PEDCD-1.1: Pediatric Cardiac Imaging Age Considerations

For this condition imaging is medically necessary based on the following criteria:

† Heart disease in the pediatric population involves predominantly congenital lesions. Pediatric members can have acquired heart disease unique to children. For those diseases which occur in both pediatric and adult populations, differences exist in management due to member age, comorbidities, and differences in disease natural history between children and adults.

† Individuals who are<18 years old should be imaged according to the Pediatric Cardiac Imaging Guidelines, and individuals who are age ≥18 years should be imaged according to the Cardiac Imaging Guidelines, except where directed otherwise by a specific guideline section.

PEDCD-1.2: Pediatric Cardiac Imaging Appropriate Clinical Evaluation

For this condition imaging is medically necessary based on the following criteria:

† A recent (within 60 days) face-to-face evaluation should be performed prior to considering advanced imaging unless the member is undergoing guideline-supported scheduled follow-up imaging evaluation. This evaluation should include:

® A detailed history
® Physical examination
® Appropriate laboratory studies

† Unless otherwise stated in a specific guideline section, the use of advanced imaging to screen asymptomatic members for disorders involving the heart is not supported.

† Members starting ADHD medications, in the absence of other appropriate indications listed in these guidelines, imaging is not indicated.

† Asymptomatic Members with known or suspected syndromes, which may be associated with congenital heart disease, can have an initial echocardiogram.

† Asymptomatic members with family history of aortopathy, cardiomyopathy, congenital heart disease with known inheritance pattern, can have an echocardiogram as an initial study. Additional studies are determined based on findings.

† Asymptomatic members with exposure to cardiotoxic drugs can have serial echocardiograms as per Pediatric Oncology Imaging Policy (Policy #166 in the Radiology Section); PEDONC-19.2: Cardiotoxicity and Echocardiography

† Advanced imaging of the heart should only be approved in members who have documented active clinical signs or symptoms of disease involving the heart or as follow-up for findings on echocardiograms.

† Unless otherwise stated in a specific guideline section, repeat imaging studies of the heart are not necessary unless:

® There is evidence for progression of disease
® New onset of disease and/or documentation of how repeat imaging will affect member management or treatment decisions.

For this condition imaging is medically necessary based on the following criteria:

† MRI

® MRI and MRA studies are frequently indicated for evaluation of congenital heart defects not well visualized on echocardiography, thoracic arteries and veins not visualized on echocardiography, cardiomyopathies, and right ventricular disease, as well as in follow-up for these indications.
® Due to the length of time for image acquisition and the need for the member to be motionless during the acquisition, anesthesia is required for almost all infants and young children (age <7 years), as well as older children with delays in development or maturity. In this member population, MRI imaging sessions should be planned with a goal of avoiding a short-interval repeat anesthesia exposure due to insufficient information using the following considerations:
¡ MRI is typically performed without and with contrast.
¡ If multiple body areas are supported by Horizon BCBSNJ guidelines for the clinical condition being evaluated, MRI of all necessary body areas should be obtained concurrently in the same anesthesia session.

® CT is primarily used to evaluate the coronary and great vessels in congenital heart disease if cardiac MR is contraindicated.
® Coding considerations are listed in PEDCD-10: CT Heart and Coronary Computed Tomography Angiography (CCTA)–Other Indications)

® Echocardiography is the primary modality used to evaluate the anatomy and function of the pediatric heart, and is generally indicated before considering other imaging modalities.
® Coding considerations are listed in PEDCD-8: Echocardiography Other Indications.

SPECT, PET stress may be indicated for members with anomalous CA, angina chest pain, and follow-up for Kawasaki. See specific sections for those indications.
® Multi Gated Acquisition (MUGA) studies (CPT^®78472, CPT^®78473, CPT^®78481, CPT^®78483, CPT^®78494, or CPT^®78496) are rarely performed in pediatrics, but can be approved for the following:
¡ Certain pediatric oncology members when echocardiography is insufficient: See: Pediatric Oncology Imaging Policy (Policy #166 in the Radiology Section); PEDONC-1.2: Appropriate Clinical Evaluations for imaging guidelines.
¡ Quantitation of left ventricular function when recent echocardiogram shows ejection fraction of <50% and MUGA results will impact acute member care decisions.
® SPECT/CT fusion imaging involves SPECT (MPI) imaging and CT for optimizing location, accuracy, and attenuation correction combines functional and anatomic information.
¡ There is currently no evidence-based data to formulate appropriateness criteria for SPECT/CT fusion imaging.
¡ Combined use of nuclear imaging, including SPECT, along with diagnostic CT (fused SPECT/CT) is considered investigational.
® Central C-V Hemodynamics (CPT^®78414) is not an imaging study and is an outdated examination
® Cardiac Shunt Detection (CPT^®78428) is rarely performed in pediatrics but can be approved for members in whom Cardiac MR is not diagnostic
¡ Calculation of left and right ventricular ejection fractions
¡ Assessment of wall motion
¡ Quantitation of right to left shunts
® Myocardial Tc-99m Pyrophosphate Imaging
¡ Infarct Avid Myocardial Imaging studies (CPT^® 78466, CPT^® 78468, and CPT^® 78469), historically this method of imaging the myocardium , Myocardial Tc-99m Pyrophosphate Imaging , was used to identify recent infarction, hence, the term "infarct-avid scan.” Although still available, the sensitivity and specificity for identifying infarcted myocardial tissue is variable and the current use for this indication is limited
¡ CPT^® 78466, CPT^® 78468, and CPT^® 78469, CPT^® 78800 or CPT^® 78803 may be used, for identification of myocardial ATTR (transthyretin) amyloidosis. Refer to Adult Cardiac Imaging Policy (Policy #149 in the Radiology Section); CD-3.7: Myocardial Tc-99m Pyrophosphate Imaging and Adult Cardiac Imaging Policy (Policy #149 in the Radiology Section); CD-3.8: Cardiac Amyloidosis

MUGA (Multi Gated Acquisition) – Blood Pool Imaging	CPT®
Myocardial Imaging, infarct avid, planar, qualitative or quantitative	78466
Myocardial Imaging, infarct avid, planar, qualitative or quantitative with ejection fraction by first pass technique	78468
Myocardial Imaging, infarct avid, planar, qualitative or quantitative tomographic SPECT with or without quantification	78469
Radiopharmaceutical Localization Imaging Limited area	78800
Radiopharmaceutical Localization Imaging SPECT Note: When reporting CPT® 78803, planar imaging of a limited area or multiple areas should be included with the SPECT	78803

