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KEY FACTS

Terminology
- Congenital narrowing of aorta, most commonly occurring just distal to left subclavian artery origin
Imaging
- Chest radiograph: Inferior rib notching, figure 3 sign
- Esophagram: Reverse figure 3 sign
- CTA: Focal, shelf-like narrowing of posterior/lateral aorta just distal to left subclavian origin
- MR - Contrast-enhanced 3D MR angiography (MRA) for vessel morphology and depiction of enlarged collateral arteries - Velocity-encoded cine is used to estimate pressure gradients and flow volumes
- Angiography - Morphology of coarctation and collateral vessels - Measurement of pressure gradients
Top Differential Diagnoses
- Pseudocoarctation
- Takayasu arteritis
- Interrupted aortic arch
Pathology
- Associations - Bicuspid aortic valve, ventricular septal defect, patent ductus arteriosus - Turner syndrome
Clinical Issues
- Surgical correction used for infants
- Balloon angioplasty used for children and adults
- Stent placement typically for recoarctation
Diagnostic Checklist
- Search for subtle signs of coarctation in any young patient with hypertension

TERMINOLOGY

Abbreviations
- Coarctation of aorta**(CoA**, coarc)
Definitions
- Congenital narrowing of aorta, most commonly occurring just distal to left subclavian artery origin
- Atypical coarctation: Not involving isthmus (usually abdominal aorta)

IMAGING

General Features

Location

- May occur anywhere in aorta or at multiple sites
- Preductal, ductal, or post ductal

Size

- Longer segment stenosis referred to as tubular hypoplasia
- Focal or diffuse

Radiographic Findings
- Radiography - Inferior rib notching (Roesler sign) - Related to enlargement of intercostal arteries serving as collaterals - Rare before 5 years of age - Affects ribs 3-8; ribs 1 and 2 are not affected, as they arise from costocervical trunk and do not anastomose with distal aorta - May regress post repair - Unilateral rib notching may indicate aberrant subclavian artery - Figure 3 sign in up to 50% of cases - Dilated left subclavian artery produces proximal convexity - Indentation at coarctation - Poststenotic descending aorta produces distal convexity - Ill-defined or obscured aortic arch - Mediastinal widening - Heart: Rounded apex from left ventricular hypertrophy - May exhibit bicuspid aortic valve
- Esophagram - Reverse figure 3 sign - Compression on esophagus from dilated left subclavian artery and poststenotic dilatation of descending aorta

CT Findings

CTA

- Excellent morphologic characterization
        - Defines location and severity of stenosis
        - Focal, shelf-like narrowing of posterior/lateral aorta just distal to left subclavian origin
        - Enlarged collateral arteries indicate hemodynamic significant obstruction at coarctation site
- Gradient cannot be calculated
- Multiplanar reformations (sagittal oblique plane) and 3D volume-rendered images
- Useful for pre- and postoperative stent repair

MR Findings
- Allows morphologic and functional assessment
- Morphology - Achieved with several MR protocols: HASTE, SSFP (TrueFISP, FIESTA), contrast-enhanced MR angiography (MRA) - HASTE (dark blood) and SSFP (bright blood) - Single-shot sequences; can be axial, sagittal, or coronal - ECG-gated technique; can be free breathing - Helpful to determine associated cardiac anomalies - "Gothic" or angulated aortic morphology after coarctation surgery is associated with high risk of arterial hypertension - Useful to assess for complications: Aneurysm, pseudoaneurysm, recoarctation - Contrast-enhanced MRA - 3D acquisition on oblique sagittal axis (i.e., "candy cane" axis) after intravenous contrast - Better results with ECG-gated and breath-holding techniques - Planimetry: Determine orthogonal diameters and areas from proximal to distal to ductus - Indexed minimal aortic cross-sectional area (cm²/m²) - Adjusted to body surface area - Best predictor of severity - < 0.33 cm²/m² indicates severe coarctation (gradient ≥ 20 mmHg) - Demonstrates enlarged collateral arteries
- Velocity-encoded cine MR - Flow-sensitive phase-contrast technique, segmented acquisition - Requires breath-holding and ECG-gating techniques - Heart rate-corrected deceleration time (sec⁻⁰**·**⁵) - Adjusted to heart rate - Flow value at cessation of flow - peak flow rate/time interval from peak to cessation - Deceleration time = [(flow at end of deceleration) - (peak systolic flow)]/(deceleration time) - Adjustment to heart rate with Bazett formula: 1/(R-R interval)⁰·⁵ - Excellent predictor of severity - ≥ 0.30 sec⁻⁰·⁵ indicates severe coarctation (gradient ≥ 20 mmHg) - Amount of collateral flow - Enlarged collateral vessels are reliable indicator of hemodynamic significance - Retrograde blood flow in relevant mediastinal or intercostal arteries is indicative of collateral flow - Percentage increase in flow between aorta immediately distal to coarctation and just above diaphragmatic crura due to collateral flow - Normal subjects: 7% ± 6% decrease - Coarctation (gradient < 20 mmHg): No increase - Coarctation (gradient ≥ 20 mmHg): 83% ± 50% mean increase - Pressure gradient (ΔP) - Calculated with peak velocity (v) - Modified Bernoulli equation (short-segment stenoses only): ΔP = 4v² - Gradient ≥ 20 mmHg indicates severe coarctation - May be used to quantify aortic valve stenosis and regurgitation
- Combination of indexed minimal cross-sectional area and heart rate-corrected deceleration time best****predicts hemodynamically significant coarctation
- Cine MR - Cine SSFP or GRE (segmented acquisition) - Requires breath-holding and ECG-gating techniques - Gold standard to assess left ventricular hypertrophy (myocardial thickness and mass) - Enables characterization of bicuspid aortic valve - Allows identification of turbulent and jet flows that may indicate stenosis or regurgitation
- 4D flow MR - Calculates collateral blood flow - Vortical flow pattern in regions of poststenotic dilatation - Helical flow pattern may be seen in angulated postsurgical aorta

