Exam 1 Flashcards
complete or partial absences of a structure caused by environmental or genetic factors
malformation
due to mechanical forces that mold a previously normal part of the fetus over a prolonged period
-often MSK and reversible
deformations
destructive processes which result in morphological alterations of already formed structures
disruption
ex. limb defects caused by amniotic bands
groups of anomalies occuring together which have a common cause*
syndromes
groups of anomalies that often occur together more often than chance alone would allow but whose cause has not yet been determined
associations
ex. CHARGE association
agents, generally chemical, radioactive or infectious which can produce birth defects. These are especially destructive when exposure occurs during the period of organogenesis
teratogens
____ the most common cause of low birth weight babies in the developing world
malnutrition
macrosomia
excessive birth weight
insulin acts as a powerful growth hormone in the fetus, causing
macrosomia
excessive insulin results in risk of ___
hyperglycemia in the newborn period
Describe insulin and glucose and how it relates to the fetus
insulin does not cross the placenta but glucose does. The fetus secretes increased level of insulin to regulate hyperglycemia
glucose is more teratogenic to the fetus when
1st trimester
hyperthyroidism in mom (ie. Grave’s disease) affects the baby how?
- thyroid hormone can cross the placenta slowly
- can cause growth retardation or prematurity in newborns
How does hypothyroidism affect baby
can cause decreased IQ or small stature
Pre-eclamplsia
HTN that generally begins in 3rd trimester and is associated with edema (maternal) and proteinuria
Eclamplsia
HTN, edema, proteinuria and maternal seizures
HELLP
severe form of eclampsia with associated Hemolysis, Elevated Liver, Low Platelets
How does HTN in mom affect baby?
related to insufficient blood supply to placenta bc of vasocontriction
- IUGR (intrauterine growth restriction)
- premature delivery
pregnancy risks in extreme younth
- pregnancy induced HTN
- prematurity
pregnancy risks with advanced maternal age
- Down syndrome (or other chromosomal non-disjunctions)
- increased fetal loss
- inadequate milk supply
- increased risk of pre-eclampsia and HTN
Taking Dilantin (phenytoin) during pregnancy is associated w/
fetal hydantoin syndrome- consisiting of cleft lip and palate as well as mental retardation
Taking Valproic acid during pregnancy is associated w/
neural tube defects (eg. spina bifida)
Taking Keppra during pregnancy
has limited study but shows no teratogenicity
taking Lamictal during pregnancy shows
0.9% (8.9/1000) incidence of cleft lip +/- palate
taking Lithium during pregancy is associated w/
risk of Ebstein abnormality (displacement of TV into RV)
taking Phenothiazines (older anti-psychotic med ex. Haldol and Thorazine) during pregancy is associated w/
no known teratogenic effects but had disagreeable side-effects for adult pt.
what antidepressants are ok to take during pregnancy?
- Tricyclics (ex. amitriptyline and nortriptyline)
- SSRI (ex. Celexa, Prozac, Zoloft)
- Wellbutrin
**avoid Paxil- doubles risk of septal defects in fetus
Xrays during pregnancy are known to cause
microcephaly, spina bifida, celft palate, limb defects
most common cause of IUGR in developed countries an its mechanism
Tobacco!
