254 lines
14 KiB
Markdown
254 lines
14 KiB
Markdown
---
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title: "Persistent Fifth Arch"
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docid: "931c64bc-e8a0-4a99-848b-1429f3c1d500"
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authors:
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- key: "9fea2857-d729-4fe4-b4fd-3b7bf1db23cf"
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value: "Mortadha Al-Kinani, MD, MBChB"
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- key: "3d1e4c57-c1cf-4c89-b0f0-5d82b29a31e1"
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value: "Suhny Abbara, MD, FACR, MSCCT, FNASCI"
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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: "Persistent Fifth Arch"
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slug: "persistent-fifth-arch"
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treeNodeId: null
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category: "Cardiac"
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documentVersionId: "2c81f654-df79-4360-b8b4-b5042f45e8e5"
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imageCount: 4
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lastUpdated: "01/24/25"
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pageDescription: "Persistent Fifth Arch"
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pageKeywords: "Cardiac, Diagnosis, Aorta, Persistent Fifth Arch"
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pageTitle: "Persistent Fifth Arch | STATdx"
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enhancedTitle: "Persistent Fifth Arch"
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type: "DX"
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references: true
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cases: 1
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breadcrumbs:
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- "Cardiac"
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- "Diagnosis"
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- "Aorta"
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- "Persistent Fifth Arch"
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---
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# KEY FACTS
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- ## Terminology
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- Rare congenital vascular anomaly
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- May be isolated or associated with other abnormalities
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- Complex congenital cardiac heart disease
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- Vascular anomalies
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- Skeletal anomalies
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- 2 distinct forms
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- **Systemic-to-systemic connection**: 5th arch arises at brachiocephalic trunk and reconnects at descending aorta
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- **Systemic-to-pulmonary connection**: 5th arch connects with embryologic remnant of 6th aortic arch, which is usually left pulmonary artery
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- ## Imaging
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- Contrast-enhanced MRA most appropriate in children with suspected persistent 5th arch
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- Short segment of duplication aortic arch with 2 parallel distinct lumina in systemic-to-systemic connection
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- Abnormal vessel connecting aorta with isolated pulmonary artery in systemic-to-pulmonary connection
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- ## Clinical Issues
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- Presentation depends on which type of connections exists and on associated cardiac and vascular anomalies
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- Associated cardiovascular anomalies
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- Ventricular septal defect (most common)
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- Pulmonic valve or artery stenosis/atresia
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- Interruption of aortic arch
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- Coarctation of aorta
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- Transposition of great arteries
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- Pentalogy of Fallot
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- Patent ductus arteriosus
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- Tricuspid atresia
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- In case of coarctation/obstruction, surgical patching or conduit interposition may be indicated
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# TERMINOLOGY
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- ## Synonyms
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- Ipsilateral double aortic arch
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- Double lumen aortic arch
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- ## Definitions
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- Rare congenital vascular anomaly of aortic arch
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- May be isolated
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- Often associated with other congenital cardiac, vascular, or skeletal anomalies
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- 2 distinct forms
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- Systemic-to-systemic connection
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- 5th arch arises at brachiocephalic trunk and reconnects at descending aorta
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- Systemic-to-pulmonary connection
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- 5th arch connects with embryologic remnant of 6th aortic arch, which is usually left pulmonary artery
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# IMAGING