References
1. Karmazyn BK, John SD, Siegel MJ, et al. ACR–ASER–SCBT-MR–SPR Practice parameter for the performance of pediatric computed tomography (CT).Am Coll Radiology (ACR). Revised 2014 (Resolution 3).https://www.acr.org/-/media/ACR/Files/Practice-Parameters/ct-ped.pdf?la=en.
2. Bridges MD, Berland LL, Kirby AB, et al. ACR Practice Parameter for performing and interpretingmagnetic resonance imaging (MRI). Am Coll Radiology (ACR). Revised 2017 (Resolution 10).https://www.acr.org/-/media/ACR/Files/Practice-Parameters/mr-perf-interpret.pdf?la=en.
3. Sorantin E and Heinzl B. What every radiologist should know about paediatric echocardiography.Eur J Radiol.2014 Sep;83(9):1519-1528.
4. Ing C, DiMaggio C, Whitehouse A, et al. Long-term differences in language and cognitive function after childhoodexposure to anesthesia.Pediatrics. 2012 Aug;130(3):e476-e485.
5. Monteleone M, Khandji A, Cappell J, et al. Anesthesia in children: perspectives from nonsurgical pediatric specialists. J Neurosurg Anesthesiol, 2014 Oct 01;26(4):396-398.
6. DiMaggio C, Sun LS, and Li G. Early childhood exposure to anesthesia and risk of developmental and behavioral disorders in a sibling birth cohort.Anesth Analg, 2011 Nov;113(5):1143-1151.
7. Moss AJ, Adams FH, Allen HD, et al. Moss and Adams' Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult. Philadelphia: Wolters Kluwer; 2016
8. Lai WW, Geva T, Shirali GS, et al. Guidelines and Standards for Performance of a Pediatric Echocardiogram: A Report from the Task Force of the Pediatric Council of the American Society of Echocardiography. Journal of the American Society of Echocardiography. 2006;19(12):1413-1430. doi:10.1016/j.echo.2006.09.001.
9. Prakash A, Powell AJ, Geva T. Multimodality Noninvasive Imaging for Assessment of Congenital Heart Disease. Circulation: Cardiovascular Imaging. 2010;3(1):112-125. doi:10.1161/CIRCIMAGING.109.875021.
10. Doherty JU, Kort S, Mehran R, Schoenhagen P, et al. ACC/AATS/AHA/AYSE/ASNC/HRS/SCAI/SCCT/SCMR/STS 2017 Appropriate Use Criteria for Multimodality Imaging in Valvular Heart Disease. Journal of Nuclear Cardiology. 2017;24(6):2043-2063.
11. Chowdhury D, Gurvitz M, Anderson J, et al. Development of Quality Metrics in Ambulatory Pediatric Cardiology. JACC: J Am Coll Cardiol. 2017 Feb, 69 (5) 541-555.doi: 10.1016/j.jacc.2016.11.043.
12. Valente AM, Cook S, Festa P, et al. Multimodality Imaging Guidelines for Patients with Repaired Tetralogy of Fallot: A Report from the American Society of Echocardiography. Journal of the American Society of Echocardiography. 2014;27(2):111-141. doi:10.1016/j.echo.2013.11.009.
13. Simpson J, Lopez L, Acar P, et al. Three-dimensional Echocardiography in Congenital Heart Disease: An Expert Consensus Document from the European Association of Cardiovascular Imaging and the American Society of Echocardiography. Journal of the American Society of Echocardiography. 2017;30(1):1-27. doi:10.1016/j.echo.2016.08.022.
14. Cohen MS, Eidem BW, Cetta F, et al. Multimodality Imaging Guidelines of Patients with Transposition of the Great Arteries: A Report from the American Society of Echocardiography Developed in Collaboration with the Society for Cardiovascular Magnetic Resonance and the Society of Cardiovascular Computed Tomography. Journal of the American Society of Echocardiography. 2016;29(7):571-621. doi:10.1016/j.echo.2016.04.002.
15. Silvestry FE, Cohen MS, Armsby LB, et al. Guidelines for the Echocardiographic Assessment of Atrial Septal Defect and Patent Foramen Ovale: From the American Society of Echocardiography and Society for Cardiac Angiography and Interventions. Journal of the American Society of Echocardiography. 2015;28(8):910-958. doi:10.1016/j.echo.2015.05.015.
16. Paridon SM. Clinical Stress Testing in the Pediatric Age Group: A Statement From the American Heart Association Council on Cardiovascular Disease in the Young, Committee on Atherosclerosis, Hypertension, and Obesity in Youth. Circulation. 2006;113(15):1905-1920. doi:10.1161/CIRCULATIONAHA.106.17437D.
17. Gidding SS, Champagne MA, Ferranti SDD, et al. The Agenda for Familial Hypercholesterolemia. Circulation. 2015;132(22):2167-2192. doi:10.1161/CIR.0000000000000297.
18. National Cancer Institute. Radiation Risks and Pediatric Computed Tomography. National Cancer Institute. https://www.cancer.gov/about-cancer/causes-prevention/risk/radiation/pediatric-ct-scans. Published September 4, 2018.
19. Hundley WG, Bluemke DA, Finn JP, et al. ACCF/ACR/AHA/NASCI/SCMR 2010 Expert Consensus Document on Cardiovascular Magnetic Resonance. Circulation. 2010;121(22):2462-2508. doi:10.1161/cir.0b013e3181d44a8f.
20. Kane DA. Suspected heart disease in infants and children: Criteria for referral. UpToDate. https://www.uptodate.com/contents/suspected-heart-disease-in-infants-and-children-criteria-for-referral. Published April 3, 2019.

PEDCD-2: Congenital Heart Disease

PEDCD-2.1: Congenital Heart Disease General Considerations

PEDCD-2.2: Congenital Heart Disease Echocardiography Coding

PEDCD-2.3: Congenital Heart Disease Modality Considerations

PEDCD-2.4: Congenital Heart Disease Timing Considerations

PEDCD-2.1: Congenital Heart Disease General Considerations

For this condition imaging is medically necessary based on the following criteria:

† Congenital heart disease accounts for the majority of cardiac problems occurring in the pediatric population. Members may be diagnosed any time spanning prenatal evaluation to adolescence. For members over 18 year of age, see Adult Congenital Guidelines.

† There are a number of variables that influence the modality and timing of imaging members with congenital heart disease, which results in a high degree of individuality in determining the schedule for imaging these members, including:

® Gestational age
® Member age
® Physiologic effects of the defect
® Status of interventions (catheterization and surgical)
® Rate of member growth
® Stability of the defect on serial imaging
® Comorbid conditions
® Activity level

For this condition imaging is medically necessary based on the following criteria:

† Any of the following echocardiography code combinations are appropriate for re-evaluation of members with known congenital heart disease:

® CPT^®93303, CPT^®93320, and CPT^®93325
® CPT^®93304, CPT^®93321, and CPT^®93325
® CPT^®93303
® CPT^®93304

† All requested CPT^® combinations other than those listed in this section should be forwarded for Medical Director Review.

PEDCD-2.3: Congenital Heart Disease Modality Considerations

For this condition imaging is medically necessary based on the following criteria:

† Echocardiography is the primary imaging modality used for diagnosing and monitoring congenital heart disease and is generally required before other imaging modalities are indicated unless otherwise indicated in a specific guideline section.

† Cardiac MRI either without contrast (CPT^®75557) or without and with contrast (CPT^®75561) is indicated, when a recent echocardiogram is inconclusive, needs confirmation of findings, or does not completely define the disease (for subsequent follow-up studies, a recent echocardiogram is not a requirement):

® CPT^®75565 is also indicated for members with valvular disease or a need to evaluate intracardiac blood flow. These members will usually have CPT^®93320 and CPT^®93325 performed with their echocardiography studies.
® MRA Chest (CPT^®71555) may be added if the aorta or pulmonary artery needs to be visualized beyond the root, or if aortopulmonary collaterals, pulmonary veins, or systemic veins need to be visualized.
¡ MRA Chest alone (CPT^®71555) should be performed if the member cannot cooperate with full cardiac MRI exam.

® Coarctation of the aorta, tetralogy of Fallot, anomalous pulmonary veins, Transposition of the great arteries, Truncus arteriosus, vascular rings, and other lesions of the great arteries, with inconclusive recent echocardiography findings

® Report CPT^®75574 for evaluating coronary artery anomalies
® Report CPT^®75573 for congenital heart disease
® CPT 71275 Determination of vascular extra-cardiac anatomy in members with complex congenital heart disease
® Pulmonary artery (PA) and Pulmonary vein (PV) assessment
® CTA of the chest is indicated to assess, Coarctation of the aorta, tetralogy of Fallot, anomalous pulmonary veins, and other lesions of the great arteries, vascular rings, with inconclusive recent echocardiography findings

For this condition imaging is medically necessary based on the following criteria:

† Echocardiography is repeated frequently throughout a child’s life, and can generally be approved regardless of symptoms according to the following schedule, with some modifications listed below:

® Members 0-2 months:
¡ Can have one repeat echocardiogram if prior echocardiogram is abnormal (either in hospital or as newborn outpatient)
® Member’s age 0 to 2 years:
¡ every 3 months
¡ Members with single ventricle physiology (e.g., Hypoplastic left heart syndrome [HLHS], Mitral atresia, Unbalanced atrioventricular septal defect [uAVSD]) may require echocardiograms very frequently and can be approved:
§ Birth to 6 months of life: every 2 weeks
§ 7-12 months of life: 1 per month
§ Then every 3 months until 2 years of age
¡ Members with unrepaired asymptomatic isolated secundum atrial septal defect (ASD) without syndromes (such as Down Syndrome) or evidence of pulmonary hypertension:
§ Every 3 months until they are 1 year
§ Then once a year, unless consideration for surgery
® Member’s age 3 to 12 years:
¡ Non-ASD members: every 6 months
¡ Members with unrepaired asymptomatic isolated secundum atrial septal defect (ASD), without syndromes (such as Down Syndrome) or evidence of pulmonary hypertension:
§ Follow the above schedule until they are 1 year
§ Then they can have echocardiogram once a year, unless consideration for surgery.
® Member’s age 13 years and older: every 12 months
® Modifiers to the above schedule:
¡ Some congenital conditions may require more frequent testing, especially with more complex heart disease, congestive heart failure, obstructive heart lesions, ductal dependent lesions, changes in clinical status, repeat interventions, and/or in neonates
¡ Any member being treated for heart failure, with consideration for changing medical regimen can have an echocardiogram

