Common Cardiac Problems in Pediatric Practice

Dr. Pravin Asir Abraham.

DISCLAIMER:

This lecture is based on generalizations and common cases presented at the pediatrics dept, AMH. There are many more CHDs than what Ive listed and I hope you can use these principles to help you out with those.

Cardiac cases at AMH

Newborn referralsCardiac murmurs. Cyanosis. Respiratory symptoms. Pediatric referrals-Cardiac murmurs. Chest pain evaluation. Palpitation evaluation. Syncope evaluation. Evaluation of genetic syndromes. Pre-operative evaluation. Evaluating cardiac involvement in systemic diseases.

NEWBORN REFERRALS

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
Pulmonary resistance (PVR) is high; so initially RV pressure ~ LV pressure RV pumps to pulmonary circulation and LV pumps to systemic circulation 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

Normal cardiac findings in newbornsHR range 100-180. Varying degrees of acrocyanosis. Maximal cardiac impulse at LLSBRV hyperactive. S2 may be single for first few days of life. An ejection click representing pulmonary hypertension heard in first hours of life.

Normal cardiac findings in newborns- contd.

Pulmonary flow murmur-grade 12/6 systolic ejection murmur(innocent murmur)characteristically radiates to sides and back of the chest. This prevalence and loudness increased in preterms. Peripheral pulses are bounding due to lack of subcutaneous tissue.

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(5-10/15-20)

ASD(5-10)

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 pulmvenous 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(8-12/3-6) Critical CoA/IAA(8-10/1) 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

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

COMMON

PEDIATRIC REFERRALS

Innocent murmurs

Stills murmur
Classic innocent murmur Heard most commonly in

young children (35 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

CHEST PAIN IN CHILDREN

Idiopathic(12-45)-precordial catch,mastalgia Costochondritis(9-22) Musculoskeletal trauma(21) Lung(15-21) Psychogenic(5-9) GI(4-7) Sickle cell crisis(2) Cardiac(0-4)

Cardiac pains
Severe AS. Severe PS. Mitral Valve prolapse. HOCM. CAD(ALCAPA or kawasaki disease sequelae) Cocaine abuse. Pericarditis-pericardiotomy syndrome)/Myocarditis. Arrhythmias(WPW syndrome,LQT)

Palpitations in children

Syncope in children
Vasovagal syncope- with prodromedizziness, pallor, palpitation, nausea, diaphoresis, hyperventilation followed by LOC. Orthostatic hypotension- No prodrome-light headedness. CVOD. Cardiac causes- AS, PS, HOCM, Myocardial dysfunction, arrhythmias(WPW, LQTS,RV dysplasia,SVT,VT, CHB,SSS)

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

Pre-operative evaluation

Indications for prophylaxis - High risk- prosthetic cardiac valve, previous bacterial endocarditis, CCHD, Surgically constructed systemic to pulmonary artery conduits. - Moderate risk- Other CHD, Acquired Valvular disease, HCM, MVP with MR or thickened leaflets.

IE prophylaxis for DORE procedures

Oral- Amoxicillin 50mg/kg 1 hr before Sx. Inj- Ampicillin 50mg/kg IM or IV within 30 minutes before Sx. Allergic to PCN- Cephalexin or Cefadroxil 50mg/kg/ Azithromycin 15mg/kg 1 hr before Sx. Allergic to PCN and unable orallyClindamycin 20mg/kg or Cefazolin 25mg/kg within 30mts.

IE prophylaxis for GI and GU

High risk-Ampi 50mg/kg IM or IV(2g)+genta 1.5mg/kg within 30mts, Ampi 25mg/kg IM or IV 6hr(OR)Vanco 20mg/kg IV over 1-2 hr+genta 1mg/kg IM or IV. Moderate risk-Amox 50mg/kg orally 1hr or ampi 50mg/kg IM or IV within 30mts(OR)Vanco 20mg/kg IV over 1-2hr.

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

HEART-FELT THANKS