IPCCC: 09.29.22, 09.29.12, 01.04.04, 01.03.00, 02.03.01,

02.06.02, 07.02.00, 07.10.00, 01.05.01, 01.06.01, 07.14.01
 

AEPC Derived Term:    

Aortic arch atresia: fibrous cord distal to subclavian artery (type A) (09.29.22)
Aortic arch hypoplasia (tubular) distal to subclavian artery (isthmal) (09.29.12)
Double inlet left ventricle (01.04.04)
Usual atrial arrangement (atrial situs solitus) (01.03.00)
Right hand pattern ventricular topology (02.03.01)
Aortic orifice anterior right with respect to pulmonary orifice (02.06.02)
Right ventricular hypoplasia (07.02.00)
VSD (07.10.00)
Discordant VA connections (TGA) (01.05.01)

Two AV valves in double inlet ventricle (01.06.01) 

Restrictive VSD (07.14.01) 

EACTS-STS Derived Term:    

Aortic pathology, Abnormality involving aortic arch, Aortic arch atresia, Fibrous cord, Atresia distal to subclavian artery (type A) (09.29.22)
Aortic arch hypoplasia, Hypoplasia of aortic arch, Isthmus hypoplasia (distal to the subclavian artery) (09.29.12)
Single ventricle, DILV, {SDD}, Subaortic RV outlet chamber with VSD (Bulboventricular foramen) (01.04.04, 01.03.00, 02.03.01, 02.06.02, 07.02.00, 07.10.00, 01.05.01)
AV connection(s) = Double inlet AV connection (univentricular): Mode of connection = Two AV valves in double inlet ventricle (01.06.01)
VSD-modifier, Restrictive (07.14.01)

ICD-10 Derived Term:    

Double inlet ventricle  (Q20.4)

Definition:  pending

Common Synonyms:  pending

Modality: Anatomic specimen

Orientation: Right anterior view

Description: This view shows the outward appearance of a heart with aortic arch atresia and transposition of the great arteries with double inlet left ventricle (ventricular inlet not shown). The ascending aorta (A) is relatively small and is located to the right of the pulmonary trunk (PT). The aortic isthmus is hypoplastic between the left common carotid (LCCA) and the patent arterial duct (PAD). This severely stenotic segment is patent up to and including the left subclavian artery (LSA). The aorta then becomes an atretic, fibrous segment where it attaches to the arterial duct (seen on further dissection). Note the left subclavian artery is smaller than the other brachiocephalic vessels. (BCT-brachiocephalic trunk, DAo-descending aorta, LAA-left atrial appendage, LVA-vertebral artery, PT-pulmonary trunk, RAA-right atrial appendage, RCCA-right common carotid, RSA-right subclavian artery, SCV-superior caval vein)

Contributor: Diane Spicer, BS

Institution: The Congenital Heart Institute of Florida (CHIF)

Image Label: A010404-18a

Source of Image: Van Mierop Archive, University of Florida, Gainesville, Florida

Image Certification: 5 Nov 2011

AWG Rating:

Modality: Anatomic specimen

Orientation: Long axis view

Description: This view is a long axis view of the great arteries as they exit the ventricular mass. It shows the luminal surface of the vessels and illustrates the atretic portion of the aorta (red arrow) as well as the severe coarctation of the isthmus. Note the relatively small ascending aorta (A) arising from the rudimentary right ventricle (RudRV) along with the ventricular septal defect and the pulmonary trunk arising from the left ventricle (LV). This view shows that the ventricular septal defect (VSD) is smaller than the aortic orifice; therefore, likely restrictive. (IVS-interventricular septum, LCCA-left common carotid artery, LVA-vertebral artery, PAD-patent arterial duct, PT-pulmonary trunk, RCCA-right common carotid artery, RSA-right subclavian artery, SCV-superior caval vein, VA-vertebral artery).
Contributor: Diane Spicer, BS

Institution: The Congenital Heart Institute of Florida (CHIF)

Image Label: A010404-18b

Source of Image: Van Mierop Archive, University of Florida, Gainesville, Florida

Image Certification: 5 Nov 2011

AWG Rating:

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