References
1. Kliegman R, Lye PS, Bordini BJ, et al. Nelson Pediatric Symptom-Based Diagnosis. Philadelphia, PA: Elsevier; 2018.
2. Riveros R and Riveros-Perez E. Perioperative considerations for children with right ventricular dysfunction. Seminars in Cardiothoracic and Vascular Anesthesia. 2015 Jul 10;19(3):187–202.
3. Lai WW, Geva T, Shirali GS, et al. Guidelines and Standards for Performance of a Pediatric Echocardiogram: A Report from the Task Force of the Pediatric Council of the American Society of Echocardiography. Journal of the American Society of Echocardiography. 2006;19(12):1413-1430. doi.:10.1016/j.echo.2006.09.001.
4. Prakash A, Powell AJ, Geva T. Multimodality Noninvasive Imaging for Assessment of Congenital Heart Disease. Circulation: Cardiovascular Imaging. 2010;3(1):112-125.doi:10.1161/CIRCIMAGING.109.875021.
5. Simpson J, Lopez L, Acar P, et al. Three-dimensional Echocardiography in Congenital Heart Disease: An Expert Consensus Document from the European Association of Cardiovascular Imaging and the American Society of Echocardiography. Journal of the American Society of Echocardiography. 2017;30(1):1-27. doi:10.1016/j.echo.2016.08.022
6. Truong UT, Kutty S, Broberg CS, Sahn DJ. Multimodality Imaging in Congenital Heart Disease: an Update. Current Cardiovascular Imaging Reports. 2012;5(6):481-490. https://doi.org/10.1007/s12410-012-9160-6.
7. Wernovsky G, Rome JJ, Tabbutt S, et al. Guidelines for the Outpatient Management of Complex Congenital Heart Disease. Congenital Heart Disease. 2006;1(1-2):10-26. doi:10.1111/j.1747-0803.2006.00002.x.
8. Altman CA. Identifying newborns with critical congenital heart disease. UpToDate. https://www.uptodate.com/contents/identifying-newborns-with-critical-congenital-heart-disease/print. Published June 14, 2018.
9. Valente AM, Cook S, Festa P, et al. Multimodality Imaging Guidelines for Patients with Repaired Tetralogy of Fallot: A Report from the American Society of Echocardiography. Journal of the American Society of Echocardiography. 2014;27(2):111-141. doi:10.1016/j.echo.2013.11.009.
10. Margossian R, Schwartz ML, Prakash A, et al. Comparison of Echocardiographic and Cardiac Magnetic Resonance Imaging Measurements of Functional Single Ventricular Volumes, Mass, and Ejection Fraction (from the Pediatric Heart Network Fontan Cross-Sectional Study)††A list of participating institutions and investigators appears in the Appendix. The American Journal of Cardiology. 2009;104(3):419-428. doi:10.1016/j.amjcard.2009.03.058.
11. Hundley WG, Bluemke DA, Finn JP, et al. ACCF/ACR/AHA/NASCI/SCMR 2010 Expert Consensus Document on Cardiovascular Magnetic Resonance. Circulation. 2010;121(22):2462-2508. doi:10.1161/cir.0b013e3181d44a8f.
12. Silvestry FE, Cohen MS, Armsby LB, et al. Guidelines for the Echocardiographic Assessment of Atrial Septal Defect and Patent Foramen Ovale: From the American Society of Echocardiography and Society for Cardiac Angiography and Interventions. Journal of the American Society of Echocardiography. 2015;28(8):910-958. doi:10.1016/j.echo.2015.05.015.
13. Franklin RCG, Béland MJ, Colan SD, et al. Nomenclature for congenital and paediatric cardiac disease: the International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Iteration of the International Classification of Diseases (ICD-11). Cardiology in the Young. 2017;27(10):1872-1938. doi:10.1017/s1047951117002244.
14. Cohen MS, Eidem BW, Cetta F, et al. Multimodality Imaging Guidelines of Patients with Transposition of the Great Arteries: A Report from the American Society of Echocardiography Developed in Collaboration with the Society for Cardiovascular Magnetic Resonance and the Society of Cardiovascular Computed Tomography. Journal of the American Society of Echocardiography. 2016;29(7):571-621. doi:10.1016/j.echo.2016.04.002.
15. Canobbio MM, Warnes CA, Aboulhosn J, et al. Management of Pregnancy in Patients With Complex Congenital Heart Disease: A Scientific Statement for Healthcare Professionals From the American Heart Association. Circulation. 2017;135(8). doi:10.1161/cir.0000000000000458.
16. Hare GFV, Ackerman MJ, Evangelista J-AK, et al. Eligibility and Disqualification Recommendations for Competitive Athletes With Cardiovascular Abnormalities: Task Force 4: Congenital Heart Disease. Journal of the American College of Cardiology. 2015;66(21):2372-2384. doi:10.1016/j.jacc.2015.09.036.
17. Giglia T, Stagg A. Infant Single Ventricle Monitoring Program (ISVMP): Outpatient Interstage Pathway: Stage I Discharge to Second Operation. Clinical Pathways Program. https://www.chop.edu/clinical-pathway/single-ventricle-fetus-or-newborn-clinical-pathway. Published July 2011. Revised January 2018

PEDCD-3: Heart Murmur

PEDCD-3.1: Heart Murmur General

PEDCD-3.1: Heart Murmur General

For this condition imaging is medically necessary based on the following criteria:

† Heart murmurs are extremely common in pediatric members. The thinner chest wall in children allows clearer auscultation of blood flowing through the chambers of the heart, which may result in a murmur on physical exam.

† The majority of murmurs are innocent and do not require further evaluation. More than 30% of children may have an innocent murmur detected during physical examination. Innocent murmurs are typically systolic ejection murmurs with a vibratory or musical quality, and generally change in quality when the member changes position.

† Other types of murmurs can be pathologic and require additional evaluation, usually by a pediatric cardiologist. Echocardiography is indicated, and is performed as part of the office visit. When evaluating a member with a murmur for the first time, it will not be known whether the member has congenital heart disease or not. The cardiologist only submits charges for the procedure actually performed.

† The following echocardiography code combinations should be approved for evaluation of any pathologic murmur or any innocent murmur with associated cardiac signs or symptoms:

® CPT^®93303, CPT^®93306, CPT^®93320, and CPT^®93325
® CPT^®93303, CPT^®93306
® CPT^® 93306, CPT^®93320 and CPT^®93325 are included with CPT^®93306 and should not be approved separately.

References
1. Nelson Textbook of Pediatrics, 20th Edition, Robert M. Kliegman, MD, Bonita M.D. Stanton, MD, Joseph St. Geme, MD and Nina F Schor, MD, PhD, p2182 to p2292.
2. Campbell RM, Douglas PS, Eidem BW, Lai WW, Lopez L, Sachdeva R. ACC/AAP/AHA/ASE/HRS/SCAI/SCCT/SCMR/SOPE 2014 Appropriate Use Criteria for Initial Transthoracic Echocardiography in Outpatient Pediatric Cardiology. Journal of the American College of Cardiology. 2014;64(19):2039-2060. doi:10.1016/j.jacc.2014.08.003.
3. Lai WW, Geva T, Shirali GS, et al. Guidelines and Standards for Performance of a Pediatric Echocardiogram: A Report from the Task Force of the Pediatric Council of the American Society of Echocardiography. Journal of the American Society of Echocardiography. 2006;19(12):1413-1430. doi:10.1016/j.echo.2006.09.001.
4. Allen HD, Shaddy RE, Penny DJ, Cetta F, Feltes TF. Moss and Adams' Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult. 9th ed. Philadelphia, PA: Wolters Kluwer; 2016.
5. Prakash A, Powell AJ, Geva T. Multimodality Noninvasive Imaging for Assessment of Congenital Heart Disease. Circulation: Cardiovascular Imaging. 2010;3(1):112-125. doi:10.1161/circimaging.109.875021.
6. Geggel RL. Approach to the infant or child with a cardiac murmur. UpToDate. https://www.uptodate.com/contents/approach-to-the-infant-or-child-with-a-cardiac-murmur. Published July 25, 2019.
7. Geggel RL. Overview of common causes of cardiac murmurs in infants and children. UpToDate. https://www.uptodate.com/contents/overview-of-common-causes-of-cardiac-murmurs-in-infants-and-children Published June 1, 2017.

PEDCD-4: Chest Pain

PEDCD-4.1: Chest Pain General

PEDCD-4.1: Chest Pain General

For this condition imaging is medically necessary based on the following criteria:

Chest pain in pediatric members is caused by a cardiac etiology in <5% of cases, yet causes great anxiety for parents resulting in requests for testing.

† A recent (within 60 days) face-to-face evaluation including a detailed history, physical examination, EKG, and appropriate laboratory studies should be performed prior to considering advanced imaging.

† Echocardiography is indicated for pediatric members with chest pain and one or more of the following:

® Exertional chest pain
® Non-exertional chest pain with abnormal EKG
® Chest pain with signs or symptoms of pericarditis
® First-degree relative with sudden unexplained death or cardiomyopathy
® Recent onset of fever
® Recent illicit drug use
® Other signs or symptoms of cardiovascular disease

† The following echocardiography code combinations should be approved for evaluation of chest pain:

® CPT^®93303, CPT^®93306, CPT^®93320, and CPT^®93325
® CPT^®93303, CPT^®93306
® CPT^®93306
® CPT^®93320 and CPT^®93325 are included with CPT^®93306 and should not be approved separately.

