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5/1/2015 I have no financial disclosures. Pediatric Cardiac Imaging: Why, Who, What, When and Where I do plan to discuss the off-label use of gadolinium contrast for pediatric cardiac MRI/MRA Austin H Wong, MD FACC, FAAP May 22, 2015 HILTON HEAD ISLAND, SC Goals WHY Image the Heart? Why is this baby blue? Discuss the five W’s but not the H (how) Review common cardiac imaging modalities Strengths, Limitations, Liabilities Discuss new appropriate use criteria for initial pediatric echocardiography Apply guidelines to several common pediatric clinical scenarios Bradford Cox, Deerhunter lead singer • Are these symptoms of CHF? Does this patient have underlying heart disease Are there risk factors for sudden death? Shane Pope, Georgetown Fury Youth Basketball 1 5/1/2015 Goals of Imaging WHAT tests are available? Ionizing Radiation Röentgenography Angiography CT Scan Nuclear scans Define the anatomy Assess contractility Follow the blood flow Assess myocardial viability Röentgenography (Chest X-ray) Simple, convenient, and inexpensive. Assessment of and monitoring for CHF: Cardiomegaly Pulmonary vascularity Associated extracardiac anomalies Small radiation exposure (0.1 mSv per CXR) Non-ionizing Ultrasound Cardiac MRI Won’t discuss EP tests ECG, Holter, loop recorders, EST, EPS Fluoroscopy / Angiography Real-time visualization of chambers, vasculature Especially good for small/distal vessels, e.g. coronaries, PA branches, collaterals (e.g. TOF) Moderate radiation dose (median 4.6 mSv) Labor intensive, limited access, invasive, anesthesia risks Rarely used in isolation Coronary aneurysm due to Kawasaki disease 5/1/2015 Computerized Tomography Coronary-Artery Aneurysms in a 9-Year-Old Boy, 6 Years after the Diagnosis of Acute Kawasaki Disease Excellent anatomic resolution Contrast allows visualization of small vessels, e.g. coronaries Quick, fairly accessible, sedation rarely needed. Newer techniques have reduced radiation dose (median doses 1.4-6.3 mSv) Most common applications: coronary anomalies, complex CHD and aortic pathology Burns J. N Engl J Med 2007;356:659-661 Nuclear Scans: Perfusion Imaging Echocardiography e.g. stress thallium, PET, lung perfusion scan Uses radiolabelled tracers to assess cardiac metabolism e.g. viability p MI, coronary perfusion p KD, TGA Quantification of differential pulmonary blood flow (e.g. PE, branch PA stenosis). Relatively poor spatial resolution, moderate radiation dose (5-18 mSv) Accessible, non-invasive, potentially high resolution Color-flow and Doppler techniques allow quantification of blood velocities, pressures, some myocardial properties Limited by acoustic windows, patient cooperation TEE, contrast and sedation overcome some limitations but increase risk 5/1/2015 Magnetic Resonance Imaging WHICH Modality to Choose? Excellent resolution for anatomic details. Not limited by acoustic windows. No ionizing radiation Contrast allows detailed quantification and fibrosis detection. Older implants / devices maybe contraindications Sedation required, limited availability to date MR Case courtesy of Dr Frank Gaillard, Radiopaedia.org CT Case courtesy of Dr David Cuete, Radiopaedia.org Everyone Needs an Echo! WHO & WHEN: Pediatric AUC Criteria ACC/AAP/ASE/AHA/HRS/SCAI/SCMR/SOPE Highly accessible, can be performed portably, No associated risks As a result, utilization has skyrocketed 2007 American College of Cardiology Foundation established appropriate use criteria (AUC) for adult echocardiography, revised in 2011 Goal to improve patient care, outcomes Improve cost-effectiveness Reporting of compliance mandated for lab accreditation 100 clinical scenarios when echo might be ordered Addresses initial outpatient echocardiography only Independent panel of experts rated scenarios as: “Appropriate”, “May be appropriate”, “Rarely