When do symptoms of ASD usually present?
not in infancy- takes a while to develop symptoms
What are the long term risks of untreated ASDs?
development of pulm vascular disease atrial arrhythmias HF
____ is the most common cyanotic heart defect and accounts for 15% of all congenital heart defects.
Tetralogy of Fallot
In Tetrology of Fallot, the RV and LV pressures are _____.
equal
Why isn’t a murmur going away a good thing?
could be a large VSD with equalizing R and L pressures elevation in PVR
When can a VSD be a right to L shunt?
with pulmonary stenosis
What are the exam findings with a small VSD?
precordial activity normal normal 2nd heart sound 2-4/6 early systolic murmur NO diastolic rumble
DX? 2-3/6 systolic ejection murmur at L upper sternal border with possible diastolic rumble widely split 2nd heart sound
ASD
Turner’s Syndrome is associated with ______.
coarctation of the aorta
Where do coarctations of the aorta occur?
in the descending aorta opposite of the ductus arteriosus or lower
DX? 1. tachycardia 2. difference in upper extremity and lower extremity BP 3. pulmonary rales 4. hepatomegaly 5. S2 and S3 gallop 6. soft systolic murmur
Coarctation of the aorta
What happens with pulmonary vascular disease at high altitudes?
it’s more common and occurs at an earlier age
10% of VSDs are which type?
muscular VSDs
What are the exam findings with a large VSD?
active precordium loud 2nd heart sound 2-3/6 harsh, holosystolic murmur loudest at the LLSB diastolic murmur
How is VSD treated?
diuretics surgical close if muscular
How does Tetrology of Fallot present?
- blue baby 2. loud murmur
What are some of the s/s of VSD?
respiratory distress diaphoresis (esp with feeding) failure to thrive tachypnea
Dx? hypoxic or hypercyanotic episode, usually in the 2-6th month of life can be life threatening unk mechanism
Tet spell (Tetralogy of Fallot)
What are the steps of atrial septal embryology?
- septum primum closes 2. apoptosis of the middle primum cells to form the osteum secundum 3. septum secundum closes to enclose the foramen ovale
What is a fixed split S2 and what is it diagnostic of?
when the S2 is split at any point in the respiration cycle; ASD
Why isn’t ASD caught at infancy?
there’s usually not a loud murmur or symptoms until later in life
What are the phys exam findings in a large ASD?
- increased respiratory rate 2. sweating with feeding 3. hepatomegaly 4. 2-3/6 systolic ejection murmur at L upper sternal border with possible diastolic rumble 5. split 2nd heart sound
What is the normal pressure within the LV?
120
How is VSD treated?
diuretics surgical close if muscular
What will happen in untreated Tetrology of Fallot?
death at the time of ductus arteriosus closure (if severe RVOT obstruction) OR cyanosis, clubbing, poor dental enamel, bleeding tendencies, squat with exercise, arrhythmias AND MAYBE cerebral abscesses
Why does a baby develop symptoms of coarctation 2 days to 2 weeks post delivery after being fine at birth?
the ductus arteriosus closes and worsens the problem
How does a secundum ASD happen?
the osteum secundum was too large or the septum secundum inadequately develops
When should you decide to surgically correct a VSD?
- pulmonary vascular changes 2. persistent symptoms, failure to thrive 3. 2a complications (aortic insuff, double chambered RV)
What is coarctation of the aorta?
a narrowing of the aortic lumen
What does the left endocardial cushion become?
posterior leaflet of the mitral valve
How does Tetralogy of Fallot happen?
abnormal development of the conal crests resulting in an infundibular septum
What will the phys exam findings be in Coarctation of the aorta?
- tachycardia 2. difference in upper extremity and lower extremity BP 3. pulmonary rales 4. hepatomegaly 5. S2 and S3 gallop 6. soft systolic murmur
Why would you want to keep the ductus arteriosus open in a Tetrology of Fallot pt?
to help offset the effects of the RV outflow tract obstruction
Why do atrial arrhythmias occur in ASD patients?
atrial enlargement over time
How does Tetralogy of Fallot happen?
abnormal development of the conal crests resulting in an infundibular septum that is displaced anteriorly, superiorly, and rightward
What are the phys exam findings in Tetrology of Fallot?
- tachycardic and cyanotic OR diaphoretic and tachypnic 2. precordial impulse at the L lower sternal border 3. 2-3/6 short systolic murmur of pulm stenosis
What causes the murmur in ASD?
- systolic ejection murmur = excessive blood flow across the pulmonic valve 2. diastolic rumble = excessive blood flow in diastole across the tricuspid valve
When should you decide to surgically correct a VSD?
