medium CAAs. We determined the optimal definition of CAA to be small if the z-score is ≥2.5 to <5.0, large if the z-score is ≥5.0 to <10.0, and giant if the z-score is ≥10.0. This classification seems to appropriately apply to the circumflex branch despite a lack of normal values for this branch. The current AHA classification might not accurately classify CAAs in KD patients. Accurate classification is important for defining management and prognosis consistently across patient age and size.

Clinical Features, Management, and Outcome of Children With Fetal and Postnatal Diagnoses of Isomerism Syndromes

Background— Isomerism is associated with a complex spectrum of anomalies. There is paucity of data on prenatally detected cases. Methods and Results— Between January 1990 and February 2004, 83 of 166 cases (50%) had a prenatal diagnosis of left isomerism (LAI; 52 of 97) or right isomerism (RAI; 31 of 69) at our institution. The spectrum of anomalies, management, and outcomes was compared for fetal and postnatal diagnoses of LAI and RAI. RAI more often than LAI was associated with AV septal defect (90% versus 56%; P<0.0001), pulmonary outflow obstruction (91% versus 37%; P<0.0001), total anomalous pulmonary venous drainage (73% versus 13%; P<0.0001), and abnormal VA connections (68% versus 33%; P<0.0001), whereas inferior vena cava interruption (3% versus 93%; P<0.0001), complete AV block (0% versus 13%; P=0.004), aortic obstruction (6% versus 33%; P<0.0001), and extracardiac defects (5% versus 25%; P=0.006) were less common. The spectrum of lesions was comparable for fetal and postnatal cases, except for AV block (fetal, 25%; postnatal, 0%; P=0.0002) and AV septal defect (fetal, 67%; postnatal, 42%; P=0.023) in LAI. Fetal demise was due mainly to pregnancy termination (LAI, 42%; RAI, 45%). Survival of actively managed children with LAI was significantly better than for those with RAI (P<0.0001) but did not differ with regard to fetal versus postnatal diagnosis. Most LAI cases required no intervention or underwent successful biventricular cardiac surgery (65%), unlike RAI cases (13%; P<0.0001). Conclusions— Prenatal diagnosis did not affect overall survival despite facilitated care. The prognosis of RAI was worse compared with LAI because of more complex associated cardiac defects and the inability to perform successful surgical procedures.

Extent of myocardial noncompaction: comparison between MRI and echocardiographic evaluation

Background: Noncompaction of the left ventricular myocardium is an important cause of cardiomyopathy. There is no clear consensus about its diagnostic criteria or the diagnostic test of choice. MRI is increasingly used in the pediatric cardiac field because of its superior and objective image quality. Objective: To compare the echocardiographic and MRI findings in four patients with recently diagnosed ventricular noncompaction. Materials and methods: We compared the extent of myocardial involvement shown at MRI and echocardiography in four individuals, two patients with echocardiographic diagnosis of left ventricular noncompaction, and two family members of one of the patients. Results: In all patients, MRI showed wider area of involvement than echocardiography. A definite diagnosis was entertained in only two patients by echocardiography but in all by MRI. Cine imaging was diagnostic of the disease in all patients. Black-blood pool imaging with double-inversion recovery sequence also helped to visualize the abnormal areas by showing slow flow artifacts in the four- and two-chamber images. Conclusion: MRI provided better delineation of the extent of the abnormal trabeculation in patients with noncompaction of the left ventricular myocardium. It was particularly useful when the myocardial involvement was subtle, as in the asymptomatic family members.

The ventricular volume variability study of the Pediatric Heart Network: study design and impact of beat averaging and variable type on the reproducibility of echocardiographic measurements in children with chronic dilated cardiomyopathy.

BACKGROUND Clinical trials often rely on echocardiographic measures of left ventricular size and function as surrogate end points. However, the quantitative impact of factors that affect the reproducibility of these measures is unknown. To address this issue, the National Heart, Lung, and Blood Institute-funded Pediatric Heart Network designed a longitudinal observational study of children with known or suspected dilated cardiomyopathy aged 0 to 22 years from eight pediatric clinical centers. METHODS Clinical data were collected together with 150 echocardiographic indices of left ventricular size and function. Separate observers performed duplicate echocardiographic imaging. Multiple observers performed measurements from three cardiac cycles to enable assessment of intraobserver and interobserver variability. The impacts of beat averaging (BA), observer type (local vs core), and variable type (areas, calculations, dimensions, slopes, time intervals, and velocities) on measurement reproducibility were studied. The outcome measure was percentage error (100 × difference/mean). RESULTS Of 173 enrolled subjects, 131 met criteria for dilated cardiomyopathy. BA, variable type and observer type all influenced percentage error (P < .0001). Core interobserver percentage error (medians, 11.4%, 10.2%, and 9.3% for BA using one, two, and three beats, respectively) was approximately twice the intraobserver percentage error (medians, 6.3%, 4.9%, and 4.2% for BA using one, two, and three beats, respectively). Slopes and calculated variables exhibited high percentage error despite BA. Chamber dimensions, areas, velocities, and time intervals exhibited low percentage error. CONCLUSIONS This comprehensive evaluation of quantitative echocardiographic methods will provide a valuable resource for the design of future pediatric studies. BA and a single core lab observer improve the reproducibility of echocardiographic measurements in children with dilated cardiomyopathy. Certain measurements are highly reproducible, while others, despite BA, are poorly reproducible.

