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S4 W9-12 Congenital Heart Disease > Congenital LVOT Obstruction > Flashcards

Congenital LVOT Obstruction Flashcards

1
Q

Sites of LVOTOB?

A
  • Valvular (most common site, 70% CHD)
  • Subvalvular (in LVOT)
  • Supravalvular (in ascending aorta)
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2
Q

Associated lesions with valvular AS (BAV)?

A
  • Coarctation (50% of CoAo have BAV)
  • ASD
  • VSD
  • PDA
  • Common in Shone syndrome
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3
Q

Associated lesions with subvalvular AS (fixed)?

A
  • CoAo
  • PDA
  • VSD
  • Part of Shone Syndrome
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4
Q

Associated lesions with supravalvular AS?

A
  • ASD
  • PDA
  • VSD
  • CoAo
  • PA stenosis (valvular or branch stenosis)
  • Coronary artery anomalies
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5
Q

Shone’s Syndrome: What are the four anomalies associated with Shone’s syndrome?

A
  1. CoAo
  2. Supravalvular mitral ring
  3. Parachute MV
  4. Subaortic stenosis/obstruction
    - BAV also commonly seen
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6
Q

Characteristics of Valvular AS: UAV?

A
  • Very rare
  • Occurs when fusion between 2 of 3 developing aortic cusps, or fusion of all 3 cusps
  • Results in solitary opening
  • UAV most common type of AV structure in infants and young children with congenital AS
  • Can be acommissural or unicommissural
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7
Q

Characteristics of Valvular AS: Acommissual UAV?

A
  • Central opening ‘volcano’
  • No commissural attachment to aortic root
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8
Q

Characteristics of Valvular AS: Uni commissural UAV?

A
  • Most common type
  • Single commissural attachment to aortic root
  • Eccentric opening ‘keyhole’
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9
Q

Characteristics of Valvular AS: BAV?

A
  • BAV occurs in 1-2% of the population
  • M:F ratio around 3:1
  • Familial occurrence around 9%
  • Stenosis of BAV most common cause of isolated AS in patients under 50 years
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10
Q

Characteristics of Valvular AS: BAV with Raphe?

A
  • Most common type
  • Most common site of cusps fusion between RCC and LCC (85%)
  • Followed by fusion between RCC and NCC; LCC and NCC fusion rare
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11
Q

Characteristics of Valvular AS: True BAV?

A
  • Less common (10% BAVs)
  • Near-equal sized cusps
  • Orientation variable; commissures anterior & posterior, or medial & lateral
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12
Q

Characteristics of Subvalvular AS?

A
  • Obstruction proximal to AV (below AV, on LVOT side)
  • Accounts for around 30% of congenital AS cases
  • May occur as fixed or dynamic obstruction (as in HOCM)
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13
Q

What are the three types of subvalvular (fixed obstruction)?

A
  1. Membranous (most common, 75-85%)
  2. Fibromuscular
  3. Fibromuscular tunnel
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14
Q

Characteristics of Supravalvular AS?

A
  • Obstruction to distal AV (above AV, on aortic side)
  • Less common site of congenital AS (5% of cases)
  • Most often associated with William Syndrome
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15
Q

What are the three morphological types of supravalvular AS?

A
  1. Membranous (least common)
  2. Hourglass (most common)
  3. Tubular
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16
Q

Characteristics of membranous supravalvular AS?

A
  • Immediately above AV
  • Thin membrane or fibrous diaphragm with a central orifice
  • Least common
17
Q

Characteristics of hourglass supravalvular AS?

A
  • Dysplasia of aortic wall immediately above aortic cusps
  • Most common
18
Q

Characteristics of tubular supravalvular AS?

A
  • Uniform diffuse hypoplasia of the tubular portion of the ascending aorta
  • Begins immediately above the sinuses of Valsalva, and extending to origin of innominate artery
  • Most severe form of supravalvular AS which sometimes includes branch arteries
19
Q

Clues to BAV?

A
  • Eccentric aortic valve closure
  • Diastolic prolapse and systolic doming
  • “Skipping rope” appearance in PSAX
  • Normal commissural attachments at 2, 6 and 10 o’clock; commissures other than these locations can suggest abnormal or BAV
  • BAV diagnosed in systole when leaflets are open!
20
Q

Characterisitcs of subaortic membrane?

A
  • Tag of tissue attached to IVS
  • Best identified when membrane runs perpendicular to ultrasound beam: PLAX and A5C
21
Q

Characteristics of subaortic obstruction (M-Mode AV)?

A
  • When dynamic LVOTOB as in HOCM: usually mid-systole closure of the AV
  • WHEn fixed obstruction: almost always see early systolic notching of the valve

S4 W9-12 Congenital Heart Disease (10 decks)

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