- pulmonary vascular changes 2. persistent symptoms, failure to thrive 3. 2a complications (aortic insuff, double chambered RV)
How are Tet spells prevented (after the 1st one)?
beta blockers
DX? 2-3/6 systolic ejection murmur at L upper sternal border with possible diastolic rumble widely split 2nd heart sound
ASD
What are the components of S2 and what do they represent?
P2 = pulmonic valve closure A2 = atrial valve closure
What are the long term risks of untreated ASDs?
development of pulm vascular disease atrial arrhythmias HF
What is a swiss cheese septum?
multiple muscular VSDs
The murmur in a VSD is caused by?
blood flow across the defect
What is the most common congenital heart defect? How common is it?
VSD; 20% of all
What is considered a large ASD?
diameter is greater than or equal to the mitral valve size
How common are ASDs?
7-8% of all congenital heart defects
In Tetrology of Fallot, the RV and LV pressures are _____.
equal
How will a child present with coarctation of the aorta?
systemic HTN, intermittent lower extremity claudication, headaches
Why isn’t a louder murmur bad in VSD?
the VSD could be closing the PVR is low
How do you treat a Tet spell?
- knee-chest position 2. phenylephrine (increase BP) 3. morphine (sedative) 4. IV fluids
Turner’s Syndrome is associated with ______.
coarctation of the aorta
What is the tx for ASD?
- diuretics 2. percutaneous device closure
What syndrome is associated with coarctation of the aorta?
Turner’s Syndrome
How does a secundum ASD happen?
the osteum secundum was too large or the septum secundum inadequately develops
What is the tx for coarctation of the aorta?
surgery for infants and young children; balloon angioplasty with stent for adolescents/adults
What are the 4 components of Tetralogy of Fallot?
- RV outflow tract obstruction 2. RVH 3. dextraposition of the aorta 4. VSD
What is a tet spell?
hypoxic or hypercyanotic episode, usually in the 2-6th month of life can be life threatening unk mechanism
What will happen in untreated Tetrology of Fallot?
death at the time of ductus arteriosus closure (if severe RVOT obstruction) OR cyanosis, clubbing, poor dental enamel, bleeding tendencies, squat with exercise, arrhythmias AND MAYBE cerebral abscesses
The murmur in a VSD is caused by?
blood flow across the defect
What will happen in untreated Tetrology of Fallot?
death at the time of ductus arteriosus closure (if severe RVOT obstruction) OR cyanosis, clubbing, poor dental enamel, bleeding tendencies, squat with exercise, arrhythmias
What does ASD stand for?
atrial septal defect
What causes the murmur in ASD?
- systolic ejection murmur = excessive blood flow across the pulmonic valve 2. diastolic rumble = excessive blood flow in diastole across the tricuspid valve
What does the left endocardial cushion become?
posterior leaflet of the mitral valve
What are some of the s/s of VSD?
respiratory distress diaphoresis (esp with feeding) failure to thrive tachypnea
What is an RV outflow obstruction in Tetralogy of Fallot?
narrowing of the infundibular region stenosis of the pulmonary valve
How does VSD present?
asymptomatic until pulmonary vascular resistance falls
What does a deficiency or lack of the membranous portion of the interventricular septum cause?
a perimembranous VSD
DX? 1. RV outflow tract obstruction 2. RVH 3. dextraposition of the aorta 4. VSD
Tetralogy of Fallot
How can coarcation of the aorta present?
- necrotizing enterocolitis 2. leg muscle pain w/ exercise 3. increased RAAS activation
What will the xray findings be in Coarcation of the Aorta?
- 3 sign along the L edge of the heart 2. rib notching from dilated intercostal a’s
What definitively diagnoses VSD?
echo
The atrial septum fuses with the endocardial cushions, which are involved in ______.
ventricular septation
What is the result of abnormal development of the conal crests resulting in an infundibular septum that is displaced anteriorly, superiorly, and rightward?
Tetralogy of Fallot
What is the tx for ASD?
- diuretics 2. percutaneous device closure
Large, untreated VSDs can cause?
- Eisenmenger’s Syndrome
The murmur of an ASD is NOT related to?
blood flowing across the defect
What are the characteristics of Eisenmenger’s Syndrome?
- muscularization of the Pulm arterioles 2. pulmonary HTN 3. increased RV pressure 4. shunt reverses to R to L 5. no treatment other than transplant
What is the result of abnormal development of the conal crests resulting in an infundibular septum that is displaced anteriorly, superiorly, and rightward?
Tetralogy of Fallot
When do symptoms of ASD usually present?
not in infancy- takes a while to develop symptoms
What is the most common ASD?
secundum ASD
How common are coarctations of the aorta?
