Paediatric Cardiology:Paediatric Cardiology:
An Outline of Congenital An Outline of Congenital Heart DiseaseHeart Disease
Dr. H.C. [email protected]
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?