Echocardiographic Findings

Echocardiogram

- Suprasternal long-axis view
- Color Doppler estimates gradient across coarctation
- Classic findings: Narrowing of isthmus and posterior indentation or shelf

Angiographic Findings
- Vessel morphology and direct measurement of pressure gradient (ΔP) - < 20 mmHg: Mild coarctation - ≥ 20 mmHg: Suggests need for intervention

Imaging Recommendations

Best imaging tool

- MR and MRA often fully characterize and determine needs of treatment
- Angiography remains gold standard; used when MR is inconclusive

Protocol advice

- MR to include sagittal oblique plane through aortic arch and perpendicular plane through coarctation for measurement of cross-sectional diameter

DIFFERENTIAL DIAGNOSIS

Pseudocoarctation
- Older adult with elongation and kinking of aorta related to atherosclerosis
- No hemodynamically significant stenosis or collateral vessels
Takayasu Arteritis
- Inflammatory narrowing of unknown etiology
- Narrowing &/or occlusion of aorta and branch vessels, rarely isolated to aortic isthmus
Interrupted Aortic Arch
- Complete absence of continuity between 2 segments of aorta
- Nearly always manifests in neonates
Traumatic Pseudoaneurysm
- History of trauma, healed rib, and other skeletal fractures
- Narrowing of descending thoracic aorta may coexist with pseudoaneurysm
Inferior Rib Notching Differential
- Neurofibromatosis
- Venous collaterals (superior vena cava obstruction)
- Decreased pulmonary blood flow (tetralogy of Fallot, pulmonary atresia)
- Blalock-Taussig shunt (ribs 1 and 2)

PATHOLOGY

General Features

Etiology

- Abnormal development of embryologic left 4th and 6th aortic arches
- Muscular theory
        - Migration of tissue from ductus arteriosus into aortic wall and subsequent contraction
- Hemodynamic theory
        - Decreased aortic blood flow during fetal development may not allow proper aortic growth
        - Increased incidence of coarctation in disorders in which left ventricular outflow tract obstruction reduces aortic blood flow
        - Decreased incidence of coarctation in disorders in which decreased ductal flow is present (e.g., tetralogy of Fallot)

Genetics

- Association with Turner syndrome (up to 20% of patients have coarctation)

Associated abnormalities

- **Bicuspid aortic valve (reported in 50-85%)**
- Ventricular septal defect
- Patent ductus arteriosus
- Shone syndrome: Aortic coarctation, subaortic stenosis, parachute mitral valve, supravalvular mitral membrane
- Cerebral aneurysms (2.5-10.0%)
- Variable evidence regarding increased risk of coronary artery disease

Staging, Grading, & Classification
- Classification (controversial) - Previously used classifications (e.g., infantile and adult type) discouraged due to overlapping manifestations
- Pathophysiologic classification - Preductal: Stenosis proximal to ductus arteriosus - Distal blood flow relies on patency of ductus; closure can be life threatening - 5% of all children with Turner syndrome - Ductal: At insertion of ductus arteriosus - Clinically evident after ductus closure - Post ductal: Stenosis distal to ductus arteriosus
- Simple coarctation - Occurs in isolation - Often localized just beyond left subclavian artery origin (post ductal)
- Complex coarctation - Occurs in presence of other intracardiac anomalies, thus, tends to manifest in infancy - Often preductal
Gross Pathologic & Surgical Features
- Obstructing membrane or ridge of tissue near aortic isthmus
- May develop cystic medial necrosis adjacent to coarctation site; predisposes to aneurysm or dissection
- 3 main collateral pathways - Subclavian artery → internal mammary artery → intercostal arteries - Subclavian artery → thyrocervical and costocervical trunks → thoracoacromial and descending scapular arteries → intercostal arteries - Subclavian artery → vertebral artery → anterior spinal artery → intercostal arteries
Microscopic Features
- Loss of smooth muscle cells and cystic medial necrosis when associated with bicuspid aortic valve

CLINICAL ISSUES

Presentation

Most common signs/symptoms

- Presentation depends on degree of stenosis and associated abnormalities
- **Neonates**
        - Asymptomatic if coarctation not severe or patent ductus arteriosus
        - If severe coarctation or closed ductus arteriosus, may have heart failure
        - Decreased femoral pulses, associated murmurs
- **Children****and****adults**
        - May be asymptomatic
        - Leg claudication
                  - Differential blood pressure between upper and lower extremities, diminished femoral pulses
        - Angina pectoris
        - Severe hypertension
                  - Often leads to chronic heart failure
                  - Intracranial aneurysm ± subarachnoid bleeding
                  - Stroke
        - Murmur associated with bicuspid aortic valve
        - Epistaxis

