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CONGENITAL HEART EMERGENCIES: KEEPING IT

SIMPLEHelena Wang-Flores, DO, MHSA

v

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vObjectives

• Focus on hypoplastic left heart

• Overview of the 3 stage palliation surgery

• Understand the complications that can

occur at each stage and how to manage

vCase 1

EMERGENCY MEDICINE

• 5 month old CC: Cyanosis

• HR: 110, RR: 42, BP:

84/Doppler, SpO2: 60%, Temp:

36.7 C

• HLHS, s/p BT shunt with LPA

augmentation and PDA ligation

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vBut what does that mean?

EMERGENCY MEDICINE

vBreaking it all down

• Where do they get their pulmonary blood

flow?

• Where do they get their systemic blood

flow?

• 1V or 2V?

EMERGENCY MEDICINE

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vHypoplastic Left Heart Syndrome: HLHS

• 960 babies each year

• Unknown cause

• The left ventricle is

underdeveloped and too small.

• The mitral valves is not formed

or is very small.

• The aortic valve is not formed

or is very small.

• The ascending portion of

the aorta is underdeveloped or

is too small.

• May have ASD.

vHypoplastic Left Heart Syndrome (HLHS)

EMERGENCY MEDICINE

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v3 stage palliation surgery

1. Norwood

2. Bidirectional Glenn

3. Fontan

vNorwood Procedure/ BT Shunt

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vNorwood (BT Shunt )– sats 75-85%

Post-operative changes• Uncontrolled PBF• Re-constructed aortic

outflow tract• Fluid balance sensitive• Widened pulse pressures• Tenuous coronary

circulation• Single ventricle for all

circulation

vNorwood (Sano Shunt)

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vNorwood (Sano Shunt)

▪ Post-operative changes

– Direct PA

communication with RV

– Uncontrolled PBF

– Neo-aortic

reconstruction

– Higher diastolic

pressures

– Better coronary

perfusion

vCase 1: Exam

• Cyanotic, no acute distress.

• Comfortable work of breathing,

good air entry bilaterally.

• Right ventricular heave. Irregular

rhythm with a normal S1 and

single S2. There is a grade I/VI

continuous murmur at the LUSB

• Abdomen is soft, nontender, and

non-distended. The liver is

palpated 1.5 cm below the right

costal margin.

• No clubbing or edema. Warm and

well perfused with 3+ pulses.

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vED Presentation

vDifferential Diagnosis

• Respiratory infection

– Low PVO2

• Sepsis

– Low SVR

– Low SVO2

• Increased demand

• Shunt stenosis

• Anemia

EMERGENCY MEDICINE

SVO2

O2 sat

PVO2

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vHypoxic

• Look for underlying cause

o Pulmonary edema or infiltrate

oDecreased pulmonary blood flow

oMixed venous desaturation

vChest X-ray

Pulmonary vascular congestion Decreased pulmonary blood flow

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vWhat’s happening?

• BT Shunt narrowing

• At risk for shunt occlusion

• Can occur at any time, though more at risk when

older and outgrowing shunt

EMERGENCY MEDICINE

vWhat to do for Shunt Failure?

• Impending cardiac emergency, call on

arrival

• Oxygen

– Goal oxygen saturations >70%

– Supplement oxygen to assist in

maintaining oxygen saturation

• Heparin may be considered

EMERGENCY MEDICINE

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vWhat about oxygen therapy?

• Limit O2 therapy for cyanosis

• Maintain sats 75-85%

– Use blended O2 with range of up to FiO2 0.4

• If sats >85% - O2 is vasodilator

– PVR PBF Pulmonary

edema and circulatory shock

vWhat to do for resuscitation?

▪ Limit oxygen (remember: relative uncontrolled

PBF)

▪ Hemoglobin

▪ Auscultate for murmur:

– Continuous murmur at RUSB (? BT shunt)

– Systolic murmur at RLSB/ LUSB (Sano shunt)

▪ Fluid balance:

– Palpate liver

– +/- rales and CXR to evaluate for CHF

– Reverse dehydration

▪ Reverse acidosis

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v

• PVR affects flow in the BT shunt

• Forward flow in both systole and diastole = continuous murmur

• Low PVR results in more flow through the shunt rather than to the body AND CORONARIES

• Higher alveolar pO2 = less flow to body

• ALWAYS TALK TO A CARDIOLOGIST BEFORE OXYGEN ADMINISTRATION TO A CHILD WITH A BT SHUNT

• PVR does not affect flow

through a RV to PA shunt

(aka Sano).

• Lower baseline sats but less

volatile cardiac output &

coronary flow

– 2007 multi center trial

with improved survival to

12 months

– Fewer “death” spells

• Still talk to a cardiologist

about oxygen

BT Shunt Sano Shunt

vStage 2: Bidirectional Glenn

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vStage 2: Hemi Fontan

vCase 2

• 10 year old CC:

Diarrhea, fever,

abdominal pain

• HR: 110, RR: 24,

BP: 95/52, SpO2:

96%, Temp: 37.9 C

• HLHS s/p Fontan

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vStage 3: Fontan

Hemi Fontan Fontan

vCase 2:

EMERGENCY MEDICINE

• HEENT: Mucous membranes

are dry. No tonsillar exudate.

Oropharynx is clear. No

adenopathy.

• Cardiovascular: Normal rate,

regular rhythm and S1 normal.

S2 single. No murmur.

• Pulmonary/Chest: No

respiratory distress. Good air

entry.

• Abdominal: Full and soft. He

exhibits no distension. Bowel

sounds are increased. There

is tenderness.

• Skin: Skin is warm. Capillary

refill takes 3 to 5 seconds.

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vChest X-ray

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No infiltratesSternotomy wiresPacer

vLabs

EMERGENCY MEDICINE

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vFontan Post-op

• Pulmonary blood flow is

passive

• Cyanosis

– Increase in PVR

– Decrease in SVR

• Dehydration

– Non fenestrated:

decrease perfusion

– Fenestrated: more

cyanotic due to

shunting

vFontan Other Considerations

• Fluid resuscitation

• Anasarca due to protein loosing

eneteropathy

• Increase risk of arrhythmias and

thromboembolic events.

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vA dry Fontain is a dead Fontan

– Be worried when Fontan’s are at risk for

dehydration

– When dehydrated, boluses of 20mL/kg and

repeated are ok

EMERGENCY MEDICINE

vWhat information does cardiology require?

• 4 extremity BP’s, weight %iles

• H&P

Murmurs

Organomegaly

Pulses

ECG

Labs, CXR findings, saturations

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vTake home pearls

• Oxygen can kill

• A dry Fontan is a dead Fontan

vHow do you reach Peds Cardiology?

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vAcknowledgements

• Stacey Noel, MD

• Courtney Strohacker, MD