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(Peds) Abnormalities in Conotruncal Septation
Terms in this set (83)
The incomplete closure of the aortopulmonary septum is called?
The complete failure to form aortopulmonary septum is called?
What conditions are a result of the uneven conotruncal septation?
Pulmonary artery stenosis and atresia
Aortic stenosis, atresia, and hypoplasia
Tetralogy of flow
Communication between the aorta and the pulmonary trunk with two distinct and separate semilunar valves is called?
What angle of septal rotation is considered normal?
What angle of septal rotation is considered transposition?
What angle of septal rotation is considered side by side, often seen in double outlet RV?
T/F: Aortooulmonary window is the rarest of the four septal defects (ASD, VSD, AVCD, aortopulmonary septation)
Aortopulmonary window is seen in ______% of all CHD.
What type of AP window is located above the sinus of Valsalva, a few millimeters above semilunar valves, with a superior rim but little inferior rim separating AP window from semilunar valves?
What type of AP window is located in the uppermost portion of the ascending aorta, with a well formed inferior rim but little superior rim?
What type of AP window involves the majority of ascending aorta with little superior and inferior rims?
What type of AP window is similar to total defect but with adequate superior and inferior rims?
What type of AP window is most common?
What type of AP window is more suitable for closure device?
The hemodynamics of AP window is similar to PDA and VSD that it will have _______ pulmonary artery blood flow and _____ sided volume overload
What are the associated conditions of AP window?
Interrupted AO arch
Tetralogy of flow
Pulmonary and tricuspid atresia
Anomalous origin of RCA from PA and Ao arch
Transposition of great arteries
If patient with AP window presents with small shunt, will they be symptomatic or asymptomatic?
Patient with AP window present with large shunt, what are the conditions associated with it?
CHF (LV overload )
Failure to thrive
What kinds of murmur would you hear in patients with AP window?
Systolic ejection click
Harsh systolic ejection murmur
May be continuous or absent
Apical mid-diastolic murmur due to increased flow thru MV
What are the complications for patients with AP window?
What are the available treatments for AP window?
Closure of defect with Dacron patch
When should AP window be treated?
Early infancy to avoid PHTN
What echo views are used to interrogate AP window?
PSAX of Ao
RVOF (subcostal, Apical, PSAX)
What do we interrogate with 2D/MM for AP window ?
T sign artifact
LV, LA dilation
LV volume overload
Pulmonary vein dilatation
MPA, RPA, LPA dilatation
Measure diameter of defect
Determine distance from defect to semilunar valves
Determine distance from defect to PA bifurcation
Determine any associated lesions
What do we interrogate with PW/CW/Color for AP window?
High velocity, turbulent continuous flow
Diastolic flow reversals in desc Ao
Increased pulmonary venous return
Determine severity and presence of regurgitation
What do we interrogate after post operation of AP window?
Residual AP window
LV volume overload recovery
Where is Qp/Qs measured for residual AP window?
MV and TV
The condition where a single arterial trunk arises from the heart, giving origin to the coronary arteries, the pulmonary arteries, and the systemic arterial circulation with single truncal valve is called?
Truncus arteriosus is seen in ____ - _____% of all CHD
Truncus arteriosus is a genetic anomaly associated with what two things?
Maternal drug abuse
Truncus arteriosus is the result of absent formation of ___________ _________during embryology.
What are the two classification systems fir truncus arteriosus?
Collet and Edwards
Which type of truncus arteriosus has MPA present and arises from the truncal root?
Which type of truncus arteriosus has right and left PA's arise separately from the lateral portion of the truncal root?
What are the three characteristics of pseudotruncus?
Which type of truncus arteriosus has the right and left PA's arise separately from the posterior portion of the truncal root
Which type of truncus arteriosus is most common?
What is an additional variant description of truncus arteriosus where right and left pulmonary arteries are absent
What are the complications of truncus arteriosus?
Interrupted aortic arch
What are the important description modifiers for truncal arteriosus?
Truncus receives mixed blood from both _______ and ______. Some flow enters the pulmonary system and systemic system.
T/F the VSD associated with truncus arteriosus is bidirectional
What are the associated defects of truncus arteriosus?
Right aortic arch (most common 30%)
ASD secundum (9-20%)
Interrupted aortic arch ( 11-19%)
Coronary artery abnormalities
- anomalous origins
- stenotic coronary ostia
What are the clinical features of truncus arteriosus?
Failure to thrive
Death (6-12 months of CHF)
What is the palliative technique used for truncus arteriosus?
What are the available treatments for truncus arteriosus?
