Paediatric Cardiology:

Congenital Heart Disease and Clinical

ProblemsDr. Suzie Lee

Pediatric CardiologistAssistant Professor, University of Ottawa

Objectives

To provide an outline of congenital heart disease

List criteria for Kawasaki syndrome Describe the common innocent murmurs

of childhood

An Outline of Congenital Heart Disease

Pink (Acyanotic)

Blue (Cyanotic)

Critical outflow tract

obstruction

Acyanotic Congenital Heart Disease

Normal Pulmonary Blood Flow↑ Pulmonary Blood Flow

Acyanotic Congenital Heart Disease

Normal Pulmonary Blood Flow Valve Lesions

•Not fundamentally different from adults

Acyanotic Congenital Heart Disease

↑ Pulmonary Blood Flow

Shunt Lesions

Atrial Level Shunt

ASD

Physiology Left to Right shunt because of greater

compliance of right ventricle Loads right ventricle and right atrium Increased pulmonary blood flow at normal

pressure

ASD

History Usually asymptomatic in childhood

Occasionally frequent respiratory tract infections

Presentation with murmur in childhood

ASD

Physical Examination Right ventricular “lift”

Atrial level shunts result in right-sided volume overload

Wide fixed S2 Blowing SEM in pulmonic area

Murmur due to increased flow across the pulmonary

ASD

ASD

ASD

Natural History

Generally do well through childhood Major complication atrial fibrillation Can develop pulmonary hypertension / RV

failure but not before third or fourth decade of life

ASD

Management Device closure around three years of age

or when found Surgery for very large defects or outside

fossa ovalis (eg. sinus venosus defect)

ASD

Shunt Lesions

Ventricular Level Shunt

VSD

Physiology Left to Right shunt from high pressure left

ventricle to low pressure right ventricle Loads left atrium and left ventricle (right

ventricle may see pressure load)

VSD

History Small defects

Presentation with murmur in newborn period Large defects

Failure to thrive (6 wks to 3 months)• Tachypnea, poor feeding, diaphoresis

VSD

Physical Examination Active left ventricle Small defect

Pansystolic murmur, normal split S2 Large defect

SEM, narrow split S2, diastolic murmur at apex from high flow across mitral valve

VSD

VSD

VSD

Natural History Small defect

Often close No real significance beyond endocarditis risk

Large defect Failure to thrive Progression to pulmonary hypertension as

early as 1 year

VSD

Management Small defect

Conservative management Large defect

Semi-elective closure if growth failure or evidence of increased pulmonary hypertension

Occasionally elective closure if persistent cardiomegaly beyond 3 years of age

Shunt Lesions

Great Artery Level Shunt

PDA

Physiology Left to Right shunt from high pressure

aorta to low pressure pulmonary artery Loads left atrium and left ventricle (right

ventricle may see pressure load)

PDA

History Premature duct

Failure to wean from ventilator +/- murmur

Older infant Usually murmur from early infancy Occasionally signs of heart failure

PDA

Physical Examination Active left ventricle Hyperdynamic pulses Premature duct

SEM with diastolic spill Older infant

Continuous murmur

PDA

Management Premature Duct

Trial of indomethacin Surgical ligation

Older infant Leave till 1 year of age unless symptomatic Coil / device closure Rarely surgical ligation

CoarctationObstruction of the

aortic archClassically juxtaductal,

although may occur anywhere along the aorta

May develop over time Femoral pulses should

be checked routinely throughout childhood

Coarctation of the Aorta

History Presentation varies with severity

Severe coarct• Failure (shock) in early infancy

Mild coarct• Murmur (in back)• Hypertension

Coarctation

Physical Examination Absent femoral pulses Arm leg gradient +/- hypertension Left ventricular “tap” Bruit over back

Coarctation

Management Newborn with CHF

Emergency surgical repair Infant

Semi-elective repair in uncontrolled hypertension Older child

Balloon arterioplasty +/- stenting Surgery on occasion

Failure to repair prior to adolescence recipe for life long hypertension

Cyanotic Congenital Heart Disease

“Blue” blood (deoxygenated hemoglobin) enters the arterial circulation

Systemic oxygen saturation is reduced Cyanosis may or may not be clinically

evident• 5g% deoxygenated HgB

Causes of Cyanosis

RespiratoryCardiac Hematologic

Polycythemia Hemoglobins with decreased affinity

Neurologic Decreased Respiratory drive

Cyanosis

RespiratoryCardiac

Hyperoxic test – response to 100% O2• Lung disease should respond to 02• PO2 should rise to greater than 150 mmHg

