Studied by 9 people
What can premature closure of the foramen ovale lead to?
hypoplastic left heart syndrome (HLHS)
What is dextrocardia?
cardiac apex is on the right side of the chest
What is situs ambigus?
identification of the 2 atria is impossible
What is situs inversus?
anatomic right atrium to the left of the anatomic left atrium
What is the most common congenital disorder in newborns?
CHD
What is the MC overall CHD lesion?
VSD
What is the MC valvular CHD lesion?
bicuspid aortic valve
What RF inc the odds of CHD?
Fhx, premature birth, genetic syndromes, in utero infxn, ART, maternal factors (DM, HTN, obesity, thyroid, epilepsy)
What infxns inc risk of CHD?
TORCHES CLAP
toxoplasmosis, other (zika), rubella, CMV, Herpes & Hepatitis, Entero virus, Syphilis, Chicken pox, Lyme disease, AIDs, Parvovirus B19
What is ductal-dependent CHD?
life-dependent on maintaining PDA
In critical right heart obstructive lesions, what does the PDA supply?
pulmonary circulation
In critical left heart obstructive lesions, what does the PDA supply?
systemic circulation
What is critical CHD?
requires surgery or intervention in the first year of life
What CHDs are acyanotic?
CoA, AS, PDA, VSD, ASD, A-V canal defect, PS
What is the effect of the L→R shunts seen in acyanotic heart defects?
inc pulm pressure → inc pulm blood flow
What does VSD do to the hearts structure?
enlarges the PA, LA, LV due to the double blood flow
What are signs of prolonged untreated VSD?
RV hypertrophy ( d/t dec pulm resistance) & Eisenmenger Syndrome
What murmur is heard w/ VSD?
loud, harsh, pansystolic @ LLSB
*large = no murmur, but S2 will be loud or have no split
What does Lithium use during pregnancy cause?
Epstein anomaly
What heart defect does Fetal Alcohol Syndrome cause?
ASD or VSD
What would a CXR show in pts w/ a VSD?
cardiomegaly, LAE, inc pulm vasculature
What is the tx for VSD?
usually closes w/ time; severe = surgery
What is the MC type of ASD?
ostium secundum
How does ASD affect the hearts structure?
RA & RV enlargement due to the double blood flow
What CHD is common in Down’s syndrome pts?
ASD
What would you see on a EKG of ASD?
RVH, RAE, RAD, ± RBBB
What would you see on a CXR of ASD?
cardiomegaly, inc pulm vasculature
What murmur is present w/ ASD?
soft systolic ejection @ ULSB; wide split S2
What is an AV Canal Defect characterized by?
primum ASD + AV valve defect + inlet type of VSD
What are most of the hemodynamic problems associated with AV Canal Defects caused by?
VSD
What is the tx for an AV canal defect?
surgery: perform before the onset of pulm vascular occlusive disease
*PAB if infant is too small for definitive repair
What is the MC type of PS?
valvular
What is Valvular PS?
PV is dome-shaped w/ a narrow opening
*usually d/t leaflet fusion
What is subvalar PS?
muscle under the PV is thickend
What is supravalvar PS?
narrowing in the PA above the PV
What murmur is heard w/ PS?
loud w/ expiration @ ULSB, ejection systolic click; S2 split
What is the MC R-sided obstructive lesion?
valvular PS
What CHD is common in Noonan syndrome?
PS
What is the tx for PS?
mild = no tx; mod/severe = balloon dilation via cath
Why is a PDA not a problem at birth?
PVR = SVR → minimal shunting; as PVR falls shunting occurs
PDA are normal and usually close on their own. What inc the likelihood that the ductus arteriosus doesn’t close spontaneously?
premature birth
If left untreated, what are the potential complications of a PDA?
pulm HTN & heart failure
What effects the severity of the sx of a PDA?
defect size & gestational age at birth
What are sx of a large PDA?
poor eating/growth, sweating w/ crying or play, persistent tachypnea or breathlessness, easy tiring, bluish/dusky skin tone
What murmur is associated with a PDA?
pansystolic, machinery-like w/ bounding peripheral pulses & wide PP
What is the tx for a PDA?
Indomethacin or Ibuprofen OR surgery
How do NSAIDs help close PDAs?
block the prostaglandins that keep the hole open
How does HLHS affect the hearts structure?
small LV & large RV due to reverse blood flow
How does AS cause pulm HTN?
narrow AV → LV failure → backup of blood in LA → inc pressure
What is the triad of sx associated w/ AS?