Other signs/symptoms

- Turner syndrome: Short, webbed neck; broad chest; pigmented facial nevi; short 4th metacarpals

Demographics

Age

- Related to degree of narrowing and presence of associated abnormalities

Sex
```
- M:F = 2:1
```
Ethnicity
```
- White:Asian = 7:1
```

Epidemiology

- Incidence: 2-6 per 10,000 births
- Comprises 5-10% of cases of congenital heart disease
- Represents 7% of all critically ill infants with congenital heart disease

Natural History & Prognosis
- Without repair - Average age of death: 35-42 years - 75% mortality rate by age 46 - Due to aortic dissection or rupture, heart failure, myocardial infarct, and cerebral hemorrhage
- With repair - ~ 90% survival rate at 20 years; decreased chance of survival with increased age at repair - Recoarctation (2-14%) - Associated with younger age at surgery - Postoperative aneurysms (increased risk after patch aortoplasty) - Long-term survival rate decreased due to hypertension, coronary artery disease, dissection
- Pregnancy-related issues - Untreated coarctation: Increased risk of dissection and intracranial hemorrhage - Treated coarctation: Increased rate of miscarriage and preeclampsia
- All patients require lifelong follow-up with cardiologist trained in congenital heart disease
Treatment
- Indications for treatment - Infant with severe stenosis and heart failure - Longstanding hypertension - Hemodynamically significant stenosis (gradient > 20 mmHg) - Extensive collateral flow - Female patient planning pregnancy
- Surgical correction: 1st-line treatment for infants - Resection with end-to-end anastomosis - Higher risk of spinal artery injury and restenosis - Left subclavian flap aortoplasty - Sacrifice left subclavian artery and vertebral artery (to avoid subclavian steal) - Bypass graft - Used if area of narrowing is too long for end-to-end repair - Prosthetic patch or interposition graft - Rarely used due to long-term risk of infection or aneurysm with prosthetic material - Acute complications - Surgical mortality rare - Paradoxical hypertension, recoarctation, hypertension, paraplegia due to spinal artery damage, recurrent laryngeal or phrenic nerve injury, subclavian steal - Postcoarctectomy syndrome - Abdominal pain that may progress to intestinal wall hemorrhage or even perforation - Late complications - Aortic aneurysm, recurrent coarctation, hypertension
- Balloon angioplasty - 1st-line treatment for older children and adults for native coarctation or recoarctation - Not recommended for infants due to increased rate of recurrence - Acute complications (rare) - Dissection, stroke - Late complications - Recoarctation, aneurysm, endocarditis, hypertension
- Stent placement - Generally reserved for recoarctation - Complications - Acute rupture, dissection, stent fracture or migration - Aneurysm in up to 11% - More long-term data needed

DIAGNOSTIC CHECKLIST

Consider
- Search for subtle signs of coarctation in any young patient with hypertension
Image Interpretation Pearls
- Enlarged collaterals imply significant stenosis

