General Information
It is an expectation that all patients receive care/services from a licensed clinician. All appropriate supporting documentation, including recent pertinent office visit notes, laboratory data, and results of any special testing must be provided. If applicable: All prior relevant imaging results and the reason that alternative imaging cannot be performed must be included in the documentation submitted.

Where a specific clinical indication is not directly addressed in this guideline, medical necessity determination will be made based on widely accepted standard of care criteria. These criteria are supported by evidence-based or peer-reviewed sources such as medical literature, societal guidelines and state/national recommendations.

Purpose
Magnetic resonance angiography (MRA) generates images of the blood vessels (arteries and/or veins) in the chest that can be evaluated for evidence of stenosis, occlusion, or
aneurysms without use of ionizing radiation. Chest MRA (non-coronary) is used to evaluate the blood vessels outside the heart in the chest (thorax).

NOTE: Authorization for MR Angiography covers both arterial and venous imaging. The term angiography refers to both arteriography and venography.

Policy:
INDICATIONS 
Suspected Pulmonary Embolism

• Suspected pulmonary embolism when CTA is contraindicated or cannot be performed:
  • High risk for PE based on shock or hypotension, OR a validated pre-test high probability score (such as Well’s > 6, Modified Geneva score > 11),
    • (D-dimer is NOT needed for high-risk patients; can approve high-risk even with normal D dimer)
  • Intermediate and Low risk require elevated D-dimer
• Follow up of known pulmonary embolism when CTA is contraindicated or cannot be performed AND either symptoms (such as dyspnea, fatigue, lightheadedness and/or edema) that recur OR are persistent at 3 months following initial diagnosis. (Follow-up imaging in asymptomatic patients to determine if embolus has resolved or to determine cessation of anticoagulation is not indicated as imaging changes may persist)

THORACIC AORTIC DISEASE^1,2,3,4,5
Suspected Thoracic Aortic Aneurysm

• Asymptomatic suspected thoracic aortic aneurysm
  • Based on other imaging such as echocardiogram or chest x-ray
  • Screening in individuals with a personal history of bicuspid aortic valve when TTE (Transthoracic Echocardiogram) is inconclusive or insufficient:
    • Baseline study at diagnosis
    • Every 3 years thereafter
  • Screening in individuals at elevated risk due to family history as below when TTE (Transthoracic Echocardiogram) is inconclusive or insufficient:
    • First-degree relatives of individuals with a known thoracic aortic aneurysm (defined as > 50% above normal) or dissection
    • First and second-degree relatives of individuals with familial thoracic aortic aneurysm and dissection (FTAAD)/nonsyndromic heritable thoracic aortic disease (NS-TAD)
    • First degree relatives of individuals with a known bicuspid aortic valve
  • See Genetic Syndromes and Rare Diseases section for additional screening indications
• Symptomatic known or suspected thoracic aortic aneurysm
  • Symptoms may include:
    • Abrupt onset of severe sharp or stabbing pain in the chest, back or abdomen
    • Acute onset of pain with asymmetric blood pressure between limbs
    • Acute chest or back pain and at high risk for aortic aneurysm and/or aortic syndrome (risk factors include hypertension, atherosclerosis, prior cardiac or aortic surgery, underlying aneurysm, connective tissue disorder (e.g., Marfan syndrome, vascular form of Ehlers-Danlos syndrome, Loeys-Dietz syndrome), and bicuspid aortic valve)
• Suspected vascular cause of dysphagia or expiratory wheezing with other imaging that is suggestive or inconclusive.

Follow-up of Known Thoracic Aortic Aneurysm

• Baseline imaging at diagnosis then every 6 – 24 months
  • If there is a change in clinical status or cardiac exam, then imaging sooner than 6 months is indicated.

Thoracic Aortic Syndromes

• For suspected acute aortic syndrome (AAS) such as aortic dissection, intramural hematoma and penetrating atherosclerotic ulcer:
  • Other imaging (such as echocardiogram) is suggestive of AAS OR
  • Individual is either:
    • high risk and one sign/symptom OR non-high risk and two or more signs/symptoms of AAS:
      • High risk conditions:
        • Marfan's syndrome or other connective tissue disease, family history of aortic disease, known aortic valve disease, recent aortic manipulation and/or known thoracic aortic aneurysm
      • Signs and symptoms concerning for AAS:
        • Chest, back or abdominal pain described as abrupt onset, severe in intensity and/or ripping or tearing in quality
        • Pulse deficit or systolic blood pressure differential
        • Focal neurologic deficit with pain
        • New heart murmur with pain
        • Hypotension or shock
• For follow-up of known aortic syndromes, including aortic dissection, intramural hematoma and penetrating atherosclerotic ulcer: frequency for follow up is as clinically indicated.

