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Pediatric Cardiology

Terms in this set (148)

oxygenated
_____ blood enters from the umbilical vein
Ductus Venosus
some oxygenated blood perfuses the liver---most bypasses the liver into the inferior vena cava via the _____ _____
right atrium
the blood then enters the _____ _____
foramen ovale
1/3 of blood goes into the left atria through the patent _____ _____
right ventricle
2/3 of blood mixes with the venous return from the superior vena cava and enters the _____ _____
pulmonary arteries
the blood the goes out through the _____ _____
8-10%
_____-_____% of blood goes to the lungs
patent ductus arterioles (PDA)
905 of blood goes through the _____ _____ _____ to the descending aorta which supplies the fetal body
descending aorta
the _____ _____ supplies the fetal body
high pulmonary resistance
the amount of blood going to the lungs remains low due to what?
right ventricle
in utero, the _____ _____ is dominant
left ventricle
in neonate life the _____ _____ is dominant
air
with onset of breathing...
-_____ replaces lung fluid
resistance
with onset of breathing...
-pulmonary vascular _____ falls
blood flow
with onset of breathing...
-increase in _____ _____ to the lungs
left atria
with onset of breathing...
-increased pulmonary return to the _____ _____
left atrial pressure now > than right
which atrial pressure is greater?
foramen ovale
with onset of breathing...
-_____ _____ closes (functionally)
ductus arteriosus
with continued increase in pulmonary flow and O2 tension..._____ _____ begins to constrict
-functional closure usually within 24-48hrs in a team neonate
-permanent closure needs fibrosis and may take several week (sometimes 3 months)
24-48hrs
the ductus arterioles has it functional closure at what time?
several weeks sometimes 3 months
-needs fibrosis
the ductus arterioles has its permanent closure at what time?
innocent (functional) and significant
most important job in primary care is to distinguish between _____ and _____ murmurs
innocent murmurs
_____ _____ are not associated with significant hemodynamic abnormalities
-40-45% of children have these at some time
characteristics of innocent (functional murmurs)
-always systolic
-never higher than grade I-II
-not associated with symptoms of cardiac disease (cyanosis, poor feeding, SOB)
-vary with position or inspiration
evaluated
any murmurs that do not fall within the guidelines must be _____
grade I
-soft with heard with difficulty
grade II
-soft but easily heard
grade III
-loud but without a thrill
grade IV
-loud with a thrill
grade V
-audible with the edge of a stethoscope on chest
grade VI
-audible with stethoscope off the chest or by naked ear
Congenital Heart Disease (CHD)
-incidence is 8/1000 live births in the US and European populations
-there may be no identifiable cause

known causes include: teratogens and genetic disorders
Teratogens and Genetic Disorders
2 known causes of Congenital Heart Disease (CHD)?
Teratogens
-congenital rubella, fetal alcohol syndrome etc
Genetic Disorders
-Trisomy 21, Turner syndrome etc
Heart Failure
Cyanosis (distinguish from a respiratory etiology)
signs / most important clues for Congenital Heart Disease (CHD)? (2)
heart failure

cyanosis

major defects affect ability to grow (failure to thrive)
signs of Congenital Heart Disease (CHD)? (3)
history for neonates with CHD
-poor feeding
-easy fatigue
-lethargy
-vomiting
Acyanotic Heart Disease
no right to left shunt? no blue coloration of the neonate due to this heart disease.
Ventricular Septal Defect (VSD)
-hole between ventricles
-most common heart defect
-25-30% of CHD
Ventricular Septal Defect (VSD)
what is the MOST COMMON heart defect?
Ventricular Septal Defect (VSD)
harsh, medium - high pitched, grade II-V pansystolic murmur maximal @ LLSB (3rd and 4th ICS)
heave or systolic thrill
larger Ventricular Septal Defect (VSD) (6-10mm) may have a left ventricular _____ or _____ _____ at LLSB
asymptomatic
Ventricular Septal Defect (VSD)... small holes (<3mm) may be _____
-failure to thrive
-tachypnea
-diaphoresis with feeling
-easy fatiguability
Ventricular Septal Defect (VSD)... larger holes may cause what by 3-6 months old?
Ventricular Septal Defect (VSD)
often presents before 2 months old with heart failure
older children with Ventricular Septal Defect (VSD)
what age group?
-experience exercise into;erance and easy fatiguability
-may have frequent respiratory infections and slow weight gain
close spontaneously
treatment for Ventricular Septal Defect (VSD)
-80-85% are small (<3mm) and _____ _____
asymptomatic
_____ pts with normal heart size need NO treatment

-Ventricular Septal Defect (VSD)
2y/o

6y/o
50% of small VSD close by age _____

90% by age _____
serial echoes or catherizations
moderate size VSD (3-5mm) can be followed with _____ _____ or _____
treatment for Ventricular Septal Defect (VSD)---if HF, failure to thrive, cardiomegaly, or pulmonary hypertension
with Ventricular Septal Defect (VSD)...