Truncal valve repair or replacement
Aortic arch repair
What is Rastelli?
A RV to PA conduit
What do we interrogate with PLAX for patients with truncus arteriosus?
Demostraste dilated Ao and malalignment VSD
What do we interrogate with subcostal and parasternal for patients with truncus arteriosus?
Demonstrate large truncal valve cusp
PA or absence of PA
What do we demonstrate with 2D/MM for truncus arteriosus?
Type of truncus
Origins of coronary arteries
Distance of coronary ostia to branch PA
Degree of truncal override
What do we demonstrate with PW/CW/Color for truncus arteriosus?
Color flow to locate origins of PA's
Pressure gradients of PA's
Truncal valve gradients
Magnitude of VSD
What do we demonstrate post-op for truncus arteriosus?
VSD patch repair
Truncal valve regurgitation/stenosis
Ventricular size and function
Adequacy of Rastelli repair
Anomalous pulmonary artery originating from ascending aorta is called?
Hemitruncus is an inaccurate word because it implies that the anomalous vessel arises from common persistent arterial trunk when it actually arises from the ______
A congenital heart defect characterized by four different cardiac abnormalities is called?
Tetralogy of Fallot (TOF)
What are the four cardiac abnormalities associated with TOF?
TOF is due to the underdevelopment of the _________, with all other cardiac abnormality secondary. Anterior and rightward deviation of the ________. And abnormal separation of the _______ ______.
TOF is seen in ______% of all CHD.
T/F: TOF is the most common cyanotic lesion in adults.
What are the pulmonary outflow tract obstruction variants for TOF ?
Valvular and subvalvar
Valavalar, subvalvar, and Supravalvar
What are the variants of TOF ?
Pseudotruncus-pulmonary atresia with VSD
Atrioventricular canal defect
The most important factor in determining the severity of the lesion in TOF is the degree of ___________ ________________.
In cases where patients with TOF presents with severe RVOT obstruction, what direction does blood flow through VSD
VSD flow right to left
In cases where patients with TOF presents with mild RVOT obstruction, what direction does blood flow through VSD
VSD flow left to right
What are the hemodynamics for an acyanotic "Pink TET"
Nonrestrictive VSD equalized pressure between ventricles
Mild RVOT obstruction
Flow across VSD is bidirectional, but mostly left to right
What are the hemodynamics for an cyanotic "Blue TET"
Nonrestrictive VSD equalized pressure between ventricles
Severe RVOT obstruction
RV pressures are Supra systemic
Flow across VSD is all right to left
What are the progression and complications of acyanotic patients with TOF?
75% become cyanotic in one year
Hypoxic spells "TET Spells"
What are the progression and complications of an cyanotic patient with TOF?
Severity depends of size of the PA branches
Secondary damage to heart, lungs, and brain due to constant hypoxia
30% fatality within the first year of life if untreated
Hypoxic spells "TET Spells" presents as the following?
Irritability and crying
During the Tet spell, something triggers the RV pressure to become higher than the LV pressure which changes VSD flow direction from (_______ to ______) to (_______to _______)
L to R
R to L
During Tet spells, cyanosis increases as oxygen ________, which then triggers __________ respiration
During Tet spell, increased respiration result in an ________ in venous return, which then results in ________ right to left shunt
What are the associated conditions of TOF ?
Right aortic arch (30% most common)
Coronary artery anomalies
Complete AV canals
Supravalular MV ring
What are the clinical presentation of TOF
What are the auscultation findings of TOF?
Aortic systolic ejection click
Loud systolic ejection murmur
Systolic murmur LSB (VSD)
Diastolic decrescendo murmur (semilunar regurgitation)
Continuous murmur (PDA)
What palliative techniques are uses for TOF ?
Systemic to pulmonary shunt
What are the available treatments for TOF ?
Ventriculotomy (widening RVOT)
RV to PA conduit ( Rastelli)
Pulmonic valve repair
What treatments are used for cyanotic TOF?
Balloon dilatation of branch PA's
Closure of VSD
Usually done is separate stages
What are the echo views used to interrogate TOF ?
Apical/subc 4 with anteriortilt to LVOT and RVOT
What do we demonstrate with 2D/MM for TOF?
Distribution of coronary arteries
Identify any associated lesions
Dimensions of VSD, MPA, pulmonary branches
What do we demonstrate with PW/CW/Color for TOF?
Direction of shunt flow (VSD, PDA )
Degree of RVOT obstruction
What do we demonstrate with post op echo for TOF ?
Competency of RV to PA conduit
Residual VSD flow
Ventricular size and function(right and left)
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