Post-Operative Follow-Up of Aortic Repair

• Follow-up thoracic endovascular aortic repair (TEVAR):
  • Baseline post-EVAR at 1 month post-EVAR
  • Annually thereafter if stable
    • more frequent imaging (as clinically indicated) may be needed if there are complications or abnormal findings on surveillance imaging.
• Follow up open repair at the following intervals:
  • Baseline follow-up study at one year post-operatively
  • Every 5 years thereafter
  • If abnormal findings are seen on any surveillance imaging study, imaging is then done annually.

Vascular Disease^6,7,8,9

• Superior vena cava (SVC) syndrome
• Subclavian Steal Syndrome after positive or inconclusive ultrasound
• Thoracic Outlet Syndrome
• Suspected pulmonary hypertension when other testing (echocardiogram or right heart catheterization) is suggestive of the diagnosis 

Congenital Malformations^10,11,12,13

• Suspected thoracic malformation based on other imaging (such as chest x-ray, echocardiogram, gastrointestinal study or CT)
• Congenital heart disease with pulmonary hypertension or extra-cardiac vascular anomalies
• Suspected coarctation of the aorta (clinical sign is a disparity in the pulsations and blood pressures in the legs and arms)
• Pulmonary sequestration

Evaluation of Tumor

• When needed for clarification of vascular invasion from tumor 

Pre-operative/procedural Evaluation

• Pre-operative evaluation for a planned surgery or procedure (including prior to planned ablation for atrial fibrillation)
• Evaluation of interventional vascular procedures for luminal patency versus restenosis due to conditions such as atherosclerosis, thromboembolism, and intimal hyperplasia
• Evaluation of vascular anatomy prior to solid organ transplantation
• Evaluation prior to endovascular aneurysm repair (EVAR)
• Evaluation prior to Transcatheter Aortic Valve Replacement

Post-operative/procedural Evaluation^14,15

• Follow-up study may be needed to help evaluate a patient’s progress after treatment, procedure, intervention, or surgery. Documentation requires a medical reason that clearly indicates why additional imaging is needed for the type and area(s) requested.
• Evaluation of endovascular/interventional abdominal vascular procedures for luminal patency versus restenosis due to conditions such as atherosclerosis, thromboembolism, and intimal hyperplasia
• Evaluation of post-operative complications, e.g., pseudoaneurysms, related to surgical bypass grafts, vascular stents, and stent-grafts in abdomen and pelvis
• Suspected complications of IVC filters

Genetic Syndromes and Rare Diseases

• For patients with fibromuscular dysplasia (FMD):^16,17
  • One-time vascular study from brain to pelvis
• Vascular Ehlers-Danlos syndrome:^18,19
  • At diagnosis and then every 18 months
  • More frequently if abnormalities are found
• Marfan Syndrome:²⁰
  • At diagnosis and then every 3 years
  • More frequently (annually) if EITHER: history of dissection, dilation of aorta beyond aortic root OR aortic root ascending aorta are not adequately visualized on TTE^3,21
• Loeys-Dietz:²²
  • At diagnosis and then every two years
  • More frequently if abnormalities are found
• Williams Syndrome:²³
  • When there is concern for vascular disease based on abnormal exam or imaging findings (such as diminished pulses, bruits or signs of diffuse thoracic aortic stenosis
• Turner Syndrome
  • Screening with no known vascular abnormality at the following intervals:
    • At diagnosis
    • Every 5 years until age 18
    • Every 10 years in adults
    • Prior to pregnancy/pregnancy planning
  • Annually if any one of the following are present: coarctation of the aorta, aortic dilation, bicuspid aortic valve, hypertension
• Takayasu's Arteritis:²⁴
  • For evaluation at diagnosis then as clinically indicated
• For other syndromes and rare diseases not otherwise addressed in the guideline, coverage is based on a case-by-case basis using societal guidance.

Combination Studies
Brain/Neck/Chest/Abdomen/Pelvis MRA

• For patients with fibromuscular dysplasia (FMD), a one-time vascular study from brain to pelvis^16,17
• Vascular Ehlers-Danlos syndrome: At diagnosis and then every 18 months; more frequently if abnormalities are found^18,19
• Loeys-Dietz: at diagnosis and then every two years, more frequently if abnormalities are found²²
• For assessment in patients with spontaneous coronary artery dissection (SCAD), can be done at time of coronary angiography²⁵

Chest/Abdomen/Lower Extremity MRA

• To evaluate for an embolic source of lower extremity vascular disease. Echocardiography is also often needed, since the heart is the most commonly reported source of lower extremity emboli, accounting for 55 to 87 percent of events.