appropriate” Assumes other diagnostic tests, e.g. ECG, CXR have been considered before echo Insurance companies may use statement to validate payment, so clinicians must clearly document symptoms prompting echo 5/1/2015 From: ACC/AAP/AHA/ASE/HRS/SCAI/SCCT/SCMR/SOPE 2014 Appropriate Use Criteria for Initial Transthoracic Echocardiography in Outpatient Pediatric Cardiology: A Report of the American College of Cardiology Appropriate Use Criteria Task Force, American Academy of Pediatrics, American Heart Association, American Society of Echocardiography, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Pediatric Echocardiography J Am Coll Cardiol. 2014;64(19):2039-2060. doi:10.1016/j.jacc.2014.08.003 Chest Pain Appropriate Rarely Appropriate Exertional chest pain Non-exertional CP w/ abnormal ECG CP w/ family history of sudden death or cardiomyopathy CP w/o other symptoms or signs of CV disease, benign FHx, normal ECG Non-exertional CP w/ normal or no ECG Reproducible CP w/ palpation or deep inspiration Figure Legend: Chest Pain Each indication is preceded with a number sign. The rating of A, M, or R is then followed by the median score in parenthesis for that particular indication. ∗See Discussion section. Abbreviations: A = Appropriate; M = May Be Appropriate; R = Rarely Appropriate; ECG = Electrocardiogram. Date of download: 4/14/2015 Copyright © The American College of Cardiology. All rights reserved. From: ACC/AAP/AHA/ASE/HRS/SCAI/SCCT/SCMR/SOPE 2014 Appropriate Use Criteria for Initial Transthoracic Echocardiography in Outpatient Pediatric Cardiology: A Report of the American College of Cardiology Appropriate Use Criteria Task Force, American Academy of Pediatrics, American Heart Association, American Society of Echocardiography, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Pediatric Echocardiography J Am Coll Cardiol. 2014;64(19):2039-2060. doi:10.1016/j.jacc.2014.08.003 Syncope Appropriate Figure Legend: Syncope Each indication is preceded with a number sign. The rating of A, M, or R is then followed by the median score in parenthesis for that particular indication. Abbreviations: A = Appropriate; M = May Be Appropriate; R = Rarely Appropriate; ECG = Electrocardiogram; ICD = Implantable Cardioverter Defibrillator. Date of download: 4/14/2015 Copyright © The American College of Cardiology. All rights reserved. Syncope w/ abnl ECG Family history of sudden cardiac arrest, SCD or AICD <50 y Exertional syncope Unexplained postexertional syncope Rarely Appropriate Syncope w/o recent ECG Syncope w/o other signs or symptoms of CV dx, a benign FHx, normal ECG Probable vasovagal syncope Syncope w/ known noncardiac etiology 5/1/2015 From: ACC/AAP/AHA/ASE/HRS/SCAI/SCCT/SCMR/SOPE 2014 Appropriate Use Criteria for Initial Transthoracic Echocardiography in Outpatient Pediatric Cardiology: A Report of the American College of Cardiology Appropriate Use Criteria Task Force, American Academy of Pediatrics, American Heart Association, American Society of Echocardiography, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Pediatric Echocardiography J Am Coll Cardiol. 2014;64(19):2039-2060. doi:10.1016/j.jacc.2014.08.003 Palpitations & Arrhythmia Appropriate FHx of sudden cardiac arrest/death or AICD less than 50 years old FHx or pers hx of cardiomyopathy If confirmed SVT or VT Rarely Appropriate Palpitations w/o other signs or symptoms of CV disease, benign FHx, normal ECG FHx of channelopathy PAC’s Sinus brady/arrhythmia Figure Legend: Palpitations and Arrhythmias Each indication is preceded with a number sign. The rating of A, M, or R is then followed by the median score in parenthesis for that particular indication. ∗See Discussion section. Abbreviations: A = Appropriate; M = May Be Appropriate; R = Rarely Appropriate; ECG = Electrocardiogram; ICD = Implantable Cardioverter Defibrillator; PACs = Premature Atrial Contractions; PVCs = Premature Ventricular Contractions. Date of download: 4/14/2015 Copyright © The American College of Cardiology. All rights reserved. From: ACC/AAP/AHA/ASE/HRS/SCAI/SCCT/SCMR/SOPE 2014 Appropriate Use Criteria for Initial Transthoracic Echocardiography in Outpatient Pediatric Cardiology: A Report of the American College of Cardiology Appropriate Use Criteria Task Force, American Academy of Pediatrics, American Heart Association, American Society of Echocardiography, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Pediatric Echocardiography J Am Coll Cardiol. 