RVOT, LVOT, & Aortic Arch Anomalies Flashcards
Subvalvular obstruction involves hypertrophy of the ____ and usually occurs alongside other defects.
Infundibulum
____ obstruction may be in the form of a bicuspid pulmonary valve with varying degrees of pulmonary valve stenosis or atresia.
Valvular
Obstruction above the valve is usually in the form of ____ stenoses of the branch pulmonary arteries.
Discrete
There are two major forms of pulmonary valve stenosis:
- Fusion of the raphe, often with a bi-leaflet pulmonary valve which domes
- Severely thickened valve tissue with a tri-leaflet valve
____ will usually develop if RVOT obstruction is present.
RVH
The degree of RVH is dependent upon the amount of ____.
Obstruction
Most infants with an RVOT obstructions are ____.
Asymptomatic
Since the RV is not built to withstand high ____, early surgical intervention is necessary to reduce the stress on the RV.
Pressure
If there is a moderate to severe stenosis of the RVOT, they will have a loud ____ murmur.
Systolic
Pulmonary ____ is the complete obstruction of the RVOT with an atretic pulmonary valve.
Atresia
With pulmonary atresia, infants have no pulmonary blood flow except what is provided by the ____.
PDA
What are the two types of pulmonary atresia?
- Those with an intact IVS
- Those with a large VSD
Pulmonary atresia with intact IVS (PAIVS):
- Associated with a poor ____.
- Since there was not any blood exiting the RV during fetal development, the TV may be either severely ____ or ____.
- The RV is often ____ because of the lack of circulation in utero so it’s growth is restricted
- The RV will usually contract ____.
- Prognosis
- Stenotic/atretic; insufficient
- Hypoplastic
- Minimally
Pulmonary Atresia with Intact IVS:
- The smaller the RV and the more hypoplastic the TV, the poorer the ____
- There is a PFO or ____ ASD with a right-to-left shunt present (Venous return passes through PFO to get to LA and LV)
- There must be a ____ to supply blood flow to the lungs
- Prognosis
- Secundum
- PDA
____ is given to keep PDA open.
Prostaglandin
Placement of a systemic-to-pulmonary artery ____ is the most common procedure for PAIVS.
Shunt
What are most helpful views for visualizing RVOT obstruction?
- PSAX view at aortic level
- PLAX view of the RV, RVIT, and RVOT
- Subcostal/Apical 4 view of the RV
What are the steps to assessing an RVOT obstruction on echo?
- Assess RV size, function, and hypertrophy
- Assess TV morphology and function
- Evaluate PDA
- Evaluate IVS
- Evaluate ASD
- Measure main PA and branches
An LVOT obstrcution is any lesion that impedes the forward flow of blood from the LV to the ____.
Aortic arch
What are the three main types of LVOT obstruction?
Subvalvular, Valvular (bicuspid or stenosis), and Supravalvular
The mildest form of aortic valve disease is the ____
Bicuspid valve
Speed with which bicuspid valve calcification occurs depends upon:
- Severity of initial obstruction
- Calcium metabolism/cholesterol levels
- Gender (males more frequently)
Calcification of aortic valve isn’t seen in the ____ population.
Pediatric
What are the three major forms of subvalvular AS?
Discrete, fibromuscular, and tunnel
Discrete subaortic AS is when there is a fixed fibrous membrane located immediately ____ aortic valve.
Below
Fibromuscular subaortic AS is when there is a fibromuscular ring involving ____ and ____ areas of LVOT.
Anterior; posterior
____ is when there is a fibromuscular narrowing of LVOT.
Tunnel
What is the most common type of subvalvular AS?
Discrete
Supravalvular AS is associated with ____ Syndrome.
Williams
What are some characteristics of Williams syndrome?
- Abnormal facial characteristics (elfin look)
- Low I.Q.
- Abnormalities of calcium metabolism
What are the three major types of supravalvular AS?
Discrete, hourglass, and tubular/diffuse
Discrete supravalvular AS is when there is a fixed fibrous membrane immediately ____ the aortic valve.
Above
Hourglass supravalvular AS is when there is a narrowing at the ____.
STJ
Tubular/diffuse supravalvular AS is when there is a uniform narrowing of entire ____ aorta.
Ascending
When assessing for an LVOT obstruction, a Doppler velocity over ____ m/s is suspicious for MS and/or AS.
2 m/s
What are the three most common types of aortic arch anomalies?
- Coarctation of the aorta
- Interrupted aortic arch
- Right aortic arch
____ of the aorta is defined as the discrete narrowing of the descending thoracic aorta located generally just opposite the insertion of the ductus arteriosus causing LV pressure overload.
Coarctation
What are the three types of coarct?
Preductal, juxtaductal, and postductal
A preductal coarct is located ___ the ductus insertion.
Above
A juxtaductal coarct is located ___ the ductus insertion
At the level of
A postductal coarct is located ___ the ductus insertion.
Below
With ____ you will usually see a different blood pressure between arms and legs.
Coarctation
Use ____ to determine location and extent of coarct.
SSN
Use ____ Doppler to demonstrate high peak systolic velocity across coarct.
CW
What are some treatment options for coarctation?
- Percutaneous balloon angioplasty and stent
- Resection with end-to-end anastomoses
- Dacron patch
- Dacron graft
Turner’s syndrome affects ____ exclusively – absence of an X chromosome.
Females
Webbed neck, short stature, swelling of hands and feet, and infertility are all side effects of having ____ syndrome.
Turner’s
Shone’s complex is a rare CHD complex consisting of 4 defects:
- Coarct of Aorta
- Valvular and Subvalvular AS
- Parachute MV
- Supravalvular Mitral Ring
A ____ aortic arch is characterized by an absence of a segment between the aortic arch and the descending thoracic aorta.
Interrupted
Interrupted aortic arch is associated with:
VSD, AV deformity, and subaortic stenosis
Blood flow of an interrupted aortic arch is as follows:
LV > Ascending aorta > neck vessels
PA > PDA > Descending aorta > lower body
Surgical ____ of upper and lower segments of aorta is used to treat an interrupted aortic arch.
Anastomosis
A right aortic arch crosses over the right main bronchus, passing to the ____ of the trachea.
Right
A right aortic arch Usually courses to the right side of the ____.
Spine
Right aortic arches are most commonly seen in children who have ____.
TOF
