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Paediatric Cardiology: Congenital Heart Disease and Clinical Problems
Dr. Suzie Lee Pediatric Cardiologist Assistant Professor, University of Ottawa
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Objectives To provide an outline of congenital heart disease
List criteria for Kawasaki syndrome Describe the common innocent murmurs of childhood
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An Outline of Congenital Heart Disease
Pink (Acyanotic) Blue (Cyanotic) Critical outflow tract obstruction
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Acyanotic Congenital Heart Disease
Normal Pulmonary Blood Flow ↑ Pulmonary Blood Flow
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Acyanotic Congenital Heart Disease
Normal Pulmonary Blood Flow Valve Lesions Not fundamentally different from adults
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Acyanotic Congenital Heart Disease
↑ Pulmonary Blood Flow
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Shunt Lesions Atrial Level Shunt
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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
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ASD History Usually asymptomatic in childhood
Occasionally frequent respiratory tract infections Presentation with murmur in childhood
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ASD Physical Examination Right ventricular “lift” Wide fixed S2
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
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ASD
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ASD
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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
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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)
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ASD
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Shunt Lesions Ventricular Level Shunt
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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)
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VSD History Small defects Large defects
Presentation with murmur in newborn period Large defects Failure to thrive (6 wks to 3 months) Tachypnea, poor feeding, diaphoresis
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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
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VSD
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VSD
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VSD Natural History Small defect Large defect Often close
No real significance beyond endocarditis risk Large defect Failure to thrive Progression to pulmonary hypertension as early as 1 year
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VSD Management Small defect Large defect Conservative management
Semi-elective closure if growth failure or evidence of increased pulmonary hypertension Occasionally elective closure if persistent cardiomegaly beyond 3 years of age
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Shunt Lesions Great Artery Level Shunt
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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)
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PDA History Premature duct Older infant
Failure to wean from ventilator +/- murmur Older infant Usually murmur from early infancy Occasionally signs of heart failure
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PDA Physical Examination Active left ventricle Hyperdynamic pulses
Premature duct SEM with diastolic spill Older infant Continuous murmur
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PDA Management Premature Duct Older infant Trial of indomethacin
Surgical ligation Older infant Leave till 1 year of age unless symptomatic Coil / device closure Rarely surgical ligation
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Coarctation Obstruction of the aortic arch
Classically juxtaductal, although may occur anywhere along the aorta May develop over time Femoral pulses should be checked routinely throughout childhood
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Coarctation of the Aorta
History Presentation varies with severity Severe coarct Failure (shock) in early infancy Mild coarct Murmur (in back) Hypertension
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Coarctation Physical Examination Absent femoral pulses
Arm leg gradient +/- hypertension Left ventricular “tap” Bruit over back
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Coarctation Management Newborn with CHF Infant Older child
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
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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
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Causes of Cyanosis Respiratory Cardiac Hematologic Neurologic
Polycythemia Hemoglobins with decreased affinity Neurologic Decreased Respiratory drive
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Cyanosis Respiratory Cardiac Hyperoxic test – response to 100% O2
Lung disease should respond to 02 PO2 should rise to greater than 150 mmHg
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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
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Cyanotic Congenital Heart Disease
↑Pulmonary Blood Flow
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Cyanotic Congenital Heart Disease
Increased Pulmonary Blood Flow TGA TAPVD Truncus arteriosus
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d-Transposition
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Normal Heart Body RA RV PA AO LV LA Lungs Circulation is in “series”
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d-Transposition Circulation is in “parallel” Body RA RV Ao
Lungs LA LV PA
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d-Transposition Circulation is in “parallel” Need for mixing
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TGA Must 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 arteriosus Balloon atrial septostomy (BAS) Increase intracardiac shunting across the atrial septum
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d-Transposition Body RA RV Ao PFO BAS PDA PGE Lungs LA LV PA
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Transposition History Presentation
Profound cyanosis shortly after birth (as duct closes) Minimal or no murmur
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TGA Physical Examination Profound cyanosis Right ventricular “tap”
Loud single S2 Little or no murmur
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TGA Management Prostaglandins to maintain mixing
Balloon atrial septostomy Arterial switch repair in first week
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Balloon Atrial Septostomy
