Critical Congenital Heart DiseaseDr. Leong Ming Chern

Consultant Paediatric CardiologistPaediatric & Congenital Heart Centre,

IJN, Kuala Lumpur.

Outline of the talk

• What is a critical congenital heart disease?

• Why are they critical?

• Tips to detect critical heart disease.

• Management of critical heart disease in the ICU.

Introduction

• Congenital heart disease (CHD) is the most common type of birth defect, affecting approx 1% of birth.

• Critical CHD is a group of heart defects that cause serious, often life threatening symptoms.

Oster M. Paediatrics 2013; 131(5): 1502-1508

Types of heart Critical CHD

1. Ductal dependent pulmonary circulation

(Critical pulmonary stenosis, pulmonary atresia, Severe Ebsteins anomaly)

2. Ductal dependent systemic circulation

(Critical aortic stenosis,aortic atresia, coarctationof aorta, interrupted aortic arch)

3. TGA with inadequate mixing

4. Obstructed TAPVD.

Ductal dependent Pulmonary Circulation

Ebsteins

How do we suspect ductal dependent pulmonary circulation?

• Clinical cyanosed. SpO2 : stable.

• Patient is clinically stable (haemodynamically)

• Upper limb & lower limb show no difference in saturation.

CXR

Echocardiogram

Single ventricles

Single ventricles

Tips to diagnose Cyanotic CHD

• Cyanosed and stable

• Single S2 or ESM

• CXR oligaemic lung fields

Echo - tips

4 chamber view

Single ventricle

Biventricleventricle

Cyanosed

Not cyanosed

RVOT obstruction

Ebsteins & severely cyanosed

Prostin dependent

Not prostindependent

Prostin

Prostin

How to differentiate between cyanotic heart disease and PPHN?

Cyanotic heart disease PPHN

SpO2 Same upper and lower limb

Upper limb higher than lower limb

Clinical condition Stable (don’t even need intubation)

Unstable. Fluctuating BP

CXR Oligaemic Ground glass appearance

RVOT PS or not seen Unobstructed RVOT

What should we do?

• Start Prostaglandin.• Titrate the SpO2 to 75% - 85%.• <75% too little flow; >85% too much flow• Wean off oxygen if possible. Oxygen encourages

flow into the PA.

Ductal dependent Systemic Circulation

How do we suspect ductal dependent systemic circulation?

• Well. Suddenly collapsed at home.• Pale and circulatory shut down.• Apart from sepsis, IEM. Do consider CHD!• Clinical pink. SpO2 : stable after prostaglandin is started.• Immediately turned stable after prostaglandin.

• Lower limb pulses less well felt.

Treatment

• Start prostaglandin

• Titrate the prostaglandin to improved pulse volume. (upper and lower limb pulses same)

• If there is wide pulse pressure, the PDA is huge.

TGA with poor mixing.

TGA vs PPHN

Pure TGA

Saturation is stable

BP is stable

No lactate acidosis

Asc & Des Ao same SpO2

TGA with PPHN / PPHN

Unstable saturation

BP unstable

Lactate acidosis

Asc Ao low SpO2, Des Aohigh SpO2 (TGA, PPHN)

Asc Ao high SpO2, Des Aolow SpO2 (PPHN)

Stabilisation

• Start prostaglandin.

• Intubate the patient, sedate the paralyse if the upper limb saturation is low.

• Refer for BAS urgently!

Early BAS

Balloon atrial septostomy

Obstructed TAPVD

What if the ASD or the connecting vein is block?

Obstruction !

Difficult to differentiate from PPHN

• Remember, if

– Patient has no history of MAS

– Patient with PPHN and already on iNO and not improving clinically

– Pls call for echocardiogram

Strategy

• All obstructed TAPVC is an emergency.

• Needs early surgery.

• Get the patient to be stable enough to transfer the patient.

• Patient is stable refer early

• Patient is not stable intubate, introduce PEEP. Avoid iNO.

Summary

• Critical heart disease can be divided into PDA dependent and non PDA dependent.

• Patients are usually stabilised with the initiation of prostaglandin.

• Obstructed TAPVD may mimic PPHN and hence should be considered in patients who do not response to iNO.

Questions ?

Questions for participants

• A previously well 2 week old child collapsed suddenly at home.

• Brought to the hospital.

• Appears shut down with feeble pulse.

• She was resuscitated by the doctor at casualty.

• Intubated. IV inotropes started. IV antibiotics started.

• You arrived and reviewed the child. What else would you start?

• Antibiotics

• Prostaglandin

• iNO

• Echocardiogram

• Antibiotics

• Prostaglandin• iNO

• Echocardiogram