Pediatric Cardiology Flashcards
What congenital heart defect does PDA stand for?
Patent Ductus Arterious
What congenital heart defect does ASD stand for?
Atrial Septal Defect
What congenital heart defect does VSD stand for?
Ventricular Septal Defect
What congenital heart defect does TET stand for?
Tetrology of Fallot
What congenital heart defect does Coarc stand for?
Coarctation of the Aorta
A ____ heart defect is defined as a defect that occurs as a result of abnormal development of the heart and related structures during development
Congenital
Congenital heart defects affect between __-__ babies per 1000 live births
4-10
Congenital heart defects are the leading cause of ___ (excluding prematurity) during the first year of life
Death
During the first year of life, ___% of infants with heart defects require treatment or diet within the first year
35
The cause of the heart defects is only known in about ___% of cases
10
In the United States, there seems to be an ____ in cases of congenital heart defects; the cause is not known but it is thought that we just have better detection methods today
Increase
The risk for development of congenital heart disease increases when there is an increase in the number of ___ ___ relatives affected
First degree
What heart defects can be caused by trisomy 13?
-VSD
-PDA
-ASD
What heart defects can be caused by trisomy 21?
-Endocardial cushion defect
-VSD
-PDA
-ASD
-TET
-Transportation of great vessels
-Coarctation of the aorta
What types of heart defects might be caused by Turner’s Syndrome?
-Coarctation of the aorta
-PDA
-Pulmonary stenosis
-Septal defects
What types of heart defects might be caused by Cru du chat Syndrome?
-PDA
-Mixed defects
About ___% of congenital heart defects are thought to be caused by a combination of genetic and environmental factors
90
Because of the improved treatment methods, many children are growing up and having children; the risk of a child having a congenital defect is ___-___%
5-15%
If two siblings have cardiac anomalies, the risk is about ___% for the next child, but if three children are affected, the risk jumps to ____%
9; 50
Intrauterine infections, along with viral infections and Rubella, may cause what types of heart defects?
-PDA
-Pulmonary stenosis
-Coarctation of aorta
___ ____ can infect endothelial cells, but the specific cardiac effects are unknown
Herpesvirus cytomegalovirus
Diabetes might cause what types of heart defects?
-VSD
-Cardiomegaly
-Transposition of great vessels
What types of heart defects might be caused by PKU?
-PDA
-Coarctation of the aorta
Hypercalcemia may cause what types of heart defects?
-Supravalvular aortic stenosis
-Pulmonary stenosis
Alcohol abuse may cause what heart defects?
-TET
-ASD
-VDS
Warfarin use may cause what heart defects?
-ASD
-PDA
Increased maternal age increased risk for what cardiac defects?
-VSD
-TET
Prematurity increases risk of what heart defects?
-PDA
-VSD
Living at a high altitude increase risk for what heart defects?
-PDA
-ASD
Approximately the ___-___ day of fetal life, two lateral endothelial heart tubes fuse to form a single endocardial tube
21st-22nd
By the ___ day, the tube thickens and the first fetal heart contractions occur
28th
The tube them coils to the right and by the end of the ___ week, the tube is completely coiled and the major chambers are identified
4th
By the 22/23rd day, what structures can be identified in the heart?
-1st aortic arch
-Bulbus cordis
-Ventricle
-Atrium
By the 23/24th day, what structures can be identified in the heart?
-Truncus arteriosus
-Bulbus cordis
-Ventricle
-Atrium
-Sinus venosus
By the 24/25th day, what structures can be identified in the heart?