Cyanotic Congenital Heart Disease

Increased pulmonary blood flow Truncus arteriosus Transposition of the great arteries Total anomolous pulmonary venous return

Decreased pulmonary blood flow Tetralogy of Fallot/pulmonary atresia Tricuspid atresia Critical pulmonary stenosis

Cyanotic Congenital Heart Disease

↑Pulmonary Blood Flow

d-Transposition

Normal Heart

Body RA RV PA

LALVAO Lungs

Circulation is in “series”

d-Transposition Circulation is in “parallel”

Body RA RV Ao

Lungs LA LV PA

d-Transposition

Circulation is in “parallel”

Need for mixing

TGAMust bring oygenated blood into the systemic

circulation Great artery level shunt - PDA Atrial level shunt – PFO

Prostaglandin E1 (PGE) Re-opens and maintains patency of the ductus

arteriosusBalloon atrial septostomy (BAS)

Increase intracardiac shunting across the atrial septum

d-Transposition

Body RA RV Ao

PFO BAS PDA PGE

Lungs LA LV PA

Transposition

History Presentation

Profound cyanosis shortly after birth (as duct closes)

Minimal or no murmur

TGA

Physical Examination Profound cyanosis Right ventricular “tap” Loud single S2 Little or no murmur

TGA

Management Prostaglandins to maintain mixing Balloon atrial septostomy Arterial switch repair in first week

Balloon Atrial Septostomy

Total Anomalous Pulmonary Venous Return

Pulmonary veins fail to connect to left atrium

Pulmonary veins communicate with systemic vein

Total Anomalous Pulmonary Venous Return - Supracardiac

Pulmonary veins fail to connect to left atrium

Pulmonary veins communicate with systemic vein

Total Anomalous Pulmonary Venous Return - Infracardiac

Pulmonary veins fail to connect to left atrium

Pulmonary veins communicate with systemic vein

TAPVD

History Presentation depends on presence or

absence of obstruction to venous return Infradiaphragmatic

• Almost always obstructed• Cyanosis and respiratory distress shortly after

birth Cardiac or supracardiac

• Rarely obstructed• Can present like big ASD with cyanosis

Not a PGE dependent lesion

TAPVD

Physical Examination Variable cyanosis (again depends on

obstruction) Right ventricular “tap” Wide split S2 Blowing systolic ejection murmur

TAPVD

TAPVD

Management If severe cyanosis in newborn

Emergency surgical repair Unobstructed

Semi-elective surgical repair when discovered

Truncus arteriosus 1. common, single outflow

tract with pulmonary arteries originating from the ascending aorta

2. abnormal truncal valve 3. large VSD

4. not a PGE dependent lesion

Cyanotic Congenital Heart Disease

Decreased Pulmonary Blood Flow

Cyanotic Congenital Heart Disease - ↓ Pulmonary Flow

= RVOT Obstruction + Shunt

Cyanotic Congenital Heart Disease

Tetralogy of Fallot 1. Pulmonary stenosis

2. Overriding aorta

3. RVH

4. VSD

Generally not a PGE dependent lesion

Tetralogy of Fallot

History Presentation depends on severity of PS

Severe stenosis• Cyanosis shortly after birth (as duct closes)

Mild stenosis• May present as heart murmur (from shortly

after birth)

Tetralogy of Fallot

Physical Examination Variable cyanosis (remember the 50g/l

rule) Right ventricular “tap” Decreased P2 +/- ejection click “Tearing”/harsh SEM

Tetralogy of Fallot

Management Outside the newborn period, surgical

repair if symptomatic Elective repair at 6 months Role for beta blockers to palliate

hypercyanotic spells

Tetralogy of Fallot

Hypercyanotic Spells (“Tet” Spells) Episodes of profound cyanosis Most frequently after waking up or

exercise

Tetralogy of Fallot

Hypercyanotic Spells (“Tet” Spells)