- direct teratogen
- vasoconstriction
tobacco increases risk of (4)
spontaneous abortion, prematurity, perinatal mortality, and SIDS
most prevalent problem for cocaine babies is
prematurity
(stimulates uterine contractions, coupled with decreased blood flow causes premature labor, premature rupture of membranes and attendant complications )
most prevalent problem for amphetamine babies is
neglect
3 major components of Fetal alcohol syndrome
- prenatal and postnatal growth deficiencies
- Microcephaly w/ cognitive impairment
- Typical facies (short palpebral fissure, indistinct philtrum, thin upper lip)
When do most malformations originate and give an example
during the period of organogenesis, the 3rd to 8th weeks of gestation
-ex. CHD, tetralogy of fallot
components of tetralogy of fallot
- VSD
- pulmonic stenosis or infundibular stenosis
- dextroposition of the aorta so taht it overrides the ventricular septum and receives venous and arterial blood
- RVH
when do deformations occur and give an example
during fetal period (after 9 weeks)
ex. clubfoot
what pregnancy risks to obese women have
- diabetes
- pre-eclampsia
- higher likelihood of c-section and more difficulty healing
obesity is considered BMI > __
BMI > 31
compare birth weights of obese women compared to non-obese women
- equal weights in comparison
- BUT have 30% more fat, at expense of lean body mass
malnutrition can cause
- life long immunodeficiency
- short stature
- congnitive impairment
a B-complex vitamin which can reduce the risk of neural tube defects by 70%
folic acid
when should you start takign folic acid
2 months prior to conception
what vitamins should mom take during pregnancy
- folic acid
- calcium
- iron
- Vit. D
- Vit. A
for bones, teeth, muscle, CNS
calcium
for development of blood cells, CNS
iron
for bones and teeth and improved immunity
Vit. D
important for development of eyes
Vit. A
excess Vit. A and isotretinoin can cause
major malformations (isotretinoin= accutane)
Effects of different types of DM (pregestational, gestational, Type I, Type II)
all have same effect on fetus
excessive glucose can cause
- caudal regression syndromes
- malformations of hips and legs
extreme youth is defined as
less than 15 y/o
advanced maternal age is defined as
greater than 35 y/o
effects of general anesthesia to a pregnant woman
it is not teratogenic
can surgery be performed on MOC during pregnancy
yes
how does marijuana affect the fetus
- measurable cognitive impairment
- mild neurologic abnormalities such as jitteriness, irritability in newborn period
- IUGR
* side effects are controversial
Prescription: fentanyl, oxycodone, methadone, morphine
Non-prescription: heroin
opiates
effects of opiates on the baby
- physical abnormalities are rare
- acute intoxication at delivery can cause respiratory suppression
- born dependent and exhibit signs of withdrawl
treat withdrawl symptoms of a baby with
methadone taper
Effects are result of inhibition of re-uptake of epinepherine, norepinepherine, dopamine and serotonin neurotransmitters by sympathetic nerve endings, thus increasing concentration of neurotransmitters
cocaine
dopamine can casue
euphoria and addiction
Norepinepherine and epinepherine are neurotransmitters which cause
- vasoconstriction
- hypertension
- tachycardia
- diminshed blood flow
- fetal hypoxia
- IUGR
causes of IUGR
- malnutrition
- tobacco
- Marijuana
- Cocaine
- Ampetamines
cocain can cause
- microcephaly,
- IUGR,
- local defects to eyes, limbs, heart and urogenital system, attributable to ischemic events in utero
- hypoxia
- tachycardia
- vasoconstriction
- intoxication at birth
- long-term: cognitive and behavioral problems
- **no motor deficits
synthetic chemicals with properties similar to cocaine but with longer half-life
amphetamines
amphetamines can cause
- neglect
- -IUGR,
- microcephaly*
- cleft lip* and
- palate* w/o specific syndrome
- neurobehaviors (ADHD and aggression)
- *** no intellectual deficits defined at this time
alcohol can cause
- neurodevelopmental problems (ADHD, communication and socialization problems)
- FAS
how much alcohol is needed to cause FAS?
unknown, therefore recommend abstention during pregnancy
what does APGAR stand for and how is it scored?
A-appearance (color) -2 P- pulse (HR)- 2 G- Grimace (reflex irritability/ response to stimuli)- 2 A- activity (tone)- 2 R- respiratory (RR)- 2
Total of 10
* Colorado babys never get 10
how to you check for grimace
rub babe’s back or feet to stimulate a cry
Neonatal circulation shunts
- ductus arteriosus
- Umbilical arteries
- Umbilical vein
- Ductus vensosus
- Foramen ovale
- Functional shunt
shunt between pulmonary artery & aorta
Ductus arteriosus
branches of common iliac arteries- to placenta
umbilical arteries
from placenta to liver or DV
umbilical vein
option to bypass liver, direct to IVC
ductus venosus
interatrial shunt
Foramen ovale
increased pulmonary pressure (shunt)
functional shunt
no valves so has bidirectional flow (high to low pressure)
carries 40% of cardiac output
DA
what filters out impurities of the blood for the baby
placenta
describe the pressure system for system vs lungs in a fetus and adult
fetus: higher pressure in lungs than systemic circulation (blood bypasses lungs via functional shunt and DA)
adult: higher systemic pressure than lungs
how much blood goes to the lungs in the fetus
10-30%
where does most of the oxygenated blood go in the heart?
