Paediatric cardiology congenital heart disease and clinical problems
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Paediatric Cardiology: Congenital Heart Disease and Clinical Problems. Dr. Suzie Lee Pediatric Cardiologist Assistant Professor, University of Ottawa. Objectives. To provide an outline of congenital heart disease List criteria for Kawasaki syndrome

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Paediatric Cardiology:Congenital Heart Disease and Clinical Problems

Dr. Suzie Lee

Pediatric Cardiologist

Assistant 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


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


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

  • Respiratory

  • Cardiac

  • Hematologic

    • Polycythemia

    • Hemoglobins with decreased affinity

  • Neurologic

    • Decreased Respiratory drive


Cyanosis

  • Respiratory

  • Cardiac

    • 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

AO

LV

LA

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


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


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 communicate with systemic vein

Pulmonary veins fail to connect to left atrium


Total Anomalous Pulmonary Venous Return - Supracardiac

Pulmonary veins communicate with systemic vein

Pulmonary veins fail to connect to left atrium


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 R to L shunt

Increased Return of deeply desaturated venous blood


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/VSD

Tetralogy 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 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)


Case 1 (continued)

BTS


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


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


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


Coarctation - treatment

Surgical 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


HLHS

  • Initially cyanotic

  • With 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?


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