Paediatric Cardiology:Paediatric Cardiology:

An Outline of Congenital An Outline of Congenital Heart DiseaseHeart Disease

Dr. H.C. Rosenberghrosenberg@cheo.on.ca

ObjectivesObjectives

To provide an outline of congenital heart disease

List criteria for Kawasaki syndrome Describe the common innocent

murmurs of childhood

An Outline of Congenital An Outline of Congenital Heart DiseaseHeart Disease

Pink (Acyanotic)

Blue (Cyanotic)

Resistance= ?

Acyanotic Congenital Acyanotic Congenital Heart DiseaseHeart Disease

Normal Pulmonary Blood Flow ↑ Pulmonary Blood Flow

Acyanotic Congenital Acyanotic Congenital Heart DiseaseHeart Disease

Normal Pulmonary Blood Flow Valve Lesions

Not fundamentally different from adults

Acyanotic Congenital Acyanotic Congenital Heart DiseaseHeart Disease

↑ Pulmonary Blood Flow

Shunt LesionsShunt LesionsAtrial Level Shunt

ASDASD

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 Low resistance

ASDASD

History Usually asymptomatic in childhood

Occasionally frequent respiratory tract infections Presentation with murmur as pre-schooler or older

ASDASD

Physical Examination Right ventricular “lift” Wide fixed S2 Blowing SEM in pulmonic area

ASDASD

ASDASD

ASDASD

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

ASDASD

Management Device closure around three years of age or when

found Surgery for very large defects or outside fossa

ovalis (eg. sinus venosus defect)

ASDASD

Shunt LesionsShunt LesionsVentricular Level Shunt

VSDVSD

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)

VSDVSD

History Small defects

Presentation with murmur in newborn period Large defects

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

VSDVSD

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

VSDVSD BVH

VSDVSD

VSDVSD

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

VSDVSD

Management Small defect Large defect

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

Occasionally elective closure if persistent cardiomegally beyond 3 years of age

Shunt LesionsShunt LesionsGreat Artery Level Shunt

PDAPDA

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)

PDAPDA

History Premature duct

Failure to wean from ventilator +/- murmur

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

PDAPDA

Physical Examination Active left ventricle Hyperdynamic pulses Premature duct

SEM with diastolic spill Older infant

Continuous murmur

PDAPDA

Management Premature Duct

Trial of indomethacin Surgical ligation

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

Truncus ArterisosusTruncus Arterisosus

Cyanotic Congenital Cyanotic Congenital Heart DiseaseHeart Disease

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

Systemic oxygen saturation is reduced

Cyanosis may or may not be clinically evident

Causes of CyanosisCauses of Cyanosis

Respiratory Cardiac Hematologic

Polycythemia Hemoglobins with decreased affinity

Neurologic Decreased Respiratory drive

CyanosisCyanosis

Respiratory Cardiac

Hyperoxic test Place infant in 100% 02

Lung disease should respond to 02 Failure of saturation to rise to > 85%

suggest cardiac disease

Cyanotic Congenital Cyanotic Congenital Heart DiseaseHeart Disease

↓Pulmonary Blood Flow↑Pulmonary Blood Flow

Cyanotic Congenital Cyanotic Congenital Heart DiseaseHeart Disease

Decreased Pulmonary Blood Flow

Cyanotic Congenital Cyanotic Congenital Heart Disease - ↓ Heart Disease - ↓ Pulmonary FlowPulmonary Flow

= RVOT Obstruction + Shunt

Tetralogy of Tetralogy of FallotFallot

VSD Over-riding aorta Pulmonary stenosis RVH

Tetralogy of FallotTetralogy 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 FallotTetralogy of Fallot

Physical Examination Variable cyanosis (remember the 50g/l rule) Right ventricular “tap” Decreased P2 +/- ejection click “Tearing” SEM

Tetralogy of FallotTetralogy 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 FallotTetralogy of Fallot

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

Tetralogy of FallotTetralogy of Fallot

Hypercyanotic Spells (“Tet” Spells)

Fall in P02

Hyperventilation

Increased Return of deeply desaturated

venous blood

Increased R to L shunt

Tetralogy of FallotTetralogy of Fallot

Hypercyanotic Spells (“Tet” Spells Treatment

Tuck knees to chest (pinches off femoral veins) In hospital

O2 Bicarbonate Phenylephrine Morphine IV beta blocker

Tetralogy of FallotTetralogy of Fallot

Tetralogy of FallotTetralogy of Fallot

Decreased Pulmonary Blood Flow

Duct Dependent Duct Dependent Congenital Heart Congenital Heart DiseaseDisease Which of the following are

examples of duct dependent CHD?

