Ao Arch Anomalies (10)

Qs what does D stand for in D TGA?

dextra looping

Qs what does L stand for in D TGA?

levo looping

Qs what is another name for L loop TGA?

congenitally corrected TGA

Qs what is it caled if there is atrioventricular concordance and ventriculoarterial discordance?

d TGA

Qs What is it called if there is double discordance? What does this mean? **

CC TGA

Qs which type of TGA is more dangerous?

D-TGA

Qs atrial situs inversus can be described as what?

L TGA

malposition of great arteries

Qs what is collet and edwards type 1 truncusarteriosus?

Qs what is C & E type 2?

Qs what is C & E type 3?

Qs what is van praagh A2?

Qs what is van praagh A3?

Qs whats the most common number of truncal leaflets?

Qs what type is most common ^^?

what is the aortic isthmus?

slight constricion of the aorta immediately distal to the left subclavian artery at the point of attachment of the ductus arteriosus

what is an interrupted ao arch (IAA)?

absence of a segment of the ao arch

what is interrupted ao arch type A?

interruption found at isthmus

• origin of the subclavian artery and the insertion of the ductus arteriosus

• distal to left subclavian artery

type A IAA is associated with

50% VSD

how can type A IAA present clinically?

difference of BP of upper and lower extremities

in type B IAA where is the interruption?

between left subclavian and the LCCA

type B IAA is associated with what syndrome?

50% association with digeorge syndrome

how does type B present clinically?

right arm BP > LT arm

what is type C IAA?

when interruption occurs proximal to the LCCA between the brachiocephaic art

which type of IAA is most common?

type b

IAA is typically associated with

VSD (nonrestrictive)

• most common in concordant ventriculo arterial connections

ductus arteriosus

• most common in concordant ventriculo arterial connection

truncus arteriosus

how is IAA treated?

• before surgery prostglandins are administered to maintain patency of PDA

• reconnect ao arch to create a continuous tube (end to end anastamosis)

• close VSD to prevent blood mixing (or ASD/PDA)

what are the signs and symptoms of IAA?

• cyanosis of lower extremities

• similar to severe coarctation

• weak/absent peripheral pulses

• tachypnea

• congestive heart failure

• systolic murmur (VSD)

• shock

what are the best views for IAA?

suprasternal and subcostal views

what is coarctation of the ao?

discrete narrowing or obstruction of the ao arch located in the descending ao

• usually in area of ao isthmus

coarctation of the ao is highly associated with

VSD, PDA, bicsupid ao valve

what are the hypothesized causes of coarctation of the aorta?

• decreased blood flow through the ao arch during fetal life

• constriction of ao at juxtaductul position secondary a deformity in the media of the ao

what is a juxtaductal coarctation?

narrowing of ao isthmus as its junction with prox descending ao at insertion of arterial duct (lig arteriosum)

• basically where lig art inserts into ao

how can coarctation affect hemodynamics?

normally blood flows from right to left

narrowing of ao isthmus causes resistance for LV output

• LV afterload increases and output decreases

distal to the narrowing may have lower pressure now than pulm art

• RV output increases and pressure loaded

therefore blood may now go from ao to pa

compare size of pa and ao in utero

they should be relatively the same size

recognize

what will happen in coarctation after the ductus arteriosus begins to constrict/close after birth?

• increases LV pressure and afterload

  • LVH

• increases LAP

• increase in pulm venous pressure occurs leading to eventual RV dilation

• prominent collaterals

• BP differences of upper and lower extremities

  • more elevated in upper extremeties

how does the ao appear distal to the coarctation?

dilated

what are the types of coarctation?

A : ductal (juxtaductal)

B : preductal

C : post ductal

how is hemodynamics affected with each coarctation?

A & C : overload lungs (blood will go from ao → lig art → lungs)

B : deoxy blood going to LE (pulm art shunting deoxy blood to ao)

what are the clinical signs of coarctation of the ao?

systemic hypertension

headache

lower extremity fatigue/claudication

congestive heart failure

elevated upper extremity BP with lower BP in the lower extremity

delayed/blunted fem pulses

palp systolic thrill

dilated left subclavian artery

LVH on EKG

post stenotic dilation of descending thoracic ao

how is coarctation of the ao medically managed?

balloon angioplasty

resectionf of coarctation with end to end anastamoses

grafts/patch enlargement

treat CHF (antihypertensives)

subclavian artery flap angioplasty (use subclavian artery to widen coarcted segment)

coarctation is associated with

• bicuspid ao valve (46%)

• PDA

• abn of subclavian art

  • origin relative to duct or ligament determines the lenght of the ao isthmus

what is tubular hypoplasia and what does it typically affect?

tubular narrowing of entire ao arch segment

typically affects ao isthmus

what does this image depict?

left ao arch with aberrant right subclavian artery

what is a right aberrant subclavian artery?

rare anomaly in which right subclavian artery arises directly from ao arch instead of originating from the brachiocephalic artery

what does a double ao arch/right ao arch result in?

vascular ring

• vascular structures encircling and compressing the esophagus/trachea

• two transverse arches cross trachea and both bronchi

what are the types of right ao arches?

mirror image type (most common)

• lt innominate

• RCCA

• RSCA

right ao arch with aberrant LSA

right ao arch with isolation of left subclavian artery

mirror image type right ao arch is usually associated with

TOF

truncus arteriosus

tricuspid atreisia

transposition of great arteries

the left main coronary artery arises from

left ao sinus of valsalva

the left main coronary artery branches into

LAD

circumflex

the right coronary artery arises from

the right ao sinus of valsalva

what are the types of anomalous coronary arteries?

1. left coronary artery arising off the right coronary sinus

2. right coronary artery arising off the left coronary sinus

3. anomalous left coronary artery arising off the pulm artery (ALCAPA)

Qs when would a difference of BP occur from the upper and lower extremities?

in type A IAA

Qs what about difference between left vs right upper extremity BPs?

type b and c in IAA

Qs what type of coarctation would result in the least amount of blood flow to the lower extremities?

preductal coarctation (B)

Qs which type of interrupted ao arch is most common?

type b

• interruption between left subclavian and left CCA

Qs what type of IAA results in same blood pressures of the arms?

type A