-1st and 2nd aortic arches
-Truncus arteriosus
-Ventricle
-Premature left atrium
The cranial end of this primitive heart is known as the ___ ___ which will further divide into the great vessels
Truncus arteriosus
The caudal end of the primitive heart will become the…
Superior and inferior cava
____ is also occuring at this time which creates the beginning of the heart valves
Septation
The heart is completed by __ __ after conception, therefor some congenital defects are formed before the pregnancy is recognized
6 weeks
Fetal ____ is anatomically and physiologically different from postnatal
Circulation
Oxygenation occurs in the ____ and not in the lungs
Placenta
Fetal lungs are filled with fluid and have low ___ ___ tension which produces pulmonary vasoconstriction
Arterial O2
There is ___ resistance to pump blood into the fetal lungs, but we don’t want much in there anyway
High
Because of the pulmonary constriction, only ___% of the fetal cardiac output is received by the pulmonary circulation
8
Fetal systemic vascular resistance is low, and nearly half of the blood flow enters the ____ which provides low resistance to flow
Placenta
The fetus is ____ with an O2 saturation of approximately 60-70%; however the fetus is not hypoxic because of the extremely high cardiac output
Hypoxemic
The cardiac output of a fetus averages about ___-__ ml/kg/min
400-500
The best oxygenated blood from the placenta travels to the fetal ___
Brain
The fetal blood is oxygenated in the placenta, which is a less efficient oxygenator than the ___
Lungs
The oxygenated blood enters the fetus through the ___ ___ to the liver
Umbilical vein
About 50% of oxygenated blood enters the ___ ___, bypassing the hepatic circulation and flowing into the inferior vena cava
Ductus venosis
When this blood reaches the right atrium, it is diverted by the ___ ___ toward the atrial septum, and flows through the foramen ovale into the left atrium
Crista dividens
The blood then passes through the left ventricle and ___ ___ to perfuse the head and upper extremities
Ascending aorta
This pathway allows the best-oxygenated blood from the placenta to perfuse the ___ ___
Fetal brain
Venous blood from the heat and upper extremities results to the fetal heart through the ___ ___ ___, enters the right atrium and ventricle, and flows into the pulmonary artery
Superior vena cava
Since pulmonary vascular resistance is high, this blood is diverted through the ductus arteriosus into the ___ ____
Descending aorta
Ultimately, much of this blood will return to the ____ through the umbilical cord to be reoxygenated
Placenta
Within the first few breaths, the lungs are filled with air and it becomes a more efficient ____
Oxygenator
With this in increase in O2 and decrease in CO2, the ___ ___ ___ starts to drop
Pulmonary vascular resistance
As the peripheral vascular resistance starts to decrease, right atrium and right ventricle pressures ____
Decrease
Systemic vascular resistance increases due to the elimination of the placenta, therefore increasing ___ ___ and ___ ___ pressure
Left atrium, left ventricle
This change in pressure gradient functionally closes the ___ ___
Foramen ovale
Anatomic closure, due to ___ deposits, is completed within the first few months of life
Fibrin
The change in O2 and CO2 levels is caused by constriction of the ___ ___
Ductus arteriosus
Another factor in constriction of the ductus arteriosus is the sudden drop in ____
PGE1
The ductus is usually functionally closed by ___ to ___ hours after birth with anatomical closure within ___-___ days of life
15-18 hours; 10-21 days
If a defect is present, the duct can stay open or be reopened with medications like _____
Prostaglandin
Due to the vasoconstriction of the umbilical arteries and the elimination of blood flow through the ___ ___, by 7 days post-natal, there is no flow through the duct
Ductus venosus
The ductus venosus because then becomes the ___ ___
Ligamentum venosum
There is marked change in ___ ___ after birth
Pulmonary resistance
The change in pulmonary resistance in a neonate is ___ and related to the infant’s condition
Variable
Problems with increased peripheral vascular resistance that occur are related to factors that cause constriction of the ___ ___
Pulmonary bed
Constriction of the pulmonary bed can result in ___ ___ (2/1000 live births- children may be very sick)
Pulmonary hypertension
With a ____ lesion, there is usually an obstruction to pulmonary blood flow
Cyanotic
With a cyanotic lesion, blood shunts from ___ to ___, decreasing blood flow to the pulmonary circulation
Right to left
Cyanotic lesions cause a mixing of ____ and ___ blood
Oxygenated and deoxygenated
With an ____ lesion, blood usually shunts from left to right
Acyanotic
With acyanotic lesions, blood moves from ____ pressure to ___ pressure
High to low
Usually, acyanotic lesions cause ___ ___ of pulmonary blood flow
Over circulation
____ lesions are a stenotic area with restrictive blood flow
Obstructive
With obstructive lesions, there is hypertension ___ to obstruction
Proximally
With obstructive lesions, there is hypoperfusion ____ from obstruction
Distally
A patent ductus arteriosus (PDA) is caused by…
Failure of ductus arterious to close
With PDA, blood shunts normally from ___ to ___
Left to right
Is a PDA a cyanotic or acyanotic defect?