Stress leading to fall in P02

Tachycardia and Hyperventilation

Increased Return of deeply desaturated

venous blood

Increased R to L shunt

Tetralogy of Fallot

Hypercyanotic Spells (“Tet” Spells Treatment

Tuck knees to chest • Reduces venous return by compressing

femoral veins• Increases systemic vascular resistance

In hospital• O2• Phenylephrine• Morphine • IV beta blocker

Tetralogy of Fallot

Tetralogy of Fallot

Decreased Pulmonary Blood Flow

Pulmonary atresia/VSDTetralogy of Fallot

with atretic pulmonary valve

Variable pulmonary artery anatomy

Generally a PGE dependent lesion

Critical pulmonary stenosis

Severe pulmonary stenosis with inadequate pulmonary flow Pulmonary atresia/intact

ventricular septum

PGE dependent lesion

Tricuspid atresia 1. tricuspid atresia 2. severely hypoplastic RV 3. VSD 4. ASD – large 5. pulmonary stenosis

Variable

Generally a PGE dependent lesion

Cyanotic Heart DiseaseDecreased blood flow due to RVOT

obstruction may require augmentation of pulmonary blood flow via creation of a surgical systemic to pulmonary shunt

Blalock-Taussig Shunt (BTS)

Case 1 (continued)BTS

Duct Dependent Congenital Heart Disease

Which of the following are examples of duct dependent CHD?

1. Pulmonary atresia2. Patent ductus arteriosus3. Transposition of the great arteries

Critical Left-Sided Obstruction

Neonatal presentation

Coarctation

Critical aortic stenosis

Hypoplastic left heart syndrome

Cardiogenic shock

PGE dependent lesion

Left-sided Obstruction

Coarctation of the aorta Critical narrowing

of the “juxtaductal” aorta

Blood cannot get past the obstruction

SHOCK

CoarctationCharacterized by weak or absent pulses

particularly in the lower limbsInitiation of PGE lifesaving

‘splitting’ of saturations seen in critical narrowings with patency of ductus arteriosus ie: normal saturation in right arm and lower saturation in the lower limbs due to right to left shunting across the PDA

Coarctation - treatmentSurgical correction following initiation of

PGE and stabilization

Left-Sided Obstruction Critical Aortic

Stenosis CRITICAL

Inadequate forward

flow to maintain

cardiac output

SHOCK

Critical Aortic Stenosis

Management Prostaglandins to provide source of systemic

blood flow Balloon valvuloplasty Rarely surgery

Left Ventricular Outflow Tract Obstruction

Hypoplastic Left Heart Syndrome (HLHS)

1. Mitral atresia

2. Aortic atresia

3. Hypoplastic left ventricle

4. Hypoplastic ascending aorta

PDA is the only source of systemic blood flow

PGE dependent lesion

HLHSInitially cyanoticWith closure of the PDA SHOCK

Tachycardia, tachypnea, low blood pressure, weak pulses, poor perfusion, cyanotic/grey colour

PGE

Hypoplastic left heart Management Prostaglandins Norwood procedure Heart Transplant

Kawasaki Syndrome

Small artery arteritis Coronary arteries most seriously effected Dilatation/aneurysms progressing to (normal)

stenosis

Kawasaki Syndrome

5 days of fever plus 4 of Rash Cervical lymphadenopathy (at least 1.5

cm in diameter) Bilateral conjuctival injection Oral mucosal changes Peripheral extremity changes

Swelling Peeling (often late)

Kawasaki Syndrome

Associated Findings Sterile pyuria Hydrops of the gallbladder Irritability

Kawasaki Syndrome

Epidemiology Generally children < 5 years Male > Female Asian > Black > White

Kawasaki Syndrome

Management Gamma globulin 2g/kg 80 mg/kg ASA until afebrile then 5 mg/kg for 6

weeks

Innocent Murmurs

Characteristics Always Grade III or less Always systolic (occasionally continuous) Blowing or musical quality Not best heard in back

Innocent Murmurs

Types Still’s

• Vibratory SEM best heard mid-left sternal border Pulmonary Flow murmur

• Blowing SEM best heard in PA Venous Hum

• Continuous murmur best heard in R infraclavicular

• Decreases lying flat or occlusion of neck veins Physiologic peripheral pulmonary artery stenosis

• Blowing SEM best heard in PA radiating out to both axillae

Questions?