SAD: syncope, angina, dyspnea
What murmur is associated with AS?
systolic thrill @ RUSB; suprasternal notch, narrow PP
What is the MCC of AS?
rheumatic fever
*in infants = bicuspid valve
What is the tx for AS in children?
ballon valvuoplasty
How does CoA present in infants?
CHF or shock
How does CoA present in older children?
HTN
What is the typical location of a CoA?
thoracic aorta distal to the origin of the L subclavian artery at the level of the ductal structure; dilation following immediately
What are the MC clinically significant defects associated w/ CoA?
PDA, VSD, AS
What PE findings are associated with CoA?
SEM @ ULSB; bounding radial pulses, diminished femoral pulses, BP elevated in UE & lower in LE, cardiogenic shock
What is the tx for CoA?
surgery/cath = definitive
severe: prostaglandins to temp. reverse PDA closure
What would you see on a CXR of CoA?
figure 3
What are cyanotic CHD?
ToF, TGA, HLH, tricuspid atresia, truncus arteriosus, total anomalous pulm venous connection
What are the 4 components of ToF?
PROV: PS, RVH, Overriding aorta, VSD
What extra defect can less the effects of ToF?
PDA, allows adequate blood flow to pulm
What are sx of ToF?
“tet spells” in infants (hypoxic episodes, thromboembolism, squatting, polycythemia
What would you see on a CXR of ToF?
normal size heart; boot-shaped heart; dec pulm vasculature
What is the tx for ToF?
Blalock-Taussig procedure
What cause the “boot-shape” of the heart in ToF?
RVH & upward tilt of the apex
What is the MC CHD presenting in cyanosis w/in the first week of life?
TGA
How is TGA ductal dependent?
only access route to the lunge is via reverse BF through a PDA
What do see on a CXR of TGA?
cardiomegaly “egg-on-string”; inc pulm vasculature; narrow mediastinum
What is the tx for TGA?
ductal dependent = IV prostaglandin E1 to maintain PDA
surgery -balloon atrial septostomy (emergency)
What is HLH caused by?
underdeveloped LV; hypoplastic MV & AV; aorta & coronary arteries are small
What are signs of HLH?
weak/absent peripheral pulses; enlarged heat, inc PVM/PV congestion
What is the tx for HLH?
Ductal dependent: Prostaglandin (PDA dilate), diuretics
Definitive = surgery or transplant
What is Total Anomalous Pulm Venous Return?
pulm veins drain into other vessels or directly into the RA instead of the LA
How does Total Anomalous Pulm Venous Return present?
full-term baby w/ inc RV activity; cyanosis, pulm edema, single S2, short systolic murmur, pulm congestion on CXR
Why does TAPVC result in critical failure?
no way for oxygenated blood to return to the rest of the body
What would you see on a CXR of TAPVC?
cardiomegaly, “snowman” appearance, inc pulm vasculature
What is Tricuspid Atresia?
blood from RA → LA via ASD → oxy/deoxy blood mix → LV → majority to aorta some to RV via VSD → lungs
What are sx of Tricuspid Atresia?
cyanosis, absent tricuspid valve, hypoplastic RV & PA, MUST have ASD, PDA, or VSD
What is the tx for Tricuspid Atresia?
temp: prostaglandins if needed
Definitive: multiple surgeries, possible Blalock-Taussig shunt (artificial PDA)
What is Truncus Arteriosus?
pulm artery originate from a common truncus (causes blood to mix)
*always accompanied by VSD
What sx are associated with Truncus Arteriosus?
cyanosis, pansystolic murmur 4/6 + diastolic murmur; ejection click, single S2
What would an EKG show if a pt has Truncus Arteriosus?
LVH ± RVH
What is the tx for Truncus Arteriosus?
remove PA from TA; close VSD, replace truncal valve
What is the MC cardiomyopathy?
congestive/dilated CM
What causes congestive CM?
myocardial damage (myocarditis, ETOH, immunologic)
What is the tx for congestive CM?
CHF management, diuretics, digoxin, rest
In hypertrophic CM, what is impaired?
filling
What causes most cases of hypertrophic CM?
genetics (autosomal dominant)
What are sx of hypertrophic CM?
easy fatigue, anginal pain w/ exercise, SOB
Which form of CM is very rare in children?
restrictive
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