05.13 Approach To Congenital Heart Disease
05.13 Approach To Congenital Heart Disease
05.13 Approach To Congenital Heart Disease
CONGENITAL ACQUIRED
ACYANOTIC CYANOTIC
Congenital Heart Disease
Others
Congenital Heart Disease
Atrial Septal Defect
Ventricular Septal Defect Left to Right
Patent Ductus Arteriosus Shunts (40%)
Pulmonary Stenosis
Obstructive
Aortic Stenosis
Lesions (25%)
Coarctation of the Aorta
Tetralogy of Fallot
Transposition of the Great Arteries Right to Left
Tricuspid Atresia / Pulmonary Atresia Shunts (20%)
Congenital Heart Disease
ACYANOTIC DEFECTS
ACYANOTIC DEFECTS
- VSD - ASD - AS or AR - PS
- PDA - PAPVR - CoA - CoA (infants)
(Partial
-CAVSD anomalous - Mitral - Mitral
pulmonary venous
return)
regurgitation stenosis
ASD
Hemodynamic Consequence
Diastolic overload of RV
ASD
• PE
– Soft systolic murmur, fixed split S2
• ECG
– Normal to RAD
– IRBBB pattern; RVH
• CXR
– Mild RV cardiomegaly
– Dilated MPA; Hypervascular markings
ASD
VSD
Hemodynamic
Consequence
• MODERATE SIZE
– Volume overload of LV
• LARGE SIZE
– Volume overload of LV
– Pressure overload of RV
VSD
• Most common L to R shunt
• Shunt is actually from left ventricle into
pulmonary artery
• Into right ventricle only with pulmonary
hypertension
VSD
Natural History
• Location of defect
– Muscular and perimembranous have high
incidence of spontaneous closure
• Size of the defect
– Larger the defect, more likely to develop
congestive heart failure sooner
– Smaller the defect, more likely to be asymptomatic
• Pulmonary vascular resistance
– Pulmonary hypertension or high resistance will
limit total shunt flow
VSD
Natural History
• Small VSD
– Normal growth & development
– No CHF
• Mod. to large VSD
– Easy fatigability, intermittent feeding
– Delayed growth & development
– Repeated respiratory tract infection
– CHF
VSD
• Signs of CHF – large VSD
• Dynamic & bulging precordium
• Systolic thrill
• Pansystolic murmur at LPSB
• Normal to increased P2
VSD
• ECG
– Small – Normal
– Moderate to large – LVH to CVH
– Severe PHTN – RVH
• CXR
– Normal to cardiomegaly (LV, combined to RV)
– Dilated MPA; hypervascular markings
VSD Small
VSD Large
CAVSD
90-
100ml
RA pressure
8 mmHg 100ml
90-
100ml
100ml
RV systolic /
EDP
60/8 mmHg
return
PS
• 5 – 8% of CHD
• Associated with congenital rubella;
Noonan & William syndrome
• Types:
– Valvar, subvalvar (infundibular), supravalvar
or peripheral
• Manifestations: asymptomatic unless
severe
PS
• Physical examination
– RV heave
– Systolic thrill (valve PS)
– Systolic ejection murmur LUSB with radiation
to the back (interscapular area and bilateral
axilla)
– Soft P2
PS
• ECG
– RAD
– IRBBB if mild
– RVH
• CXR
– Normal or RV cardiomegaly
– Normal or dilated MPA (post-stenotic
dilatation)
AS
• Hemodynamic
Consequence
• Pressure
overload of LV
Coarctation of the Aorta
• Hemodynamic
Consequence
• Pressure
overload of LV
Coarctation
• Physical examination
– LV heave
– Systolic murmur at the back
– Delayed or weak lower extremity pulses versus upper
extremity
• ECG
– LVH
– Left axis deviation
• CXR
– LVH
– Rib notching
Coarctation of the Aorta
Rib Notching
Cyanotic Heart Disease
Approach to Congenital Heart
Disease
HEART DISEASE
CONGENITAL ACQUIRED
CYANOTIC
ACYANOTIC CYANOTIC
Cyanotic Heart Disease
• Cyanotic heart disease exist when one
defect or association of defects allow the
mixture of oxygenated and de-saturated
blood to reach the systemic circulation
• R to L shunt
• 5 gm de-saturated Hgb/dL
• Ideal Hct for cyanotic hearts is 55-60 (58)
Cyanotic Heart Disease
• O2 sat < 85% --clinically apparent
• Reduced Hgb >= 5 gm/dL
• Rule out other non-cardiac causes
• Central vs. peripheral cyanosis
• Hyperoxic test
Do you suspect that patient is
Cyanotic?
