2] Pediatric <3 Disorders Flashcards
What is incidence
of new cases
Incidence of peds and heart conditions
Congenital heart defects occur in about 1% = 40,000/year
What is prevalence
Total # of cases of diseases in a period of time
Prevalence of congenital heart disease
1 million kids
1.4 million adults
15% of babies born with CHD have ?
Other genetic conditions
How can you identify kids with CHD?
Newborn screening - add pulse ox
Cause of CHD?
Unknown
What’s the genetic association with CHD?
Down syndrome
The heart begins as
Two strands
The heart has two adjacent tubes at
Day 18
The two heart tubes fuse at
Day 21
The heart is beating on
Day 22
Single atria chamber and single ventricular chamber pump
Blood by day 27
Ventricle forms the
Truncus arteriosus
What grows in the truncus arteriosus
Septum
Septum forms
Aorta and pulmonary artery
Atria wall forms an opening when? And what?
Days 27-37 and foramen ovale
Heart is completely developed by
Weeks 7-10
What’s formed by weeks 7-10
Ductus arteriosus
What kind of resistance is in the fetal lungs and why
Higher resistance and fluid follow spath of least resistance
Connection from the pulmonary artery to the aorta
Ductus arteriosus
What’s between the atria
Foramen ovale
What happens as the baby takes first breath and air fills the lungs
Pulmonary arteries and capillaries DIATE and fluid moves into arterioles
Resistance is what in lungs
Lower
What happens to foramen ovale ?
Closes due to high pressure on L side
When does foramen ovale fuse
Before month 3
When oxygen levels rise, muscle in the ductus arteriosus contracts and
DA closes 10-15 hours after birth
2 types of congenital heart defects
Cyanotic vs acyanotic
Low oxygen saturation
- tetralogy of Fallot
- hypoplastic left heart syndrome
Cyanotic
Normal oxygen saturation’s
Acyanotic
Volume issues to lungs
Acyanotic
Right to left shunt
Cyanotic
Left to right shunt
Acyanotic
Correction of aorta
PDA
ASD
VSD
Acyanotic
Signals increase for RC formation in which defect
Cyanotic
Increased risk for cerebrovascular insult
Cyanotic
What is ASD
Atrial septal defect
Which way does the shunt go for ASD
Left to right shunt
Characteristics of ASD
L heart is less compliant.
Mor epressure on L side.
Which results in increased blood flow to R side
Sx of ASD
Dysrythmia
SOB
FTT/poor weight gain
Exercise intolerance
What does ASD present like in adults
• In adults, may present with SOB, leg swelling, dysrhythmia’s in 30’s or stroke.
How is ASD repaired
By sewing the opening closed or with a patch
What is VSD
Ventricular septal defect
40% of congenital heart diseases
VSD
Shunt for VSD
Most commonly results in L to R shunt
VSD results in
R sided HF
Irreversible lung damage
Turbulent blood flow that damages aortic valve
Some may close on their own
– If symptomatic (same sx as ASD), requires patch
• Can now be done percutaneous
– If asymptomatic, will be repaired if large and there is a lot ofblood flow to the lungs
Tx for VSD
What is PDA
Patent ductus arteriosis
What does the PDA connect
Pulmonary artery to the aorta
In PDA, failure to close results in blood flowing
From aorta to PA and lungs
PDA has an increased risk of
Endocarditis
a medicine that helps close
PDAs in premature infants. This medicine triggers the PDA to
constrict or tighten, which closes the opening. Indomethacin usually doesn’t work in full-term infants.
Indomethacin
What’s another medium used to close PDAs in premature infants
Ibuprofen
What is COA
Coarctation of the aorta
What does COA mean
Narrowing where the DA attached to aorta
Can occur in isolation or with other congenital heart abnormalities (VSD)
COA
Sx of COA
Decreased blood flow to body leads to organ damage and diminished pulse
FTT
• HTN
• Heart failure
Other Sx of COA
Treatment for COA
Surgery
Acyanotic defects (4)
ASD
VSD
COA
PDA
2 CYANOTIC congenital conditions
TOF
HLHS
Muscle that separates Aortic valve from pulmonary valve is not in theright location.