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Total Anomalous Pulmonary Venous Return
Pulmonary veins communicate with systemic vein Pulmonary veins fail to connect to left atrium
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Total Anomalous Pulmonary Venous Return - Supracardiac
Pulmonary veins communicate with systemic vein Pulmonary veins fail to connect to left atrium
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Total Anomalous Pulmonary Venous Return - Infracardiac
Pulmonary veins fail to connect to left atrium Pulmonary veins communicate with systemic vein
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TAPVD History Not a PGE dependent lesion
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
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TAPVD Physical Examination
Variable cyanosis (again depends on obstruction) Right ventricular “tap” Wide split S2 Blowing systolic ejection murmur
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TAPVD
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TAPVD Management If severe cyanosis in newborn Unobstructed
Emergency surgical repair Unobstructed Semi-elective surgical repair when discovered
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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
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Cyanotic Congenital Heart Disease
Decreased Pulmonary Blood Flow
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Cyanotic Congenital Heart Disease
Decreased Pulmonary Blood Flow Tetralogy of Fallot/Pulmonary Atresia Tricuspid Atresia Critical pulmonary valve stenosis
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Cyanotic Congenital Heart Disease - ↓ Pulmonary Flow
= RVOT Obstruction + Shunt
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Cyanotic Congenital Heart Disease
Tetralogy of Fallot 1. Pulmonary stenosis 2. Overriding aorta 3. RVH 4. VSD Generally not a PGE dependent lesion
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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)
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Tetralogy of Fallot Physical Examination
Variable cyanosis (remember the 50g/l rule) Right ventricular “tap” Decreased P2 +/- ejection click “Tearing”/harsh SEM
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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
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Tetralogy of Fallot Hypercyanotic Spells (“Tet” Spells)
Episodes of profound cyanosis Most frequently after waking up or exercise
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Tetralogy of Fallot Hypercyanotic Spells (“Tet” Spells)
Stress leading to fall in P02 Tachycardia and Hyperventilation Increased R to L shunt Increased Return of deeply desaturated venous blood
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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
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Tetralogy of Fallot
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Tetralogy of Fallot Decreased Pulmonary Blood Flow
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Pulmonary atresia/VSD
Tetralogy of Fallot with atretic pulmonary valve Variable pulmonary artery anatomy Generally a PGE dependent lesion
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Critical pulmonary stenosis
Severe pulmonary stenosis with inadequate pulmonary flow Pulmonary atresia/intact ventricular septum PGE dependent lesion
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Tricuspid atresia Generally a PGE dependent lesion
2. severely hypoplastic RV 3. VSD 4. ASD – large 5. pulmonary stenosis Variable Generally a PGE dependent lesion
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Cyanotic Heart Disease
Decreased 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)
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Case 1 (continued) BTS 80
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Duct Dependent Congenital Heart Disease
Which of the following are examples of duct dependent CHD? Pulmonary atresia Patent ductus arteriosus Transposition of the great arteries
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Critical Left-Sided Obstruction
Neonatal presentation Coarctation Critical aortic stenosis Hypoplastic left heart syndrome Cardiogenic shock PGE dependent lesion
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Left-sided Obstruction
Coarctation of the aorta Critical narrowing of the “juxtaductal” aorta Blood cannot get past the obstruction SHOCK
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Coarctation Characterized by weak or absent pulses particularly in the lower limbs Initiation 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
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Coarctation - treatment
Surgical correction following initiation of PGE and stabilization
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Left-Sided Obstruction
Critical Aortic Stenosis CRITICAL Inadequate forward flow to maintain cardiac output SHOCK
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Critical Aortic Stenosis
Management Prostaglandins to provide source of systemic blood flow Balloon valvuloplasty Rarely surgery
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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
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HLHS Initially cyanotic With closure of the PDA SHOCK PGE
Tachycardia, tachypnea, low blood pressure, weak pulses, poor perfusion, cyanotic/grey colour PGE
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Hypoplastic left heart
Management Prostaglandins Norwood procedure Heart Transplant
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Kawasaki Syndrome Small artery arteritis
Coronary arteries most seriously effected Dilatation/aneurysms progressing to (normal) stenosis
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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)
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Kawasaki Syndrome Associated Findings Sterile pyuria
Hydrops of the gallbladder Irritability
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Kawasaki Syndrome Epidemiology Generally children < 5 years
Male > Female Asian > Black > White
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Kawasaki Syndrome Management Gamma globulin 2g/kg
80 mg/kg ASA until afebrile then 5 mg/kg for 6 weeks
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Innocent Murmurs Characteristics Always Grade III or less
Always systolic (occasionally continuous) Blowing or musical quality Not best heard in back
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Innocent Murmurs Types Still’s Pulmonary Flow murmur Venous Hum
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
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Questions?
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