® Increased severity or change in quality of the chest pain
® New signs or symptoms of cardiovascular disease other than pain
® New abnormality on EKG

References
1. Kliegman R, Nelson WE. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016.
2. Friedman KG, Alexander ME. Chest Pain and Syncope in Children: A Practical Approach to the Diagnosis of Cardiac Disease. The Journal of Pediatrics. 2013;163(3). doi:10.1016/j.jpeds.2013.05.001.
3. Campbell RM, Douglas PS, Eidem BW, Lai WW, Lopez L, Sachdeva R. ACC/AAP/AHA/ASE/HRS/SCAI/SCCT/SCMR/SOPE 2014 Appropriate Use Criteria for Initial Transthoracic Echocardiography in Outpatient Pediatric Cardiology. Journal of the American College of Cardiology. 2014;64(19):2039-2060. doi:10.1016/j.jacc.2014.08.003.
4. Allen HD, Shaddy RE, Penny DJ, Cetta F, Feltes TF. Moss and Adams' Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult. 9th ed. Philadelphia, PA: Wolters Kluwer; 2016
5. Geggel RL. Nontraumatic chest pain in children and adolescents: Approach and initial management. UpToDate. https://www.uptodate.com/contents/nontraumatic-chest-pain-in-children-and-adolescents-approach-and-initial-management. Published March 8, 2018.

PEDCD-5: Syncope

PEDCD-5.1: Syncope

PEDCD-5.1: Syncope

For this condition imaging is medically necessary based on the following criteria:

Syncopein pediatric members is common, with up to 15% of members experiencing at least one episode by age 21. Syncope is caused by neurocardiogenic syndrome (vasovagal syncope) in 75 to 80% of cases, which is a benign and self-limiting condition. Despite this, syncope causes great anxiety for parents resulting in requests for testing.

† A recent (within 60 days) face-to-face evaluation including a detailed history, physical examination, EKG, and appropriate laboratory studies should be performed prior to considering advanced imaging.

† Echocardiography is not indicated for most members with isolated syncope.

† Echocardiography is indicated for pediatric members with syncope and one or more of the following:

® Exertional syncope
® Unexplained post-exertional syncope
® Abnormal EKG
® First-degree relative with any of the following before age 50:
¡ Sudden cardiac arrest or death
¡ Pacemaker or implantable defibrillator placement
® First-degree relative with cardiomyopathy
® Known congenital heart disease
® History of Kawasaki disease, or other coronary pathology.
® Pathologic murmur, irregular rhythm, gallop, or click on physical examination

† The following echocardiography code combinations should be approved for evaluation of syncope:

® CPT^®93303, CPT^®93306, CPT^®93320, and CPT^®93325
® CPT^®93303, CPT^®93306
® CPT^®93306
¡ CPT^®93320 and CPT^®93325 are included with CPT^®93306 and should not be approved separately.

® Increased severity or change in quality of the syncope
® New signs or symptoms of cardiovascular disease other than syncope
® New abnormality on EKG

References
1. Kliegman R, Nelson WE. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016.
2. Friedman KG, Alexander ME. Chest Pain and Syncope in Children: A Practical Approach to the Diagnosis of Cardiac Disease. The Journal of Pediatrics. 2013;163(3). doi:10.1016/j.jpeds.2013.05.001.
3. Campbell RM, Douglas PS, Eidem BW, Lai WW, Lopez L, Sachdeva R. ACC/AAP/AHA/ASE/HRS/SCAI/SCCT/SCMR/SOPE 2014 Appropriate Use Criteria for Initial Transthoracic Echocardiography in Outpatient Pediatric Cardiology. Journal of the American College of Cardiology. 2014;64(19):2039-2060. doi:10.1016/j.jacc.2014.08.003.
4. Cannon B, Wackel P. Syncope. Pediatrics in Review. 2016;37(4):159-168. doi:10.1542/pir.2014-0109.
5. Salerno JC. Causes of syncope in children and adolescents. UpToDate. https://www.uptodate.com/contents/causes-of-syncope-in-children-and-adolescents. Published November 28, 2017.
6. Allen HD, Shaddy RE, Penny DJ, Cetta F, Feltes TF. Moss and Adams' Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult. 9th ed. Philadelphia, PA: Wolters Kluwer; 2016.
7. Kane DA. Suspected heart disease in infants and children: Criteria for referral. UpToDate. https://www.uptodate.com/contents/suspected-heart-disease-in-infants-and-children-criteria-for-referral. Published April 3, 2019.


PEDCD-6: Kawasaki Disease

PEDCD-6.1: Kawasaki Disease Initial Imaging

PEDCD-6.2: Acute Phase

PEDCD-6.3: Chronic phase

PEDCD-6.1: Kawasaki Disease Initial Imaging

For this condition imaging is medically necessary based on the following criteria:

† Kawasaki disease (KD) is the leading cause of acquired pediatric cardiac disease in the developed world. It is an acute febrile illness characterized by a medium vessel vasculitis, which predominantly affects the coronary arteries.

® A recent (within 60 days) face-to-face evaluation including a detailed history, physical examination, and appropriate laboratory studies should be performed prior to considering advanced imaging.
® Scheduled indicated follow-up imaging does not require 60 day contact, if indicated based on the below follow-up schedule.
® Members who do not fulfill the diagnostic criteria for classic KD may be considered to have incomplete (atypical) KD.
® If Kawasaki disease is strongly suspected, treatment will often begin even before cardiac evaluation, since early treatment is associated with a lower risk for coronary aneurysm development.

® Coronary CTA (CPT^®75574), Cardiac MRI without contrast (CPT^®75557), Cardiac MRI without and with contrast (CPT^®75561), or MRA Chest (CPT^®71555) are indicated for evaluation of inconclusive echocardiogram findings, or significant coronary artery abnormalities.
® Screening of other body areas for aneurysms is not routinely indicated in Kawasaki disease, but MRA or CTA (contrast as requested) of the affected body area can be approved for evaluation of signs or symptoms suggesting aneurysm development.
® See acute and chronic phase for imaging

For this condition imaging is medically necessary based on the following criteria:

† The acute phase of Kawasaki disease (KD)can last up to 4-6 weeks from the onset of fever until acute systemic inflammation has resolved and coronary artery dimensions are no longer expanding

† Based on AHA recommendations, the following classifications are used in risk stratification of coronary artery abnormalities

® Z-Score classification accounts for the effects of body size and age through use of baseline coronary dimensions adjusted for body surface area. The Z score value represents the number of standard deviation above the mean. (e.g., z=0 pt. has coronary artery dimension value equal to mean, z=2 person has value 2 standard deviation above the mean, based on age, gender, BSA).
® Coronary Artery Abnormalities Risk Classification based on Z-Score:
¡ 1 - No involvement at any time point (Z score always <2)
¡ 2 - Dilation only (Z score 2 to <2.5)
¡ 3 - Small aneurysm (Z score ≥2.5 to <5)
§ 3.1 - Current or persistent
§ 3.2 - Decreased to dilation only or normal luminal dimension
¡ 4 - Medium aneurysm (Z score ≥5 to <10, and absolute dimension <8 mm)
§ 4.1 - Current or persistent
§ 4.2 - Decreased to small aneurysm
§ 4.3 - Decreased to dilation only or normal luminal dimension
¡ 5 - Large and giant aneurysm (Z score ≥10, or absolute dimension ≥8 mm)
§ 5.1 - Current or persistent
§ 5.2 - Decreased to medium aneurysm
§ 5.3 - Decreased to small aneurysm
§ 5.4 - Decreased to dilation only or normal luminal dimension⁴
® Additional Clinical Features That May Increase the Long-Term Risk of Myocardial Ischemia
¡ Greater length and distal location of aneurysms that increase the risk of flow stasis
¡ Greater total number of aneurysms
¡ Greater number of branches affected
¡ Presence of luminal irregularities
¡ Abnormal characterization of the vessel wall (calcification, luminal myofibroblastic proliferation)
¡ Presence of functional abnormalities (impaired vasodilation, impaired flow reserve)
¡ Absence or poor quality of collateral vessels
¡ Previous revascularization performed
¡ Previous coronary artery thrombosis
¡ Previous myocardial infarction
¡ Presence of ventricular dysfunction

† Echocardiography should be performed when the diagnosis of KD is considered,

® Uncomplicated members, echocardiography can be repeated after treatment both:
¡ Within 1 to 2 weeks
¡ Within4 to 6 weeks
® For members with important and evolving coronary artery abnormalities (Z score >2.5) detected during the acute illness, more frequent echocardiography (at least twice per week) should be performed until luminal dimensions have stopped progressing to determine the risk for and presence of thrombosis.
® Expanding large or giant aneurysms:
¡ Twice per week while dimensions are expanding rapidly
¡ Once weekly after dimension is stabilized for the first 45 days of illness
¡ Then monthly until the third month after illness onset

† Transesophageal echocardiography, invasive angiography, CMRI, and CTA can be of value in the assessment of selected members but are not routinely indicated for diagnosis and management of the acute illness.

