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Objectives. Discovery of the incidence and nature of heart disease identified among children with murmurs clinically ambiguous to the expert examiner, and elucidation of clinically recognizable features which correlate with the discovery of actual heart disease in this group. Design. Prospective, blinded study of diagnostic accuracy of the expert examination was made using echocardiography (echo) as the diagnostic standard. Setting. Pediatric cardiology outpatient department. Patients. 804 outpatients with heart murmur under 21 years old, without prior echo or pediatric cardiology consultation. Intervention. Echo as clinically indicated for evaluation of heart murmur of uncertain cause. Measurements. Expert examiners' clinical diagnoses and echo results in patients (pts) grouped by the examiner's level of suspicion of heart disease, by age of pt, and by indication for echo. Outcome Measures. Clinical diagnoses were compared with echo results across groups using chi square testing for dichotomous variables, and Wilcoxon 2-sample rank test for continuous or multi-level variables. Results. Expert's level of suspicion of heart disease was strongly and positively associated with presence of disease (P [is less than] 0.0001). Clinical IM not on IM on diffl dx, but less Impression diff'l dx likely than other dx IM likelihood 26/389, 6.7% 44/176, 40.5% IM most likely, IM the only but other dx consideration also listed 116/144, 80.6% 143/155, 92.3% Abbreviations: diff'l dx=differential diagnosis; IM=innocent murmur. Age of pt at presentation to cardiology clinic has strong negative association with likelihood of heart disease (p [is less than] 0.0001). Patient age [is less than] 0.10 years IM likelihood 39/185, 21.1% Patient age 1.00-4.00 years IM likelihood 73/154, 47.4% Patient age 0.10-1.00 years IM likelihood 87/243, 35.8% Patient age [is greater than] 4.00 years IM likelihood 133/222, 59.9% Among the 155 cases in which the experts' diff'l dx contained IM as the sole consideration there were 12 in which heart disease was discovered by echo. Four of these 12 have had catheter or surgical intervention (atrioventricular septal defect repair-1, cor triatriatum repair-1, secundum atrial septal defect repair-1, and patent ductus arteriosus coil-1). When IM was the sole consideration (n= 155) and the only stated reason for echo was pt, family, or referring physician anxiety (n=43) there was only 1 case of heart disease discovered (2.3%). When symptoms, an abnormal electrocardiogram, or chest X-ray were among the considerations compelling the echo (n=112) 11 cases of heart disease were discovered (9.8%); p [is less than] 0.05. Conclusions. Recognizing that in many cases IM is diagnosed clinically, conclusions drawn from this study are limited to those murmurs in which diagnostic ambiguity remains after the expert clinical examination. In this context, the examiner's clinical impression of likelihood of heart disease correlates well with presence of actual disease. However, important disease is occasionally discovered even when the expert formulates no plausible diagnosis except IM, especially when reasons beyond family and referring physician anxiety compel the cardiologist to obtain an echo. Even when a murmur sounds innocent to the expert examiner, clinical and laboratory clues suggestive of heart disease should not be suppressed as indications for echo in an effort to contain costs. David A. Danford, MD, FAAP, Ameeta B. Martin, MD, FAAP, Scott E. Fletcher, MD, FAAP, and Carl H. Gumbiner, MD, FAAP. Joint Section of Pediatric Cardiology, University of Nebraska Medical Center and Creighton University School of Medicine, Childrens Hospital (Omaha, NE) and St. Elizabeth Hospital (Lincoln, NE).3, David A. Danford, MD, FAAP, Ameeta B. Martin, MD, FAAP, Scott E. Fletcher, MD, FAAP, and Carl H. Gumbiner, MD, FAAP. Joint Section of Pediatric Cardiology, University of Nebraska Medical [...]

The clinical presentation, diagnostic workup, and surgical therapy of a coarcted right-sided aortic arch associated with a vascular ring are described. [ABSTRACT FROM AUTHOR]

Aims: We sought to evaluate the efficacy of ultrasound contrast (UC) and low mechanical index real-time perfusion (RTP) in the haemodynamic and anatomic assessment of repaired congenital heart disease (CHD) at rest and during supine bicycle stress echocardiography (BSE)., Methods and Results: Patients with CHD (n = 51, median age 21.5 years) were prospectively studied. All had compromised image quality, 20 (39%) had arrhythmias, and 10 (20%) had pacemakers. RTP was performed at rest and during BSE using Definity and Contrast Pulse Sequencing, with assessment of Doppler pressure gradients. Diagnoses included tetralogy of Fallot (n = 27), transposition of the great arteries (TGA) atrial switch (n = 10), TGA arterial switch (n = 2), aortic valve disease (n = 4), Fontan (n = 4), and Kawasaki disease (n = 4). UC with RTP improved endocardial border definition, with increased number of left ventricular (LV) and right ventricular (RV) segments visualized at rest (P < 0.0001) and during stress. LV ejection fraction (EF) and RV fractional area change (FAC) were measurable at rest and peak stress, RV FAC correlating closely with same-day magnetic resonance EFs (r = 0.72; P < 0.001). UC enhanced Doppler signals, enabling subpulmonary ventricular systolic pressure measurements at rest and stress. In six patients, marked elevations of subpulmonary ventricular systolic pressure were detected with UC during BSE, and quantifiable ventricular dysfunction. No adverse events occurred, other than transient low back pain in one patient., Conclusion: UC at rest and with supine BSE enables safe and comprehensive assessment of anatomy, haemodynamics, and biventricular functional and perfusion reserve in adolescents and young adults with surgically modified CHD.