Acyanotic
A PDA can be part of a ____ defect
Complex
A PDA can be ___ ___ is more complex defects
Medically necessary
With a PDA, there is increased blood flow to the ____, resulting in increased workload on the left side of the heart
Lungs
Clinical manifestations of PDA:
-Continuous machinery-type murmur heart beat at the upper left sternal border through systole and diastole (aortic area)
-If the lesion is significant, infants will also have bounding pulses, and active precordium (you can see their heart beating), a thrill upon palpation, and signs and symptoms of CHF from the pulmonary overcirculation
A small PDA is usually ____
Asymptomatic
Atrial septal defects (ASD) are caused by…
Improper formation of the septal wall
Is an ASD cyanotic or acyanotic?
Acyanotic
An ASD causes a failure of the ___ ___ to close
Foramen Ovale
ASD is the ___ most common congenital heart defect
4th
___ ___ ___ is an opening found low in the septum that may be associated with AV valve abnormality
Ostium primum defect
___ ___ ___ is an opening in the center of the septum and is the most common
Ostium secundum defect
___ ___ ___ is an opening high up in the atrial septum near the superior vena cava and right atrium junction
Sinus venosus defect
With ASD, blood is shunted ___ to ___ because of the higher pressure of the left atrial chamber
Left to right
ASD might cause right atrium and right ventricle ___
Enlargement
Children with ASD are usually ____
Asymptomatic
With an ASD, you will hear a ____-____ systolic ejection murmur due to increase blood flow the pulmonary valves
Crescendo-decrescendo
Erb’s point is…
2nd-3rd intercostal space, left sternal border
With an ASD, you might also hear a wide fixed splitting of the ____ heart sound due to volume overload in the right ventricle
Second
Volume overload causes a prolonged ___ ___ and delay of pulmonic valve closure resulting in the splitting sound
Ejection time
Small ASD might go undetected until adulthood when ____-like symptoms appear
Stroke
A ventricular septal defect (VDS) is a defect in the ___ ___
Septal wall
Is a VSD cyanotic or acyanotic?
Acyanotic
Those with VSD are usually asymptomatic until there is a fall in ___ ___ ___ at 2-4 weeks of age
Peripheral vascular resistance
A VSD is the most common congenital heart defect, accounting for approximately ___-___% of all defects
25-33%
With VSD, blood is shunted from ___ to ___
Left to right
The amount of shunting with VSD is dependent on the size of the defect as well as the degree of ___ ___
Pulmonary resistance
With VSD, blood is shunted on ____, so blood goes right out the pulmonary artery into pulmonary circulation
Systole
With VSD, you will see ____ of the main pulmonary artery, left atrium, and left ventricle
Enlargement
With VSD, ___ ___ hypertrophy occurs to effectively pump the additional volume
Left ventricle
With VSD, eventually the heart can’t handle the increased volume and ___ ___ ____ develops
Congestive heart failure
Clinical manifestations in children with VSDs depend on…
-Age of the child
-Size of the defect
-Level of pulmonary resistance
Newborns with VSD are relatively ___
Asymptomatic
The high ___ ___ ___ equalizes pressure in the ventricles
Peripheral vascular resistance
Once peripheral vascular resistance drops, left-right shunting occurs resulting in…
Murmur (load, holosystolic left sternal border
Large VSDs lead to symptoms like…
-Symptoms of congestive heart failure
-Poor weight gain
-Loud, harsh, holosystolic murmur
-Systolic thrill at left lower sternal border
Adults with unrepaired VSD may develop ___ ___ ___ ___ which may cause cyanosis and clubbing
Pulmonary vascular obstructive disease
Tetralogy of fallot (TET) is the most common ____ defect
Cyanotic
TET is what four defects in one?
