Common Cardiac Emergencies

Agustin E. Rubio, MDSibley Heart Center CardiologyChildren’s Healthcare of Atlanta

Emory School of Medicine

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Topics

• Cyanosis & Ductal Dependence• Emergency Room Diagnoses:

Tetralogy of FallotHypoplastic Left Heart SyndromeCoarctation of AortaSVT

• Shunt Dependent vs Non-shunt Dependent

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Epidemiology

Cardiac malformations • 10% of infant mortality

Incidence:• 4-6/1000 live births

Most common lethal diagnosis:• Left ventricular outflow tract obstruction

Hypoplastic left heart syndrome Coarctation of aorta Aortic stenosis

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Circulatory Transitions

Conversion from right sided (placental oxygenation) to left sided circulation (pulmonary oxygenation)

Progression is secondary:• Decreasing PVR• Closure of ductal shunts

Clinical presentations:• Cyanosis• Respiratory failure• Shock

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Cyanosis

Typically, 2 g/dL of reduced hemoglobin• 5g/dL of reduced Hb clinical cyanosis

The higher the Hb the less likely to have severe cyanosis

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Ductal Dependent Lesions

Cyanosis CHF/Shock

Rt to Lt shunting:

Tricuspid atresia

TOF/ Pulm atresia

Ebstein’s anomaly

Lt Ventricular Outflow Tract Obstruction:

HLHS

Coarctation of Aorta/ AS

Truncus arteriosus

TGA with VSD

TAPVR

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Left Ventricular Outflow Tract Obstruction

Major source of neonatal M&M from CHD • Accounts for ~ 12% of congenital cardiac

disease in infancy• ~ 75% discharged from hospital w/o

diagnosis• ~ 65% - normal newborn screen

examination• 6% died before diagnosis• 96% symptoms by 3 wks of life

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Symptoms in Real-Time

Timeline of Clinical Diagnosis

Week #1 HLHS

Coarctation of aorta

TAPVR - obstucted

Week #2-6 Transposition of Great Arteries

Total Anomalous Venous Return

Truncus arteriosus

Tetralogy of Fallot

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Tetralogy of Fallot

Prevalence: - 10% of CHD

Most common cyanotic heart defect beyond infancy

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Tetralogy of Fallot

+/- Cyanosis

Small to Nl cardiac silhouette

pulmonary vasculature

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Tetralogy of Fallot

“Tet spell”• Hyperpnea• Worsening

cyanosis• Disappearance of

murmur• RBBB pattern on

ECG

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Tetralogy of Fallot

“Tet spell”• Treatment objectives:

Reverse the right-to-left shunt systemic vascular resistance (SVR) Correct potential acidosis with NaHCO3 &

volume Consider peripheral vasoconstriction

(phenylephrine – 0.02 mg/kg IV) Ketamine

– increase SVR and sedates 2 mg/kg over 1 min Morphine sulphate Oxygen

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Tetralogy of FallotSurgical Options

Trans-annular patch

VSD closure

Blalock-Taussig shunt

Delayed repair

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Tetralogy of FallotPost-operative Concerns

• Post-pericardiotomy syndrome ~ 4 weeks post-op (25-30% of open heart pts) Fever, elevated ESR and CRP Increased work of breathing (? pericardial

effusion) Cardiomegaly, pleural effusions ECG – persistent ST segment elevation with

flat or inverted T waves in limb & left lateral limb leads

Pericardiocentesis – performed when tamponade physiology present

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Tetralogy of FallotPost-operative Concerns

• Endocarditis Dx after >2 BCx or echo evidence

• Residual VSD• Arrhythmias

AV block, ventricular arrhythmias

• Remember: Any incision in the ventricle produces a

RBBB pattern (rSR’ in V1; wide complex QRS)

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Tetralogy of FallotPost-operative Concerns

Arrhythmias• TOF - 40% increased

incidence of lethal arrhythmias

• Syncopal events- lethal ventricular arrhythmias ??

Hypoplastic Left Heart Syndrome

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HLHS

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HLHS

Uncommon form of cyanotic heart disease

Most common cause of death in the first month of life

Critically ill infant within the first 7 days with low O2 saturations

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HLHS

Clinically:• Progressive cyanosis and hypoxemia• Hx of poor feeding, tachypnea and poor

weight gain• Cardiovascular shock• Severe acidosis• Congestive heart failure

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Consequences and Complications

Polycythemia (erythrocytosis) Clubbing (>6 mos of age) Hypoxic spells CNS

• Cyanotic heart disease accounts for 5-10% of brain abscesses

• Cerebral venous thrombosis - <2 yrs, cyanotic and microcytic anemia

Dyscrasias

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HLHSPre-operative Resuscitation

Medical management:• Intubation• Ventilate and oxygen• Intravenous access

Central/ umbilical/ intra-osseos• Glucose• Na HCO3

• PGE1 (get that PDA open!!)