through the foramen ovale
of any gas is a measurement, in essence, of how much gas there is in any environment
partial pressure
what does a pulse ox measure
% of blood carrying oxygen
what is the % saturation of oxygen air
21% regardless of altitude
- the number of molecules of oxygen are fewer as altitude increases
normal oxygen saturation after transition period
> 95%
What happens to the babys pO2 after the baby starts to breathe air
increase in pO2 in blood
Increase in pO2 in blood when baby starts to breathe air causes:
- Dilatation of vessels in pulmonary bed drops pulmonary pressures; therefore, right sided pressures become lower than left (functional shunt gone)
- Constriction (Closure) of ductus arteriosus
what does cutting off the umbilical circulation cause during the transition period
- systemic pressure to increase
2. therefore, increased volume elsewhere
in the fetus, what side of the heart has higher pressure
right sided pressure is always higher
pressure effect of having LA pressures greater than RA pressure causes
Foramen ovale to close
why does the ductus venosus close
bc there is no longer flow through it
DA normally closes within
48 hrs
DA closes by
- increase pO2 (DA constricts)
2. Decrease in prostaglandins
Umbilical vein and arteries close becasue
blood supply is cut off
DV closes because
blood supply is cut off
Foramen ovale closes because
there is a change in pressure to LA>RA
- Time from onset of ventricular contraction until closure of aortic and pulmonary valves
- Begins with first heart sound- closure of tricuspid and mitral valves
systole
- Time of filling of chambers
- Time between second and first heart sounds
Diastole
most common congenital malformation in newborns
congenital heart disease
-at least 35 variations
1% in general population
Recurrence of CHD is greater in
- those with siblings that have CHD, 1-3% recurrence
- those whose mom has CHD, 15% recurrence
* *not necessarily the same defect
Critical congenital heart disease requires intervention within
first 28 days
serious congential heart disease requires intervention within
1st year
causes/risk factors of CHD
- sibling or parent has CHD
- genetic abnormalities
- Maternal conditions (ex. DM , alcohol use)
___% of childen with Down Syndrome also have CHD
40%
How does congenital heart disease present in a newborn?
- Cyanosis
- Heart failure (or its extreme, shock)
- Cardiac murmur
*varies on nature and severity of defect and Alterations in cardiovascular physiology in the transitional period
Two possible causes of central cyanosis
- Decreased pulmonary blood flow causing a shunt
2. Mixing of oxygenated and deoxygenated blood in heart
cyanosis is typically not recognized until pulse ox is in ___
low 80s
Most babies that are cyanotic is due to ____
pulmonary origin
DDX of central cyanosis
- primary lung disease such as pneumonia, RDS, meconium aspiritation, etc.
- airway obstruction
- Hypoventilation (drug induced or neurological)
- polycythemia
- Congential heart disease
Origin of cyanosis?
- Labored breathing
- Improves with 100% oxygen supplementation
- Cyanosis often improves with crying
Pulmonary
Origin of cyanosis?
- Non-labored respirations
- No or minimal response to 100% oxygen
- Cyanosis worsens or doesn’t improve with crying
Cardiac
Most sensitive tool for differentiating between primary pulmonary and cyanotic congenital heart disease
hyperoxitest
how do you perform a hyperoxitest?
measure pO2 by blood gas or pulse on in room air, then check pO2 on 100% oxygen after 10 min, note pulse ox/blood gas again,
**A patient with primary pulmonary disease will usually dramatically increase saturation when given oxygen
Cardiac etiology will have no improvement
signs and symptoms of congestive heart failure
- increased LA pressure causes congestion and pulmonary edema
- tachypnea
- tachycardia
- Hepatomegaly
- Sweating and feeding difficulties
Descriptors of cardiac murmur
- Intensity
- Timing in systole or diastole
- Quality
- Location
- Transmission
Grade Intensity on a 6 point scale
- Barely audible
- Soft but easily audible
- Moderately loud, but without thrill
Louder and associated with a thrill - Audible with stethoscope barely on chest
- Audible with stethoscope off the chest (usually a mechanical valve)
what does it mean if the murmur is vibratory or muscial
likely innocent
a murmur that is high-pitched and blowing/or coarse indicates
VSD
grating murmur indicates
aortic stenosis (AS) or pulmonic stensosis (PS)
