Peds E1 -Cardio pt 1

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