5-7% of all congenital heart disease
How is VSD treated?
diuretics
What does the superior endocardial cushion become?
L outlet of the interventricular septum mitral valve
What is considered a large VSD?
those as big as the aortic orifice
What are the characteristics of Eisenmenger’s Syndrome?
- muscularization of the Pulm arterioles 2. pulmonary HTN 3. increased RV pressure 4. shunt reverses to R to L 5. no treatment other than transplant
DX? 1. tachycardia 2. difference in upper extremity and lower extremity BP 3. pulmonary rales 4. hepatomegaly 5. S2 and S3 gallop 6. soft systolic murmur
Coarctation of the aorta
What are the xray findings in VSD?
cardiomegaly with enlarged pulm arteries
What is the normal pressure within the RV?
20
The atrial septum fuses with the endocardial cushions, which are involved in ______.
ventricular septation
When can a VSD be a right to L shunt?
with pulmonary stenosis
DX? precordial activity normal normal 2nd heart sound 2-4/6 early systolic murmur NO diastolic rumble
small VSD
How is ASD definitively diagnosed?
echo
What does the inferior endocardial cushion become?
inlet portion of the interventricular septum membranous portion of the intervent. septum tricuspid and mitral valves
What syndrome is associated with coarctation of the aorta?
Turner’s Syndrome
DX? 1. RV outflow tract obstruction 2. RVH 3. dextraposition of the aorta 4. VSD
Tetralogy of Fallot
What is a fixed split S2 and what is it diagnostic of?
when the S2 is split at any point in the respiration cycle; ASD
DX? 2-3/6 harsh, holosystolic murmur loudest at the LLSB diastolic murmur
VSD
How does Tetrology of Fallot present?
- blue baby 2. loud murmur
What are the 4 components of Tetralogy of Fallot?
- RV outflow tract obstruction 2. RVH 3. dextraposition of the aorta 4. VSD
DX? 2-3/6 harsh, holosystolic murmur loudest at the LLSB diastolic murmur
large VSD
What are the xray findings in VSD?
cardiomegaly with enlarged main pulm artery
What is an RV outflow obstruction in Tetralogy of Fallot?
narrowing of the infundibular region stenosis of the pulmonary valve
How is VSD treated?
diuretics surgical close if muscular
What are the phys exam findings in Tetrology of Fallot?
- tachycardic and cyanotic OR diaphoretic and tachypnic 2. precordial impulse at the L lower sternal border 3. 2-3/6 short systolic murmur of pulm stenosis
What does the right endocardial cushion become?
tricuspid valve
What are the exam findings with a large VSD?
active precordium loud 2nd heart sound 2-3/6 harsh, holosystolic murmur loudest at the LLSB diastolic murmur
DX? 1. tachycardic and cyanotic OR diaphoretic and tachypnic 2. precordial impulse at the L lower sternal border 3. 2-3/6 short systolic murmur of pulm stenosis
Tetrology of Fallot
What does the left endocardial cushion become?
posterior leaflet of the mitral valve
What will the xray findings be in Coarcation of the Aorta?
- 3 sign along the L edge of the heart 2. rib notching from dilated intercostal a’s
What is considered a large VSD?
those as big as the aoric orifice
What are the long term risks of untreated ASDs?
development of pulm vascular disease atrial arrhythmias HF
Where is the most common place for a VSD?
the membranous portion
____ is the most common cyanotic heart defect and accounts for 15% of all congenital heart defects.
Tetralogy of Fallot
What are the steps of atrial septal embryology?
- septum primum closes 2. apoptosis of the middle primum cells to form the osteum secundum 3. septum secundum closes to enclose the foramen ovale
What causes the murmur in Tetrology of Fallot?
pulmonary stenosis
What is S2?
the 2nd heart sound
The atrial septum fuses with the _____, which are involved in ventricular septation.
endocardial cushions
What are the s/s of cerebral abscesses that happen in cyanotic heart diseases?
pt older than 2 years persistent, unexplained fevers behavioral changes
In Tetrology of Fallot, the RV and LV pressures are?
equal
Dx? hypoxic or hypercyanotic episode, usually in the 2-6th month of life can be life threatening unk mechanism
Tet spell (Tetralogy of Fallot)
What is the most common type of VSD?
perimembranous VSD
How will a baby present with coarctation of the aorta?
tachypnea, diaphoresis, poor feeding; cardiac shock with HF; LACK OF FEMORAL PULSES
What are the s/s of cerebral abscesses that happen in cyanotic heart diseases?
pt older than 2 years persistent, unexplained fevers behavioral changes
What is the normal pressure within the RV?
20