2014;64(19):2039-2060. doi:10.1016/j.jacc.2014.08.003 Other Symptoms and Signs Appropriate Signs/symptoms suggestive of CHF Murmur Each indication is preceded with a number sign. The rating of A, M, or R is then followed by the median score in parenthesis for that particular indication. Abbreviations: A = Appropriate; M = May Be Appropriate; R = Rarely Appropriate. Date of download: 4/14/2015 Copyright © The American College of Cardiology. All rights reserved. Respiratory distress Poor perfusion Failure to thrive Oliguria Edema Hepatomegaly Endocarditis signs Central cyanosis Rarely Appropriate Fatigue w/o other symptoms or signs of CV disease, benign FHx, normal ECG Acrocyanosis 5/1/2015 Systemic Disorders Case 1 Appropriate Chemotherapy Sickle-cell disease Connective tissue dx Genetic syndromes assoc w/ CHD HIV Kawasaki disease Rheumatic fever Lupus Muscular dystrophy Systemic hypertension Renal failure Stroke Storage diseases 18 yo w/ 2 week hx of exertional chest pain Rarely Appropriate Obesity w/o other CV risk factors Diabetes mellitus Lipid disorders Seizures, ADHD, other neurologic, psychiatric dx Gastrointestinal dx NOS Chest Pain Began with onset of track season, elicited only w/ high levels of exertion Physical examination normal Boston Children’s Hospital SCAMP Standardized Clinical Assessment & Mgmt Plan Common, >650,000 pediatric visits annually Cardiac etiologies rare, 0-5% Saleeb et al (Boston CH, Pediatrics 2011) 3,700 pts/10 yrs Idiopathic 52%, musculoskeletal 36%, respiratory 7%, GI 3% Cardiac 1% (SVT, pericarditis), 9/3700 had conditions putting them at risk of SCD: myocarditis 4, AOCA 3, HCM 1, DCM 1 All CV etio identifiable by H&P, selected ECG use No deaths from missed CV dx High levels of pt/family anxiety Work-up extensive, costly, low-yield Designed to reduce practice variation, optimize resource utilization, and improve patient care Originally targeted clinic pediatric cardiologists, expanded to primary care physicians. OPD clinic experience showed that stress tests did not identify any cardiac etiologies for chest pain and should not be performed Holter had no role in absence of palpitations Echo low yield in absence of red flags 5/1/2015 Chest Pain SCAMP Case 1 cont’d Friedman & Alexander, Journal of Pediatrics, 163(3):896-901, 2013 Specific history & physical exam red flags to prompt subspecialty evaluation and cardiac imaging ECG read as normal Echo performed in adult lab was read as normal Underwent CT angiography – coronary anomaly found WHERE: Pediatric Lab Preferred Case 2 Kids are not little adults. 16 year old presents after two syncopal episodes Reviews by Stanger et al (Am J Card 1999) and Ward & Purdie (J Paed Ch Health 2001) show significant diagnostic errors in studies performed on pediatric patients at adult echo labs. Of patients going on to surgical/cath procedures, adult lab had major errors in 44% (e.g. missed coarct, coronary aneurysms), moderate in 28% (e.g. missed/misjudged shunts), small 12% No pediatric lab interpretative or technical errors Errors tended to occur more in younger and more complex patients. Exertional chest pain prompts further testing First after standing to leave classroom at school Second while taking shower. Both with prodrome of nausea, dizziness, blurry vision Physical