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---
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title: "Interrupted/Hypoplastic Aorta"
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docid: "100ba59a-24e8-47b4-b2b2-bbfdc0a21e92"
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authors:
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- key: "770e1d77-2287-436e-910b-48232afc7842"
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value: "Prabhakar Rajiah, MBBS, MD, FACR, FRCR, FACC, FAHA, FSCCT"
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- key: "e915766e-8102-46e4-a33e-c83f8ae12f29"
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value: "Harold Goerne, MD"
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breadcrumbs:
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-
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name: "Cardiac"
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slug: "cardiac"
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treeNodeId: "fa90100b-619c-430e-8074-b5b9789bab39"
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-
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name: "Diagnosis"
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slug: "diagnosis"
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treeNodeId: "5c92cf4f-e9d5-4059-9c13-22255c51c121"
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-
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name: "Aorta"
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slug: "aorta"
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treeNodeId: "4d206a6b-1a82-467c-9199-0df25ab749d7"
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-
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name: "Interrupted/Hypoplastic Aorta"
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slug: "interruptedhypoplastic-aorta"
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treeNodeId: null
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category: "Cardiac"
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documentVersionId: "00b8d951-9dbc-40d4-9499-c8705f77762e"
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imageCount: 15
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lastUpdated: "01/28/25"
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pageDescription: "Interrupted/Hypoplastic Aorta"
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pageKeywords: "Cardiac, Diagnosis, Aorta, Interrupted/Hypoplastic Aorta"
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pageTitle: "Interrupted/Hypoplastic Aorta | STATdx"
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enhancedTitle: "Interrupted/Hypoplastic Aorta"
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type: "DX"
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references: true
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breadcrumbs:
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- "Cardiac"
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||||
- "Diagnosis"
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- "Aorta"
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- "Interrupted/Hypoplastic Aorta"
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---
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# KEY FACTS
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- ## Terminology
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- Interrupted aortic arch
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- Complete luminal and anatomic wall discontinuity between ascending aorta and descending aorta
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- Hypoplastic aortic arch
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- Tubular narrowing without luminal discontinuity
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- ## Imaging
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- CTA
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- Best noninvasive modality to assess aortic arch and supraaortic vessels pattern
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- US
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- 1st-line modality to assess aortic arch abnormalities
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- Best diagnostic clue
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- Interrupted aortic arch
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- Blind ends in distal ascending aorta and proximal descending aorta with luminal discontinuity in between
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- Hypoplastic aortic arch
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- Tubular narrowing of aortic arch; proximal aortic arch < 60% &/or distal aortic arch < 50% of diameter of ascending aorta
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- ## Top Differential Diagnoses
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- Coarctation of aorta
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- ## Pathology
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- Type A (13%)
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- Interruption distal to left subclavian artery
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- Type B (84%)
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- Interruption between left common carotid artery and left subclavian artery
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- Type C (3%)
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- Interruption between brachiocephalic trunk and left common carotid artery
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- ## Clinical Issues
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- Rare condition
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- 1% of congenital heart disease
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# TERMINOLOGY
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- ## Abbreviations
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- Interrupted aortic arch (IAA)
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- Hypoplastic aortic arch (HAA)
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- ## Synonyms
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- Atresia of aortic arch
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- ## Definitions
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- IAA