Prolongation of the Atrioventricular Conduction in Fetuses Exposed to Maternal Anti-Ro/SSA and Anti-La/SSB Antibodies Did Not Predict Progressive Heart Block A Prospective Observational Study on the Effects of Maternal Antibodies on 165 Fetuses

OBJECTIVES We prospectively examined the prevalence and outcome of untreated fetal atrioventricular (AV) prolongation in the presence of maternal anti-Ro antibodies. BACKGROUND It has been suggested that antibody-mediated congenital complete atrioventricular block (CAVB) may be preventable if detected and treated early when low-grade block is present. With this rationale in mind, dexamethasone has been advocated by others to treat prolonged fetal AV conduction >2 z-scores, consistent with first-degree heart block. METHODS Between July 2003 and June 2009, 165 fetuses of 142 anti-Ro/La antibody-positive women were referred to our center for serial echocardiography. Our protocol included weekly evaluation of the fetal AV conduction between 19 (range 17 to 23) and 24 (range 23 to 35) gestational weeks. AV times were compared with institutional reference data and with post-natal electrocardiograms. RESULTS Of 150 fetuses with persistently normal AV conduction throughout the observation period, a diagnosis of CAVB was subsequently made in 1 at 28 weeks, after the serial evaluation had ended. Of 15 untreated fetuses either with AV prolongation between 2 and 6 z-scores or with type 1 second-degree block, progressive heart block developed in none of them. Three of these 15 fetuses (20%) had a neonatal diagnosis of first-degree block that spontaneously resolved (n = 2) or has not progressed (n = 1) on follow-up examinations. No other cardiac complications were detected. CONCLUSIONS Fetal AV prolongation did not predict progressive heart block to birth. Our findings question the rationale of a management strategy that relies on the early identification and treatment of fetal AV prolongation to prevent CAVB.

Real-Time Subcostal 3-Dimensional Echocardiography for Guided Percutaneous Atrial Septal Defect Closure

We report the closure of a secundum atrial septal defect (ASD) using subcostal real-time 3-dimensional (3D) echocardiographic guidance. A 6-year-old girl (wt 22.6 Kg) with a 9- mm ASD underwent percutaneous closure. The indication for intervention was a dilated right ventricle with an increased end-diastolic dimension for her age. The ASD was assessed using a new, truly real-time, 3D imaging system (Philips Sonos 7500 with live 3D). Subcostal 3D views, supplemented by 2-dimensional Doppler color flow mapping were utilized. A sterile barrier between the echocardiographic probe and …

Etiology and outcome of prenatally detected paracardial cystic lesions: a case series and review of the literature

Isolated paracardial cysts, defined as cystic structures adjacent to or originating from the heart, are rare and etiologically heterogeneous congenital abnormalities. The purpose of this study was to review our experience with prenatally diagnosed isolated cysts.

Utility of Contrast Echocardiography for Pulmonary Arteriovenous Malformation Screening in Pediatric Hereditary Hemorrhagic Telangiectasia

OBJECTIVE To evaluate the utility of transthoracic contrast echocardiography (TTCE) as a screening tool for pulmonary arteriovenous malformations (PAVMs) in children with hereditary hemorrhagic telangiectasia (HHT). STUDY DESIGN This was a single-center study of children who underwent baseline screening for PAVMs using both TTCE and chest computed tomography (CT) for evaluation of HHT. The CT and TTCE results were prospectively reviewed independently by 2 radiologists and 2 cardiologists blinded to the study results. RESULTS Both intraobserver and interobserver agreement for interpreting TTCE results were excellent (κ = 0.97 and 0.92, respectively) and higher than the interobserver agreement for CT interpretation (κ = 0.75). The sensitivity and specificity of TTCE to predict PAVMs were 1 and 0.82, respectively, and the positive predictive and negative predictive values were 0.39 and 1, respectively. CONCLUSION TTCE is a sensitive test for PAVMs in children with suspected HHT and can be a useful initial screening tool in pediatric HHT.