32fcf972-f7c9-4372-aa4a-24d0fc34829f

References

Selected References

Differential diagnosis

Left Ventricular Enlargement

DDX:fbb972de-3e13-4c67-b7a4-f8901aa2efb8

Left Ventricular Enlargement

DDX:f62409ca-8fb2-46b3-9919-2e1e6adf07b7

Narrowed Aorta

DDX:763503a4-a7b8-4aff-8846-3dfbe312125c

Cases

{'cases': [{'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}, {'key': '509cdc73-5701-4807-90e5-cf244eb473c8', 'value': 'Tan-Lucien H. Mohammed, MD, FCCP'}], 'caseVersionId': 'bab06bb9-d576-4b97-ad6b-dc7e810c3367', 'description': 'Typical CT features of abnormal aortic contour from aortic coarctation.\n\nCTA (#1-5) shows enlarged systemic collateral (open arrows). Segment of descending aorta (arrows, #2,3) is either small or nearly absent. Sagittal oblique shows characteristic narrowing (arrow) from coarctation.', 'history': 'Uncontrolled hypertension.', 'imagePoolId': '793035bb-240a-46fe-913b-9cf3bf6c60c7', 'name': 'Aortic contour', 'teachingPoint': None, 'demographics': '45 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}], 'caseVersionId': 'd68ea291-9e92-4251-97bf-6397569c066b', 'description': 'Typical radiographic features of mediastinal contour from aortic coarctation.\n\nRadiographs (#1,2) show abnormal mediastinal contours (open arrows) from aortic coarctation. Cardiac contour (arrows) consistent with left ventricular hypertrophy.', 'history': 'Hypertension.', 'imagePoolId': 'cd41adb1-6ea4-4276-9a20-fffa91f8e412', 'name': 'Mediastinal contour', 'teachingPoint': None, 'demographics': '28 Years old male'}, {'authors': [{'key': 'b00d2bdb-66e1-41ed-90b4-c52904f4d598', 'value': 'Seth Kligerman, MD, MS'}], 'caseVersionId': 'd9926f5c-6b27-4c12-9606-0671843ddbb1', 'description': 'Coned-down PA radiograph (#1) shows subtle areas of rib notching (arrows), giving the undersurface of the ribs a wavy appearance. Additionally, there is rapid narrowing of the proximal descending thoracic aorta (curved arrow), which then returns to normal size more distally (open arrow). This pattern is sometimes referred to as the "figure of 3" sign. Axial images from a cardiac CT at the level of the arch (#2), the proximal descending thoracic aorta (#3) and more distal descending thoracic aorta (#4) show a normal size arch (arrow, #2) with marked focal narrowing of the proximal descending aorta (arrow, #3). Distal to this, the aorta returns to normal size (arrow, #4). Note the extensive collaterals in the chest wall and mediastinum (open arrows, #2-3). Full field of view image from a cardiac CT (#5) shows intercostal collateral arteries (arrows, #5), which cause the rib notching. Sagittal multiplanar reformat from the cardiac CT (#6) shows the location of the coarctation in the descending thoracic aorta (arrow).', 'history': 'Patient with uncontrolled hypertension and blood pressure gradient of over 30 mmHg between the left arm and left leg. ', 'imagePoolId': 'a8be3956-8383-4977-aa09-910d438d5366', 'name': 'Coarctation, aorta, post-ductal', 'teachingPoint': None, 'demographics': '37 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}], 'caseVersionId': 'dcd4c2ac-af2a-4e83-a495-9bc9c6ccc6eb', 'description': 'Typical CT and radiographic features of rib notching from aortic coarctation.\n\nCTA (#1-6) shows numerous collaterals (open arrows). Descending aorta is dilated (post-stenotic) (arrows, #2-6). Coarctation (open black arrow, #3) was less than 5 mm in diameter. Coronal reconstruction (#7) shows "figure 3 sign" (arrows). Volume reconstruction (#8) shows coarctation (arrow). Radiographs (#9,10) shows rib notching (open arrows).', 'history': 'Uncontrolled hypertension on three anti-hypertensive drugs.', 'imagePoolId': '852cb0d1-d115-4fe2-b917-7fad134c72e2', 'name': 'Rib notching', 'teachingPoint': None, 'demographics': '27 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}, {'key': '509cdc73-5701-4807-90e5-cf244eb473c8', 'value': 'Tan-Lucien H. Mohammed, MD, FCCP'}], 'caseVersionId': '4cd95bfe-e455-44d4-88a1-75b02f480c80', 'description': 'Typical radiographic and MR features of aortic narrowing from coarctation.\n\nRadiograph (#1) shows normal aortic contours. Multiple small pulmonary calcifications are probably from previous histoplasmosis. T1WI MRs (#2-3) show abrupt narrowing (arrows) of the descending aorta. Cine MRs (#4-6) show short segment aortic narrowing (open arrows) and collateral vessels (arrow, #6).', 'history': 'Hypertension.', 'imagePoolId': 'ae06339c-44dc-4d6a-90ca-a927e5de6f21', 'name': 'Aortic narrowing', 'teachingPoint': None, 'demographics': '32 Years old male'}, {'authors': [{'key': '8e8c445a-2b2f-435d-b348-855b7921ad53', 'value': 'Christopher G. Anton, MD'}], 'caseVersionId': 'ec2164ac-e323-4ab0-bc64-a5b3d4fff986', 'description': 'Typical case of aortic coarctation on chest radiographs and angiography.\n\nRadiographs (#1, 2) show mild left ventricular prominence and post stenotic dilation of the descending aorta ("3" sign, arrow, #1). Aortic angiography (#3-5) show aortic coarctation (arrows) and post stenotic dilation (open arrows) of the descending aorta. 4 years after balloon angioplasty; radiograph (#6) and aortic angiography (#7) were performed to evaluate for re-coarctation. Radiograph (#6) again shows the "3" sign (arrow). Aortic angiography (#7) shows no re-coarctation or aneurysm with mild residual narrowing.', 'history': 'Patient with known aortic coarctation presents for precatheterization chest radiograph.', 'imagePoolId': '821f74d5-7a31-4407-8de8-0b1822c58aca', 'name': 'Balloon dilation', 'teachingPoint': None, 'demographics': '5 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}, {'key': '55c0cff5-49cc-4f10-af0f-9bbe4379c141', 'value': 'Howard Mann, MD'}], 'caseVersionId': 'f1ac2367-736e-42c1-a4f1-f6172213cfd3', 'description': 'Typical rib notching in aortic coarctation.