® Invasive angiography is rarely performed during the acute illness. Transesophageal echocardiography, CTA, and CMRI can be useful for the evaluation of older children and adolescents in whom visualization of the coronary arteries with transthoracic echocardiography is inadequate and results will impact immediate management decisions.
® These requests will be forwarded to Medical Director for evaluation

† Atypical or incomplete Kawasaki. Echo is indicated when atypical KD is being considered, may require repeat echocardiograms if treatment decisions will be affected by results (e.g., treating with ivig), if new signs or symptoms (such as typical peeling) develop.

PEDCD-6.3: Chronic phase

For this condition imaging is medically necessary based on the following criteria:

† Long-term management begins at the end of the acute illness, usually at 4 to 6 weeks after fever onset. Management is based on two pieces of data:

® The dimensions of the largest Aneurysm at any point during the disease
® The dimensions of the largest current aneurysm

® Greater length and distal location of aneurysms that increase the risk of flow stasis
® Greater total number of aneurysms
® Greater number of branches affected
® Presence of luminal irregularities
® Abnormal characterization of the vessel wall (calcification, luminal myofibroblastic proliferation)
® Presence of functional abnormalities (impaired vasodilation, impaired flow reserve)
® Absence or poor quality of collateral vessels
® Previous revascularization performed
® Previous coronary artery thrombosis
® Previous myocardial infarction
® Presence of ventricular dysfunction
® Long term routine surveillance in asymptomatic imaging for Kawasaki disease-see chart

® Echocardiogram can be performed at anytime with new or progressing cardiac symptoms
® Stress imaging when there are new or progressing symptoms of ischemia or ventricular dysfunction
® Invasive or coronary imaging Coronary angiography (CT, MRI, invasive) when the above studies are Positive, inconclusive, or otherwise lead to a conclusion that intervention is needed

References
1. Kliegman R, Nelson WE. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016.
2. Son MBF, Newburger JW. Kawasaki Disease. Pediatrics in Review. 2013;34(4):151-162. doi:10.1542/pir.34-4-151.
3. Campbell RM, Douglas PS, Eidem BW, Lai WW, Lopez L, Sachdeva R. ACC/AAP/AHA/ASE/HRS/SCAI/SCCT/SCMR/SOPE 2014 Appropriate Use Criteria for Initial Transthoracic Echocardiography in Outpatient Pediatric Cardiology. Journal of the American College of Cardiology. 2014; 64(19):2039-2060. doi:10.1016/j.jacc.2014.08.003.
4. Mccrindle BW, Rowley AH, Newburger JW, et al. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals from the American Heart Association. Circulation. 2017; 135(17). doi:10.1161/cir.0000000000000484.
5. Sundel R. Kawasaki disease: Clinical features and diagnosis. UpToDate. https://www.uptodate.com/contents/kawasaki-disease-clinical-features-and-diagnosis. Published November 13, 2018.
6. Mccrindle BW, Rowley AH, Newburger JW, et al. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals from the American Heart Association. Circulation. 2017;135(17). doi:10.1161/cir.0000000000000484.

PEDCD-7: Pediatric Pulmonary Hypertension

PEDCD-7: Pediatric Pulmonary Hypertension General

PEDCD-7.0: Pediatric Pulmonary Hypertension General

For this condition imaging is medically necessary based on the following criteria:

† Pulmonary hypertension in children can be caused by cardiac, pulmonary, or systemic diseases, and idiopathic disease occurs as well.

† A recent (within 60 days) face-to-face evaluation including a detailed history, physical examination, and appropriate laboratory studies should be performed prior to considering advanced imaging.

† If pulmonary hypertension is suspected, initial evaluation should consist of chest x-ray, EKG, and echocardiography (CPT^®93306, or CPT^®93303, with CPT^®93320, and CPT^®93325, see: PEDCD-8.1: Transthoracic Echocardiography (TTE) Coding for echocardiography coding considerations).

† Repeat echocardiography intervals are variable depending on age of member, etiology, and severity.

® After a comprehensive initial evaluation, echocardiograms using PH-specific protocols may be performed every 4 to 6 months.
® Echocardiography is indicated at any time for new or worsening symptoms or to evaluate a recent change in therapy.
® Right heart and /or left heart catheterization may be utilized for PAH members, including before and after initiation of PAH-targeted therapy, and for members with concomitant congenital heart disease

† Cardiac MRI without and with contrast (CPT^®75561) is indicated for evaluation of inconclusive echocardiogram findings, or for monitoring right ventricular function during follow-up.

† Stress echocardiograms may be indicated (as in adult guidelines) see Adult Cardiac Imaging Policy (Policy #149 in the Radiology Section); CD-2.7: Stress Echocardiography – Indications, other than ruling out CAD.

References
1. Abman SH, Hansmann G, Archer SL, et al. Pediatric Pulmonary Hypertension. Circulation. 2015;132(21):2037-2099. doi:10.1161/cir.0000000000000329.
2. Latus H, Kuehne T, Beerbaum P, et al. Cardiac MR and CT imaging in children with suspected or confirmed pulmonary hypertension/pulmonary hypertensive vascular disease. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK. Heart. 2016;102(Suppl 2):ii30-ii35. doi:10.1136/heartjnl-2015-308246.
3. Mullen MP, Kulik T. Pulmonary hypertension in children: Classification, evaluation, and diagnosis. UpToDate. https://www.uptodate.com/contents/pulmonary-hypertension-in-children-classification-evaluation-and-diagnosis. Published March 6, 2019.
4. Allen HD, Shaddy RE, Penny DJ, Cetta F, Feltes TF. Moss and Adams' Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult. 9th ed. Philadelphia, PA: Wolters Kluwer; 2016.
5. Galiè N, Humbert M, Vachiery J-L, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. European Respiratory Journal. 2015;46(4):903-975. doi:10.1183/13993003.01032-2015.


PEDCD-8: Echocardiography-Other Indications

PEDCD-8.1: Transthoracic Echocardiography (TTE) Coding

PEDCD-8.2: Initial Transthoracic Echocardiography (TTE) Indications

PEDCD-8.3: Repeat Transthoracic Echocardiography Indications

PEDCD-8.4: Transesophageal Echocardiography (TEE)

PEDCD-8.1: Transthoracic Echocardiography (TTE) Coding

For this condition imaging is medically necessary based on the following criteria:

† CPT^® codes for echocardiography are listed in PEDCD-1: General Guidelines

Echocardiogram coding Notes	CPT®
† The most commonly performed study is a complete transthoracic echocardiogram with spectral and color flow Doppler (CPT® 93306). ® CPT® 93306 includes CPT® 93320 and CPT® 93325, so those codes should not be approved along with CPT® 93306.	93306
† Doppler codes (CPT® 93320, CPT® 93321, and CPT® 93325) are add-on codes and are assigned in addition to code for the primary procedure, and should not be approved alone.	+93320 +93321 +93325
† For a 2D transthoracic echocardiogram without Doppler, report CPT® 93307.	93307
† A limited transthoracic echocardiogram is reported with CPT® 93308. ® Limited transthoracic echocardiogram should be billed if the report does not “evaluate or document the attempt to evaluate” all of the required structures. ® Unlike CPT® 93306, the Doppler CPT® 93321 and CPT® 93325 are not included with CPT® 93308. ® CPT® 93321 (not CPT® 93320) should be reported with CPT® 93308 if Doppler is included in the study. ® CPT® 93325 should also be reported with CPT® 93308 if color flow Doppler is included in the study.	93308
† For members with known congenital heart disease, a limited transthoracic echocardiogram is reported with CPT® 93304, +/- CPT® 93321 and CPT® 93325.	93304

† Providers performing an initial echo on a pediatric member will not know what procedure codes they will be reporting until the initial study is completed.

® If congenital heart disease is found on the initial echo, a complete echo is reported with codes CPT^®93303, CPT^®93320, and CPT^®93325 because CPT^®93303 does NOT include Doppler and color flow mapping.
® If no congenital issue is discovered, then CPT^®93306 is reported alone and includes 2-D, Doppler and color flow mapping.