PGE1 0.05 mcg/kg/min

• Volume – NS/ 5% Albumin/ PRBC’s• NIRS probe

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HLHSNorwood/ Blalock-Taussig Shunt

Post-operative changes• Uncontrolled PBF

• Re-constructed aortic outflow tract

• Fluid balance sensitive

• Widened pulse pressures

• Tenuous coronary circulation

• Single ventricle for all circulation

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HLHSNorwood/ Sano shunt

Post-operative changes• Direct PA

communication with RV• Uncontrolled PBF• Neo-aortic

reconstruction• Higher diastolic

pressures• Better coronary

perfusion

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HLHSPost-Operative 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

Coarctation of Aorta

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Coarctation of Aorta

Common cause of left sided heart failure

95% located in juxtaductal region

Associated with other congenital anomalies

May be short segments or long segments

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Coarctation of Aorta

Associations:• HLHS

• Aortic stenosis

• TOF

• Truncus arteriosus

• VSD

• DORV

• Turner’s syndrome

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Coarctation of Aorta

Clinical• Poor feeding, dyspnea & poor weight gain• Upper arm vs lower extremity BP

discrepancy >10-20 mmHg systolic upper vs. lower 20-30% develop CHF by 2-3 months

• Hx of lower extremity weakness or pain after exercise

• 50% will have no murmur

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Coarctation of Aorta

Acute clinical presentation:• Cardiovascular shock

Somnolent & lethargic Poor po intake/ dehydrated, poor U/O Cold, clammy & diaphoretic Poor pulses +/- organomegaly Bradycardia/ tachycardia

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Coarctation of Aorta

Laboratory Evaluation:• CBC & ABG/VBG

• CMP, Magnesium & Phos

• Lactate

• BNP level

• CXR & 12 lead ECG

• Blood cultures

• NIRS probe

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Coarctation of Aorta

Neonatal Coarctation• rSR’ in the right precordial leads (V1 &

V2)• Deep S waves in the lateral leads• RAD

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Coarctation of Aorta

Infant Coarctation• LVH apparent (left lateral leads)• Deep S waves in the right chest• Large R waves in lateral leads

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Coarctation of AortaSurgical repairs

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Coarctation of AortaPost-operative State

Re-coarctation• Occurs most commonly within the first 12

months• Evaluated by 4 extremity BP’s• Physical examination of upper & lower

extremity pulses

Tachyarrhythmia:Sinus Tach vs. SVT

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Clinical Signs of Tachyarrhythmia

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Symptoms from History

Neonate: • Sudden onset of

irritability& sudden relief

• Poor po intake & somnolence

• Inconsolable• “Rapid heart

beat”– felt by parents

Older Child:• Stops activity

abruptly• “Palpitations”/

“feels funny”• Sudden relief with

vasovagal manuever

• Chest pain - rare

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ECG Findings

Sinus Tach

Sinus Tach

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Rhythms

SVT

Sinus Tach

Regular rhythm, narrow QRS, HR >200, p buried in T wave

Regular rhythm <200, distinct p waves, nl intervals

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Sinus Tachycardia vs. SVT

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SVT – Hemodynamically Stable

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SVT – Hemodynamically Unstable

** Cardioversion should be performed in a location which can provide for continuous monitoring and potential complications of sedation.

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Medications for SVT

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Laboratory Evaluation

Electrolytes• Calcium, Magnesium & Phosphorus

CBC with diff

CXR & 12 lead EKG

• looking for pre-excitation – WPW

Shunt Dependent vs. Non-dependent

What’s the big deal !!!

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The Difference

Shunt Dependent• The only source of PBF = SHUNT

Non-Dependent• Two sources of PBF = Shunt + some

antegrade flow through diminuitive PV

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Shunt Dependent

Oxygen therapy• Limit O2 therapy for cyanosis• Maintain sats 75-85%• Sats can drop significantly and quickly

• If sats >85%: PVR PBF Pulmonary edema

and circulatory shock

• Use blended O2 with range of up to FiO2 0.4

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Non-Dependent

Oxygen therapy• Two sources of PBF:

One with fixed obstruction and the other is uncontrolled

• If BT shunt present: Limit O2 O2 saturations should not drop as far nor as

quickly

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Summary

CHD &/or arrhythmias should be suspected neonates with cardiovascular shock

Evaluation should include:• CBC, cultures, electrolytes, lactate levels, Blood

gases• CXR, 12 Lead EKG

H&P provide 90% of diagnoses

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Medical Management

Airway, Breathing, Circulation

What disease and what was the repair?

Prostaglandins• 0.03 to 0.1 mcg/kg/min• Side effects:

Hyperpyrexia Apnea Flushing

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Miscellaneous

What information do we require?• 4 extremity BP’s, weight %iles

• H&P Murmurs Organomegaly Pulses ECG Labs, CXR findings, saturations

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Sources

Internet websites:• www.childrenshospital.org• www.cincinattichildrens.org• www.ucsfhealth.org/childrens/

Pediatric Cardiology for the Practioners. MK Park 4th ed.

Congenital Heart Disease - Moss and Adams