Pediatric Cardiology 101

Misty Carlson, M.D.

DISCLAIMER:
This lecture is based on generalizations. In reality, a congenital heart defect (CHD) can act completely different from one patient to the next (eg- classic ToF vs pink ToF). There are many more CHDs than what Ive listed and I hope you can use these principles to help you out with those.

Fetal Circulation

For the fetus the placenta is the oxygenator so the lungs do little work RV & LV contribute equally to the systemic circulation and pump against similar resistance Shunts are necessary for survival

ductus venosus (bypasses liver) foramen ovale (RL atrial level shunt)

ductus arteriosus (RL arterial level shunt)

Transitional Circulation
With first few breaths lungs expand and serve as the oxygenator (and the placenta is removed from the circuit) Foramen ovale functionally closes Ductus arteriosus usually closes within first 1-2 days

Neonatal Circulation
RV pumps to pulmonary circulation and LV pumps to systemic circulation Pulmonary resistance (PVR) is high; so initially RV pressure ~ LV pressure By 6 weeks pulmonary resistance drops and LV becomes dominant

Normal Pediatric Circulation

LV pressure is 4-5 x RV pressure (this is feasible since RV pumps against lower resistance than LV) RV is more compliant chamber than LV

100% 90/ 60 20/8 75% 100%

75%

20/ 90/

No shunts No pressure gradients Normal AV valves Normal semilunar valves If this patient was desaturated what would you think?

If you have a hole in the heart what affects shunt flow?

1. 2.

Pressure easy enough to understand Resistance impedance to blood flow

Remember, the LV has higher pressure and a higher resistive circuit relative to the RV. Now onto the nitty-gritty

Congenital Heart Disease (CHD)

Occurs in 0.5-1% of all live births Simple way to classify is:

shunts Cyanotic CHD (RL shunts) Obstructive lesions

LR

LR Shunts (Acyanotic CHD)

Defects
VSD 2. PDA 3. ASD 4. AVSD (or complete atrioventricular canal)
1.

May not be apparent in neonate due to high PVR (ie- bidirectional shunt)

LR Shunts General Points

PDA & VSD

ASD

Presents in infancy w/ heart failure, murmur, and poor growth Left heart enlargement (LHE) Transmits flow and pressure

Presents in childhood w/ murmur or exercise intolerance (AVSD or 1o ASD presents earlier) Right heart enlargement (RHE) Transmits flow only

AVSD can present as either depending on size of ASD & VSD component

Increased PBF

Left Heart Overload

Right Heart Overload

Pulm vasc markings equal in upper and lower zones

Cardiomegaly

Eisenmengers Syndrome
A long standing LR shunt will eventually cause irreversible pulmonary vascular disease This occurs sooner in unrepaired VSDs and PDAs (vs an ASD) because of the high pressure Once the PVR gets very high the shunt reverses (ie- now RL) and the patient becomes cyanotic

RL Shunts (CCHD)
Blue blood bypasses the lungs Degree of cyanosis varies Classify based on pulmonary blood flow (PBF)

PBF Truncus arteriosus Total anomalous pulm. venous return (TAPVR) Transposition of the great arteries (TGA)

PBF Tetralogy of Fallot Tricuspid atresia Ebsteins anomaly

Please note: This is a generalization. In reality most of these defects can present with low or high PBF (eg- ToF with little PS acts more like a VSD with high PBF)

RL Shunts
PBF

There is unimpeded PBF; thus, extreme pulmonary overcirculation.

Equal pressures here too

Presents more often with heart failure (except TGA) Pulmonary congestion worsens as neonatal PVR lowers Sats can be 93-94% if there is high PBF

RL Shunts

PBF Presents more often with cyanosis See oligemic lung fields Closure of PDA may worsen cyanosis

Dynamic subvalvular obstruction here causes Tet spells

Why are pressures equal?

90%

Pulmonary overcirculation 70%

Too little PBF

70%

99%

60%

99%

99% 99%

70%

60%

Different amounts of PBF

(Truncus vs ToF)

Obstructive Lesions
1. 2. 3.

Ductal Dependent Critical PS/AS Critical CoA/IAA HLHS Presents in CV shock at 2-3 days of age when PDA closes +/- cyanosis Needs PGE1

Non-Ductal Dependent 1. Mild-moderate AS 2. Mild-moderate CoA 3. Mild-moderate PS Presents in older child w/ murmur, exercise intolerance, or HTN (in CoA) Not cyanotic

Ductal-Dependent Lesion
Without a PDA there is no blood flow to the abdomen and lower extremities.
(Blue blood is better than no blood.)