you can distinctly hear S1, closing of the tricuspid and mitral valves
ejection murmur
aka “crescendo-decrescendo
Caused by flow through stenotic or deformed semilunar valve
ejection murmur
you can’t separate the murmur from S1
regurgitant murmur
caused by flow of blood from a chamber that is at higher pressure to one of lower pressure eg. VSD
regurgitant type murmur
Most common benign murmurs
- pulmonic flow murmur aka peripheral pulmonic stenosis or PPS** (most common)
- Physiologic ductus arteriosus
- Tricuspid jet
Loudest at left upper sternal border but radiates throughout the precordium and to the axillae and back
-Usually Grade I-II/VI
pulmonary flow murmur or PPS
Caused by relatively hypoplastic pulmonary arteries- normally formed but underused in fetal life
(Hearing the turbulence against the pulmonary vessels)
pulmonary flow murmur or PPS
Systolic murmur heard at ULSB and below left clavicle
- Usually Grade I-II/VI
physiologic ductus arteriosus murmur
*usually only heard on 1st day of life bc ductus closes off by 2nd day
when the PVR is low enough to function but still high enough to cause a little bit of blood to squirt through and cause a murmur
tricupsid jet murmur
What is the cause of pathologic murmurs that occur at birth
those of stenotic lesions (isolated)
*not affected by changes in pulmonary vascular resistance
what is the cause of pathologic murmurs that present later
murmurs of left to right shunts (like VSD) depend on reduction in pulmonary vascular resistance
Further evalution of a murmur
Fetal echocardiogram History- maternal medical, pregnancy Physical exam Signs of CHF, Cyanosis, murmur 4-point blood pressures (all extremities) Pulse oximetry study Chest x-ray Electrocardiogram Echocardiogram
benefits of fetal echos
- 60% diagnosed before birth
- most severe diagoses can be determined (except: coarctation, small VSD, ASD)
when are the most accurate fetal echos done
at 18- 20 weeks
BP is done to look for __ in a newborn
coarctation of the aorta
___mm Hg difference between right arm and legs is significant for coarctation
10 mmHg
Select the right size blood pressure cuff
- width should be 125%-155% of the diameter of the limb
- length should be long enough to completely encircle the limb
cyanosis does not show up until
for a pulse ox reading to be considered accurate what needs to occur
HR on monitor match manual HR and regular respiratory pattern on screen
______ baby looks more cyanotic than an anemic one at the same saturation
polycythemic baby
Enlarged heart and pulmonary edema (diffusely opaque lung fields) on chest xray are signs of
CHF
dark lung fields on chest xray indicates
decreased pulmonary vascular flow
Boot-shaped heart xray
Fallot Tetralogy
Egg on a string chest xray
Transposition of great vessels
primary diagnostic tool for anatomic definition of congenital heart lesion
echocardiogram
costs $3000
Types of neonatal CHD
- Obstruction to the flow of blood in the heart or great vessels caused by underdevelopment of structures
- Blood flows in abnormal patterns
example of obstruction CHD
- Valvular atresia/stenosis
- Hypoplastic left or right heart syndromes
- Coarctation of the aorta/interrupted aortic arch
From right to left (blue mixes with red)-leads to
cyanosis
From left to right (red mixes with blue)-leads to
CHF
ex. VSD
pressure from RA to LA presents as
cyanotic lesion
ex. PFO
most common cyanotic lesions in newborn (7)
- Transposition of the Great Vessels
- Truncus arteriosus
- Tricuspid atresia
- Tetralogy of Fallot
- Total anomalous pulmonary venous return (TAPVR)
- Pulmonary atresia
- Hypoplastic left heart
most common acyanotic lesions in newborn (5)
- Patent ductus arteriosus (PDA)
- Large septal defects (VSD, ASD)
- Critical aortic stenosis
- Coarctation of the Aorta
- Hypoplastic left heart
what causes a drop in prostaglandins
loss of placenta
direction of blood flow in fetus
R–> L
pathologic direction of blood flow
L–> R
15% of infants with ____ have other cardiac defects
PDA
*associated defects may cause the ductus to close more slowly (ex. coarctation of the aorta)
Most frequent disorder in premature newborns
PDA
patent ductus arteriosus
How is PDA treated
Treated with indomethacin (in preterm babies) or surgical ligation
what could happen if mother takes advil or NSAID during pregnancy
could close off DA
**Take tylenol
most common congenital heart disease
VSD
The magnitude of the Left to Right shunt is determined by
the size of the defect and the pulmonary vascular resistance (PVR).
