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119 Cards in this Set

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What does Atrial Septal defect (ASD) result from?
Failure of the interatrial septum to close during fetal development
What does an ASD allow?
blood to flow between the left and right atrium
What is the prevalence of Ostium Primum ASD?
20%
Where is Ostium primum ASD located?
The inferior portion of the intertribal septum
Why does an Ostium primum ASD occur?
The septum primum fails to fuse with the endocardial cushion during septation of the atria
What are associated anomalies of the Ostium Primum ASD
Deformed Mitral Valve
What is the most common ASD?
Ostium secundum 70%
Where is a Ostium Secundum located?
Mid portion of the interatrial septum
What is the prevalence of Sinus Venosus ASD?
10 %
Where is a sinus venosus ASD located?
the superior portion of the interatrial septum near the junction of the superior vena cava and the right atrium.
What are associated findings with Sinus Venosus ASD?
Partial anomalous pulmonary venous return (one or more of the pulmonary veins do not return to the left atrium.
What is the prevalence of Coronary Sinus ASD?
RARE
Where does Coronary Sinus ASD occur?
inferior septal area close to the coronary sinus
What is a common atrium?
the absence or near absence of the interatrial septum resulting in a common atrial chamber
What do hemodynamic effect of the ASD depend on?
1. size and direction of shunt
2. compliance of ventricles
3. response of the pulmonary vascular bed to the increased blood flow.
What does the direction of the shunt depend on
pulmonary vascular resistance (PVR) and systemic vascular resistance (SVR)
As the PVR elevates the resistance to pulmonary blood flow increases resulting in what?
reduction in pulmonary blood flow
What is Eisenmenger Syndrome?
When shunt becomes right to left as in utero.
What will Eisenmenger cause
less than normal pulmonary flow and cyanosis b/c pulmonary vascular pathology is now reversed
Qp/Qs shunt ratio equation
Qp = SV rvot
Qs = SV lvot

FYI: opposite of PDA equation
What is a VSD
failure of the inter ventricular septum to close during fetal development, which allows blood to shunt across the inter ventricular septum between left ventricle and right ventricle
Another name for atrioventricular defect
Endocardial cushion defect
What VSD is common with Trisomy 21 (Down Syndrome)
Endocardial cushion defect
what is an Endocardial cushion defect
A shunt between ventricle and atria
VSDs vary in size from....?
7mm to 3cm
Where is a Inlet septal VSD located?
bordered by tricuspid valve, mitral valve and muscle. (SUPRAVALVULAR)

associated with atrioventricular septal defects.
What is the prevalence of Inlet Septal defect
3-5%
Where is a Trabecular or muscular septal VSD located?
between the bodies of the left ventricle and right ventricle. Low on the septal wall in the thicker more muscular portion of the septum close to the apex. "SWISS CHEESE" appearance
What is another name for Outlet septal VSD?
SUPRACRISTAL VSD
What is the prevalence of Outlet septal VSD?
3-5%
Where is an Outlet septal VSD located?
between the left ventricular (SUBVALVULAR) outflow tract and right ventricular outflow tract bordered by the aortic valve, pulmonic valve and muscle
What VSD has a strong association with aortic valve prolapse and aortic insufficiency?
Outlet Septal VSD
What VSD has a strong prevalence with Asian population
Outlet Septal VSD
What is the most common VSD with and 80% occurrence
Membranous septal or perimembranous septal VSD
Where is the Membranous septal VSD located
bordered by tricuspid valve, aortic valve and muscle, usually high on the septal wall in the thinner, more flexible portion of the septum, closer to the valves and great vessels
What VSD si aneurysmal ...thin stretchy portion of tissue extending into RV
Membranous septal or perimembranous septal VSD
When does a malalignment septal VSD occur?
when 2 portions of the inter ventricular septum have failed to align properly during development
What are examples of malalignment septal VSD
Tetralogy of Fallot and Truncus Arteriosus
What occurs with malalignment septal VSD
Blood through both ventricles mixes together as it exits through a single valve from the heart.
What is a Truncus Arteriosus
an abnormality where the aorta and pulmonary artery rise from a common trunk causing blood to mix from both ventricles and exit through a single valve.
Why does VSD place a burden not the heart?
volume overload on both ventricles due to large amount of blood ejected into the RV and lungs, flow returns to the LV therefore both ventricles experience overload
What can a VSD cause?
PVR resulting in pulmonary hypertension
VSD IMAGE
PDA IMAGE
ASD IMAGE
When does the ductus arteriosus close?
15 hours after birth and it is structurally sealed within 2 to 3 weeks
What does the ductus arteriosus become?