Physical Exam

Inspection and palpation

Cardiac

cyanosis must be central Differential cyanosis = RL PDA shunt Differential edema/congestion implies obstruction of SVC or IVC Increased precordial activity Displaced PMI RV heave = RV hypertension

Physical exam

Lungs
Respiratory

rate and work of breathing Oxygen saturations

Abdominal exam
Liver

size

Extremities
Perfusion

Edema
Clubbing

Physical Exam

Pulses (very important)

Differential

pulses (weak LE) = CoA Bounding pulse = run-off lesions (LR PDA shunt, AI, BT shunt) Weak pulse = cardiogenic shock or CoA Pulsus paradoxus is an exaggerated SBP drop with inspiration tamponade or bad asthma Pulsus alternans altering pulse strength LV mechanical dysfunction

Physical Exam

Heart sounds
Ejection

click = AS or PS Mid-systolic click = MVP Loud S2 = Pulmonary HTN Single S2 = one semilunar valve (truncus), anterior aorta (TGA), pulmonary HTN Fixed, split S2 = ASD, PS Gallop (S3) may be due to cardiac dysfunction/ volume overload Muffled heart sounds and/or a rub = pericardial effusion tamponade

Physical Exam

Types of Murmurs
Systolic

Ejection Murmur (SEM) = turbulence across a semilunar valve Holosystolic murmur = turbulence begins with systole (VSD, MR) Continuous murmur = pressure difference in systole and diastole (PDA, BT shunt)

Innocent murmurs

Peripheral pulmonic stenosis (PPS)

in newborns disappears by one year of age (often earlier) Soft SEM at ULSB w/ radiation to axilla and back (often heard best in axilla/back) Need to differentiate b/w PPS and actual pulmonic stenosis. PS often associated with a valvular click and heard best over precordium
Heard

Innocent murmurs

Stills murmur
Classic innocent murmur Heard most commonly in

young children (3-5 yrs of age) but can be heard in all ages Vibratory low-frequency murmur often heard along LSB and apex Positional increases in intensity when pt is in supine position Also louder in high output states (i.e. dehydration, fever) Need to differentate from VSD

Innocent murmurs

Pulmonary flow murmur

Often

heard in older children and adolscents Soft SEM at ULSB, little radiation; normal second heart sound Not positional Need to differentiate b/w mild PS and especially an ASD

Hint: ASD would have a fixed split second heart sound

Innocent murmurs

Venous hum
Often

heard in toddlers, young children Low pitched continuous murmur often heard best in infraclavicular area, normal heart sounds Positional diminishes or goes completely away when pt in supine position or with compression of jugular vein Need to differentiate between a PDA

Syndrome Associations
Down AV canal and VSD Turner CoA, AS Trisomies 13 and 18 VSD, PDA Fetal alcohol LR shunts, ToF CHARGE conotruncal (ToF, truncus)

Hereditary Diseases

Marfan (AD) aortic root aneurysm dissection, MVP, MR, AI HCM (AD) outflow tract obstruction, arrhythmias Noonan (AD) HCM, PS DMD/BMD (X-link) DCM (>12 y.o.) Williams (AD) supravalvar AS Tuberous sclerosis rhabdomyoma Romano-Ward AD LQTS Jervell & Lange-Nielsen AR LQTS & deafness

Kawasaki Disease (KD)

Now the #1 cause of acquired heart disease A systemic vasculitis (etiology-unknown) Tests CBC, CMP, CRP, ESR, EKG, ECHO Rx IVIG at 2g/kg and high-dose ASA Prognosis Coronary artery dilatation in 15-25% w/o IVIG and 4% w/ IVIG (if given within 10 days of fever onset). Risk of coronary thrombosis.

Kawasaki Clinical criteria

Fever for at least 5 days AND 4 of the following 5 criteria:

Eyes - conjunctival injection (ie- no exudate) Lips & mouth - erythema, cracked lips, strawberry tongue Hands & feet - edema and/or erythema Skin - polymorphous exanthem (ie- any rash) Unilateral, cervical lymphadenopathy

Rheumatic Fever

A post-infectious connective tissue disease Follows GAS pharyngitis by 3 weeks (vs. nephritogenic strains of GAS) Injury by GAS antibodies cross-reacting with tissue Dx JONES criteria (major and minor) Tests Throat Cx, ASO titer, CRP, ESR, EKG, +/ECHO Rx PCN x10 days and high-dose ASA or steroids 2o Prophylaxis daily po PCN or monthly IM PCN

Rheumatic Fever organs affected

1.
2. 3.

4.

5.

Heart muscle & valves myocarditis & endocarditis (pericarditis rare w/o the others) Joints polyarthritis Brain Sydenhams Chorea (milkmaids grip or better yet, motor impersistance) Skin erythema marginatum (serpiginous border) due to vasculitis Subcutaneous nodules non-tender, mobile and on extensor surfaces

In case you havent had enough.

A ductal-dependent lesion One ventricle pumps both PBF & SBF Difficult to balance PBF & SBF

Norwood Procedure

What is the purpose of the BT shunt? Is there a murmur? What is your guess for the arterial saturation?

Bidirectional Glenn

What is the purpose of the Glenn? Is there a murmur? What is your guess for the arterial saturation?

Fontan circuit

What is the path of blood? Is there a murmur? What is your guess for the arterial saturation?