Cyanosis

1. Blue mucous membranes and tongue indicate ____
2. __ gms/dL of unoxygenated hgb to appear cyanotic
3. ____ is very common as infant transistions in the first couple hours

1. central cyanosis (does not include lips)
2. 5 gms/dL
3. acrocyanosis

Left to Right shunts

• systemic blood circulated to pulmonary circuit
• as pulmonary vascular resistance drops, blood shunted from high pressure L to low pressure R

Give 3 examples

1. PDA
2. VSD
3. ASD

Patent Ductus Arteriosus (PINK)

1. functionally closed within __hrs, completely in up to __wks
2. large PDA can cause ____ d/t pulm and L heart volume overload
3. ____ babies at greatest risk
4. associated with a ____ murmur heard best ____
5. can be closed medically with ____

1. 15-48 hrs; 3 wks
2. heart failure
3. premature
4. continuous, "machine-like"; below L clavicle
5. indomethacin

ventricular septl defect (VSD)

VSD (PINK)

1. associated with a ____ murmur
2. get ____ with age
3. __ spontaneously close by 1 year, __ by 5 years
4. ____ VSDs more likely to close than ____
5. small VSDs may lead to ____
6. large VSDs may lead to ____

1. harsh, holosystolic
2. smaller
3. 1/3; 2/3
4. muscular; membranous
5. endocarditis if jet against aortic valve disrupts endothelial membrane
6. pulmonary and L heart volume overload

PA CXR shows cardiomegaly

pulmonary arterial markings increased

start to become symptomatic as PVR drops

poor weight gain, tachycardia, tachypnea

• initially medical managed - diuretics, increase calories
• closure - surgical, device closure in cath lab

increased flow to lungs has been reversed by increased resistance in the pulmonary bed

• no murmur!
• EKG: R ventricular hypertrophy
• once flow is reversed, pt becomes cyanotic
• requires heart transplant

Atrial Septal defect

do not usually cause symptoms in infancy/childhood

1. associated with a ____
2. large defects lead to ____

1. fixed split S2 (may dev. midsystolic pulm/systolic ejection murmur 2ndary to increased blood flow across pulm valve)
2. pulmonary and Right heart volume overload

87% spontaneous closure

surgical patch or occlusion: Amplatzer device at age 2-4

Name the defect

• involves malformation of tricuspid and mitral valves
• assoc w/ Down's syndrome
• often present with heart failure by 2 mos

AV Canal

(atrio-ventricular defect)

Name the defect

• Right outflow tract obstruction (pulm stenosis)
• Right ventricular hypertrophy
• large VSD
• over-riding aorta
• BLUE (R-L shunt)

Tetralogy of Fallot

• child suddenly becomes cyanotic, hyperpnic, altered consciousness and squats on the floor

peripheral dilation of blood vessels - systemic vascular resistance drops - less blood flow to pulm bed - blood chooses path of least resistance, across VSD

squatting augments venous return - increasing intravascular volume and systemic resistance

• calm infant, sedate if necessary
• give morphine (helps spasm around pulm valve)
• give oxygen
• knee chest position
• phenylephrine to increase afterload
• beta-blocker to decrease infundibulum stenosis
• operate

1. Classic CXR of older tets shows ____
2. Classic EKG of older tets shows ____

Hypertrophic RV develops ectopic foci, runs of Vtach triggered by these can lead to sudden death

1. boot shaped heart
2. RVH

systolic ejection murmur d/t pulm stenosis

S2 single - pulm component barely audible

Transposition of great arteries (TGA)

open ductus arteriosus

Management issues: keep ductus open, establish mixing lesion more stable than ductus

Hyperoxia test:

check arterial PaO2, give 100% O2 for 10 min, recheck

if PaO2 >150, TGA less likely

What do you do if pt fails hyperoxia test?

Life saving measure?

start prostaglandin (Alprostadil)

*PGE 1 opens PDA but puts pt at risk for apnea*

Balloon atrial septostomy life saving

appearance of heart with TGA on CXR

Name the defect

• one great vessel that branches to bring blood to body and lungs
• always assoc w/VSD
• dyspnea and heart failure

Truncus arteriosus (BLUE)

surgically

• pt placed on bypass
• single trunk fashioned into aorta, pulm vessels removed
• VSD patched
• conduit created to take blood from RV to pulm arteries

Name the defect

• valve b/t R atrium and R vent doesn't develop
• blood from RA must pass through ASD to LA
• needs to be repaired to single ventricle
• BLUE

tricuspid atresia

Name the defect

• cyanotic, respiratory distress, grunting, labored breathing
• requires surgical repair, ECMO for stabilization if can't be fixed immediately
• all have ASD also

TAPVR

all pulm veins return to R atrium instead of L

1. truncus
2. transposition
3. tricuspid atresia
4. tetrology of fallot
5. TAPVR

• feeding difficulties - failure to thrive
• easy fatigability
• sweating while feeding (esp forehead)
• tachypnea

echocardiogram

Coarctation of the Aorta

1. occurs most commonly ____
2. classically presents at __ wks
3. may develop ____
4. chest x-ray shows ____
5. repair with ____

1. next to the ductus
2. 2 wks when ductus closes
3. LVH, heart failure
4. rib notching
5. balloon angioplasty (not for infants <6mos)

What is the most common, symptomatic arrhythmia in children?

How is it characterized?

What are the BPM parameters?

SVT

by abrupt onset and termination

>220 bpm in infants

>180 bpm in older children

IV adenosine

if that fails, synchronized cardioversion

Name the defect

• autosomal-dominant
• assoc w/syncope and sudden cardiac death
• EKG diagnostic
• B-blockers, implantable defibrillators for treatment

Long QT syndrome

Name the defect

• familial disorder
• pain, lightheadedness, syncope
• at risk for arrhythmias - sudden death
• +/- harsh SEM at LLSB, louder as pt stands from sitting/squatting position
• ECHO diagnostic

hypertrophic obstructive cardiomyopathy

murmur

1. Systolic ejection murmur louder at ____
2. Regurgitant/holosystolic murmur louder in ____

1. base of heart
2. lower chest

1. soft, barely audible
2. easily audible
3. loud
4. loud with a thrill, can be heard w/steth partially off chest
5. loud, heard w/steth off chest
6. loud, heart w/o steth

Name the murmur

• common in newborns
• disappears by 3-6 mos
• 1-2/6 SEM heard best at ULSB
• radiates to axilla and back

Peripheral pulmonic stenosis murmur

Name the murmur

• common in kids b/t 3-6 yrs
• heard only when child is upright, disappears when lying down
• heard in R and L infraclavicular areas

venous hum

Name the murmur

• uncommon before age 2
• mid L sternal border
• mid-systolic
• musical, vibratory quality

Still's murmur

Name the murmur

• early systolic ejection murmur
• best heard in supraclavicular fossa or over carotid arteries
• found in children of any age

carotid bruit

• diastolic
• continuous (except venous hum)
• holosystolic/pansystolic/regurgitant
• late systolic
• palpable thrill
• other abnml CV exam finding

Name the defect

• M/C cause of death from CHD during first month of life
• symptoms develop as PDA closes
• inadequate systemic circulation with pulmonary venous HTN
• cyanosis, dyspnea, hepatomegaly

Hypoplastic Left heart syndrome

Norwood procedure