® The following echocardiography code combinations should be approved for any initial echocardiogram:
¡ CPT^®93303, CPT^®93306, CPT^®93320, and CPT^®93325
¡ CPT^®93303, CPT^®93306
¡ CPT^®93306
§ CPT^®93320 and CPT^®93325 are included with CPT^®93306 and should not be approved separately.
® Depending upon individual health plan payer contracts, post-service audits may be completed to ensure proper claims submission.

For this condition imaging is medically necessary based on the following criteria:

† In addition to indications listed in previous guideline sections, initial TTE evaluation is indicated for any of the following:

® Any signs/symptoms that are possibly cardiac in nature, including (but not limited to) central cyanosis, dyspnea, edema, poor peripheral pulses, feeding difficulty, decreased urine output, hepatomegaly, or desaturation on pulse oximetry.
® Abnormal EKG or cardiac biomarkers
® Abnormal chest x-ray suggesting cardiovascular disease
® Palpitations and one of the following:
¡ Abnormal EKG
¡ First-degree relative with any of the following before age 50:
§ Sudden cardiac arrest or death
§ Pacemaker or implantable defibrillator placement
¡ First-degree relative with cardiomyopathy
® Supraventricular Tachycardia (SVT), Ventricular Tachycardia, or Premature Ventricular Contractions (PVCs)
® Known or suspected valvular dysfunction
® Persistent systemic hypertension
® Obesity (BMI >30) with additional cardiovascular risk factors
® Stroke
® Renal failure
® Preoperative evaluation of members with chest wall deformities or scoliosis
® Known or suspected vascular ring
® Planned administration of cardiotoxic chemotherapy
¡ Generally anthracyclines (doxorubicin, daunorubicin, mitoxantrone, idarubicin, epirubicin)
® Planned radiation therapy involving heart muscle or hematopoietic stem cell transplant
® Sickle cell disease or other hemoglobinopathy causing chronic anemia
® Known or suspected vasculitis, acute rheumatic fever, or other systemic autoimmune disease
® Muscular dystrophy
® Metabolic, mitochondrial, and storage disorders
® Abnormalities of cardiac or other viscera situs
® Signs, symptoms, or blood culture suggestive of endocarditis
® Known or suspected mass lesion involving the heart or great vessels
® Known or suspected clot in atrium or ventricle
® Known or suspected pulmonary hypertension
® Known or suspected pericardial effusion
® Complications during prenatal development:
¡ Known or suspected cardiovascular abnormality on fetal echocardiogram
¡ Maternal phenylketonuria (PKU)
¡ Maternal diabetes with no fetal echo
¡ Maternal teratogen exposure
¡ Maternal infection during pregnancy with potential cardiac sequelae
® Genetic abnormality known to be associated with cardiovascular disease
® First-degree relative family history of:
¡ Unexplained sudden death before age 50
¡ Hypertrophic cardiomyopathy
¡ Non-ischemic dilated cardiomyopathy
¡ Genetic abnormality known to be associated with cardiovascular disease
¡ Congenital left-sided heart lesion
¡ Heritable pulmonary arterial hypertension

For this condition imaging is medically necessary based on the following criteria:

† Repeat echocardiograms may be required for members with no new symptoms.

† In addition to indications listed in previous guideline sections, repeat TTE evaluation is indicated for any of the following:

® New or worsening symptoms in a member with known cardiac disease, previously normal echocardiogram with one of the following:
¡ New or worsening cardiac symptoms
¡ New EKG abnormality
¡ Newly recognized family history suggestive of heritable heart disease
® Every 12 months for members age 12 to 18 years with first-degree family history of hypertrophic cardiomyopathy.
® Members who are status post heart transplant can have echocardiograms repeated as often as requested by heart transplantteam.
® Every 12 months for members receiving active therapy for ventricular hypertrophy, valvular dysfunction, cardiomyopathy.
¡ One time repeat TTE can be approved at 6 months to assess response to a change in therapy.
® Every 12 months for members with chronic pericardial effusions
® Every 12 months for sickle cell disease or other hemoglobinopathy causing chronic anemia and one of the following:
¡ High risk genotype (Hgb SS or Sß⁰, severe thalassemia, etc.)
¡ History of acute chest syndrome or intrinsic lung disease
¡ History of stroke
¡ Receiving chronic transfusion therapy
® As needed for monitoring cardiotoxicity during chemotherapy administration
® After completion of chemotherapy and/or radiation therapy. See Pediatric Oncology Imaging Policy (Policy #166 in the Radiology Section); PEDONC-19.2: Cardiotoxicity and Echocardiography for imaging guidelines.

For this condition imaging is medically necessary based on the following criteria:

† Transesophageal echocardiography imaging indications in pediatric members are identical to those for adult members. See Adult Cardiac Imaging Policy (Policy #149 in the Radiology Section); CD-2.5: Transesophageal Echocardiography (TEE) – Indications.

References
1. Campbell RM, Douglas PS, Eidem BW, Lai WW, Lopez L, Sachdeva R. ACC/AAP/AHA/ASE/HRS/SCAI/SCCT/SCMR/SOPE 2014 Appropriate Use Criteria for Initial Transthoracic Echocardiography in Outpatient Pediatric Cardiology. Journal of the American College of Cardiology. 2014;64(19):2039-2060. doi:10.1016/j.jacc.2014.08.003.
2. Ambrusko SJ, Gunawardena S, Sakara A, et al. Elevation of tricuspid regurgitant jet velocity, a marker for pulmonary hypertension in children with sickle cell disease. Pediatric Blood & Cancer. 2006;47(7):907-913. doi:10.1002/pbc.20791.
3. Klings ES, Machado RF, Barst RJ, et al. An Official American Thoracic Society Clinical Practice Guideline: Diagnosis, Risk Stratification, and Management of Pulmonary Hypertension of Sickle Cell Disease. American Journal of Respiratory and Critical Care Medicine. 2014;189(6):727-740. doi:10.1164/rccm.201401-0065st.
4. Evidence-Based Management of Sickle Cell Disease: Expert Panel Report, 2014. Pediatrics. 2014;134(6). doi:10.1542/peds.2014-2986.
5. Allen HD, Shaddy RE, Penny DJ, Cetta F, Feltes TF. Moss and Adams' Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult. 9th ed. Philadelphia, PA: Wolters Kluwer; 2016.


PEDCD-9: Cardiac MRI-Other Indications

PEDCD-9.1: Cardiac MRI General Guidelines

PEDCD-9.2: Cardiac MRI Coding

PEDCD-9.3: Indications for Cardiac MRI

PEDCD-9.4: Aortic Root and Aorta

PEDCD-9.5: Evaluation of Pericardial Effusion or Diagnosis of Pericardial Tamponade

PEDCD-9.1: Cardiac MRI General Guidelines

For this condition imaging is medically necessary based on the following criteria:

† Requests for cardiac MRI that contain only one CPT^® code can be completed by the Nurse Reviewer. If the request contains more than one cardiac/chest MRI CPT^® code, it should be forwarded for Medical Director Review.

PEDCD-9.2: Cardiac MRI Coding

For this condition imaging is medically necessary based on the following criteria:

Cardiac Imaging Procedure Codes
Cardiac MRI	CPT®
Cardiac magnetic resonance imaging for morphology and function without contrast.	75557
Cardiac magnetic resonance imaging for morphology and function without and with contrast and further sequences.	75561
Cardiac magnetic resonance imaging for morphology and function without contrast; with stress imaging (rarely used in pediatrics).	75559
Cardiac magnetic resonance imaging for morphology and function without and with contrast and further sequences; with stress imaging (rarely used in pediatrics).	75563
Cardiac magnetic resonance imaging for velocity flow mapping (List separately in addition to code for primary procedure).	+75565

† Only one procedure code from the set: CPT^®75557, CPT^®75559, CPT^®75561, and CPT^®75563 should be reported per session.

† Only one flow velocity measurement (CPT^® +75565) should be reported per session.