Ligamentum arteriosus
What side of the heart is burdened by the PDA
Right heart
lungs
ultimately the left side of the heart
What does the flow reversal in a PDA cause
volume overload in pulmonary artery and lungs as flow returns to the left side. Right side must work harder agains the increased pulmonary vascular resistance
What kind of murmur is heard with a PDA
high pitched "machine" murmur heard during diastole and systole
what is considered a significant PDA
QpQs ration of 1.5: 1 or greater
What associated finding and hemodynamic effectswill you find with a PDA?
left ventricular volume overload and pulmonary hypertension
What kind of appearance will a Endocardocushion defect have
butteryfly
What is an Endocardial Cushion defect
A hole in the center of the heart where the upper chambers meet the lower chambers allowing oxygenated blood to mix with deoxygenated blood
If you have a common valve in place of the mitral and tricuspid valve what defect will you have
Endocardial Cushion defect
endocardial cushion defect
endocardial cushion defect
What is the most common site for a cleft mitral valve leaflet
anterior mitral valve leaflet
What are CMVL associated findings
atrioventricular septal defect
ventricular septal defect
atrial septal defect
PDA
rotation of papillary muscles
presence of an accessory papillary muscle or MVL
mitral valve prolapse
What are the effect of pulmonic stenosis (PS)
obstructs blood flow between RV and pulmonary artery during systole
this type of PS is the most common
Valvular PS

usually due to fusion of the cusp
This type of PS involves stenosis in the RV outflow tract creating a RV outflow tract obstruction
Subvalvular (infundibular) PS
this type of PS involves stenosis of the main pulmonary artery just above pulmonic valve
Supravalvular PS
What changes occur with PS
cusp become thickened and fibrotic and calcified reducing mobility and increasing obstruction. changes in RV; myocardial fibrosis or subvalvular muscular hypertrophy
What echo finding must be evaluated with PS
right heart enlargement
hypertrophy
failure and/or infarct
What anomalies are found with PS
atrial septal defect
ventricular septal defect
double chambered RV
Pulmonic Stensis
What is coarctation of the aorta
narrowing or partial obstruction of the aorta, typically in the AORTIC ISTHMUS
Where is aortic isthmus located
between the left subclavian artery and the first intercostal artery
What is coarctation of the aorta associated with
bicuspid aorta
valvular aortic stenosis
subvalvular aortic stenosis
PDA
ventricular septal defects
What are the 3 types of aortic coarctation
Preductal
Juxtaductal
Postductal
Where is Preductal coarctation located
in aorta, superior to the ductus arteriosus or ligamentum arteriosum
Where is Juxtaductal coarctation located
in the aorta at the level of ductus arteriosus or ligamentum arteriosum
Where does the Postductal coarctation occur
n the aorta inferior to the ductus arteriosus or ligamentum arteriosum
What are the hemodynamic effects of aortic coarctation
blood pressure is elevated above the coarctation and decreased below the coarctation.
What might develop with aortic coarctation
Collateral vessels develop from the arteries above the coarctation in order to supply blood flow to the lower extremities.
What is the optimal window to rule out aortic coarctation
suprasternal
A coarctation measurement of less than or equal to _____% of the descending thoracic aorta diameter indicates server coarctation
40
Postductal Aortic Coarctation
EPSTEIN ANOMALY
epstein anomaly with septal defect
tetralogy of fallot
dextro transposition
levo transposition
What does Epstein Anomaly involve?
downward displacement of 1,2 or 3 of the tricuspid valve leaflets into the RV. A portion of the RV becomes ATRAILIZED and serves as part of the RA
What is the result of Epstein Anomaly
Large RA may cause a PFO and /or sucundum atrial septal defeat , and small RV and TR...may result in heart failure
What other anomalies are associated with Epstein Anomaly
ASD (75%)
VSD
PFO
PDA
PS
Mitral Stenosis
TOF
Transposition of Great Arteries
What is the most common cyanotic lesion in the adult population?
