Cyanotic Congenital Heart Disease

2/24/21 Pediatric Cardiology... Prof. SADIQ M. AL- HAMASH 2

 CYANOSIS IN CHILDREN
! Central cyanosis:
– It is the cyanosis of the tongue, mucous membranes
and skin . It is necessary to have >5g/dl of
deoxygenated Hb to have such cyanosis and it
usually due to Rt to Lt shunt.
! Peripheral cyanosis:
– It is visible only in the skin of the extremities with
normal arterial saturation and it usually due to
vasomotor instability like cold environment.

2/24/21 Pediatric Cardiology... Prof. SADIQ M. AL- HAMASH 3

TETRALOGY OF FALLOT (cont.):
It is the “commonest”
cyanotic congenital heart
disease in children and adults.
It is a combination of:
1. VSD
2. Pulmonary stenosis (PS).
3. Overriding of aorta.
4. Right ventricular hypertrophy
(RVH).

2/24/21 Pediatric Cardiology...Assist. Prof. SADIQ M. AL- HAMASH 5

TETRALOGY OF FALLOT (cont.):

Hemodynamics:
The degree of right ventricular outflow
obstruction (i.e. P.S.) determines the degree
of pulmonary blood flow and the severity of
cyanosis.

2/24/21 Pediatric Cardiology...Assist. Prof. SADIQ M. AL- HAMASH 6

 Hemodynamics of TOF

RV（hypertrophy） bypass LV

Pulmonic stenosis
Bypass
Aorta（blood flow ↑, dilated)
↓
Pulmonary circulation ↓
Deoxygenated blood
(clear lung field) enters systematic circulation
（cyanotic、growth delay、
squatting、anoxic spell）

Low saO2 （clubbing）and spell

2/24/21 Pediatric Cardiology.... Prof. SADIQ M. AL- HAMASH 7

TETRALOGY OF FALLOT (cont.):
C/F:
1、Cyanosis：central，worsen when exercise or fast
breathing.
most patients develop cyanosis in the first 6 month – 1 year of
life.
2、Squatting：seen older children
it is the characteristic posture of children with TOF after any
physical effort
3、Clubbing of fingers and toes
4、Hypercyanotic spell
5、Excertional dyspnea
2/24/21 Pediatric Cardiology...Prof. SADIQ M. AL- HAMASH 8
TETRALOGY OF FALLOT (cont.):
O/E:
ü Cyanosis (central and peripheral).
ü Clubbing (usually after 3 months of age).
ü Normal pulse & quiet precordium.
ü Single S2 & ejection systolic murmur at the left
sternal border.

2/24/21 Pediatric Cardiology...Prof. SADIQ M. AL- HAMASH 9

ECG

Right axis
deviation
RV hypertrophy
RA hypertrophy

2/24/21 Pediatric Cardiology...Assist. Prof. SADIQ M. AL- HAMASH 10

 TOF x-ray findings
Ø Normal heart size.
1、Upturned apex
( RV hypertrophy)
2、The absence of main PA
segment gives it the shape
described as boot shape
3、Pul. fields are oligaemic.
(decrease pulmonary
vasculature, clear lung fields)
2/24/21 Pediatric Cardiology... Prof. SADIQ M. AL- HAMASH 11
TETRALOGY OF FALLOT (cont.):
Echo: It is essential for Dx.
Catheterization: Gives the definitive Dx
preoperative step.

Echo: PLAX view showing TOF.

2/24/21 Pediatric Cardiology...Assist. Prof. SADIQ M. AL- HAMASH 12
TOF …Echo vedio

2/24/21 Pediatric Cardiology...Assist. Prof. SADIQ M. AL- HAMASH 13

 TETRALOGY OF FALLOT (cont.):
Complications:
1. Hypercyanotic spells.
2. Cerebrovascular accidents (CVA).
3. Cerebral abscess.
4. Infective endocarditis.
5. Hematological complications include bleeding and
thrombosis.
§ All complications are essentially due to cyanosis & polycythemia.
§ Polycythemia is due to hypoxia which results from right to left shunt (as the right pressure
grows higher than the left one).

2/24/21 Pediatric Cardiology...Assist. Prof. SADIQ M. AL- HAMASH 14

TETRALOGY OF FALLOT (cont.):
Rx:
Medical:
§ Rx of anemia by iron, sometimes considered as relative
anemia if we find normal Hb level; also nutritional
support.
§ Phlebotomy: in symptomatic patients with hematocrit
more than 65% (as there is a risk for CVA).
§ B-Blocker
Surgical:
§ Palliative to ­ pulmonary blood flow e.g.: Blalock-Taussig
shunt (BT shunt).
§ Total surgical repair.
2/24/21 Pediatric Cardiology... Prof. SADIQ M. AL- HAMASH 15
TETRALOGY OF FALLOT (cont.):
Indications for surgery:
1. Cyanosis
2. Hypercyanotic spells
3. Polycythemia
4. ¯ exercise tolerance
5. Appropriate age and
weight (usually 1 to 2
years).

