E A R LY D E T E C T I O N A N D M A N A G E M E N T

C R I T I C A L C O N G E N I TA L
HEART DISEASE
W H AT W E W I L L D I S C U S S T O D AY

• What is critical congenital heart disease (CCHD)

• When to suspect CCHD (duct-dependent lesions)

• How not to miss a CCHD?

• How to early manage CCHD?

 “Any potentially life-threatening duct-dependent
disorder from which infants die or undergo invasive
procedures (surgery or cardiac catheterisation) in
the first 28 days of life.”

– C R I T I C A L C O N G E N I TA L H E A R T D I S E A S E

Lancet 2011; 378: 749–50.

S O M E S TAT I S T I C S

• 1/100 baby is born with congenital heart disease

(CHD), in which one fourth are CCHD

• Indonesia: 5 million infants are born each year

• 12,500 babies are born with CCHD

J Am Coll Cardiol. 2011;58(21):2241-2247.

“Time course of the reported total CHD birth
prevalence”

–J AM COLL CARDIOL. 2011;58(21):2241-2247.

“Reported total CHD birth prevalence per
continent”

–J AM COLL CARDIOL. 2011;58(21):2241-2247.

TYPES OF CCHD

• The most critical CHD are those not compatible with

life — duct dependent lesions

• Less severe CCHD: tetralogy of Fallot, truncus

arteriosus, etc
“In foetus, most venous blood is shunted through
PFO and PDA.”

– C I R C U L AT O R Y C H A N G E S A F T E R B I R T H
 Duct-dependent lesions

Duct dependent Duct dependent

systemic circulation pulmonary circulation

Critical problems in right

Critical problems in
heart structures or
left heart structures
abnormal VA connection

Hypoplastic left heart syndrome Pulmonary atresia (PA)

Aortic atresia Critical pulmonary stenosis
Coarctation of the aorta Tricuspid atresia with PA/PS
Transposition of great arteries
“When to suspect CCHD?”
 Duct-dependent lesions

Duct dependent Duct dependent

systemic circulation pulmonary circulation

Circulatory collapse
Cyanosis/oxygen
(Septic-like) shock
desaturation
Perfusion disturbance
(‘happy tachypnoea)
Weak femoral pulse

…in previously well neonates who were mostly

vigorous at birth
ADDITIONAL SIGNS OF CCHD

• Heart murmurs

• Abnormal chest x-ray (cardiomegaly/abnormal cardiac

contour)

• ECG abnormality
“How not to miss a CCHD?”
Lancet May 2, 2012.
P U L S E O X I M E T R Y I N T E R P R E TAT I O N

• Refer for cardiology evaluation if SaO2 is consistently

below 95%
T H E M A I N C C H D S C R E E N I N G TA R G E T S

• Pulmonary atresia

• Transposition of the great arteries

• Hypoplastic left heart syndrome

• Tetralogy of Fallot

• Total anomalous pulmonary venous return (TAPVR)

• Tricuspid atresia

• Truncus arteriosus
“How to early manage CCHD?”
E A R LY M A N A G E M E N T O F C C H D

• Keep the duct open to maintain pulmonary or

systemic circulation

• Perform balloon atrial septostomy if mixing at atrial

level is desired

• General management of neonates

HOW TO KEEP THE DUCT OPEN?

• Medications:

• IV prostaglandin E1: (10-50 ng/kg/min)

• ?Oral prostaglandin: 15-65 microgram/kg/min

• PDA stent placement

 Neonate with cyanosis

Peripheral cyanosis Central cyanosis

Prostaglandin E1

Cardiology consultation
CONCLUSIONS

• CCHD is a prevalent congenital lesion

• CCHD typically presents with cyanosis or circulatory

collapse in previously well neonates

• Pulse oximetry screening should be encourage to

detect CCHD

• Prostaglandin is the live saving drug for CCHD

“Thank you”