-VSD
-Pulmonary stenosis
-Right ventricular hypertrophy
-Overriding aorta
TET occurs __-__% of the time
9-14%
Pathophysiology of TET varies widely based on…
-Degree of pulmonary stenosis
-Size of the VSD
-Pulmonary and systemic resistance to flow
If pulmonary resistance is high with TET, you will see a ___ to ___ shunt
Right to left
If pulmonary stenosis is minor and pressure is lower, you will see a ___ to ___ shunt
Left to right
Pulmonary stenosis decreases the amount of blood flow to the ____
Lungs
Pulmonary stenosis causes an increase in pressure which results in a __ to ___ shunt
Right to left
People with pulmonary stenosis have a mixing of deoxygenated blood with a decreased amount of oxygenated blood from the lungs; the body compensates by increased the amount of ___ ___ ___ (polycethmia)
Red blood cells
Clinical manifestations of tetralogy of fallot:
-While the PDA is open, the newborn has adequate pulmonary blood flow
-Once the duct closes, cyanosis occurs
-Over time, you may see clubbing of fingers and toes
A tet spell might cause…
Sudden onset of dyspnea, cyanosis, and restlessness which occurs with crying or exertion
One theory about tet spells is that right ventricle outflow tract goes into spasm, increasing pulmonary resistance and __-__ shunting
Right-left
A compensatory mechanism for older children with tet spell is to ___, which increases systemic vascular resistance while decreasing venous return to the heart
Squat
The decrease in venous return that happens when squatting allows for more ___ ___ to be available and the increase in systemic vascular resistance helps reverse the shunt to more left-right
Oxygenated blood
Coarctation of the aorta (Coarc) is severe ____ of the arch of the aorta
Narrowing
The ____ and ____ of the coarc predict presentation
Location and narrowing
Coarc commonly occurs because of an abnormal ___ ___ ___ that constricts at the time of ductal closure
Contractile ductal tissue
Coarc causes ___ pressures above the site of stenosis and ____ pressures below the site
Higher; lower
You may see ___ ___ ___ is the coarc is pre-ductal
Congestive heart failure
The high pressure above the coarc are seen in the ___ ___ served by the arteries where the pressure is higher
Systemic circulation
Clinical manifestations of coarc in newborns:
-Usually present with signs of congestive heart failure
-Once the ductus closes, infants deteriorate rapidly with the development of hypotension, acidosis, and shock
Older children with coarc might not be diagnosed until ____ is noted
Hypertension
In older children with coarc, you would see ____ in upper extremities and ____ in lower extremities
Hypertension; hypotension
If pulses are affected by the coarc, we will see…
-Mottled skin
-Leg cramps during exercise from anorexia
Hypertension may cause…
-Dizziness
-Headache
-Fainting
-Epistaxis
___ ___ is a narrowing of the right ventricle outflow tract
Pulmonary stenosis
There is a decrease in ____ blood flow with pulmonary stenosis
Pulmonary
Pulmonary stenosis can be…
-Supravalvular
-Subvalvular
-Valvular
___ ___ ___ is a result of the stenosis
Right ventricular hypertrophy
If the backup into the right atrium is severe with pulmonary stenosis, you may see reopening of the __ __, causing ASD
Foramen ovale
Clinical manifestations of pulmonary stenosis depends on ___
Severity
Systolic ejection murmur at the __ ___ ___ ___ reflects obstruction to flow
Left upper sternal border
With pulmonary stenosis, you might palpate a ___
Thrill
If child has moderate stenosis, they may have ___ ___ because of insufficient pulmonary blood flow
Exertional dyspnea
Severe pulmonary stenosis results in…
-Cyanosis
-Right sided failure
Is a transposition of the great arteries cyanotic or acyanotic?