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- Complete luminal discontinuity between ascending aorta and descending aorta
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- Not true interruption, as there is fibrotic continuity between blind ends
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- HAA
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- Tubular narrowing of aortic arch without luminal discontinuity
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# IMAGING
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- ## General Features
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- ### Best diagnostic clue
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- IAA
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- Blind-ending distal ascending aorta and proximal descending aorta with luminal discontinuity in between
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- HAA
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- Tubular narrowing of aortic arch
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- Proximal arch < 60% &/or distal aortic arch < 50% of diameter of ascending aorta
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- Transverse aortic arch z-score usually < -3
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- ### Location
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- Aortic arch: Proximal or distal
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- ### Size
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- Variable
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- ### Morphology
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- IAA: Complete anatomic discontinuity between ascending aorta and descending aorta
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- HAA: Tubular narrowing of aortic arch
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- ## CT Findings
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- ### CTA
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- Interruption: Blind ending ascending and descending aorta without luminal opacification of arch
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- Type, site, and length of interruption
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- Type A: Distal to left subclavian artery
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- Type B: Between left common carotid and subclavian arteries
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- Type C: Between right brachiocephalic and left common carotid arteries
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- Hypoplasia: Small caliber of aortic arch
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- Best modality for measuring vessel diameters
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- Best noninvasive modality to assess aortic arch and supraaortic vessels pattern
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- New-generation scanners provide faster acquisition without sedation or ECG gating
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- High-resolution 3D reconstructions provides roadmap for surgery
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- ## MR Findings
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- ### MRA
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- High-resolution images of aorta without radiation; can be performed ± contrast
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- Types of interrupted arch
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- Shows diameters of each aortic segment and length of HAA
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- Hypoplasia: External diameter of proximal arch, distal arch, or isthmus measuring < 60%, < 50%, or < 40% of that of ascending aorta
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- Transverse aortic arch z-score usually < -3
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- This assumes that ascending aorta diameter is normal
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- Dynamic MRA shows multiple vascular phases, providing information about aorta, pulmonary arteries and veins, and systemic venous return pattern with single gadolinium injection
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- Associated anomalies can be evaluated
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- ### MR cine
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- Biventricular function assessment, including ejection fraction, end-diastolic, and end-systolic volumes
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- Wall motion abnormalities
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- Evaluation of additional intracardiac abnormalities (such as septal defects)
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- ## Ultrasonographic Findings
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- 1st modality to assess aortic arch abnormalities in children
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- Define true interruption or HAA and type of IAA by looking at pattern of supraaortic trunks
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- Associated patent ductus arteriosus (PDA)
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- Size, flow, aortic and pulmonary ostial diameters
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- Associated cardiac abnormalities: Atrial septal defect (ASD), ventricular septal defect (VSD), left ventricular outflow tract (LVOT) obstruction, aorticopulmonary window defect
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- ## Imaging Recommendations
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- ### Best imaging tool
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- CTA or MRA
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- ### Protocol advice
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- Newborn and infants: Contrast injection based on body weight
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- Bolus tracking position and HU threshold are variable and depend on scanner speed to start acquisition
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- Newborn and infants: Acquisition can be started immediately after contrast injection
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# DIFFERENTIAL DIAGNOSIS
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- [Coarctation of Aorta](/document/coarctation-of-aorta/c0b23d8c-05e3-4373-b5d9-2de1590414a7)