\n\nRadiographs (#1-3) demonstrates notching (arrows) of the inferior surfaces of posterior left 5th (#2) and right 4th (#3) ribs.', 'history': 'Long-standing hypertension.', 'imagePoolId': '74d8582b-ac70-4185-bf5d-5e18aa0b3dad', 'name': 'Rib notching', 'teachingPoint': None}, {'authors': [{'key': 'fff9b5a0-8473-401a-8da6-d1366705ec01', 'value': 'Jeffrey P. Kanne, MD'}], 'caseVersionId': '112313c7-2488-4b82-9e5d-a034b9f2b5d9', 'description': 'PA chest radiograph (#1) shows a focal narrowing (arrow) in the proximal descending aorta. Sagittal T2 FSE MR image (#2) shows focal narrowing (arrow) in the proximal descending aorta with post-stenotic dilation (curved arrow). Sagittal T2* GRE cine MR image (#3) shows a dephasing jet (arrows) distal to the aortic narrowing.', 'history': 'Heart murmur.', 'imagePoolId': '20c69921-e2f6-47a3-ba49-2248520d9d32', 'name': 'Aortic narrowing', 'teachingPoint': None, 'demographics': '30 Years old male'}, {'authors': [{'key': '8e8c445a-2b2f-435d-b348-855b7921ad53', 'value': 'Christopher G. Anton, MD'}], 'caseVersionId': '20d90d0d-4c24-4ecb-8eeb-d1535c0d16d3', 'description': 'Typical case of aortic coarctation with rib notching.\n\nRadiograph (#1) shows bilateral inferior rib notching. Aortic angiography (#2, 3) show coarctation (arrow, #2) of the aorta and numerous collateral vessels (white arrows, #3) with enlargement of the internal mammary arteries (black arrows, #3). Aortic angiography (#4, 5) after stent (curved arrow, #4) placement shows improvement but mild residual narrowing (arrow, #5) at the stent across the coarctation.', 'history': 'Patient with known history of coarctation and new onset upper extremity hypertension presents for precatheterization chest radiographs and angiographic stent placement.', 'imagePoolId': '71e1fdde-8064-44bd-8c03-005ce2babf0b', 'name': 'Stent, rib notching', 'teachingPoint': None, 'demographics': '16 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}, {'key': '55c0cff5-49cc-4f10-af0f-9bbe4379c141', 'value': 'Howard Mann, MD'}], 'caseVersionId': '87b73b7e-407c-4978-ac97-2cd88d7649a9', 'description': 'Typical radiographic and CT features of aortic narrowing from aortic coarctation.\n\nRadiographs (#1-2) show rib notching (open arrows) and abnormal aortic contour (arrows). CECT images (#3-6) show large collaterals (open arrows) and aortic narrowing (arrows, #5-6). Bone window (#7) shows rib notching (open arrows). Sagittal reconstruction (#8) shows aortic narrowing (arrow). Volume reconstruction (#9) shows aortic narrowing (arrow).', 'history': 'Hypertension.', 'imagePoolId': '762d40e6-b4b5-4a3c-85d4-5e1bce136b35', 'name': 'Aortic narrowing', 'teachingPoint': None, 'demographics': '27 Years old male'}, {'authors': [{'key': 'd06dfcc4-4b3a-4c2a-b6ae-6ac081d23b98', 'value': 'Jonathan Hero Chung, MD'}, {'key': '3d1e4c57-c1cf-4c89-b0f0-5d82b29a31e1', 'value': 'Suhny Abbara, MD, FACR, MSCCT, FNASCI'}], 'caseVersionId': '2b552581-b70a-4216-9502-5bd2d595bf0f', 'description': 'Sagittal VR image from CTA (#1) shows severe narrowing (arrows) of the proximal aspect of the descending aorta consistent with focal aortic coarctation. As shown in VR CTA (#1-3), there are multiple collateral arteries (open arrows) to bypass this area of aortic narrowing. As shown on axial CTA (#4), though the aortic valve has a trileaflet morphology during diastole (arrows), functional images should be reviewed to assess the aortic valve during systole given the high association between aortic coarctations and bicuspid aortic valves. On occasion, a bicuspid valve with a prominent raphe may mimic a normal trileaflet valve during diastole.', 'history': 'Patient presented with hypertension.', 'imagePoolId': '7e9a7762-a9bb-440b-9cda-d52491f07693', 'name': 'Aortic coarctation with multiple collaterals', 'teachingPoint': None, 'demographics': '35 Years old female'}, {'authors': [{'key': 'd06dfcc4-4b3a-4c2a-b6ae-6ac081d23b98', 'value': 'Jonathan Hero Chung, MD'}], 'caseVersionId': '2d64b2db-0d26-4e46-86d9-389f99a23c7d', 'description': 'VR images from cardiac CTA (#1-2) show a bicuspid aortic valve (arrows, #1); a small partially calcified raphe is also present (open arrow, #1). There is mild narrowing (curved arrows, #2) of the proximal aspect of the descending aorta from previous aortic coarctation repair.', 'history': 'Patient with history of aortic coarctation status post repair.', 'imagePoolId': '5d5824be-aac3-49a2-ac41-33cd2b35e726', 'name': 'Bicuspid aortic valve, history of aortic coarctation', 'teachingPoint': None, 'demographics': '57 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}], 'caseVersionId': '22439123-9e23-4130-9448-b3b5a5426bf3', 'description': 'Typical CT features of aortic contour abnormality from coarctation associated with ventricular septal defect.\n\nCECT (#1-5) shows aneurysmal dilatation of proximal descending aorta (arrows). Sagittal oblique reconstructions (#6,7) shows coarctation anomaly (arrows). Ventricular septal defect (open arrow, #6). CECT (#8) shows ventricular septal defect (arrow). \n\nComment: Lack of collaterals suggests that there is no hemodynamic gradient.', 'history': 'Chest pain.', 'imagePoolId': '8f73cacd-1cd2-4b9a-8274-4a3bd9f952ce', 'name': 'Aortic aneurysm with ventricular septal defect', 'teachingPoint': None, 'demographics': '62 Years old female'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}], 'caseVersionId': '87f05bfe-9fc3-434d-a689-0eb7ab1ea444', 'description': 'Typical CT features of coarctation of aorta.\n\nCECT (#1-4) shows extensive arterial collaterals (arrows) and aortic narrowing (open arrows, #2,3). Sagittal oblique reconstructions (#5,6) show coarctation (open arrows), dilated ascending aorta (curved arrow, #5), and aortic valve prosthesis (arrows). Coronal and sagittal reconstructions (#7,8) show dilated intercostal artery causing rib erosion (open arrows). Volume reconstructions (#9,10) shows collaterals (arrows, #9) and coarctation (open arrows).', 'history': 'Hypertension.', 'imagePoolId': 'c4b83649-38db-41a0-928a-17ac7430160c', 'name': 'Rib erosion', 'teachingPoint': None, 'demographics': '41 Years old female'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}], 'caseVersionId': '1b61a4cf-a8fb-4e95-8e07-7e1135fd5c42', 'description': 'Typical radiographic and US features of aortic contour abnormality of coarctation of aorta.\n\nRadiographs (#1,2) shows "3" configuration of proximal descending aorta (open arrows). Doppler US (#3) of renal artery shows parvus tardus signifying upstream stenosis.', 'history': 'Hypertension.', 'imagePoolId': 'fdaee957-c09d-487d-a923-b64d85bb93d0', 'name': 'Aortic contour', 'teachingPoint': None, 'demographics': '9 Years old male'}], 'caseType': 'typical', 'name': 'TYPICAL'}