-primary closure with synthetic patch needed at the time; usually 3-6 months
-may try to medically control HF first
-primary repair before 2y/o is best to prevent pulmonary hypertension
Patent Ductus Arteriosus (PDA)
-continued latency of the vessels between the pulmonary artery and the aorta
-normally closes spontaneous at 3-5 days old
-10% of all CHD
-female 2x> male
-may have associated coarctation of the aorta or VSD
the vessels between the pulmonary artery and the aorta
in Patent Ductus Arteriosus (PDA)... there is a continued potency of what?
spontaneously at 3-5 days old
Patent Ductus Arteriosus (PDA) normally closes how?
females 2x >
which sex is more likely to experience Patent Ductus Arteriosus (PDA)?
hypoplastic left heart syndrome, pulmonary atresia
important to recognize pts with PDA that have other non apparent CHD that are being kept alive by the PDA ie...?
Prostaglandin E2 (PGE2)
_____ sometimes given to maintain patency

-Patent Ductus Arteriosus (PDA)
Patent Ductus Arteriosus (PDA)
-rough "machinery" murmur max. @2nd ICS that begins in systole and persistent as a decrescendo murmur in diastole
-radiates anteriorly but NOT posteriorly
Patent Ductus Arteriosus (PDA)
-spontaneous closure may occur up to 1y/o but uncommon after a few weeks of age
-not closed by one year
-large shunt
-pulmonary hypertension
due to poorer prognosis, surgery recommended in Patent Ductus Arteriosus (PDA) if what 3 things are present?

-use of Amplazter Duct Occluder
pulmonary vascular obstruction
in Patent Ductus Arteriosus (PDA)... surgery is NOT done if _____ _____ _____
pulmonary hypertension or HF
in Patent Ductus Arteriosus (PDA)... even with surgery some develop _____ _____ or _____ in the 3rd or 4th decade of life
Atrial Septal Defect (ASD)
-a hole between atria
Osmium Secundum Variety
Sinus Venous Type
Osmium Primum Type
what are the 3 types of Atrial Septal Defect (ASD)?
Ostium Secundum Variety
type of Atrial Septal Defect (ASD)

-intermediate position in the atria (area of the foramen ovale)
-most common type
Sinus Venous Type
type of Atrial Septal Defect (ASD)

-high position on the septum
Osmium Primum Type
type of Atrial Septal Defect (ASD)

-low position on the septum
-a form of AV septal defect
Ostium Secundum Variety
what is the most common type of Atrial Septal Defect (ASD)?
Osmium Primum Type
which type of Atrial Septal Defect (ASD) is a from of AV septal defect due to its LOW position?
Atrial Septal Defect (ASD)
-10% of CHD
-females 2x> males
Atrial Septal Defect (ASD)
grade I-III systolic ejection murmur @ pulmonic area

may have early to mid systolic rumble

if a significant shunt --> diastolic flow murmur @ LLSB

S2 @ pulmonic area is widely split
Atrial Septal Defect (ASD)
-most pts are asymptomatic
-pts may present with rare cases of HF unresponsive to treatment
Atrial Septal Defect (ASD)
may present in 2nd to 3rd decade of life with
-pulmonary hypertension
-atrial arrhythmias
-easy fatiguability
-exercise intolerance
RV heave
Atrial Septal Defect (ASD)
sign: _____ _____ at mid to lower LLB
symptomatic, large defect, right heart dilation
in Atrial Septal Defect (ASD)
treatment with an Amplatzer Septal Occluder would occur with an early closure if the pt has what 3 issues?
large hemodynamically
asymptomatic children with _____ _____ significant defects usually have elective surgery at 1-3 y./o
RV dysfunction
in Atrial Septal Defect (ASD) surgery / procedure...
-when done by 3/yo, late complications of _____ _____ and significant dysrhythmias are avoided
Coarctation of the Aorta
-narrowing of the aorta usually in the thoracic* portion (proximal descending aorta near the ductus arteriosus)
Coarctation of the Aorta
-6% of CHD
-males 3x> females
-associated with Turner syndrome
Coarctation of the Aorta
pathognomonic murmur is blowing systolic murmur in left axilla and back (inter scapular area) which may spill into diastole
no symptoms
with Coarctation of the Aorta... usually _____ _____ during childhood
Coarctation of the Aorta
signs for?
-pulse weak or absent in the lower extremities after the ductus arteriosus closes
-blood pressure of 20mmHg higher in the upper extremity
-decreased lower extremity pushes
-right arm hypertension
-40% present in infancy