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- Focal narrowing at aortic isthmus
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- Same location as type A interruption
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- Usually short segment of luminal narrowing
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- No complete loss of continuity, like interruption
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- Occasionally, extremely tight stenosis may be seen
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- More pronounced poststenotic dilation
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- In interruption, arch is smaller caliber, and branch vessels are straighter than normal
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- With advanced cases, distinguishing features may disappear
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- ## Focal Atresia of Aortic Arch
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- Most common at aortic isthmus, similar to type A interruption
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- Lumen is interrupted, but aortic wall is present
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- Fibrous strand between ascending and descending aorta
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# PATHOLOGY
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- ## General Features
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- ### Etiology
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- Type A
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- Abnormal regression of left 4th aortic arch late in development after left subclavian artery is in position
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- Reduced blood flow through 4th aortic arch during embryologic phase
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- Insufficient development of aortic arch with spectrum from coarctation to atresia to IAA
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- Conal septum not malaligned or deviated; no subaortic stenosis
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- Type B
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- High association with chromosome 22q11.2 microdeletion
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- Abnormal regression of left 4th arch, early in development, before cephalad migration of left subclavian artery
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- Malalignment of infundibular septum with muscular septum → LVOT narrowing → decreased growth, hypoplasia, and interruption of arch due to absolute decrease in cardiac output
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- Type C
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- Abnormal regression of ventral portion of left 3rd and 4th arches
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- ### Genetics
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- 50% of patients with IAA have chromosome 22q11.2 deletion
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- 42% of patients with DiGeorge syndrome have IAA
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- ### Associated abnormalities
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- IAA
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- PDA is essential for life in all patients (seen in 97% of cases)
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- VSD in 90% of IAA
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- Other congenital heart abnormalities are present in 98%
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- Subaortic stenosis
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- Bicuspid aortic valve
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- Truncus arteriosus
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- Aortopulmonary window
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- Transposition of great arteries
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- Double-outlet right ventricle
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- Functional single ventricle
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- Persistent 5th arch
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- Anomalous origin of subclavian artery
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- HAA
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- ASD
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- VSD
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- PDA
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- ## Staging, Grading, & Classification
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- Type A (13%)
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- Interruption distal to left subclavian artery
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- Type B (84%)
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- Interruption between left common carotid artery and left subclavian artery
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- Type C (3%)
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- Interruption between right brachiocephalic trunk and left common carotid artery
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- In any of these types, 3 subtypes may be seen depending on origin of right subclavian artery
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- Subtype 1: Normal subclavian artery origin
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- Subtype 2: Aberrant right subclavian artery distal to origin of left subclavian artery
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- Subtype 3: Isolated right subclavian artery originating from right ductus arteriosus
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# CLINICAL ISSUES
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- ## Presentation
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- ### Most common signs/symptoms
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- Differential cyanosis (ductal right-to-left shunt)
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- Type A
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- Normal saturation in both arms and head, desaturated legs
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- Type B
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- Normal saturation in right arm and head, desaturated left arm and legs