{'cases': [{'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}], 'caseVersionId': 'ff62466f-4e10-4fa4-8139-53e8adb03ef8', 'description': 'Variant CT features of narrowing aorta from restenosis coarctation.\n\nCECTs (#1-5) show aortic narrowing (open arrows, #1-2, 5) and post-stenotic dilatation (arrows, #3-5). Radiographs (#6-7) show an endovascular stint (open arrows) post treatment. CECT (#8) shows bicuspid aortic valve (arrow).', 'history': 'Previous coarctation repair.', 'imagePoolId': '5ba32dba-0932-4018-a078-312902f4c64f', 'name': 'Aortic narrowing', 'teachingPoint': None, 'demographics': '39 Years old male'}, {'authors': [{'key': '6f4e2c1e-8c5f-4fec-a244-9b7a171e7873', 'value': 'Helen T. Winer-Muram, MD'}], 'caseVersionId': '30b8348d-a052-4984-8580-0b08c93e0028', 'description': 'Variant of coarctation. Mediastinal mass represents pseudocoarctation. \n\nRadiograph (#1) shows two convexities (open arrows) at the left superior mediastinum. Radiograph (#2) shows a convexity (arrow) and concavity (open arrow) that represents kinking of an elongated thoracic aorta. CECT (#3-5) shows that there is no stenosis (open arrow) or post-stenotic dilatation (arrows) at the proximal descending aorta consistent with the diagnosis of pseudocoarctation.', 'history': 'Asymptomatic', 'imagePoolId': '0fd24734-abd2-4bf8-b138-02828ecae6ff', 'name': 'Mediastinal mass', 'teachingPoint': None, 'demographics': '73 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}], 'caseVersionId': '71dc2a7d-41c8-4f57-bec4-928e7f92bf25', 'description': 'Variant CT features of bypass surgery for aortic coarctation.\n\nCECT (#1-3) shows bypass (open arrows) for aortic coarctation.CECT (#4) shows left ventricular hypertrophy (arrows). Sagittal oblique (#5-7) reconstructions show aortic bypass (arrows) and typical contour for coarctation (open arrows).', 'history': 'Surgery for coarctation.', 'imagePoolId': '2f402172-a927-4688-a2b7-da11ecfa3ee4', 'name': 'Surgical correction', 'teachingPoint': None, 'demographics': '42 Years old female'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}], 'caseVersionId': 'a22923d2-26d3-4288-9234-d1d653f6378a', 'description': 'Variant CT radiographic and CT features of postoperative coarctation repair aortic aneurysm and aneurysmal dilatation ascending aorta from bicuspid aortic valve.\n\nRadiographs (#1, 2) show aneurysmal dilatation (arrows) at level of aortic arch and aneurysmal dilatation of ascending aorta (open arrow, #1). CTA (#3-6) shows aneurysm (arrows, #3, 4) in region of previous coarctation repair. Aneurysm neck (open arrow, #3). Aneurysmal dilatation of ascending aorta (open arrows, #5, 6) from bicuspid aortic valve. Focal stenosis main pulmonary artery (curved arrow, #5). Sagittal oblique reconstruction (#7) shows dilated ascending aorta (open arrow) and aneurysm at level of previous coarctation repair (arrow).', 'history': 'Coarctation repair as child.', 'imagePoolId': '523eb95c-6706-41fb-a600-7f922d0d56f7', 'name': 'Postoperative aortic aneurysm', 'teachingPoint': None, 'demographics': '45 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}], 'caseVersionId': 'b2567037-3209-4a0e-8cf4-d046c452a689', 'description': 'Variant radiographic and CT features of pseudoaneurysm following aortic coarctation repair.\n\nRadiographs (#1-4) shows mediastinal contour abnormality (arrows). CECT (#5-9) shows aneurysm (arrows, #5-7, 9) at site of previous repair. Endograft has been place across the aneurysm (open arrows).', 'history': 'Previous surgical repair aortic coarctation.', 'imagePoolId': '495aa2d3-f56a-4b36-8d58-18d3f07dc939', 'name': 'Pseudoaneurysm', 'teachingPoint': None, 'demographics': '59 Years old female'}, {'authors': [{'key': 'a081f7d3-2c54-4779-846e-0d82cea35329', 'value': 'Alexander Bankier, MD'}], 'caseVersionId': 'b38e9001-0bff-4176-bc09-8d412150b98f', 'description': 'Variant CT features of aortic narrowing from aortic coarctation.\n\nBoth coronal (#1-4) and parasagittal (#5-7) reformations show the manifestations of pseudocoarctation. Narrowing of the aorta (arrow, #5,6) best seen on the parasagittal images, whereas dilatation of the supraaortic branches (open arrow, #3-6) better seen on the coronal images. Note that the descending aorta (curved arrow, #7) is of normal diameter.', 'history': 'Routine screening examination, incidental finding on echocardiography.', 'imagePoolId': 'bc685044-cc2e-44fd-ade9-51ca63fd9fdd', 'name': 'Aortic narrowing', 'teachingPoint': None, 'demographics': '32 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}, {'key': '509cdc73-5701-4807-90e5-cf244eb473c8', 'value': 'Tan-Lucien H. Mohammed, MD, FCCP'}], 'caseVersionId': 'bff521b6-04d4-4e8f-a2c4-2a228a3c096e', 'description': 'Variant CT features of aortic contour from pseudocoarctation.\n\nNECT (#1-5) shows abnormal aortic contour (arrows) from pseudocoarctation. Note absence of collaterals. Sagittal oblique reconstruction (#6) shows notching of proximal aorta (arrow).', 'history': 'Asymptomatic', 'imagePoolId': '2afe9b1b-6942-4464-a01d-77fc985e6730', 'name': 'Pseudocoarctation', 'teachingPoint': None, 'demographics': '45 Years old male'}, {'authors': [{'key': '612e745f-e298-40da-8326-de2c383c4fc1', 'value': 'Jud W. Gurney, MD, FACR'}, {'key': '509cdc73-5701-4807-90e5-cf244eb473c8', 'value': 'Tan-Lucien H. Mohammed, MD, FCCP'}], 'caseVersionId': 'e33d0134-1101-487e-b575-87ef2ca58cf0', 'description': 'Variant CT and MR features of pseudocoarctation with bicuspid aortic valve.\n\nCECTs (#1-3) show a focal deformity of the aorta (open arrows). CECT reconstruction (#4) shows focal buckling of the proximal descending aorta (open arrow). The aorta is not narrowed and there are no collaterals. Bone window (#5) shows faint calcification (curved arrow) of the aortic valve. MR cine (#6) shows the bicuspid aortic valve (curved arrow).', 'history': 'Murmur.', 'imagePoolId': '37b37df3-b20b-43a8-9609-566ca12e4eb2', 'name': 'Pseudocoarctation', 'teachingPoint': None, 'demographics': '47 Years old female'}], 'caseType': 'variant', 'name': 'VARIANT'}