PEDCD-9.3: Indications for Cardiac MRI

For this condition imaging is medically necessary based on the following criteria:

† In addition to indications listed in previous guideline sections, Cardiac MRI evaluation is indicated for any of the following, when a recent TTE is inconclusive:

® Assessment of global ventricular function and mass if a specific clinical question is left unanswered by recent TTE and the MRI results will affect the management of the member’s condition
® Members with complex congenital heart disease (e.g. Tetralogy of Fallot [TOF], single ventricle, truncus arteriosis, Transposition of the Great Arteries (TGA)) may require a baseline MRI, or routine cardiac MRI, especially as they approach their teenage years, due to poor imaging windows on echocardiogram, and the need for specific clinical informationnot seen on prior echocardiograms due to these known limitations, and these studies should be forwards to the medical director. Once these members reach age 18, they can be imaging by adult congenital heart disease guideline.
® Clinical suspicion of arrhythmogenic right ventricular dysplasia (ARVD) or arrhythmogenic cardiomyopathy (ARVC).
® For pericardial disease (including constrictive pericarditis, restrictive pericarditis, and perimyocarditis), MRI should not be utilized to diagnose pericarditis but only to answer the question regarding possible constriction or restriction suggested clinically or by other techniques (TTE, etc.)
¡ MRI without and with contrast (CPT^®75561) is considered the optimal test for this disorder.
® Evaluate cardiac tumor or mass
¡ MRI without and with contrast (CPT^®75561) is considered the optimal test for this disorder.
® Evaluate anomalous coronary artery
¡ After echocardiogram, MRI without and with contrast (CPT^®75561) or CCTA (CPT^®75574) is considered the optimal test for this disorder.
® For Fabry's disease, late enhancement MRI may predict the effect of enzyme replacement therapy on myocardial changes that occur with this disease.
¡ MRI without and with contrast (CPT^®75561) is considered the preferred test for this disorder.
® Cardiac MRI can be performed to evaluate members with congenital cardiomyopathy (muscular dystrophy, glycogen storage disease, fatty acid oxidation disorders, mitochondrial disorders, etc.) or unexplained cases of cardiomyopathy in order to characterize the myocardium.
® Cardiac stress perfusion study (CPT^®75559 or CPT^®75563) can be considered on a case by case basis for members with any of the following:
¡ Anomalous coronary artery
¡ Kawasaki disease
¡ TGA
¡ Ross operation
¡ or other disorder with the potential for coronary ischemia
¡ Members in whom an exercise stress test (EST)without imaging is indicated, but they cannot perform
¡ Members in whom an exercise stress test (EST) is equivocal, positive, or concern for a false negative
® Assessment of cardiac iron overload such as in hemochromatosis, thalassemia, sickle cell (either CPT^®75557 or CPT^®71550, T2* MRI, contrast not necessary).
¡ Screening imaging may be approved every 12 months
¡ Imaging may be approved every 3 months for treatment response in members receiving active treatment (chelation +/- phlebotomy)
¡ Frequently performed along with MRI Abdomen (CPT^®74181) to assess liver iron deposition. See Pediatric Abdomen Imaging Policy (Policy #160 in the Radiology Section); PEDAB-18.2: Transfusion-Associated (Secondary) Hemochromatosis for additional imaging guidelines.

For this condition imaging is medically necessary based on the following criteria:

† For screening due to family history of aortic aneurysm or dissection, see: Adult Peripheral Vascular Disease Imaging Policy (Policy #158 in the Radiology Section); PVD-2.2: Screening for Vascular related genetic connective tissue Disorders (Familial Aneurysm Syndromes/Spontaneous Coronary Artery Dissection SCAD)/Ehlers-Danlos/Marfan/Loeys-Dietz).

† For members who have both cardiac and ascending aorta abnormalities (e.g., truncus arteriosus), the following studies may be indicated following TTE:

® Cardiac MRI (CPT^®75557 or CPT^®75561) when TTE is inconclusive.
® If aorta is involved, MRI Chest (CPT^®71552) or MRA Chest (CPT^®71555) is also indicated.

® MRI Chest (CPT^®71552)
® MRA Chest (CPT^®71555)

For this condition imaging is medically necessary based on the following criteria:

† Echocardiogram is the initial imaging study of choice to evaluate pericardial effusions or diagnose pericardial tamponade.

† If a specific clinical question is left unanswered by another recent imaging study and the answer to the clinical question will affect the management of the member’s clinical condition, contrast-enhanced cardiac MRI is useful for evaluating:

® Pericarditis
® Neoplastic effusion
® Tamponade
® Myocardial infiltration.

References
1. Nelson Textbook of Pediatrics, 20th Edition, Robert M. Kliegman, MD, Bonita M.D. Stanton, MD, Joseph St. Geme, MD and Nina F Schor, MD, PhD, p2182 to p2292.
2. Atweh LA, Orth RC, Guillerman RP, Zhang W, Kan JH. MR imaging of children and young adults with classic findings of osteonecrosis on unenhanced MR images: do contrast-enhanced sequences help? Pediatric Radiology. 2013;43(11):1502-1506. doi:10.1007/s00247-013-2714-1.
3. Cohen MS, Eidem BW, Cetta F, et al. Multimodality Imaging Guidelines of Patients with Transposition of the Great Arteries: A Report from the American Society of Echocardiography Developed in Collaboration with the Society for Cardiovascular Magnetic Resonance and the Society of Cardiovascular Computed Tomography. Journal of the American Society of Echocardiography. 2016;29(7):571-621. doi:10.1016/j.echo.2016.04.002.
4. Adler Y, Charron P, Imazio M, et al. 2015 ESC Guidelines for the diagnosis and management of pericardial diseases. European Heart Journal. 2015;36(42):2921-2964. doi:10.1093/eurheartj/ehv318.
5. Allen HD, Shaddy RE, Penny DJ, Cetta F, Feltes TF. Moss and Adams' Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult. 9th ed. Philadelphia, PA: Wolters Kluwer; 2016.
6. Hundley WG, Bluemke DA, Finn JP, et al. ACCF/ACR/AHA/NASCI/SCMR 2010 Expert Consensus Document on Cardiovascular Magnetic Resonance. Circulation. 2010;121(22):2462-2508. doi:10.1161/cir.0b013e3181d44a8f.
7. Cohen MS, Eidem BW, Cetta F, et al. Multimodality Imaging Guidelines of Patients with Transposition of the Great Arteries: A Report from the American Society of Echocardiography Developed in Collaboration with the Society for Cardiovascular Magnetic Resonance and the Society of Cardiovascular Computed Tomography. Journal of the American Society of Echocardiography. 2016;29(7):571-621. doi:10.1016/j.echo.2016.04.002.
8. Valente AM, Cook S, Festa P, et al. Multimodality Imaging Guidelines for Patients with Repaired Tetralogy of Fallot: A Report from the American Society of Echocardiography. Journal of the American Society of Echocardiography. 2014;27(2):111-141. doi:10.1016/j.echo.2013.11.009.


PEDCD-10: CT Heart and Coronary Computed Tomography Angiography (CCTA)-Other Indications

PEDCD-10.1: CT Heart and Coronary Computed Tomography Angiography (CCTA) General Considerations

PEDCD-10.2: Anomalous Coronary Artery

PEDCD-10.3: Indications for CCTA (CPT® 75574)

PEDCD-10.4: Indications for Cardiac CT (CPT® 75572)

PEDCD-10.5: Radiation Dose

PEDCD-10.1: CT Heart and Coronary Computed Tomography Angiography (CCTA) General Considerations

For this condition imaging is medically necessary based on the following criteria:

† Metal artifact reduces the accuracy of CCTA. Devices that can cause this issue include, but are not limited to, surgical clips, pacemaker devices, defibrillator devices, and tissue expanders.

† Cardiac testing that does not involve exposure to ionizing radiation should be strongly considered.

Practice Note

Relative Contraindications to CCTA Include:

Very obese members (body mass index > 40 kg/m2)

Elevated calcium score: CCTA should not be performed if there is extensive coronary calcification (calcium score >1000).

Renal insufficiency

Inability to follow breath-holding instructions

PEDCD-10.2: Anomalous Coronary Artery

For this condition imaging is medically necessary based on the following criteria:

† Evaluating coronary artery anomalies and other complex congenital heart disease of cardiac chambers or great vessels is an appropriate indication for CCTA, or cardiac MRI

® Report CPT^®75574 for evaluating coronary artery anomalies
® Report CPT^®75573 for congenital heart disease
® Can add CPT^®71275 (chest CTA) to evaluate great vessels

† Members with congenital heart disease such as TOF, Truncus Arteriosus, and , TGA have increased incidence of coronary artery anomalous and may require the above imaging as well

† Members with confirmed coronary artery anomalies may require repeat imaging based on the clinical scenario

† The use of CCTA to rule out anomalous coronary artery should be limited to one of the following:

® Members who need to have an anomalous coronary artery mapped prior to an invasive procedure.
® Members who have not had a previous imaging study that clearly demonstrates an anomalous coronary artery
® Members with a history that includes one or more of the indications in PEDCD-10.3: Indications for CCTA (CPT^® 75574)

For this condition imaging is medically necessary based on the following criteria:

† In addition to indications listed in previous guideline sections, CCTA is indicated for any of the following, when a recent TTE and/or MRI is inconclusive:

® Persistent exertional chest pain and normal stress test
® Full sibling(s) with history of sudden death syndrome before age 30 or with documented anomalous coronary artery
® Resuscitated sudden death and contraindication to conventional coronary angiography
® Unexplained new onset of heart failure if CCTA will replace conventional invasive coronary angiography
® Documented ventricular tachycardia (6 beat runs or greater) if CCTA will replace conventional invasive coronary angiography
® Equivocal coronary artery anatomy on conventional cardiac catheterization
® In infants: otherwise unexplained dyspnea, tachypnea, wheezing, episodic pallor, irritability, sweating, poor feeding, and/or failure to thrive
¡ The presence of other congenital heart disease is not a separate indication for CCTA to rule out anomalous coronary artery(except when coronary artery surgery is pending, i.e. Transposition of the great arteries, Tetralogy of Fallot, Truncus arteriosis ,aortic root surgery)
® Evaluation of the arterial supply and venous drainage in children with bronchopulmonary sequestration

For this condition imaging is medically necessary based on the following criteria:

† In addition to indications listed in previous guideline sections, CCTA is indicated for any of the following, when a recent TTE and/or MRI is inconclusive:

® Cardiac or pericardial mass
® Pericarditis
® Complications of cardiac surgery or evaluation of post-operative anatomy
® Cardiac thrombus in members with technically limited TTE, TEE, or MRI
® Clinical suspicion of arrhythmogenic right ventricular dysplasia (ARVD) or arrhythmogenic cardiomyopathy (ARVC)
® Native aortic abnormalities if echocardiogram is indeterminate

For this condition imaging is medically necessary based on the following criteria:

† ACR–NASCI–SPR Practice Parameter For The Performance And Interpretation Of Cardiac Computed Tomography (CT) states “Cardiac CT should be performed only for a valid medical indication and with the minimum radiation exposure that provides diagnostic image quality”

† ACR–NASCI–SPR Practice Parameter for the Performance of Quantification of Cardiovascular Computed Tomography (Ct) And Magnetic Resonance Imaging (MRI) states“. In younger members, MRI may be the preferred modality, particularly when functional assessment with CT would require retrospective ECG gating and relatively high radiation doses. Further, the use of time-resolved MRA and phase contrast MRI methods offer significant advantages whose relative importance will depend on the specific application”

® See table: Practice Estimate of Effective Radiation Dose chart for Selected Imaging Studies in Adult Cardiac Imaging Policy (Policy #149 in the Radiology Section); CD-1: General Guidelines

PEDCD-11.1: Cardiac Catheterization General Information

For this condition imaging is medically necessary based on the following criteria:

Cardiac Catheterization Procedure Codes
Cardiac Cath Procedures	CPT®
Congenital Heart Disease Code “Set”	93530-93533
Right Heart Catheterization (CHD)	93530
Right/Left Heart Catheterization (CHD)	93531
Right/Left Heart Catheterization (CHD-TS)	93532
Right/Left Heart Catheterization (CAD-ASD)	93533
Anomalous coronary arteries, patent foramen ovale, mitral valve prolapse, and bicuspid aortic valve	93451-93464, 93566-93568
RHC without LHC or coronaries	93451
LHC without RHC or coronaries	93452
RHC and retrograde LHC without coronaries	93453
Native coronary artery catheterization;	93454
with bypass grafts	93455
with RHC	93456
with RHC and bypass grafts	93457
with LHC	93458
with LHC and bypass grafts	93459
with RHC and LHC	93460
with RHC and LHC and bypass grafts	93461
LHC by trans-septal or apical puncture	+93462
Angiography of non-coronary arteries and veins performed as a distinct service	Select appropriate codes from the Radiology and Vascular Injection Procedures sections.
CPT® 93530 to 93533 are appropriate for invasive evaluation of congenital heart disease

† These guidelines apply to individuals with stable conditions and who are not in the acute setting. Individuals in acute settings or with unstable angina should be handled as medical emergencies.

† Pediatric catheterizations are done for many purposes, including diagnosis and intervention of congenital and acquiredheart disease.

† When device placement is planned (ASD/VSD device, transcatheter valve implantation, pda device), the procedure codes for those devices include all cardiac catheterization(s), intraprocedural contrast injection(s), fluoroscopic radiological supervision and interpretation, and imaging guidance performed to complete the procedure. Forcoarctation or aortic arch stenting, or other endovascular procedures with no intracardiac issues that require clarification by left heart cath, a left heart cath is not required along with these endovascular procedures. A right heart cath can be approved for pulmonary artery interventions (e.g., stents, coils)

PEDCD-11.2: Cardiac Catheterization Indications

For this condition imaging is medically necessary based on the following criteria:

† Diagnostic catheterization is indicated:

® When other advanced imaging has failed to resolve a clinical issue and results will impact member management
® For preoperative assessment in complex heart disease
¡ Norwood procedure
¡ Bidirectional Glenn shunt
¡ Fontan procedure
¡ Pulmonary atresia
® Pulmonary hypertension
® With some interventions such as:
¡ Valvuloplasty
¡ Stents
® See PEDCD-6.1: Kawasaki Disease Initial Imaging for specific intervals in Kawasaki Disease
® On a member who is having a device placed when:
¡ A diagnostic catheterization, or stenting is needed in addition to the device
¡ The diagnostic catheterization is indicated separate from the device placement

For members enrolled in Medicaid and NJ FamilyCare plans, Horizon BCBSNJ applies the above medical policy.

FIDE SNP:

For members enrolled in a Fully Integrated Dual Eligible Special Needs Plan (FIDE-SNP): (1) to the extent the service is covered under the Medicare portion of the member’s benefit package, the above Medicare Coverage statement applies; and (2) to the extent the service is not covered under the Medicare portion of the member’s benefit package, the above Medicaid Coverage statement applies.

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Horizon BCBSNJ Medical Policy Development Process:

This Horizon BCBSNJ Medical Policy (the “Medical Policy”) has been developed by Horizon BCBSNJ’s Medical Policy Committee (the “Committee”) consistent with generally accepted standards of medical practice, and reflects Horizon BCBSNJ’s view of the subject health care services, supplies or procedures, and in what circumstances they are deemed to be medically necessary or experimental/ investigational in nature. This Medical Policy also considers whether and to what degree the subject health care services, supplies or procedures are clinically appropriate, in terms of type, frequency, extent, site and duration and if they are considered effective for the illnesses, injuries or diseases discussed. Where relevant, this Medical Policy considers whether the subject health care services, supplies or procedures are being requested primarily for the convenience of the covered person or the health care provider. It may also consider whether the services, supplies or procedures are more costly than an alternative service or sequence of services, supplies or procedures that are at least as likely to produce equivalent therapeutic or diagnostic results as to the diagnosis or treatment of the relevant illness, injury or disease. In reaching its conclusion regarding what it considers to be the generally accepted standards of medical practice, the Committee reviews and considers the following: all credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, physician and health care provider specialty society recommendations, the views of physicians and health care providers practicing in relevant clinical areas (including, but not limited to, the prevailing opinion within the appropriate specialty) and any other relevant factor as determined by applicable State and Federal laws and regulations.

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Index:
Pediatric Cardiac Imaging Policy
Cardiac Imaging Policy, Pediatric
Computed Tomography, Cardiac, Pediatric
CT, Cardiac, Pediatric
Computed Tomography Angiography, Cardiac, Pediatric
CTA, Cardiac, Pediatric
Coronary CTA, Pediatric
CTA, Coronary, Pediatric
Coronary Computed Tomography Angiography, Pediatric
CCTA, Pediatric
Magnetic Resonance Imaging, Cardiac, Pediatric
MRI, Cardiac, Pediatric
Magnetic Resoance Angiography, Cardiac, Pediatric
MRA, Cardiac, Pediatric
Positron Emission Tomography, Cardiac, Pediatric
PET, Cardiac, Pediatric
Myocardial PET, Pediatric
PET Myocardium, Pediatric
Myocardial Perfusion Imaging with SPECT, Pediatric
SPECT, Myocardial Perfusion Imaging, Pediatric
Infarct Avid Myocardial Imaging, Pediatric
SPECT, Infarct Avid Myocardial Imaging, Pediatric
SPECT Equilibrium Cardiac Radionuclide Angiography, Pediatric
Gated Cardiac Radionuclide Angiography, Pediatric
Planar First Pass Cardiac Radionuclide Angiography, Pediatric
Nuclear Medicine Imaging, Cardiac, Pediatric
Ultrasound, Chest, Pediatric
Transthoracic Echocardiography, Pediatric
Echocardiography, Transthoracic, Pediatric
Transesophageal Echocardiography, Pediatric
Echocardiography, Transesopahageal, Pediatric
TEE, Pediatric
Doppler Echocardiography, Pediatric
Multi Gated Acquisition (MUGA) Studies, Pediatric
MUGA, Pediatric

References:


Codes:
(The list of codes is not intended to be all-inclusive and is included below for informational purposes only. Inclusion or exclusion of a procedure, diagnosis, drug or device code(s) does not constitute or imply authorization, certification, approval, offer of coverage or guarantee of payment.)

CPT*