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- Type C
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- Normal saturation in right arm and right carotid artery, desaturated left carotid artery, left arm and legs
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- ### Other signs/symptoms
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- When ductus arteriosus begins to close, neonate develops signs of hypoperfusion and cardiogenic shock
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- Death usually occurs 4-10 days after closure of ductus arteriosus
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- By 1 month, 76% of untreated infants are dead; by 1 year, > 90% are dead
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- ## Demographics
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- ### Age
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- Neonates
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- ### Sex
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- Male patients: 59%
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- Female patients: 41%
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- ### Epidemiology
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- Rare condition
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- 1% of congenital heart disease
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- 2/100,000 live births
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- ## Natural History & Prognosis
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- When untreated and ductus arteriosus closes, distal hypoperfusion leads to renal failure, lactic acidosis, and eventually death in few days
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- ## Treatment
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- Surgical correction is only treatment; goal is to establish continuity in aortic arch
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- Prostaglandin E₁ is given to maintain patency of ductus arteriosus until neonate is stable for surgical correction
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4e578d09-68e6-4af7-8a56-23527ab96783
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## References
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# Selected References
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1. [Evans WN et al: Prenatal diagnosis of hypoplastic aortic arch without intracardiac malformations: the nevada experience. J Card Surg. 37(11):3705-10, 2022](http://www.ncbi.nlm.nih.gov/pubmed/?term=36047366%5Bpmid%5D)
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1. [LaPar DJ et al: Surgical considerations in interrupted aortic arch. Semin Cardiothorac Vasc Anesth. 22(3):278-84, 2018](http://www.ncbi.nlm.nih.gov/pubmed/?term=29774793%5Bpmid%5D)
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1. [Hanneman K et al: Congenital variants and anomalies of the aortic arch. Radiographics. 37(1):32-51, 2017](http://www.ncbi.nlm.nih.gov/pubmed/?term=27860551%5Bpmid%5D)
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1. [Goudar SP et al: Echocardiography of coarctation of the aorta, aortic arch hypoplasia, and arch interruption: strategies for evaluation of the aortic arch. Cardiol Young. 26(8):1553-62, 2016](http://www.ncbi.nlm.nih.gov/pubmed/?term=28148317%5Bpmid%5D)
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1. [Roubertie F et al: Aortopulmonary window and the interrupted aortic arch: midterm results with use of the single-patch technique. Ann Thorac Surg. 99(1):186-91, 2015](http://www.ncbi.nlm.nih.gov/pubmed/?term=25440264%5Bpmid%5D)
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1. [Ramos-Duran L et al: Developmental aortic arch anomalies in infants and children assessed with CT angiography. AJR Am J Roentgenol. 198(5):W466-74, 2012](http://www.ncbi.nlm.nih.gov/pubmed/?term=22528928%5Bpmid%5D)
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1. [Hellinger JC et al: Congenital thoracic vascular anomalies: evaluation with state-of-the-art MR imaging and MDCT. Radiol Clin North Am. 49(5):969-96, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=21889017%5Bpmid%5D)
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1. [Frank L et al: Cardiovascular MR imaging of conotruncal anomalies. Radiographics. 30(4):1069-94, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20631369%5Bpmid%5D)
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1. [Kimura-Hayama ET et al: Uncommon congenital and acquired aortic diseases: role of multidetector CT angiography. Radiographics. 30(1):79-98, 2010](http://www.ncbi.nlm.nih.gov/pubmed/?term=20083587%5Bpmid%5D)
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1. [Dillman JR et al: Interrupted aortic arch: spectrum of MRI findings. AJR Am J Roentgenol. 190(6):1467-74, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=18492893%5Bpmid%5D)
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1. [Yang DH et al: Multislice CT angiography of interrupted aortic arch. Pediatr Radiol. 38(1):89-100, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=17965856%5Bpmid%5D)
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1. [Loffredo CA et al: Interrupted aortic arch: an epidemiologic study. Teratology. 61(5):368-75, 2000](http://www.ncbi.nlm.nih.gov/pubmed/?term=10777832%5Bpmid%5D)
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1. [Kaulitz R et al: Echocardiographic assessment of interrupted aortic arch. Cardiol Young. 9(6):562-71, 1999](http://www.ncbi.nlm.nih.gov/pubmed/?term=10593265%5Bpmid%5D)
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1. [Reardon MJ et al: Interrupted aortic arch: brief review and summary of an eighteen-year experience. Tex Heart Inst J. 11(3):250-9, 1984](http://www.ncbi.nlm.nih.gov/pubmed/?term=15227058%5Bpmid%5D)
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1. [CELORIA GC et al: Congenital absence of the aortic arch. Am Heart J. 58:407-13, 1959](http://www.ncbi.nlm.nih.gov/pubmed/?term=13808756%5Bpmid%5D)
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## Images
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### Selected Images