Tetralogy of Fallot
What are the findings with Tetralogy of Fallot
PS
Right Ventricular Hypertrophy
Over-riding Aorta
Malalignment
With Tetralogy of Fallot what do the symptoms depend on
Size of the VSD
degree of PS
position of the Aorta
With Tetralogy of Fallot what do PS and RVH cause
VSD to shunt right to left producing a decrease in systemic arterial oxygen saturation, cyanosis, reduced pulmonary flow and possibly a hyperplastic PA
What do children with Tetralogy of Fallot experience
1. exercise intolerance
2. squatting episodes (incerases venous return and SV and CO
3. Tets episodes- faintness and cyanosis
With Dextro-TGA where does the aorta arise from
anterior ventricle
With Dextro-TGA where does the PA arise from
posterior ventricle
What is the circulation pattern with Dextro-TGA
systemic flow returns to the RA⇨RV⇨aorta⇨body⇨RA again
Pulmonary Venous Flow returns to the LA⇨LV⇨PA⇨Lungs⇨LA again
How does life sustain with Dextro-TGA
Shunts! intermixing of the 2 circuits with occur through PFO and PDA
What medications maintains shunts with Dextro-TGA
Prostaglandins
What other anomalies are associated with Dextro-TGA
VSD
ASD
PS
Right sided Aortic Arch
What occurs with Levo-TGA
1. anatomic RV will be displaced posterior and leftward becoming arterial ventricle
2. anatomic LV will be displaced anterior and rightward becoming venous ventricle
What is the circulation pattern with Levo-TGA
1.Pulmonary Venous returns to LA⇨anatomicRV⇨aorta⇨body
2. Systemic flow returns to RA⇨anatomic LV⇨PA⇨lungs⇨back to LA
Why do Levo-TGA survive
PA arises from venous ventricle
Aorta arises from arterial ventricle
as normal
How long will a patient with Levo-TGA survive
With no other defect 15-20 years then systemic ventricle (anatomic RV) will fail
What is Hypoplastic Left Heart Syndrome? (HLHS)
Left heart is reduced in size due to restriction of the LV inflow and outflow. Due to MITRAL ATRESIA, AORTIC ATRESIA, HYPOPLASIA OF THE AORTA
When can you make diagnosis of HLHS
LV is less than 10mm, the aortic annular size is reduced, mitral valve is distorted or absent
What assist with circulation with HPHS
ductus arteries and foramen ovale
What side of the heart is burdened with HPHS
Right heart is burdened with volume and pressure working to assist the left heart.
When can HPHS be diagnosed
16-20 weeks into pregnancy
What is needed for survival with HPHS
Shunts which are kept open with the medication prostaglandin
ASD
failure of the interatrial septum to close during fetal development: allows blood to shunt between LA and RA
TYPES: ostium primum, sinus venosus, coronary sinus and common artium
VSD
failure of interventricular septum to close during fetal development: allows blood to shunt between LV and RV
TYPES: inlet, trabecular/muscular, outlet, membranous/perimembranous, malalignment
PDA
failure of the ductus arteriosus to close after birth: results in a communication between the AO (high pressure system) and PA (low pressure system)
ENDOCARDIAL CUSHION DEFECT
(aka..atrioventricular canal defect or atrioventricular septal defect)
Combination of anomalies
1. Hole in the center of the heart where the upper chamber set the lower chamber allowing oxygenated blood to mix with deoxygenated blood
2. a common valve in place of the MV and TV: common in patients with Trisomy 21 (Downs Syndrome)
CLEFT MITRAL VALVE LEAFLET (CMVL)
Split or division of the anterior (more common) and or /posterior MV leaflet: evaluate degree of cleft edge fibrosis, retraction of valve anatomy: frequently associated with atrioventriculare septal defect, VSD, ASD, PDA, MVP rotation of the papillary muscles, presence of an accessory papillary muscle or MV leaflet
PULMONIC STENOSIS (PS)
Obstruction of blood flow from the RV to the PA (subvalvular, valvular or supravalvular): the PV cusp can become thickened, fibrotic, and calcified which reduces the valve mobility and increases obstruction; changes also occur in the RV such as myocardial fibrosis or subvalvular muscular hypertrophy which can further contribute to the RV outflow tract obstruction.
COARCTATION OF THE AORTA
Narrowing of the aorta, typically in the area of the aortic isthmus (preductal, juxtaductal, or post ductal): often associated with BAV, valvular AS, subvalvular AS, PDA and VSD
EBSTEIN ANOMALY
downward displacement of one, two or three TV leaflets into the RV, a portion of the RV becomes atrailized and serves as part of the RA: results in a large RA, small RV and TR; may result in RHF; often associated with ASD, VSD, PFO, PDA, PS, MS, TOF and D-TGA
TETRALOGY OF FALLOT
Cyanotic lesion consisting of 4 defects
1. PS
2. RV hypertrophy
3. Over-riding Aorta
4. Malaignment
children typically experience exercise intolerance, squatting episodes, and "tet" spells
D-TGA
Great arteries are transposed creating two independent parallel circuits; life dependent on some intermixing of those two circuits via PFO, PDA, and possible VSD
L-TGA
Ventricles are transposed; if no other defects are present, the patients heart will function normally for 15-20 years but then the systemic ventricle (anatomic RV) will fail.
HYPOPLASTIC LEFT HEART SYNDROME (HLHS)
LV is reduced in size due to a restriction of LV inflow and/or outflow; restriction is typically due to mitral atresia, aortic atresia, or hypoplasic of the aorta; the key to treating HLHS is early detection; prostaglandin can be administered in order to maintain the shunts and circulation; keeping the shunts open is the key to survival