IPCCC: 07.09.10, 09.15.00, 09.15.28
 

AEPC Derived Term:    

Left ventricular outflow tract obstruction: subaortic (07.09.10)

Aortic valvar abnormality (09.15.00)

Unicuspid aortic valve: unicommissural (09.15.28)

EACTS-STS Derived Term:    

Ventricular outflow tract obstruction, Left (LVOTO), LV outflow tract obstruction – subaortic (07.09.10)

Aortic valve pathology, Aortic valve cusp(s)-modifier for number of cusp(s) = 1 (Unicuspid aortic valve), Unicommissural (09.15.00, 09.15.28)

ICD 10 Term:     

Congenital subaortic stenosis (Q24.4)

Congenital stenosis of aortic valve (Q23.0)

Definition:  pending

Common Synonyms:  pending

Modality: Anatomic specimen

Orientation: Short axis - base

Description: This short axis view of the aortic valve shows a markedly thickened, unicommissural valve. The two thickened raphes (red arrows) do not extend to the margin of the valvar orifice while the commissure (yellow arrow) does. There is a solitary opening or zone of apposition within the thickened valve. Within the non-coronary sinus, there is a thickened nodule of firm white tissue that nearly fills the sinus. The left coronary artery exits the aorta adjacent to the commissure (yellow arrow) and the right coronary artery exits near mid-sinus.

Contributor: Diane E. Spicer, BS

Institution: The Congenital Heart Institute of Florida (CHIF)

Image Label: A070910-23a

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

Image Certification: 17 November 2012       

AWG Rating:

Modality: Anatomic specimen

Orientation: Left ventricular outflow tract view

Description: The aortic valve is opened and viewed from the left ventricular aspect. There is a subaortic shelf with subaortic stenosis and a unicommissural valve. The cut edges of the valve are marked with black dots. As in the image in panel one, the two thickened raphes (red arrows) do not extend to the margin of the valvar orifice and the commissure (yellow arrow) does. The left coronary orifice exits the aorta adjacent to the commissure. Within the non-coronary sinus there is a thickened, firm, white nodule.

Contributor: Diane E. Spicer, BS

Institution: The Congenital Heart Institute of Florida (CHIF)

Image Label: A070910-23b

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

Image Certification: 17 November 2012

AWG Rating:

Modality: Anatomic specimen

Orientation: Left ventricular apical view

Description: A view from the apex, looking into the left ventricular outflow, shows the fibrous ring (yellow arrows) that causes the severe subaortic stenosis.   (LV - left ventricle,  MV - mitral valve)

Contributor: Diane E. Spicer, BS

Institution: The Congenital Heart Institute of Florida (CHIF)

Image Label: A070910-23c

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

Image Certification: 17 November 2012

AWG Rating:

These pathological images of an abnormal aortic valve generated a lenghty discussion about how to distinguish unicuspid/unicommissural aortic valves from bicuspid ones. With permission from the participants, we publish the following excerpts:

Prof. Robert Anderson:

This aortic valve “looks more to me like the unicommisural and unicuspid variant. The solitary opening within the valve does not extend to the margin of the valvar orifice” and most commonly, “extend(s) backwards to the zone of fibrous continuity with the mitral valve. This is the typical appearance of the so-called unicuspid and unicommissural variant”. In most cases, the “solitary zone of apposition point(s) towards the mitral valve”. In this example, “it is running from right to left. This is unusual in my experience, as is the fact that the zone of apposition does not extend to the sinutubular junction … we can only describe what we see!”.  “The subaortic shelf is very nicely shown. This is often called a membrane, but as Dr. Jane Somerville has emphasised on numerous occasions … the obstructive lesion is far from ‘membranous’. As shown in this specimen, it is a discrete fibrous shelf. It can often be more extensive, and then forms the so-called ‘tunnel’ variant”.

Dr. Paul Weinberg:

“I agree … that this looks like a unicommissural valve. This is commonly mistaken for a bicuspid valve by echocardiographers and angiographers because of the length and shape of the orifice. This differs from typical, isolated pulmonary stenosis which usually has a circular orifice in the center of a domed valve; whereas unicommissural AS typically has an eccentric elliptical orifice”.  “Regarding terminology of fibrous subAS (and PS in TGA) there are some others as well. I like the term "discrete" when there is a linear "membrane-like" obstruction. In my experience "tunnel" is usually what happens … after surgical resection. There is also fibrous subAS from abnormal attachment of an AV valve to the septum or crest of a VSD as in common AV canal. Similar to the current cases, these are often acquired after (AV) canal repair. There are also ‘ball-like excrescences’ from mitral valve chords and accessory mitral valve leaflets that can cause fibrous subAS”.

Dr. Meryl Cohen:

“I also think it looks unicommissural and that is often” mistaken by “echocardiographers. Essentially most critical AS infants have unicommissural valves”.

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