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VASCULAR RINGS & SLINGS

DR. NGUYEN HOANG LINH CHICARDIOVASCULAR DEPARTMENTVIETNAM NATIONAL HOSPITAL OF PEDIATRICS

EMBRYOLOGYDevelops during 3rd -8th week of the fetal lifeVentral aorta partly fuse Aortic sacUnfused parts remain as Rt & Lt horns of the sacThe first arteries to appear in the embryo Right & left primitive aortaContinuous with endocardial tubeEach one dorsal & ventral portion

EMBRYOLOGYSix pairs of arterial arches appear, connecting the dorsal & ventral aortaAll are not present at the same timeSelective regression & persistance of these arch vessels forms the major arteries of head, neck & thorax

EMBRYOLOGYThe 1st, 2nd, 5th arches disappear- 1st arch : dissappeared, small portion maxillary artery2nd arch : dissappeared, remain portion hyoid + stapedial arteries 5th arch : never forms/form incompletetly regresses

EMBRYOLOGY3rd arch Common carotid artery4th arch :Right : proximal part of RSALeft: part of aortic arch between LCA & LSA6th arch Pulmonary arteries & Ductus arteriosusRight side: + Proximal part proximal segment of RPA + Distal portion dissapearsLeft side: + Proximal part proximal segment of LPA + Distal part : forms ductus arterious

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EMBRYOLOGYAortic arch + Proximal segment from aortic sac + Middle segment from the Lt 4th arch arch + Distal segment from the Lt dorsal aortic

EMBRYOLOGYBrachiocephalic A Rt horn of the aortic sacRt subclavian : + Rt 4th arch artery + Rt dorsal aorta + 7th intersegmental ALt subclavian : 7th intersegmental A

NORMAL ANATOMY

VASCULAR RINGDEFINITION Vascular ring is a congenital anomaly in which the aortic arch and its branches completely or incompletely encircle and compress the trachea or esophagus or both

INCIDENCE:Based on surgical case series True prevalence : ascertain ( ~ 1-3% CHD) Double aortic arch : most commonRt Aortic arch (RAA) & aberrant LtSA

VASCULAR RINGCLASSIFICATIONComplete Vascular RingDouble aortic ringRt aortic arch (RAA) & retroesophageal component + Retroesophageal LSA & ligamentum/ductus arteriosum + Mirror-image branching & retroesophageal ligamentum arteriosum + Retroesophageal Lt brachiocephalic artery Lt aortic arch (LAA) & Rt DAo & Rt PDA/ligamenttum arteriosusCervical aortic arch complexIncomplete Vascular RingLt aortic arch & Retroesophageal RSATracheal compression brachiocephalic or Lt common carotid arteryDuctus arteriosus slingMalrotation of heart & PDAPulmonary artery Sling

VASCULAR RINGKEY FEATURES Arch locationBranching of aortaCompression of airwayDiverticulum of KommereldEscending aorta

VASCULAR RING

VASCULAR RINGCLASSIFICATIONComplete Vascular RingDouble aortic ringRt aortic arch (RAA) & retroesophageal component + Retroesophageal LSA & ligamentum arteriosum + Mirror-image branching & retroesophageal ligamentum arteriosum + Retroesophageal Lt brachiocephalic artery Lt aortic arch (LAA) & Rt DAo & Rt PDA/ligamenttum arteriosusCervical aortic arch complexIncomplete Vascular RingLt aortic arch & Retroesophageal RSATracheal compression b brachiocephalic or Lt common carotid arteryDuctus arteriosus slingMalrotation of heart & PDAPulmonary artery Sling

DOUBLE AORTIC ARCHMost common vascular ring (40%) Both of embryonic right and left arches persistAscending aorta arises normally leaves the pericardium divides 2 branches (Lt & Rt arch) join posteriorly to Dao Lt arch :pass anterior, left of trachea join by ductus /ligamentum arteriosus gives 2 vessels: LCA , LSA Rt arch:pass posterior, right of esophageus join left-side DaoGives 2 vessels : RCA, RSA Right arch dominant is most common (75%) , Left arch dominant (25%), 2 arch is equal (5%)Associated anomalies : uncommon (TOF, TGA.)

DOUBLE AORTIC ARCHCLINICAL PRESENTATIONS

RESPIRATORY SYMPTOMPSsFEEDING SYMPTOMS Inspiratory stridor ( onset