Chest/Abdomen/Pelvis MRA

• Evaluation prior to endovascular aneurysm repair (EVAR) when thoracic involvement is present
• Evaluation prior to Transcatheter Aortic Valve Replacement (TAVR) when CTA is contraindicated or cannot be performed²⁶
• Marfan syndrome:²⁰
  • At diagnosis and every 3 years
  • More frequently (annually) if EITHER: history of dissection, dilation of aorta beyond aortic root OR aortic root/ascending aorta are not adequately visualized on TTE (i.e., advanced imaging is needed to monitor the thoracic aorta)^3,21
• Williams Syndrome²³
  • When there is concern for vascular disease (including renal artery stenosis) based on abnormal exam or imaging findings (such as diminished pulses, bruits or signs of diffuse thoracic aortic stenosis)
• Acute aortic dissection²⁷
• Significant post-traumatic or post-procedural vascular complications reasonably expected to involve the chest, abdomen and pelvis

Chest MRA and Chest MRI (or CT)

• When needed for clarification of vascular invasion from tumor 

Chest MRA and Heart MRI (or CT)

• When medical necessity criteria indications are met for each Chest MRA (see above) and Heart MRI (see Clinical Guideline Evolent_CG_028) or CT (see Clinical Guideline Evolent_CG_025) (such as for certain congenital malformations when evaluation of extra cardiac and cardiac structures are needed)

Neck/Chest/Abdomen/Pelvis MRA

• Takayasu's Arteritis: For evaluation at diagnosis then as clinically indicated²⁴ 

Further Evaluation of Indeterminate Findings on Prior Imaging
Unless follow up is otherwise specified within the guideline:

• For initial evaluation of an inconclusive finding on a prior imaging report that requires further clarification
• One follow-up exam of a prior indeterminate MR/CT finding to ensure no suspicious interval change has occurred. (No further surveillance unless specified as highly suspicious or change was found on last follow-up exam.)

BACKGROUND
Contraindications and Preferred Studies

• Contraindications and reasons why a CT/CTA cannot be performed may include: impaired renal function, significant allergy to IV contrast, pregnancy (depending on trimester).
• Contraindications and reasons why an MRI/MRA cannot be performed may include: impaired renal function, claustrophobia, non-MRI compatible devices (such as noncompatible defibrillator or pacemaker), metallic fragments in a high-risk location, patient exceeds weight limit/dimensions of MRI machine.

References:

1. Borger M, Fedak P, Stephens E, Gleason T, Girdauskas E et al. The American Association for Thoracic Surgery consensus guidelines on bicuspid aortic valve-related aortopathy: Full online-only version. J Thorac Cardiovasc Surg. Aug 2018; 156: e41-e74. 10.1016/j.jtcvs.2018.02.115.
2. Ferreira Tda A, Chagas I, Ramos R, Souza E. Congenital thoracic malformations in pediatric patients: two decades of experience. J Bras Pneumol. Mar-Apr 2015; 41: 196-9. 10.1590/s1806-37132015000004374.
3. Isselbacher E M, Preventza O, Hamilton Black III J, Augoustides J G, Beck A W et al. 2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines. Journal of the American College of Cardiology. 2022; 80: e223 - e393. https://doi.org/10.1016/j.jacc.2022.08.004.
4. Mariscalco G, Debiec R, Elefteriades J, Samani N, Murphy G. Systematic Review of Studies That Have Evaluated Screening Tests in Relatives of Patients Affected by Nonsyndromic Thoracic Aortic Disease. Journal of the American Heart Association. 2018; 7: true. 10.1161/JAHA.118.009302.
5. Erbel R, Aboyans V, Boileau C, Bossone E, Bartolomeo R et al. 2014 ESC Guidelines on the diagnosis and treatment of aortic diseases: Document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC). Eur Heart J. Nov 1, 2014; 35: 2873-926. 10.1093/eurheartj/ehu281.
6. American College of Radiology. ACR Appropriateness Criteria® Thoracic Outlet Syndrome. 2019; 2022:
7. Bonci G, Steigner M, Hanley M, Braun A, Desjardins B et al. ACR Appropriateness Criteria Thoracic Aorta Interventional Planning and Follow-Up. Journal of the American College of Radiology. 2017; 14: S570 - S583. 10.1016/j.jacr.2017.08.042.
8. Friedman T, Quencer K, Kishore S, Winokur R, Madoff D. Malignant Venous Obstruction: Superior Vena Cava Syndrome and Beyond. Semin Intervent Radiol. Dec 2017; 34: 398-408. 10.1055/s-0037-1608863.
9. Osiro S, Zurada A, Gielecki J, Shoja M, Tubbs R. A review of subclavian steal syndrome with clinical correlation. Med Sci Monit. May 2012; 18: Ra57-63. 10.12659/msm.882721.
10. Bae S, Kang E, Choo K, Lee J, Kim S et al. Aortic Arch Variants and Anomalies: Embryology, Imaging Findings, and Clinical Considerations. J Cardiovasc Imaging. 2022; 30: 231 - 262.
11. Leo I, Sabatino J, Avesani M, Moscatelli S, Bianco F et al. Non-Invasive Imaging Assessment in Patients with Aortic Coarctation: A Contemporary Review. 2024; 13: 10.3390/jcm13010028.
12. Orozco V U H M F. Thoracic Vascular Variants and Anomalies: Imaging Findings, Review of the Embryology, and Clinical Features. Indian Journal of Radiology and Imaging. 2022; 32: 568 - 575. 10.1055/s-0042-1757742.
13. Pascall E, Tulloh R. Pulmonary hypertension in congenital heart disease. Future Cardiol. Jul 2018; 14: 343-353. 10.2217/fca-2017-0065.
14. Bennett K, Kent K, Schumacher J, Greenberg C, Scarborough J. Targeting the most important complications in vascular surgery. J Vasc Surg. Mar 2017; 65: 793-803. 10.1016/j.jvs.2016.08.107.
15. Choudhury M. Postoperative management of vascular surgery patients: a brief review. Clin Surg. 2017; 2: 1584.
16. Gornik H L, Persu A, Adlam D, Aparicio L S, Azizi M et al. First International Consensus on the diagnosis and management of fibromuscular dysplasia. Vascular Medicine. 2019; 24: 164 - 189. 10.1177/1358863X18821816.
17. Kesav P, Manesh Raj D, John S. Cerebrovascular Fibromuscular Dysplasia - A Practical Review. Vascular health and risk management. 2023; 19: 543-556. 10.2147/VHRM.S388257.
18. Bowen J M, Hernandez M, Johnson D S, Green C, Kammin T et al. Diagnosis and management of vascular Ehlers-Danlos syndrome: Experience of the UK national diagnostic service, Sheffield. European journal of human genetics: EJHG. 2023; 31: 749-760. 10.1038/s41431-023-01343-7.
19. Byers P. Vascular Ehlers-Danlos Syndrome. 1999 Sep 2 [Updated 2019 Feb 21]. GeneReviews®[Internet]. 2019.
20. Dietz H. FBN1-Related Marfan Syndrome. [Updated 2022 Feb 17]. GeneReviews® [Internet]. 2022; Accessed May 2024:
21. Weinrich J M, Lenz A, Schön G, Behzadi C, Molwitz I et al. Magnetic resonance angiography derived predictors of progressive dilatation and surgery of the aortic root in Marfan syndrome. PLOS ONE. 2022; 17: true.
22. Loeys B, Dietz H. Loeys-Dietz Syndrome. [Updated 2018 March 1]. GeneReviews® [Internet]. 2018.
23. Morris C. Williams Syndrome. [Updated 2023 Apr 13]. GeneReviews® [Internet]. 2023.
24. Maz M, Chung S A, Abril A, Langford C A, Gorelik M et al. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis. Arthritis & Rheumatology (Hoboken, N.J.). 2021; 73: 1349-1365. 10.1002/art.41774.
25. Teruzzi G, Santagostino Baldi G, Gili S, Guarnieri G, Montorsi P. Spontaneous Coronary Artery Dissections: A Systematic Review. Journal of clinical medicine. 2021; 10: 10.3390/jcm10245925.
26. Hedgire S, Saboo S, Galizia M, Aghayev A, Bolen M et al. ACR Appropriateness Criteria® Preprocedural Planning for Transcatheter Aortic Valve Replacement: 2023 Update. Journal of the American College of Radiology: JACR. 2023; 20: S501-S512. 10.1016/j.jacr.2023.08.009.
27. Kicska G, Hurwitz Koweek L, Ghoshhajra B, Beache G, Brown R et al. ACR Appropriateness Criteria® Suspected Acute Aortic Syndrome. Journal of the American College of Radiology: JACR. 2021; 18: S474-S481. 10.1016/j.jacr.2021.09.004.

Coding Section 

Procedure and diagnosis codes on Medical Policy documents are included only as a general reference tool for each policy. They may not be all-inclusive. 

This medical policy was developed through consideration of peer-reviewed medical literature generally recognized by the relevant medical community, U.S. FDA approval status, nationally accepted standards of medical practice and accepted standards of medical practice in this community, Blue Cross Blue Shield Association technology assessment program (TEC) and other non-affiliated technology evaluation centers, reference to federal regulations, other plan medical policies, and accredited national guidelines.

"Current Procedural Terminology © American Medical Association. All Rights Reserved" 

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