paediatric cardiology: congenital heart disease and clinical problems dr. suzie lee pediatric...
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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?