A) Clubbing
B) Polycythemia: Elevated Hgb and Hct
C) Saturation by pulse oximeter in older infants
or children
D) Hyperoxia test in neonates or older
- 100% oxygen challenge for >= 10 minutes then
extract arterial blood sample from the right arm
(1st branch of aorta) to check the PaO2
Hyperoxia Test
• Hyperoxia test is considered positive for
intracardiac shunting if PaO2 < 150 mmHg
(torr) after 10 minutes of 100% fiO2
CYANOTIC DEFECTS
• Best to have a
large atrial shunt
Transposition of the Great Arteries
(TGA)
• Palliative
– Prostaglandin infusion
– Create interatrial communication
• Balloon atrial septostomy (BAS) – Rashkind procedure
• Blade septostomy
• Atrial septostomy – Blalock-Hanlon procedure
• Stent insertion across the atrial septum
• Definitive
– Arterial switch – Jatene procedure
– Atrial switch – Senning or Mustard procedure
Tricuspid Atresia
• Hemodynamic
Consequence
• Volume overload
of LV
• Obligatory right to
left atrial shunt
TAPVR
Total Anomalous Pulmonary Venous Return
• Hemodynamic Consequence
• Volume overload of RV
• Obligatory right to left
atrial shunt
PVA – IVS
Pulmonary Valve Atresia with Intact Ventricular Septum
• Hemodynamic
Consequence
• Pressure
overload of RV
• Obligatory right to
left atrial shunt
• PDA dependent
pulmonary
circulation
PVA – VSD
Pulmonary Valve Atresia with VSD
• Hemodynamic
Consequence
• Pressure
overload of RV
• PDA or collateral
dependent
pulmonary blood
flow
PDA Dependent Pulmonary
Circulation
• Pulmonary valve atresia (PVA) with intact
interventricular septum
• Other lesions with accompanying PVA
PDA Dependent Systemic
Circulation
• Hypoplastic left heart syndrome (HLHS)
• Interrupted aortic arch
Obligatory LR shunting at the
Atrial Level
• Mitral atresia / aortic atresia
• Hypoplastic Left Heart Syndrome (HLHS)
Obligatory RL shunting at the
Atrial Level
• Tricuspid Atresia
• Total Anomalous Pulmonary Venous
Return (TAPVR)
• Pulmonary Valve Atresia with intact
ventricular septum (PVA – IVS)
Characteristic Cardiac Silhouette
Tetralogy of Fallot Coeuer en Sabot
Boot shape
Transposition of Great Egg shape
Arteries
Total anomalous Snowman
Pulmonary Venous Figure of 8
Return
TOF Boot Shaped Heart
TGA Egg shaped
Pulmonary Vascular Markings
Increased: Cyanotic
C) Combination of both
Hemodynamic Consequences
Heart Failure
• MILD
– Small VSD, PDA, ASD
– Mild Aortic Stenosis (AS) or Pulmonary
Stenosis
• MODERATE
– Mild – moderate AS or PS
– Non-critical Coarctation of the Aorta
– Large ASD
Hemodynamic Consequences
Heart Failure
• SEVERE
– All cyanotic lesions (+/- heart failure)
– Large VSD
– Large PDA
– Critical AS / HLHS
– Critical PS
– Critical Coarctation of the aorta
– Complete atrioventicular septal defect (CAVSD)
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