2/24/21 Pediatric Cardiology... Prof. SADIQ M. AL- HAMASH 16

 HYPERCYANOTIC SPELLS:
Definition;
§ Attacks of increasing cyanosis associated with
abnormal respiration and altered level of
consciousness and it is an important cause of death
in TOF patients .
§ Spells are particular problems during the 1st 2 years
of life and is more common in the morning and can
be precipitated by any activity. Most spells are self-
limited, but should be considered as an absolute
indication for surgery.
§ The cause is infundibular spasm.
2/24/21 Pediatric Cardiology...Prof. SADIQ M. AL- HAMASH 17
HYPER CYANOTIC SPELLS (cont.):
Managment:
§ Knee-chest position.
§ O2 .
§ Sodium bicarbonate to correct metabolic acidosis.
§ Morphine: 0.2 mg/Kg subcutaneously (s.c.) or i.v. can be
repeated 4 hourly.
§ B-blocker: propranolol (inderal) given 0.1-0.2 mg i.v. and
can be given orally (1-2 mg/Kg/day) as a prophylaxis
against the spells (given in 2 divided doses to patients
not indicated for surgery).
§ Surgery: emergency surgical intervention may be
necessary in severe spells resistant to medical Rx.

2/24/21 Pediatric Cardiology... Prof. SADIQ M. AL- HAMASH 18

TRANSPOSITION OF GREAT
ARTERIES (cont.):
§ It is the most common cause of
cyanosis in the newborns
§ 5% of CHD.
§ M:F is 2:1
§ More common in infants with DM
mothers.
§ In TGA, there is Ventriculoarterial
discordance.. aorta to RV, and
pulmonary artery to LV.

2/24/21 Pediatric Cardiology... Prof. SADIQ M. AL- HAMASH 20

TRANSPOSITION OF GREAT
ARTERIES (cont.):

Clinical features:
Cyanosis and tachypnoea in the first hours or days of life.

Prognosis:
Without surgery, 90% will die within the first year of life.
If Rashkind septostomy done, 90% will survive the first
year of life.
If surgery done it will have normal life span

2/24/21 Pediatric Cardiology...Assist. Prof. SADIQ M. AL- HAMASH 21

Transposition – Diagnosis and
Treatment
! Diagnosis
– Echocardiography

! Treatment
– Balloon septostomy during cardiac
cath.
» Rashkind’s Procedure
» Reestablish Foramen Ovale
– Surgical Correction
» Jantene Operation

2/24/21 Pediatric Cardiology...Prof. SADIQ M. AL- HAMASH 22

 HEART FAILURE (cont.):
Heart failure:
is inability of the heart myocardium to produce cardiac
output to sustain metabolic body need.
i.e. the cardiac output which is heart rate times the stroke
volume is not enough to supply O2 & nutrients and other
cellular essential building units, also it is not enough to
remove the waste products generated by the cells. .

2/24/21 Pediatric Cardiology... Prof. SADIQ M. AL- HAMASH 24

 HEART FAILURE (cont.):
pathophysiologic alterations include:

§ ­ preload ( volume overload) as in: Lt. à Rt. Shunt, valvular

insufficiency, severe anemia.
§ ­ after load ( pressure overload )as in: aortic stenosis, aortic
coarctation, acut HPT.
§ Myocardial abnormalities: this impair its contractility as in
myocarditis, dilated cardiomyopathy.
§ Diastolic dysfunction i.e. Impaired ventricular filling so this reduce
stroke volume as in: hypertrophic cardiomyopathy, tachy-
dysrrhythmias.

2/24/21 Pediatric Cardiology... Prof. SADIQ M. AL- HAMASH 25

HEART FAILURE (cont.):
Compensatory mechanism against HF:
1. Enlarged myocardial contractility with
increased filling volume. This is “frank-starling
law”.
2. Hypertrophy of myocardium.
3. Activation of rennin-angiotensin system.
4. Activation of sympathitic nervous system

2/24/21 Pediatric Cardiology... Prof. SADIQ M. AL- HAMASH 26

HEART FAILURE (cont.):
Causes of HF according to age:
• Asphyxial cardiomyopathy.
• Fluid overload
Neonate: • Congenital heart disease as hypoplastic
heart syndrome, large VSD, Lt. à Rt. Shunt.
• Viral myocarditis

• Lt. à Rt. Shunt as VSD, PDA.