Images

Selected Images

PA radiograph of the chest demonstrates the classic figure 3 morphology in a patient with aortic coarctation. Note the area of stenosis , dilated subclavian artery , and poststenotic dilatation .

Lateral radiograph of the chest in the same patient reveals an indentation along the aortic isthmus, representing the stenosis. While the figure 3 sign is not frequently seen, its presence suggests the diagnosis and should prompt additional evaluation.

Axial CTA of the aorta shows a classic coarctation . Note the relatively normal caliber of the aorta proximal and distal to the critical stenosis. There are extensive mediastinal collaterals seen as serpiginous vessels around the coarctation and dilated internal mammary arteries, which also reflect collateral flow.

Oblique CTA 3D reformation in the same patient shows the coarctation . Note the intercostal arteries , which appear dilated due to collateralization.

PA chest radiographs in 2 different patients with aortic coarctation show an ill-defined mediastinal widening on the left and mediastinal contour abnormality on the right. Visualization of the classic figure 3 sign is often obscured by the presence of mediastinal collaterals.

PA chest radiograph shows an inferior rib notching , the so-called Roesler sign, a classic radiographic sign of aortic coarctation produced by dilation of collateral vasculature. (Courtesy L. Heyneman, MD.)

Axial CTA images at the prestenotic level (left) and the coarctation site (right) show characteristic focal narrowing due to the coarctation and dilated internal mammary and intercostal arteries that serve as collateral pathways.

Oblique CTA MIP reformation in the same patient better delineates the coarctation , poststenotic dilatation of the descending thoracic aorta, tortuous and dilated internal mammary artery , and intercostal collateral arteries .

Volume-rendered 3D CTA in a patient with aortic coarctation allows for morphologic assessment of the coarctation and provides an overall appreciation of the extent of collateralization. 3D reformations are most helpful for clinicians/surgeons to get the overall picture of the 3D configuration of the pathology.

