Congenital heart disease final year lectures

September 2010

Cyanotic heart disease

Relevant terms
Percent O2 Saturation the proportion of RBC
sites which contain O2.
Partial pressure of O2 - indicates the availability
of O2 in alveolus and serum.
O2 dissociation curve shows the relation of O2
Sat and PO2.

Relevant terms
Oxygen content: 1 gm Hb carries approx
1.39ml of O2 if 100% sat.
O2 cont. adult Hb 15gmHb/dl, 100 % sat:
15 x 1.39 x 1(100% sat) x 10 = 208 O2/L.
O2 cont. adult Hb 15gm Hb, pO2 40(70%sat):
15 x 1.39 x .7 x 10 = 146 O2/L blood.

Relevant terms
Newborn O2 content:
20gm Hb, pO2 40(sat 75%)
20 x 1.39 x .75 x 10 = 208ml/L blood
Newborn O2 content:
12gm Hb, pO2 30(sat 60%)
12 x 1.39 x .60 x 10 = 100ml/L blood.

Congenital heart disease final year lectures

September 2010

Cyanotic CHD
Cyanosis - means blue blood a sign, not a
diagnosis - requires 5gm/dl reduced Hb.
Cyanosis - peripheral, central, differential.
Causes Cardiac, pulmonary, CNS,
haematological.

Approach to the cyanotic infant

PERIPHERAL
Pink mm
PO2 normal
Cool extremities
DD shock, sepsis,
cold

CENTRAL
Blue mm
Low pO2 & O2 sat
Warm extremities.

Cardiac or pulmonary cyanosis?

CARDIAC
PULMONARY
Increase on crying
Decrease on crying
+ or SOB
In resp. distress
Abnormal ECG
Normal ECG
Cxray changes
Cxray changes
pCO2 normal
pCO2 high
Small response to O2 Marked response to O2

Congenital heart disease final year lectures

September 2010

Laboratory studies
Pulse oximetry
Blood gas + 100% O2 challenge.
Pulm/hypovent paO2 <60 to > 200
R to L shunt

to >100
Pulm dis/CCF

to 100 200
Dextrostix. U&E. Hb PCV.
CXRay ECG 2DE

Classification cyanotic CHD

With incr. Pulm blood flow - TGA, TAPVR
truncus arteriosus.
With decr. PBF - TOF, PA, TA.
TGA is commonest cyanotic CHD in NB
period.
TOF is commonest cyanotic CHD at all ages.

Cyanotic CHD

Tetralogy of Fallot
Transposition of great arteries
Tricuspid atresia
Truncus arteriosus
Total anomalous pulmonary ven. Drainage
Hypoplastic left heart syndrome
Pulmonary atresia

Congenital heart disease final year lectures

September 2010

Complications - cyanotic CHD

Polycythemia
Thrombosis
Paradoxical emboli
Cerebral abscess
Infective endocarditis

Tetralogy of Fallot
Components

Ventricular septal defect

RV OT obstruction
RV hypertrophy
Overriding of Aorta

Congenital heart disease final year lectures

September 2010

TOF
Embryology
Anterior and superior displacement of conus
septum resulting in a nonalignment VSD in
the area of the membranous septum.

TOF - Haemodynamics

Large unrestrictive VSD.

Equal RV and LV and aortic pressures.
RVOT obstruction infundibular/valvular/
pulm art/branches.
R to L shunt through VSD to aorta.
Decreased pulmonary blood flow.

Congenital heart disease final year lectures

September 2010

TOF - Clinical features

Usually pink at birth unless defect is severe.
Signs similar to VSD at first
Blue on crying . Persistent cyanosis and
clubbing.
SOB on exercise. Squatting.
Growth and development affected.
Hypercyanotic spells.

TOF - Haemodynamics
R to L shunt increased by a fall in systemic
resistance and an increase in RVOT
obstruction (e g infundibular spasm).
If RVOT obstruction is mild, shunt is L to R
across VSD but ECG shows RVH.
Systemic resistance increases with
squatting, decreases with exercise.

TOF - Blue Spell

Congenital heart disease final year lectures

September 2010

TOF- Physical examination

- classical
General Growth impaired, cyanosis,
clubbing.
Pulses and BP normal.
Heart quiet praecordium, diffuse apex,
RV heave, single S2, ejection SM ULSE
No evidence of CCF.

TOF - Investigations

Hb PCV Pulse oximetry

ECG RAD RVH
Cxray Heart size normal. Decreased PBF.
Boot shaped heart. 25% R aortic arch.
2DE all features demonstrated.
Cardiac catheter preferred by some
surgeons.Coronary art. & pulm art (br) size.

TOF - Boot shaped heart

Congenital heart disease final year lectures

September 2010

Treatment of TOF
Maintain hydration
Treat Fe deficiency. Plebotomy if PCV>65
Anticipate and manage blue spells

Treatment of blue spells

Anticipate. Prevention propanolol p.o.
Knee chest position. Oxygen(not helpful)
IV access - bolus N saline / Hartmanns
- Propranolol / morphine
- Na HCO3, phenylephrine
If severe/persistent - anaesthetise/ventilate
- urgent Sx.

TOF BT shunt

Congenital heart disease final year lectures

September 2010

TOF - Total correction

TOF - Complications

Polycythemia. Infective endocarditis.

Hypercyanotic spells. Failure to thrive
Brain abscesses. Seizures. CVA.
Bleeding disorders.
Decreased development.
Death/ brain damage in blue spell.
Post op complications.

Congenital heart disease final year lectures

September 2010

Transposition of the great arteries

Aorta arises from the RV anterior to and to the R
of the
Pulmonary artery which arises from LV
In the normal heart there is a single circulation in
series
In TGA there are 2 circulations in parallel
Survival depends on a communication between
parallel circulations.

TGA Features
Cyanosis at birth, NB becomes hypoxic and
acidotic.
Cardiac failure in NB period.
When the ductus closes severe hypoxia,
cyanosis and death ensue if there is no other
communication.

10

Congenital heart disease final year lectures

September 2010

TGA
Treatment
maintain ductal patency by IV
prostaglandin E1 in the early NB period or
Perform balloon atrial septostomy until
Definitive surgery - e.g. arterial switch

Balloon atrial septostomy

b

11

Congenital heart disease final year lectures

September 2010

Arterial switch

Truncus arteriosus

Tricuspid atresia

12

Congenital heart disease final year lectures

September 2010

TAPVR

Pulmonary atresia

HYPOLASTIC LH SYN

13

Congenital heart disease final year lectures

September 2010

14