Spectrum and Outcome of Primary Cardiomyopathies Diagnosed During Fetal Life

OBJECTIVES The purpose of this study was to determine the phenotypic presentation, causes, and outcome of fetal cardiomyopathy (CM) and to identify early predictors of outcome. BACKGROUND Although prenatal diagnosis is possible, there is a paucity of information about fetal CM. METHODS This was a retrospective review of 61 consecutive fetal cases with a diagnosis of CM at a single center between 2000 and 2012. RESULTS Nonhypertrophic CM (NHCM) was diagnosed in 40 and hypertrophic CM (HCM) in 21 fetuses at 24.7 ± 5.7 gestational weeks. Etiologies included familial (13%), inflammatory (15%), and genetic-metabolic (28%) disorders, whereas 44% were idiopathic. The pregnancy was terminated in 13 of 61 cases (21%). Transplantation-free survival from diagnosis to 1 month and 1 year of life for actively managed patients was better in those with NHCM (n = 31; 58% and 58%, respectively) compared with those with HCM (n = 17; 35% and 18%, respectively; hazard ratio [HR]: 0.44; 95% confidence interval [CI]: 0.12 to 0.72; p = 0.007). Baseline echocardiographic variables associated with mortality in actively managed patients included ventricular septal thickness (HR: 1.21 per z-score increment; 95% CI: 1.07 to 1.36; p = 0.002), cardiothoracic area ratio (HR: 1.06 per percent increment; 95% CI: 1.02 to 1.10; p = 0.006), ≥3 abnormal diastolic Doppler flow indexes (HR: 1.44; 95% CI: 1.07 to 1.95; p = 0.02), gestational age at CM diagnosis (HR: 0.91 per week increment; 95% CI: 0.83 to 0.99; p = 0.03), and, for fetuses in sinus rhythm, a lower cardiovascular profile score (HR: 1.45 per point decrease; 95% CI: 1.16 to 1.79; p = 0.001). CONCLUSIONS Fetal CM originates from a broad spectrum of etiologies and is associated with substantial mortality. Early echocardiographic findings appear useful in predicting adverse perinatal outcomes.

The impact of additional epicardial imaging to transesophageal echocardiography on intraoperative detection of residual lesions in congenital heart surgery

OBJECTIVE Transesophageal echocardiography is the primary intraoperative imaging modality used to evaluate cardiac surgery. Its predecessor, epicardial echocardiography, enables visualization of certain cardiovascular structures that are beyond the visual scope of transesophageal echocardiography. We review the current use of epicardial echocardiography to analyze its contemporary application and benefit. METHODS A retrospective review of the intraoperative echocardiograms of 1204 children undergoing bypass cardiovascular surgery between January 2007 and December 2009 was performed. The incidence of epicardial echocardiography use, intraoperative revisions, and early reinterventions were analyzed. RESULTS Epicardial echocardiography was performed in 7.9% of all intraoperative studies: epicardial echocardiography alone (n = 38) and transesophageal echocardiography + epicardial echocardiography (n = 57). Epicardial echocardiography alone was performed in patients with contraindications for transesophageal echocardiography. In the transesophageal echocardiography + epicardial echocardiography group, indications to obtain additional information by epicardial echocardiography were for the assessment of branch pulmonary arteries (40%), coronary arteries (28%), aortic arch/Blalock-Taussig shunt (14%), Glenn/Fontan circuit (9%), pulmonary veins/baffles (7%), and residual ventricular septal defects (1.7%). The overall intraoperative surgical revision incidence was 10.2%, consisting of 21% of the transesophageal echocardiography + epicardial echocardiography group, 5.3% of the epicardial echocardiography alone group (P = .01), and 9.8% of transesophageal echocardiography alone group (P = .02). Intraoperative revisions indicated after epicardial echocardiography were mostly related to extracardiac structures (77%), whereas they were mostly related to intracardiac structures in the transesophageal echocardiography alone group (80.7%) (P = .0002). Early reintervention was indicated mostly for pulmonary artery and Glenn obstructions, the majority (75%) with previously known stenosis or interventions on the pulmonary arteries. CONCLUSIONS Epicardial echocardiography detects residual intraoperative lesions not visualized by transesophageal echocardiography, most frequently related to pulmonary arteries. Its use, in addition to standard transesophageal echocardiography, may decrease the need for early reintervention.