General Approach to

Cyanotic Spell
in Pediatric with
Congenital Heart
Disease
Noormanto
Department of Pediatric, Faculty of
Medicine, Universitas Gadjah Mada/
Sardjito General Hospital Yogyakarta
OUTLINES
Anatomy physiology of the Heart
Cyanosis and non-cyanosis CHD
Tetralogy of Fallot
Hypercyanotic spell
ANATOMY PHYSIOLOGY OF THE
HEART
 110/70
>95%

22/14
70%

8
>95%

6
70% 110/8
>95%

22/6
70%
CYANOTIC AND NON-CYANOTIC
CHD
Definition of cyanosis
 Cyanosis is a bluish discoloration of the skin and mucous
membrane
 Resulting an increased concentration of reduction
hemoglobin to about 5 gram% in the cutaneous veins
Central and peripheral cyanosis
 DIFFERENCES BETWEEN CENTRAL
AND PERIPHERAL CYANOSIS
Points Central Peripheral
Mechanism Diminished arterial SO2 Diminished flow of blood
the local part
Sites of cyanosis Skin and mucous On skin only (localized)
membrane (generalized)
Temperature of the limb warm Cold

Local heat Cyanosis remain Cyanosis abolished

Oxygen Cyanosis decreased Cyanosis persists
Tongue Always involved Never involved
Clubbing and polycytemia Usually associated Not associated
Type of cyanosis

Central cyanosis
Peripheral cyanosis
 CAUSES OF CENTRAL CYANOSIS

Shunt anomaly in heart (right to left) Defect in oxygenation of blood in lungs

Tetralogy of Fallot Fibrosis alveolus

Transposition of the great arteries Severe pneumonia
Truncus arteriosus COPD
Total anomalous pulmonary venous Severe bronchial asthma
drainage
Tricuspid atresia Pulmonary infarction
Ebstein anomaly AV fistula
 Cyanotic and non-cyanotic CHD
Non cyanotic CHD
Cyanotic CHD
TETRALOGY OF FALLOT

1. Pulmonary
stenosis
2. VSD
3. Overriding of aorta
4. RVH
CLINICAL SIGN
Cyanosis
Dyspnea on exertion
Squatting
History of hyper-cyanotic spell
Clubbng finger
Single 2nd heart sound
Ejection systolic murmur audible at birth
ECG

 RAD
 RVH
Concave pulmonary sgment  Boot shaped appearance
Pulmonary vascular markings are decreased (oligemic)
PATHOPHYSIOLOGY

Pulmonary stenosis  fixed resistant  infundibular stenosis

 relative non reactive
Decrease SVR  increase R-L shunt, decrease PBF 
increase in cyanotic

PA AO
SVR

RV LV
SpO2 >95% SpO2 <95%

110/8
110/8 SpO2 >95%
SpO2>95%
110/6
SpO2 70%

24/6
 HYPERCYANOTIC SPELL
Transient episodes of severely increased
cyanosis in patients with tetralogy of
Fallot
Hypercyanotic spell also called the hypoxic
spell, cyanotic spell, tet spell
Lethal
Unpredictable episode
CAUSES OF HYPERCYANOTIC
SPELL
Hypovolemic
Excessive tachycardia
Crying
Defecation
Increased physical activity
MANAGEMENT
Knee chest position  increases SVR
Morphine 0,2 mg/kgBW im/iv suppresses the
respiratory centre  abolishes hyperpnea
Sodium bicarbonate 1 meq/kgBW 10-15 minute
Oxygen  little effect
 Become less cyanotic, the heart murmur
become lauder  indicated increased amount of
blood flow flowing through RVOT
If the hypoxic spells do not fully respond  following
medication can be treated
Ketamine 1-3 mg/kgBW iv over 60 second  increases SVR
and sedation
Propranolol 0.01-0.25 mg/kgBW slow iv push  reduce the
heart rate and spasm of infundibular, stabilize vascular
reactivity of the systemic arteries
 CRYING INCREASED O2 DEMAND
ETC
Propranolol Sodium bicarbonat

PO2, PCO2, pH
Morphine
SPASM RVOT
GREATER RIHGT-TO- HYPERPNEA
LEFT SHUNTING

SVR

Ketamine
INCREASED SYSTEMIC DECREASED
VENOUS RETURN INTRATHORACIC
PRESSURE

Knee chest
position
Palliative
Blalock Taussig shunt
Definitive
Tetralogy of Fallot repair repair