-60% present on routine physical exam in childhood with what 2 presentations?
Coarctation of the Aorta
treatment
-usually surgery around 4 years old
-at 2 y/o if significant elevation of BP
2 weeks old
when associated with action lesion--> HF presents by _____ and surgery needed within days of stabilization
-may utilize PGE2 infusion to reopen ductus arteriosus
Pulmonary Stenosis
-majority have 3 leaflet valves with varying degrees of leaflet fusion
-creates obstruction to flow across the pulmonary valve causing increased RV pressures
-incidence 5-10% of CHD
Pulmonary Stenosis
I-IV systolic murmur max. @ pulmonic area with systolic ejection click---often radiated to back

often right ventricular lift (heave)

may have systolic thrill in pulmonic area
Pulmonary Stenosis
-cyanosis with right sided failure in severe cases
-asymptotic in others who lead normal lives but need to be evaluated on a regular basis
percutaneous balloon valvoplasty
Treatment: Pulmonary Stenosis
-in severe --> relief of obstruction with _____ _____ _____ with stent
-surgical correction sometimes necessary
Cyanotic Heart Disease
-significant right to left shunt!
Tetralogy of Fallot
1) Pulmonary Stenosis
2) VSD
3) Dextroposed Aorta receiving blood from both ventricles (overriding aorta)
4) Hypertrophy of Right Ventricle (RVH)---secondary to pumping across a large VSD
Tetralogy of Fallot
-10% if CHD
-most common form of CYANOTIC CHD
Tetralogy of Fallot
II-IV, rough, ejection type crescendo / decrescendo systolic murmur maximum at LSB in 3rd ICS

radiated over anterior and posterior lung fields (back)

aortic closure is loud over 3rd and 4th ICS

right ventricular lift
Tetralogy of Fallot
-dyspnea on exertion
-easy fatigue
-many are cyanotic at brith; almost all by 4 months old and this is progressive
-growth and development delayed
Tetralogy of Fallot
-cyanotic spells (tet spells)---many begin at 4-6 months*
cyanotic spells / tet spells
-sudden onset of cyanosis or deepening of cyanosis
-dyspnea
-alterations in consciousness from irritability to syncope
-decreased or disappearance of systolic murmur
Tetralogy of Fallot
signs and symptoms of?
-pts will squat after learning to walk to increase systemic vascular resistance to ward of cyanotic spells
increase systemic vascular resistance
acute treatment includes oxygen and placing the pts in a knee chest position both of these will do what?
2 years old
Treatment for Tetralogy of Fallot?
-most centers attempting total repair before _____ (age) but timing depends upon severity of symptoms
-1-2% mortality with surgery
cardiac dysrhythmias
with the corrective Tetralogy of Fallot surgery... after surgery pt always at risk for sudden death form _____ _____
palliatively
Tetralogy of Fallot
-those infants too high risk for surgery are treated _____ with a small surgical procedure
-some improve by 1 y/o when complete repair can be attempted
10-15 years
in Tetralogy of Fallot... pts frequently require surgery _____-_____ years after initial repair to replace the pulmonic valve
Complete Transposition of the Great Arteries
-due to the aorta attached to the right ventricle, life is not possible without mixing of the pulmonary and systemic blood

-5% of CHD
-2nd most common cyanotic CHD
-males 3x > females
right to left shunts
-->PFO and ASD
in Complete Transposition of the Great Arteries... many associated defects actually help by allowing what to occur?
Complete Transposition of the Great Arteries
signs / symptoms
-most are severely cyanotic at birth
-HF
-growth retardation
Complete Transposition of the Great Arteries
vary depending upon the type of defects