DSA in a patient undergoing angiography for subarachnoid hemorrhage shows the catheter tip proximal to an incidentally detected tight aortic coarctation.

Short-axis SSFP MR through the aortic valve shows a bicuspid aortic valve in a patient with aortic coarctation. Note that the aortic valve has only 2 cusps . This is a common association in patients with aortic coarctation.

Axial SSFP MR through the area of aortic coarctation shows an ascending aorta normal in diameter, a diminutive proximal descending aorta in the area of coarctation , and a relatively normal diameter of the more distal descending aorta .

Sagittal SSFP MR images in a patient with aortic coarctation show a well-defined long-segment area of stenosis in the proximal descending aorta .

Oblique aortic MRA MIP reformation in the same patient shows marked stenosis with extensive regional collaterals resulting from a hemodynamically significant obstruction. MRA is useful (as is CT) for determining the minimal aortic cross-sectional area and for evaluating the pressure gradients and flow volumes.

Candy cane view from MRA of the thoracic aorta in a patient with repaired aortic coarctation shows mild residual narrowing at the aortic isthmus.

4D-flow image of the same patient shows mild increased velocity at the repaired coarctation site, indicated in red. 4D flow images allow for quantification of flow at multiple levels, including valve levels, and also visually demonstrate flow patterns.

Short-axis SSFP cine MR in this patient with coarctation shows concentric thickening of the left ventricular myocardium , consistent with left ventricular hypertrophy. This is a sequela from longstanding upper body arterial hypertension.

Anterior and posterior MIP views of sagittal aortic MRA demonstrate coarctation and extensive chest wall and mediastinal collaterals . Collateral vessels are better characterized when thin-slice images (e.g., MRA) are reformatted to MIPs.

This axial phase-contrast MR is from a patient with Shone complex that includes coarctation. While this sequence does not provide good morphologic correlation, it allows calculation of flow velocities and volumes over time, which may be used to quantify heart rate-corrected deceleration time.

3D volume-rendered MRA in the same patient shows coarctation with poststenotic dilatation. Shone complex includes coarctation, supravalvular mitral ring, parachute mitral valve, and subaortic stenosis.

This oblique sagittal CTA of the aorta is from a patient with coarctation who underwent successful endovascular stent placement after a failed treatment with angioplasty. CT and MR allow for follow-up and assessment of stent complications.

Oblique sagittal CTA images in a patient with remote history of surgically corrected aortic coarctation show aneurysmatic dilatation of the left subclavian artery and the proximal descending aorta , a known complication after this type of surgery.

Additional Images

Sagittal graphic shows high-grade, short segmental narrowing of the thoracic aorta distal to the ductus arteriosus.

PA chest radiograph shows barium in the esophagus and the characteristic figure 3 sign in a patient diagnosed with aortic coarctation.

PA chest radiograph (coned down to the left lung) shows left-sided rib notching at multiple levels . The patient was subsequently diagnosed with coarctation of the aorta.

Sagittal oblique CECT shows short-segment, high-grade coarctation involving the proximal descending aorta . This patient had left upper extremity hypertension.

Sagittal CECT of a patient with aortic coarctation shows circumscribed high-grade narrowing of the proximal descending thoracic aorta.

Sagittal NECT shows high-grade stenosis of the proximal descending aorta of a 27-year-old patient with coarctation. Elongation of the supraaortic vessels is also visible.

Sagittal oblique T1 C+ FS MR shows focal narrowing of the proximal descending thoracic aorta. Turbid flow is seen as hypointensity distal to the narrowing .

Sagittal oblique CECT MIP of a 19-year-old man after repair of aortic coarctation shows an endovascular stent in the proximal descending aorta.

Frontal radiograph of the chest in a patient with aortic coarctation is shown. Note that, despite cardiomegaly, the mediastinum does not appear widened but rather has ill-defined borders. Often, the radiographic findings are nonspecific. In this case, the hazy mediastinal borders are due to mediastinal collaterals.

Oblique sagittal SSFP MR in the same patient demonstrates a marked stenosis of the proximal descending aorta in keeping with aortic coarctation.

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KEY FACTS

Terminology

Imaging

Top Differential Diagnoses

Pathology

Clinical Issues

Diagnostic Checklist

TERMINOLOGY

Abbreviations

Definitions

IMAGING

General Features

Location

Size

Radiographic Findings

CT Findings

CTA

MR Findings

Echocardiographic Findings

Echocardiogram

Angiographic Findings

Imaging Recommendations

Best imaging tool

Protocol advice

DIFFERENTIAL DIAGNOSIS

Interrupted Aortic Arch

Inferior Rib Notching Differential

PATHOLOGY

General Features

Etiology

Genetics

Associated abnormalities

Staging, Grading, & Classification

Gross Pathologic & Surgical Features

Microscopic Features

CLINICAL ISSUES

Presentation

Most common signs/symptoms

Other signs/symptoms

Demographics

Age

Sex

Ethnicity

Epidemiology

Natural History & Prognosis

Treatment

DIAGNOSTIC CHECKLIST

Consider

Image Interpretation Pearls

References

Selected References

Differential diagnosis

Left Ventricular Enlargement

Left Ventricular Enlargement

Narrowed Aorta

Cases

Images

Selected Images

Additional Images

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