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*Posterior oblique CTA cinematic rendering shows type B interrupted aortic arch <img src='img/arrows/CC.png'/> with an aberrant right subclavian artery <img src='img/arrows/WS.png'/>. Also note the collateral vessel <img src='img/arrows/WO.png'/> with right carotid artery <img src='img/arrows/WC.png'/>. Left carotid artery <img src='img/arrows/CS.png'/> and left subclavian artery (LSA) <img src='img/arrows/CO.png'/> are also shown.*
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*Posterior oblique CTA cinematic rendering shows type B interrupted aortic arch <img src='img/arrows/CC.png'/> with an aberrant right subclavian artery <img src='img/arrows/WS.png'/>. Also note the collateral vessel <img src='img/arrows/WO.png'/> with right carotid artery <img src='img/arrows/WC.png'/>. Left carotid artery <img src='img/arrows/CS.png'/> and left subclavian artery (LSA) <img src='img/arrows/CO.png'/> are also shown.*
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*Anterosuperior oblique CTA cinematic rendering shows type B interrupted aortic arch (IAA) with aberrant right subclavian artery <img src='img/arrows/WS.png'/>. Patent ductus arteriosus (PDA) <img src='img/arrows/WO.png'/> provides blood flow to both subclavian arteries and the distal descending aorta (DA).*
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*Sagittal oblique MIP CTA shows a hypoplastic aortic arch <img src='img/arrows/WS.png'/> and PDA <img src='img/arrows/WO.png'/>.*
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||||
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*Sagittal oblique CTA cinematic rendering in the same patient shows a hypoplastic aortic arch <img src='img/arrows/WS.png'/> and PDA <img src='img/arrows/WO.png'/>. CT is the best noninvasive imaging modality to assess aortic arch and supraaortic vessel patterns as well as to measure vessel diameters.*
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*Anterosuperior oblique CTA cinematic rendering shows type B IAA. The brachiocephalic trunk (BCT) <img src='img/arrows/WS.png'/> and left carotid artery <img src='img/arrows/WO.png'/> originate from the proximal aorta. The LSA <img src='img/arrows/WC.png'/> originates from the ductal arch.*
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||||
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*Sagittal oblique MIP CTA shows type B IAA <img src='img/arrows/CS.png'/>. The BCT <img src='img/arrows/WS.png'/> and left common carotid artery (LCC) <img src='img/arrows/WO.png'/> originate from the proximal aorta. The LSA <img src='img/arrows/WC.png'/> originates from the ductal arch.*
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*CTA cinematic rendering demonstrates type B IAA. The LSA <img src='img/arrows/WS.png'/> originates from the DA <img src='img/arrows/CS.png'/>. The BCT and LCC <img src='img/arrows/WO.png'/> originate from the proximal ascending aorta (AA). The PDA <img src='img/arrows/WC.png'/> provides blood flow to both the LSA and DA.*
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*Sagittal oblique CTA cinematic rendering shows a hypoplastic distal aortic arch <img src='img/arrows/WS.png'/> involving the isthmus and proximal DA <img src='img/arrows/WO.png'/>.*
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*Neonate with type A IAA shows AA terminating as the BCT <img src='img/arrows/CS.png'/>, LCC <img src='img/arrows/CO.png'/>, and LSA <img src='img/arrows/CC.png'/> (left). Notice the AP window defect <img src='img/arrows/BO.png'/> between the AA and main pulmonary artery (MPA) <img src='img/arrows/BC.png'/>. A PDA <img src='img/arrows/WO.png'/> supplies the DA <img src='img/arrows/WS.png'/>. PA branches <img src='img/arrows/BS.png'/> are visible.*
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*Coronal (left) and sagittal (right) images in a neonate with type B IAA show the AA <img src='img/arrows/CS.png'/> terminating as the BCT <img src='img/arrows/CC.png'/> and LCC <img src='img/arrows/CO.png'/>. The LSA <img src='img/arrows/WO.png'/> and DA <img src='img/arrows/WS.png'/> are supplied by a PDA <img src='img/arrows/WC.png'/>. PA branches <img src='img/arrows/BS.png'/> arise from the MPA. (Courtesy S. Kligerman, MD.)*
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||||
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||||
### Additional Images
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||||
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||||
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||||
*CTA cinematic rendering demonstrates a hypoplastic aortic arch <img src='img/arrows/WS.png'/> with severe coarctation <img src='img/arrows/WO.png'/>. Note the decreased diameter of the aortic arch due to hypoplasia.*
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||||
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||||
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||||
*Sagittal oblique MIP CTA in the same patient demonstrates decreased diameter of the aortic arch due to a hypoplastic aortic arch <img src='img/arrows/WS.png'/> and associated severe aortic coarctation <img src='img/arrows/WO.png'/>.*
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||||
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||||
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*Coronal (left) and sagittal (right) oblique images in a 1-day-old with type B IAA show a hypoplastic ascending aorta <img src='img/arrows/CS.png'/> terminating as the right common carotid <img src='img/arrows/CC.png'/> and left common carotid <img src='img/arrows/CO.png'/> arteries. The PDA <img src='img/arrows/BO.png'/> supplies the descending thoracic aorta (DTA) <img src='img/arrows/WS.png'/>. A portion of the LSA <img src='img/arrows/BS.png'/> is seen from the DTA.*
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*Coronal image in the same patient shows that an aberrant right subclavian artery <img src='img/arrows/WO.png'/> arises from the DTA distal to the LSA <img src='img/arrows/BS.png'/>, making this a type B IAA, subtype 2.*
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*3D image in a neonate with type B IAA shows the ascending aorta terminating as the right BCT <img src='img/arrows/CO.png'/> and LCC <img src='img/arrows/CC.png'/>. The PDA <img src='img/arrows/BO.png'/> supplies the descending thoracic aorta <img src='img/arrows/WC.png'/> and LSA <img src='img/arrows/CS.png'/>. (Courtesy S. Kligerman, MD.)*
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Reference in New Issue
Block a user