usually loud systolic murmurs at 2nd and 3rd ICS

may have diastolic flow murmur at apex
Complete Transposition of the Great Arteries
Treatment for?
-arterial switch surgery usually done by 4-7 days old
-large VSD or ASD closed at the same time
-if large VSD, surgery may be delayed for 3-4months
Heart Failure
-clinical condition where the heart fails to meet the circulatory and metabolic needs of the body
3 cardinal signs of peds Heart Failure
1) Cardiomegaly
2) Tachypnea
3) Hepatomegaly
Heart Failure
-increased feeding difficulties and diaphoresis is common early presentation
signs and symptoms of Heart Failure
-irritability
-tachycardia
-peripheral edema
-dyspnea
-easy fatigue
-exercise intolerance
congenital and acquired causes
causes of HF in children? (2)
congenital
-VSD
-PDA
-Coarctation of the Aorta
-Septal Defects
acquired
-myocarditis
correct underlying cause
meds to increase inotropic effect
meds to decrease preload
meds to reduce after load
Treatment for HF?
inotropic
meds to increase _____ effect (Digoxin)
preload
meds to decrease _____ (diuretics --- usually furosemide)
-Thiazide or Spironolactone may be used to complement Furosemide in refractory cases
afterload
meds to reduce _____ ACE inhibitors
-improve cardiac output by decreasing systemic vascular resistance
Rheumatic Fever
-peak 5-15
-slightly higher in females and blacks
Rheumatic Fever
_____ _____ is caused by infection of the respiratory tract of a susceptible individual
Group A beta hemolytic Strep
1) sensitization of B lymphocytes by what type of bacteria?
anti streptococcal antibody
2) formation of _____ _____
cardiac antigens
3) formation of immune complexes that cross react with _____ _____
myocardial and valvular
4) _____ and _____ inflammation
Two Major

or

One Major and Two Minor

--> Modified Jones Criteria
diagnosis rules / criteria of Rheumatic Fever?
documentation of recent strep infection
-ASO titer
-positive throat culture
-scarlet fever
2-6 weeks
Rheumatic Fever presentation is usually how long after infection?
carditis
polyarthrits
erythema marginatum
Sydneham's Chorea
subcutaneous nodules
5 types of major criteria?
carditis
any new murmur of:
-mitral or aortic insufficiency
-HF
-pericarditis (friction rub or effusion)
polyarthritis
-occurs in 80% of pts
-must have 2 or more joints
-heat, redness, swelling or severe pain associated with limitation of movement
-most commonly large joints and migratory
erythema marginatum
-erythematous, macular rash with circular boarders on thorax or extremities
-usually spares the face
Sydenham's chorea
-emotional instability
-involuntary movements of muscles
-can progress to ataxia and slurred speech
-usually self limited but can lasts for 3 months
subcutaneous nodules
-over joints, spine and scalp
-non tender
-freely moveable
fever (low grade)
polyarthralgia --> just pain (2 or more joints)
previous Rheumatic Heart Disease
elevated ESR and CRO with leukocytosis
prolonged PR interval
what are the 5 minor criteria for Rheumatic Fever?
polyarthritis (inflammation of 2 or more joints) = Major

polyarthaglia (pain of 2 or more joints) = Minor
difference between Major and Minor criteria when it comes to the joints?
Benzathine Penicillin G (IM)

(can use Pen VK and Amoxicillin)
first line treatment for Rhematic Fever?
penicillin allergy
narrow spectrum cephalosporins, clindamycin, azithromycin or clarithromycin in pts who have _____ _____
ASA (Aspirin)
this treatment option can be used usually for 2-6 weeks and is good symptomatic relief for fever and arthritis
Corticosteriods
(Prednisone 2mg for 2 weeks then taper dose to 1mg for 1 week)
_____ can be used if severe carditis and HF
Benzathine Penicillin G (IM)

(Penicillin VK, Sulfadiazine, Erythromycin, Azithromycin or Clarithromycin can also be used but are less effective)
preventing Recurrence of Rheumatic Fever... what is mot effective?
after 5-10 years of therapy or at age 21
if no cardiac involvement may consider discontinuing when?
Kawasaki Disease
Mucocutenaous Lymph Node Syndrome
Kawasaki Disease
-an acute, self limited vasculitis of childhood
Kawasaki Disease
etiology
-unclear
-infectious link highly likely
-may be immunologic response
acquired
Kawasaki Disease is the leading cause of _____ heart disease in children in the US
Kawasaki Disease
-90% of pts are <5 y/o
-median age of 2 y/o
-Asian and Pacific Islander >>> than non-hispanics Blacks > Whites
-more common in winter and early spring
Kawasaki Disease
-fever > 5 days (usually high, spiking and remittent), plus 4 of the following

1) bilateral, painless, non-exudative conjunctivitis
2) lip or oral cavity changes
3) cervical lymphadenopathy
4) polymorphous exanthema

5) extremity changes
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