Infancy: • Dilated cardiomyopathy.

• Supraventricular tachycardia.
• Acute HT a sin hemolytic uremic syndrome.

2/24/21 Pediatric Cardiology...Prof. SADIQ M. AL- HAMASH 27

HEART FAILURE (cont.):
Causes of HF according to age (cont.):

Childhood • Viral myocarditis & cardiomyopathy.

• Rheumatic heart disease
and • Anticancer therapy as radiation and adriamycin.
• Hemochromatosis, hemosiderosis à primary or
adolescence secondary as in thalassemia.
• Acute HT e.g. glomerulonephritis.

2/24/21 Pediatric Cardiology... Prof. SADIQ M. AL- HAMASH 28

HEART FAILURE (cont.):
C/F:
Hx is important in both Dx and evaluation of HF, and
knowing the cause of HF.
In infants:
§ Feeding difficulties (less volume per feeding).
§ Dyspnoea (crying, milk or formula sucking).
§ Profuse sweating, irritability.
§ Poor weight gain.
§ Weak cry and noisy labored respiration.
In children (as in adult C/F):
§ Fatigue, effort intolerance, anorexia, cough, dyspnoea,
abdominal pain which is due to (hepatic capsule stretching
or intestinal vascular congestion).

2/24/21 Pediatric Cardiology...Assist. Prof. SADIQ M. AL- HAMASH 29

HEART FAILURE (cont.):
O/E:
Infant with HF:
§ Weak peripheral pulse, narrowed pulse pressure
(pulse pressure = systolic – diastolic B.P.)
§ Tachycardia, Gallop rhythm i.e. S1,2,3, + S4, murmur
of the cause.
§ Rhonchi due to compressed airway by congested
pulmonary vessel.
§ Basal crepitations which may be due to respiratory
infection.
§ Peripheral edema is rare in infant.
In children à facial edema, and edema of dependant
body part is more common than peripheral edema.
2/24/21 Pediatric Cardiology...AProf. SADIQ M. AL- HAMASH 30
HEART FAILURE (cont.):
Dx:
§ CXR: the most important to look for is heart size,
pulmonary vascular shadow à this is usually elevated,
frank pulmonary edema is rare in children.
§ ECG: non specific but it is helpful in assessing the
cause but it does not confirm the Dx. So, ECG is best to
evaluate rhythm disorder as a cause of heart failure.
§ Echo: to diagnose structural defect, assess myocardial
function by determining stroke vol., end-systolic vol.,
End-diastolic vol.

2/24/21 Pediatric Cardiology...Prof. SADIQ M. AL- HAMASH 31

HEART FAILURE (cont.):
Rx of HF: Aims are:
1. Improve myocardial performance by digoxin (digitalis).
2. Relief of pulmonary and systemic venous congestion
by diuretic and vasodilator.
3. Rx of underlying cause.

General Rx measures:
§Bed rest.
§Semi-up right position.
§Diet.

2/24/21 Pediatric Cardiology...Assist. Prof. SADIQ M. AL- HAMASH 32

HEART FAILURE (cont.):
Medical Rx:
1. Digoxin: it remains the most widely used pharmacologic
agent in Rx of HF in infant and children, it is +ve inotropic
agent i.e. increases myocardial contractility.
§ Mechanism of action: Inhibit Na+-K+ pump à so ­
intracellular Na+ and indirectly ­ Ca+2 this Ca+2 increase
the myocardial contractility.
Slowing heart rate by direct effect on AV-node and by
vagus N. stimulation and by anti-adrenergic effect.
§ Metabolism:
§ t1/2 = 36 hr., eliminated by kidney.

2/24/21 Pediatric Cardiology... Prof. SADIQ M. AL- HAMASH 33

HEART FAILURE (cont.):
Dose:

§ There is individual variation in response to drug.

§ Don't exceed the adult dose à 1-1.5 mg
§ Digitalization dose is 0.04-0.06 mg/Kg/day, we give
1/2 dose immediately and succeeding 2 doses 8-16
hrs later.
§ Maintenance dose is 0.01 mg/Kg/day. This should
be started 12 hr after full digitalization.