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

arteries

Cyanotic Congenital Cyanotic Congenital Heart Disease With Heart Disease With ↑Pulmonary Blood Flow↑Pulmonary Blood Flow

Cyanotic Congenital Cyanotic Congenital Heart Disease With Heart Disease With ↑Pulmonary Blood Flow↑Pulmonary Blood Flow

Transposition of the great arteries Total anomalous pulmonary venous

drainage

d-Transposition d-Transposition

Normal HeartNormal Heart

Body RA RV PA

LALVAO Lungs

Circulation is in “series”

d-Transposition d-Transposition

Circulation is in “parallel”

Need for mixing

TranspositionTransposition

History Presentation

Profound cyanosis shortly after birth (as duct closes)

Minimal or no murmur

Tetralogy of FallotTetralogy of Fallot

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

Tetralogy of FallotTetralogy of Fallot

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

Total Anomalous Total Anomalous Pulmonary Venous Pulmonary Venous ReturnReturn

Pulmonary veins fail to connect to left atrium

Pulmonary veins communicate with systemic vein

Total Anomalous Total Anomalous Pulmonary Venous Pulmonary Venous Return - SupracardiacReturn - Supracardiac

Pulmonary veins fail to connect to left atrium

Pulmonary veins communicate with systemic vein

Total Anomalous Total Anomalous Pulmonary Venous Pulmonary Venous Return - InfracardiacReturn - InfracardiacPulmonary

veins fail to connect to left atrium

Pulmonary veins communicate with systemic vein

TAPVDTAPVD

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

TAPVDTAPVD

Physical Examination Variable cyanosis (again depends on obstruction) Right ventricular “tap” Wide split S2 Blowing systolic ejection murmur

TAPVDTAPVD

TAPVDTAPVD

Management If severe cyanosis in newborn

Emergency surgical repair Unobstructed

Semi-elective surgical repair when discovered

Coarctation of the aortaCoarctation of the aorta

Coarctation of the AortaCoarctation of the Aorta

History Presentation varies with severity

Severe coarct Failure (shock) in early infancy

Mild coarct Murmur (in back) Hypertension

CoarctationCoarctation

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

CoarctationCoarctation

Management Newborn with CHF

Emergency surgical repair Infant

Semi-elective repair in uncontrolled hypertension Older child

Balloon arterioplasty Surgery on occasion

Failure to repair prior to adolescence recipe for life long hypertension!

““Grey” Heart Grey” Heart DiseaseDisease

Critical LVOT

obstruction

Left Ventricular Outflow Left Ventricular Outflow Tract ObstructionTract Obstruction

Critical Aortic Stenosis “Critical”

shock

Critical Aortic StenosisCritical Aortic Stenosis

Management Prostaglandins to provide source of systemic blood

flow Balloon valvuloplasty Rarely surgery

Hypoplastic Left Heart Hypoplastic Left Heart SyndromeSyndrome

“Duct dependent “ congenital heart disease

Ductus arteriosus is the only source of systemic blood flow

Hypoplastic left heart Hypoplastic left heart Management Prostaglandins Norwood procedure

Kawasaki SyndromeKawasaki Syndrome

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

(normal) stenosis

Kawasaki SyndromeKawasaki 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 SyndromeKawasaki Syndrome

Associated Findings Sterile pyuria Hydrops of the gallbladder Irritability!!!

Kawasaki SyndromeKawasaki Syndrome

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

Kawasaki SyndromeKawasaki Syndrome

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

for 6 weeks

Innocent MurmursInnocent Murmurs

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

Innocent MurmursInnocent 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?Questions?