Cyanotic
With a transposition of the great arteries, the ___ and ___ ___ are switched
Aorta and pulmonary artery
Transposition of the great arteries is called a ___ defect because of the dependence upon mixing of the pulmonary and systemic circulation for survival
Mixing
Patients with transposition of the great arteries must have one of these things to survive:
-Patent ductus arteriosus
-ASD
-VSD
With transposition of the great arteries, there is mild cyanosis shortly after birth which worsens during the first day when the ___ ____ closes
Ductus arteriosus
When the ductus arteriosus closes, it causes hypoxemia which causes symptoms like…
-Metabolic acidosis
-Tachycardia
-Tachypnea
The presence of a ___ or ___ ___ allows for more mixing with transposition of the great arteries
PDA or septal defect
If someone with transposition of the great arteries has an intact septum, you will not hear a ____
Murmur
Fixing a transposition of the great arteries is a tricky procedure because the __ __ must be moved along with the ___
Coronary arteries, aorta
___ ___ is also known as mucocutaneous lymph node syndrome
Kawasaki Disease
Kawasaki disease is acute self-limiting ____ that may result in cardiac complications
Vasculitis
Are males or females more affected by Kawasaki disease?
Males (slightly)
Peak incidence of Kawasaki disease is in what seasons?
-Winter
-Spring
Kawasaki disease is a primary disease of ___ ___
Young children
___% of cases of Kawasaki disease are in children less than 5 years of age
80%
The first reported cause of Kawasaki was reported in ___ by Dr. Thomisakyu Kawasaki
1967
Kawasaki occurs throughout the world but the greatest number of cases are reported in ____
Japan
What U.S state has the highest incidence of Kawasaki?
Hawaii
Kawasaki disease occurs in ___ stages
4
What happens in stage I of Kawasaki disease (0-12 days)?
Small capillaries, arterioles, venules, and the heart become inflamed
What happens in stage 2 of Kawasaki disease (12-25 days)?
Inflammation spreads to larger vessels
What happens in stage 3 of Kawasaki disease (26-40 days)?
Coronary arteries begin to thicken and inflammation resolves
What happens in stage 4 of Kawasaki disease (40 days and beyond)?
Vessels develop scarring, calcification, and stenosis of coronaries
Symptoms of the acute phase of Kawasaki:
-Fever (most common)
-Conjuntivitis (begins within days of fever onset)
-Strawberry tongue
-Rash
-Lymphadenopathy (occurs in 50-70%)
-Irritability
Symptoms of the subacute phase of Kawasaki:
-Fever ends
-Clinical signs resolve
-Desquamation of palms and soles
-Thrombocytosis (high platelet count)
During the subacute phase, the child is at the highest risk for developing ___ ___
Coronary aneurysm
Symptoms of the convalescent phase of Kawasaki:
-Elevation of erythrocyte sedimentation rate and platelet count
-Possible arthritis
The convalescent phase lasts until all ____ return to normal
Labs
Eisenmenger’s Syndrome is a defect of the ___ ___ (ASD or VSD) with severe pulmonary hypertension, hypertrophy of the right ventricle, and latent or overt cyanosis
Interventricular septum
Because of the VSD with Eisenmenger’s, there is an ___ in blood flow to the lungs and an ___ in blood pressure in the lungs
Increase; increase
When the high blood pressure becomes an irreversible problems on its own, the direction of blood through the ___ in the heart is reversed
Hole
The reversal of the direction of shunting ____ pressure resulting in a narrowing of the arteries
Increases
Over time, these changes are irreversible due to ___ of the arteries
Inflammation
Much of the lung arteries become ___, leading to increase in pulmonary blood vessel resistance
Occluded
Once the resistance is so high, the shunt reverses to ___ to ___
Right to left
Symptoms of Eisenmenger’s syndrome:
-Cyanosis
-Clubbing
-Dyspnea and SOB
-Hemoptysis
-Activity intolerance
-Palpitations
-Syncope
-Erythrocytosis
-Sudden death
-Symptoms of pulmonary hypertension
-Symptoms of heart failure