PVR is highest when
first days of life, gradually falls over weeks
VSD blood flow in fetus
R –> L
VDS blood flow in newborn
L —> R
how to treat VSD
- 50% to 75% of small VSDs will close spontaneously
- try diuretics or digoxin (medications of CHF)
- large ones get closed surgically w/ patch (via cardiac catheterization and placement of an occluder)
Longterm risk of untreated VSD
development of pulmonary vascular disease (hypertension) with continued pulmonary overcirculation
aka. Eisenmenger’s complex
Fixed pulmonary pressures that are non-reversible due to chronic VSD (has a lof of cardiac output on right side)
Eisenmenger’s complex
Can occur anywhere, but usually juxtaductal and presents when DA closes
Coarctation of aorta
coarctation of aorta often has associated
bicupsid aortic valvee (50%)
causes Impaired LV output, decreased pulses below, differential BP and CHF/ shock/ renal failure are late findings
coarctation
CHD frequently in Turner Syndrome
coarctation
When do a baby’s symptoms of coarctation occur
after DA closes and prostaglandins are gone
tachypnea, appears in shock, no femoral pulse, poor peripheral perfusion (looks septic- but don’t have femoral pulses!)
early presenting coarctation of aorta
**typically has some other shunt lesion
treatment of early presenting coarctations
Prostaglandins aka PGE1 (life-saving but temporary fix)
-requires surgery once baby is stable (cath and balloon dilation)
side effects of prostaglandins
apnea
Average age of presentation is 5 years
-Presents with hypertension, easy fatigue in legs
late presenting coarctation
**great collateral circulation because no other shunt
treatment of late presenting coarctation
non-emergent
-requires surgery (cath and balloon dilation)
may open ductus and help widen area of aortic constriction
PGE1
Spectrum of undergrowth of left side of heart, including severe coarctation of aorta, severe aortic or mitral valve stenosis or atresia, hypoplasia of LV and ascending aorta. Coronary blood flow is retrograde from PDA
hypoplastic left heart syndrome (HLHS)
what presents with Poor myocardial function, no systemic cardiac output once PDA closes andsevere CHF and shock
HLHS
**in utero diagnosis becoming more common
treatments of HLHS
-prior to repair, use PGE1 or PDA stent to keep DA open or -Staged repair (Norwood operation) -Heart transplantation -Comfort care (hospice)
Congestive heart failure (severe) with impaired alveolar ventilation and O2 diffusion causes
cyanosis from CHD
4 elements of TOF
- Ventricular septal defect
- Overriding of ascending aorta
- Obstruction of RV outflow tract
- Right ventricular hypertrophy (muscle grows to overcome pressure)
most common cyanotic congenital heart defect; may present beyond newborn period
tetralogy of fallot (TOF)
treatment of TOF
- medical management of hypercyanotic episodes (“tet spells”) from transient increase in RV outflow tract obstruction)
* *use propranolol (relax vessels to increase flow), oxygne, knee-chest position - surgical repair when older
a palliative shunt procedure is done (ie Blalock-Taussig) that connects subclavian artery to pulmonary artery
is done when
a baby is severely hypoxic in newborn period due to TOF
The aorta & pulmonary a. arise from wrong sides
*2 closed systems
Transposition of the Great Arteries (TGA)
treatment of TGA
create a PFO to allow blood flow to mix
A single arterial vessel arises from the heart
Pulmonary vessels arise from the truncus
Truncus arteriososus
What CHD ALWAYS has a large VSD present
Truncus arteriososus
CHD Often associated with DiGeorge syndrome, a deletion of a portion of chromosome 12
Truncus arteriososus
- Pulmonary veins do not empty into left atrium
- Connection can be to right atrium, superior or inferior vena cava, coronary sinus
Total anomalous pulmonary venous return (TAPVR)
Obstructed veins in TAPVR leads to
pulmonary venous hypertension, edema, and severe hypoxia
treatment of TAPVR
surgical and emergic if obstructed
What CHD requires an ASD for blood flow to get to left side of heart
TAPVR
snowman heart on xray
TAPVR
Tricuspid valve is absent
resulting in functionally hypoplastic right ventricle & pulmonary arteries
tricuspid atresia
what presents with cyanosis and CHF
tricupsid atresia
treatment of tricupsid atresia
- PGE1 is initial therapy to maintain patent DA and VSD
- then surgery
Prominent LV silhouette
Variable pulmonary blood flow on xray
tricupsid atresia
Crucial in left-sided obstructive lesions & lesions with decreased pulmonary blood flow
PGE1
what medication improves pulmonary edema
diuretics
what medications improve cardiac contractility
Dopamine
dobutamine
digoxin
cardiac caths are used to
open valves, atrial septum wiht balloon
supplemental oxygen provides
increase pulmonary blood flow bc pO2 dilates pulmonary beds, alveolar oxygen delivery