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- ## General Features
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- ### Best diagnostic clue
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- Short segment of duplication of aortic arch with 2 parallel distinct lumina
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- May have interrupted arch
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- Abnormal vessel connecting aorta with pulmonary artery
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- ### Location
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- Aortic arch
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- Cephalad of arches is 4th arch, which gives rise to arch vessels
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- Lower arch is persistent 5th arch
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- ## Radiographic Findings
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- ### Radiography
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- May demonstrate associated findings, such as vertebral anomalies
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- ## CT Findings
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- ### CTA
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- 2 distinct left aortic arches with what may appear as septation separating them
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- Double-barrel appearance on coronal oblique short-axis views
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- May have interrupted arch
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- May show anomalous connection between aorta and isolated left pulmonary artery
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- May demonstrate associated cardiovascular and skeletal abnormalities
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- ## MR Findings
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- Same as CTA findings
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- Superior to echocardiography due to acoustic window restrictions near aortic arch
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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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- Contrast-enhanced MRA most appropriate in children with suspected persistent 5th arch
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# DIFFERENTIAL DIAGNOSIS
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- [Aortic Dissection](/document/aortic-dissection/57e3428e-1f18-4f38-95c6-f7fe2d93c00a)
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- Easily differentiated by double-barrel appearance of persistent 5th arch: 2 round lumina form "figure of 8" on arch short-axis views
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- [Patent Ductus Arteriosus](/document/patent-ductus-arteriosus/5ba3261d-bd13-4542-92ec-5db5274e2050)
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- Systemic-to-pulmonary connection may mimic large PDA
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- PDA would communicate distally to arch arteries
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- ## Aortopulmonary Window
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- Abnormal connection between proximal aorta and pulmonary trunk
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# CLINICAL ISSUES
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- ## Presentation
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- ### Most common signs/symptoms
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- Often presents soon after birth due to associated cardiac or vascular defects
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- Ventricular septal defect
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- Pulmonic valve or artery stenosis/atresia
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- Interruption of aortic arch
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- Complex congenital heart disease
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- Often present after birth due to associated cardiac or vascular defects
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- Ventricular septal defect (most common), pulmonic valve or artery stenosis/atresia, tricuspid atresia
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- Interruption of aortic arch, coarctation of aorta, transposition of great arteries, persistent truncus arteriosus, PDA
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- Could be hemodynamically beneficial
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- Systemic-to-systemic connection; if associated with coarctation of aorta or interrupted aortic arch
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- Systemic-to-pulmonic connection; when associated with pulmonary or tricuspid atresia
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- ### Clinical profile
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- Association with intrauterine thalidomide exposure and chromosomal disorders
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- Weinberg classification defines 3 types (A,B,C)
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- Type A: Double-lumen aortic arch (arch vessels arise from upper 4th; lower is 5th arch)
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- Type B: Single-lumen arch; 4th arch is interrupted; 5th arch originates from ascending and connects to descending aorta
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- Type C: 5th originates from proximal brachiocephalic artery off of ascending aorta and connecting to pulmonary artery via 6th arch
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- Associated cardiovascular anomalies include
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- Coarctation
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- Pulmonary atresia or stenosis
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- Transposition of great arteries
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- Truncus arteriosus
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- Pentalogy of Fallot
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- PDA, ventricular septal defect
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- Tricuspid atresia