exam normal except for mild postural tachycardia, +35 bpm when standing Like CP, syncope very common in children & teens Like CP, elicits concern/anxiety prompting extensive workup 5/1/2015 NCS vs. Cardiac Syncope Syncope SCAMP H&P + ECG will identify vast majority of cardiac syncope History: focus on characterizing prodrome, setting, trigger, associated symptoms. Physical exam including orthostatics, dynamic auscultation (r/o LVOTO), ECG r/o AV block, LQTS, WPW, CMO ,sev RVH, Brugada, etc. Neurocardiogenic syncope >80% of ped syncope Typical settings: prolonged standing, warm environment, rising suddenly. Prominent prodrome (nausea, dizziness, tunnel vision) Pallor, brief LOC with minimal residual symptoms Cardiac syncope Acute collapse, often w/ exertion Limited prodrome Friedman & Alexander, J Peds, 2013 Syncope Ritter et al (Pediatrics 2000) – 480 syncopal kids, echo did not contribute to diagnosis in any patients, H&P, ECG identified 21 of 22 w/ cardiac etiologies Saarel et al (Pediatrics 2004) – 495 patients w/ Holter, useful only if had palpitations. No yield for isolated syncope, pre-syncope or CP. Tilt-table has limited specificity (40%), poor sensitivity (65-75%) and poor reproducibility (50-87%) Reassurance and education are key to prevent recurrences. Summary CXR still useful for evaluation of CHF symptoms Angio/cath supplanted by CTA or MRA for anatomic evaluation but still useful in guiding interventional therapies, hemodynamic cath CTA especially useful for small-vessel anatomy, e.g. coronaries, distal PA’s, aortic aneurysm, esp. in ER Nuclear scans – limited utility in pediatrics MRI – high resolution, sophisticated quantification and myocardial viability assessment but limited by need for anesthesia. Echo remains cornerstone of field 5/1/2015 Mutton’s Law Know What to Do and When to Do It “They also serve who stand and wait” – John Milton “Because that’s where the money is” – Willie Sutton References: • • • • Lots of tools available to image the heart. AUC and SCAMPs help guide rational use of limited resources and will ultimately improve patient care • • • • • • Thank You! S Anwar & RW Kavey, “Pediatric Chest Pain: Findings on Exercise Stress Testing”, Clinical Pediatrics, 51(7):659-62; 2012 RM Campbell et al, “2014 Appropriate Use Criteria for Initial Transthoracic Echocardiography in Outpatient Pediatric Cardiology: A Report of the American College of Cardiology Appropriate Use Criteria Task Force, American Academy of Pediatrics, American Heart Association, American Society of Echocardiography, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Pediatric Echocardiography”, J Am Coll Cardiol, 64(19):2039-60; Nov 11, 2014 MMP Driessen et al, “Advances in cardiac magnetic resonance imaging of congenital heart disease”, Pediatr Radiol, 45:5-19; 2015 KG Friedman et al, “Management of Pediatric Chest Pain Using a Standardized Assessment and Management Plan”, Pediatrics 128(2):239-45; Aug 2011 KG Friedman & ME Alexander, “Chest Pain and Syncope in Children: A Practical Approach to the Diagnosis of Cardiac Disease”, Journal of Pediatrics, 163(3):896-901; Sept 2013 DA Kane et al, “Needles in the Hay: Chest Pain as the Presenting Symptom in Children with Serious Underlying Cardiac Pathology”, Congenit Heart Dis, 5:366-73; 2010. FG Meinel et al, “ECG-synchronized CT Angiography in 324 Consecutive Pediatric Patients: Spectrum of Indications and Trends in Radiation Dose”, Pediatr Cardiol, 36:569-78, 2015 Saleeb SF et al, “Effectiveness of screening for life-threatening chest pain in children”, Pediatrics 128:e1062-8; Nov 2011 P Stanger et al, “Diagnostic Accuracy of Pediatric Echocardiograms Performed in Adult Laboratories”, Am J Cardiol, 83:90814; 1999 JT Tertter & RW Kavey, “Distinguishing Cardiac Syncope from Vasovagal Syncope in a Referral Population”, Journal of Pediatrics, 163(6):1618-1623; Dec 2013