TOF
What does TOF stand for
Tetralogy of Fallot
4 deficits of TOF
1] Obstructs pulmonary flow
2] VSD
3] aorta lies over VSD (overriding aorta)
4] R ventricular hypertrophy
Obstruction of pulmonary flow with TOF causes
Decreased oxygen- cyanosis
What’s an overriding aorta with TOF
Aorta shifted to the R and sits over the VSD
Surgery for TOF
Shunt to address obstruction- connects a small branch off aorta to pulmonary artery
What happens with a full repair in TOF
VSD repair with patch that reduces pulmonary flow obstruction
Long term issues of TOF
If pulmonary valve is leaky- exercise intolerance need for surgery as adult.
Dysryhtmia.
Risk for endocarditis.
What does HLHS stand for
Hypoplastic left heart syndrome
What is HLHS
Underdevelopment of the L side of the heart
If HLHS is found early?
Prostaglandins is given to keep ductus arteriosis open
HLHS can be picked up on
Prenatal ultrasound
Surgical options for HLHS
Transplant or staged reconstruction
Where is the apical pulse
4th ICS
Typical newborn HRs are at ?
100-180 b/m
Femoral pulse- hyperdynamic pulses may indicate
PDA
Shape of thorax at 0-3 months
Triangle
Shape of thorax after 3 months
Rectangle
Direction of ribs 0-6 months
Horizontal
Direction of ribs 6-12 months
Angled down
Primary muscles used for inspire at 0-3 months
Diaphragm
Primary muscles used for inspire 3-6 months
Diaphragm and accessory muscles
Primary muscles used for inspiration at 6 - 12 months
Diaphragm and intercostals
Kids start sitting at
6 months
After correction of underlying problem and medical clearance, the AHA recommends ?
30 minutes of light to moderate exercise
Would static be good for CHD?
Static exercises cause a higher blood pressure response, especially with valslava maneuver.
What happens with static exercises ?
Puts an afterload pressure on the LV
What about dynamic exercises with CHD?
Dynamic exercises cause an increase in volume to the left ventricle.
Equation for dynamic exercises
For every 1/min increase in oxygen uptake, there is a 5-6 L/m COincrease needed
What happens with dynamic exercises ?
Puts a VOLUME load on the LV
If they have ASD and no PH, they can
Participate in all sports
AD with PH can
Participate in class IA sports
ASD with PH, R to L shunt can ?
No participation in competitive sports but possibly IA after Evaluation
ASD treated: Post-op 3-6 months, no PH ,no RV Dysfunction, No Dysrhythmia can ?
Participate in all sports
Small VSD, no PH can
Play all sports
Large VSD and PH can
Pay IA sports
3-6 months post op, no dysrhythmia, no PH
All sports
3-6 months post op with PH can play
IA ports
3-6 months post op with atrial or ventricular tachycardias
Must be evaluated by an
electrophysiologist before any sportacctivity
If cyanotic heart disease is stable on CPET and no dysrhythmias or significant desaturation may
May be considered for IA sports
With Marfan syndrome you have to have 1 copy of
• Autosomal dominant connective tissue disorder
What happens in Marfan syndrome?
Abnormalities in microfibrils diminish structuralintegrity of vessel walls
Can cause compression of RA/RV and decreases vital capacity
Excavatum (depression) in Marfan
Rigid chest wall, increased energy
consumption for breathing, alveolar hypoventilation, Cor pulmonale
Carinatum (pigeon chest) in Marfan
Wrap 1st and 5th digits around opposite wrist. (+) if overlap
+ stein berg
Scoliosis
• Limited elbow extension
• Visual changes
• Flat feet
Marfan syndrome
People with Marfan syndrome may participate in low/moderate static and dynamic sports (IA and IIA) unless they have:
Aortic root dilation
– Moderate or severe Mitral regurgitation
– LV systolic dysfunction c. L