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- ## Demographics
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- ### Epidemiology
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- Extremely rare congenital malformation
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- ## Treatment
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- In case of coarctation/obstruction, surgical patching or conduit interposition may be indicated
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# DIAGNOSTIC CHECKLIST
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- ## Consider
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- May have obstruction due to associated coarctation
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- Check BP difference between upper and lower extremities (BP in both arms)
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597b11ca-b733-4d2c-8265-416a2b493b11
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## References
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# Selected References
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1. [Shan H et al: Persistent fifth aortic arch: a comprehensive literature review. Front Pediatr. 11:1183345, 2023](http://www.ncbi.nlm.nih.gov/pubmed/?term=37435167%5Bpmid%5D)
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1. [Liu Y et al: Persistent fifth aortic arch: a single-center experience, case series. Transl Pediatr. 10(6):1566-72, 2021](http://www.ncbi.nlm.nih.gov/pubmed/?term=34295771%5Bpmid%5D)
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1. [Kligerman S et al: Persistent fifth aortic arch in a patient with a history of intrauterine thalidomide exposure. J Cardiovasc Comput Tomogr. 3(6):412-4, 2009](http://www.ncbi.nlm.nih.gov/pubmed/?term=19709946%5Bpmid%5D)
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1. [Kirsch J et al: Magnetic resonance angiography of an ipsilateral double aortic arch due to persistent left fourth and fifth aortic arches. Pediatr Radiol. 37(5):501-2, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17415604%5Bpmid%5D)
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1. [Zhao YH et al: Surgical treatment of persistent fifth aortic arch associated with interrupted aortic arch. Ann Thorac Surg. 84(3):1016-9, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17720425%5Bpmid%5D)
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1. [Zhong Y et al: Contrast-enhanced magnetic resonance angiography of persistent fifth aortic arch in children. Pediatr Radiol. 37(3):256-63, 2007](http://www.ncbi.nlm.nih.gov/pubmed/?term=17200843%5Bpmid%5D)
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1. [Hwang MS et al: Isolated persistent fifth aortic arch with systemic-to-pulmonary arterial connection. J Thorac Cardiovasc Surg. 126(5):1643-4, 2003](http://www.ncbi.nlm.nih.gov/pubmed/?term=14666049%5Bpmid%5D)
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## Cases
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- {'cases': [{'authors': [{'key': 'b00d2bdb-66e1-41ed-90b4-c52904f4d598', 'value': 'Seth Kligerman, MD, MS'}], 'caseVersionId': '33e370c1-f5d0-4136-8a26-f4f065378eab', 'description': "Coronal CT angiograms through the chest (Figs. 1-3) and sagittal oblique volume-rendered image (Fig. 4) with a posterior view of the aorta show the transverse aorta bifurcating into the superior <img src='img/arrows/CO.png'/>and inferior <img src='img/arrows/WO.png'/> channels just proximal to the level of the left brachiocephalic artery (<img src='img/arrows/CC.png'/>, Fig. 4) and again merging just distal to the left subclavian artery (<img src='img/arrows/WC.png'/>, Figs. 3-4). The more inferior arch is the persistent 5th arch. All of the arch vessels, including the left common carotid artery (<img src='img/arrows/CS.png'/>, Figs. 1, 4,), arise from the true arch. Volume-rendered 3-D cine clip (Vid. 1) again shows the bifurcation.", 'history': 'Patient with multiple congenital bony anomalies and a history of intrauterine thalidomide exposure undergoes preoperative CT.', 'imagePoolId': '8123c1a9-ea90-4684-985e-d9c873cc0c2d', 'name': 'Persistent Fifth Arch', 'teachingPoint': 'Persistence of the 5th aortic arch is an exceedingly rare anomaly. There are 2 mains forms. In the systemic to systemic form, the arch connects the ascending and descending aorta. In the systemic to pulmonary form, the 5th arch connects to a pulmonary artery, usually the left.', 'demographics': '45 Years old male'}], 'caseType': 'typical', 'name': 'TYPICAL'}
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## Images
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### Selected Images
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*Aortic arch "candy cane" view of CTA shows separation of the aortic arch into 2 distinct vessels (*). The superior is the normal 4th aortic arch giving rise to the arch vessels. The inferior is the persistent 5th aortic arch.*
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*Aortic arch "candy cane" view of CTA shows separation of the aortic arch into 2 distinct vessels (*). The superior is the normal 4th aortic arch giving rise to the arch vessels. The inferior is the persistent 5th aortic arch.*
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*Coronal CTA in the same patient shows the short axis of aortic arches with a double-barrel appearance (*). The 4th and persistent 5th arch have a figure of 8 configuration in the short axis, which allows differentiation from dissection.*
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*Oblique 3D volume-rendered reconstruction shows the relationship of the 4th arch <img src='img/arrows/WC.png'/> with the arch vessels. Note the abnormal persistent 5th arch <img src='img/arrows/WS.png'/> arising from the aorta at the level of the brachiocephalic trunk and reentering into the descending thoracic aorta at its isthmus.*
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*Oblique 3D reconstruction of the skull in the same patient shows a cleft palate <img src='img/arrows/WC.png'/>. Other skeletal anomalies in this patient include fused ribs and hemi vertebra (not shown).*
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