2/24/21 Pediatric Cardiology...Assist. Prof. SADIQ M. AL- HAMASH 34

HEART FAILURE (cont.):
Toxicity:
Incidence is low in infant and children and can
occur without systemic manifestations. Features
of toxicity are:
§ Cardiac:
§ brady-arrhythmia is commoner in young.
§ AV block is commonest toxicity sign.
§ Ventricular fibrillation & death may occur.
§ Extra-cardiac:
§ N. & V., anorexia, visual disturbance.

2/24/21 Pediatric Cardiology... Prof. SADIQ M. AL- HAMASH 35

HEART FAILURE (cont.):
Mx of toxicity:
1. Observation of serum digoxin level.
2. Stop administration of digoxin.
3. Correct any electrolyte disturbance.
4. Atropine or pacemaker for patients with
bradycardia.
5. Digoxin Fab antibody à effect appear in 30-
40 min.

2/24/21 Pediatric Cardiology...Assist. Prof. SADIQ M. AL- HAMASH 36

HEART FAILURE (cont.):
2. Diuretic:
§ Loop diuretic as frusemide à 1-2 up to 2-4 mg to ¯
preload.
3. Vasodilators:
§ ACE inhibitors à captopril 0.6-6 mg/Kg
§ Angiotensin II receptor blockers.

4.B-blockers
5. Inotropic agent à dopamine, dobutamine
6. Rx for underlying cause (e.g.. closing the VSD)

2/24/21 Pediatric Cardiology...Prof. SADIQ M. AL- HAMASH 37

CARDIOMYOPATHY (cont.):
§ It is a disease of the heart muscle itself, not
associated with congenital, valvular, or
coronary heart disease or systemic disorders. It
is distinct from the specific heart muscle
diseases of known cause.
§ Cardiomyopathy has been classified into three
types based on anatomic and functional
features: hypertrophic, dilated , and restrictive.

2/24/21 Pediatric Cardiology...Assist. Prof. SADIQ M. AL- HAMASH 39

CARDIOMYOPATHY (cont.):
Functional Classification of cardiomyopathy:
Dilated cardiomyopathy 80%

Hypertrophic cardiomyop10%

Restrictive cardiomyophy 10%

2/24/21 Pediatric Cardiology... Prof. SADIQ M. AL- HAMASH 40

CARDIOMYOPATHY (cont.):
Dilated Cardiomyopathy (DCM):
§ In which there is myocardial dysfunction and
ventricular dilatation.
§ Causes:
1. Idiopathic
2. Viral infection à Coxachi virus.
3. Genetic
4. Metabolic à Beri Beri and carnitine deficiency
5. Drugs à adriamycin
6. Autoimmune disease as SLE.

2/24/21 Pediatric Cardiology...Prof. SADIQ M. AL- HAMASH 41

CARDIOMYOPATHY (cont.):
Clinical features:
Symptoms:
§ Orthopnoea
§ Dyspnoea
§ Paroxysmal nocturnal dypnoea

Signs:

§ Gallop rhythm

2/24/21 Pediatric Cardiology...Assist. Prof. SADIQ M. AL- HAMASH 42

 CARDIOMYOPATHY (cont.):
CXR: cardiomegaly, plethoric lung
ECG:
1. LVH; Sinus tachycardia, and ST-T changes are the
most common findings.
2. Atrial or ventricular arrhythmias and atrio ventricular
(AV) conduction disturbances may be seen.
Echo: is the most important tool in the diagnosis of the
condition and is important in the longitudinal follow-up of
patients.

2/24/21 Pediatric Cardiology...Assist. Prof. SADIQ M. AL- HAMASH 43

CARDIOMYOPATHY (cont.):

Echo: A4C & M mode views in DCM

2/24/21 Pediatric Cardiology...Assist. Prof. SADIQ M. AL- HAMASH 44

CARDIOMYOPATHY (cont.):
Death occurs due to:
1. Dysrrhythmia
2. Severe HF
3. Severe resp. infection
4. Thromboembolism

2/24/21 Pediatric Cardiology...Assist. Prof. SADIQ M. AL- HAMASH 45

CARDIOMYOPATHY (cont.):
Rx:
§ Rx the cause.
§ Rx HF by inotropics, vasodilators, diuretics.
§ Antiplatelet (aspirin), anticoagulant (warfarin)
§ Anti-arrhythmic
§ B-blockers à metoprolol & carvidolol
§ Carnitine
§ Ventricular assisted device
§ ICD
§ Heart transplant

2/24/21 Pediatric Cardiology...Assist. Prof. SADIQ M. AL- HAMASH 46

CARDIOMYOPATHY (cont.):
Prognosis:
§ 80% à 1 year survival;
§ 25% of the above will be normal.

2/24/21 Pediatric Cardiology... Prof. SADIQ M. AL- HAMASH 47