Cyanotic Heart Disease
- Transposition of the great vessels
- Tetralogy of Fallot
- Total anomalous pulmonary venous return
- Truncus arteriosis
- Tricuspid atresia
- Severe pulmonary stenosis
Duct-Dependent lesions
Poor PULMONARY flow requires PDA to preserve blood flow from Aorta to pulmonary circulation
- Critical pulmonary stenosis
- Transposition of great vessels
- Hypoplastic Right Heart Syndrome
- (TOF)
- (Tricuspid atresia)
Presentation
- cyanosis
- Inc RR without resp distress
- Adequate perfusion - initial
Poor SYSTEMIC flow requires PDA to preserve blood flow from PA to systemic circulation
- Coarctation of aorta
- Interrupted aortic arch
- Hypoplastic left heart syndrome
- Critical aortic stenosis
Presentation
- Cardiac failure with systemic hypoperfusion
- Poor or absent peripheral pulses
- Inc metabolic acidosis
- Cyanosis may not dvp until later
AIMS of Mx
- Minimise pulmonary blood flow
- Mod PEEP
- Ventilate in air if possible
- CO2 37-45mmHg
- Sats 75-85%
- Maximise tissue perfusion
- Fluid (10mL/kg boluses) if shocked
- Low dose inotropes - NA 0.1mcg/kg/min
- NaHCO3 if BE >-10
- Consider intubation, muscle relaxants and sedation if distressed
- PGE1 0.05-0.1mcg/kg/min for ductal patency
Cyanotic Heart Dx Table
Neonatal cyanosis
- Congenital Heart disease
- Sepsis
- Respiratory disorders
- Heamoglobinopathies
Tetralogy of Fallot
- Ventricular Septal defect
- RVH
- Overriding aorta
- Pulmonary stenosis
Functional result => shunt from RV to aorta and small pulmonary artery and oligaemic lungs
TET Spells
Baseline depends on degree of Pulmonary stenosis
Mild = normal pulmonary flow
Mod-severe = significant outflow obstruction = > hypoxia
O2 sats range from 70-100% depending on pulmonary stenosis
Causes:
Sepsis, anaphylaxis, hypoxia, tachycardia, hyperventilation
Physiology:
Hypoxia => dec SVR => dec afterload => inc R→ L shunt through the VSD => worsening hypoxia
Dec pO2 / inc pCO2 / dec pH => increased Pulmonary Vascular resistance => inc venous return
Inc venous return => more shunting
Management of cyanotic spells:
- Increase SVR OR
- Dec VR
- Dec Pulmonary VR
- Oxygen
- Calm pt
- Knees to chest
- Morphine 10-50mcg/kg OR fentanyl 1mcg/kg
- Volume expansion: 10 ml/kg IV colloid/crystalloid
- Vasopressor: metaraminol 5-10 mcg/kg IV or phenylephrine 5-10 mcg/kg, then 1-5 mcg/kg/min IV/(SC)
- Esmolol 100-500 mcg/kg (titratable) +/- infusion
ECGs - Paeds
Rate - HR faster
Axis - RV predominance
- RAD
- Tall RV1
- TWI right precordial leads
Intervals - tend to be short
- PR normalises at adolescence
- QRS<80ms before 8yrs
- QT may be up to 490ms before 6/12
Pseudo-ischaemia/infarction
- Small narrow Q waves inf and lateral leads
- TWI
- V1-3
- Juvenile T-wave abN
- Upright Ts in V1 - RVH
Eisenmenger Syndrome
- L-R shunt into R-L shunt
- Irreversible PHTN
Occurs with
- Large VSD
- Ostium primum defect (lower septum - malformation of AV valve annulus)
- Transposition of great vessels with large shunt
Presentation
Cyanosis, CP, SOBOE, haemoptysis
Hyperviscosity, Paradoxical emboli, brain abscess
Mx
IV prostaglandin may reduce Pulmonary vasc pressure
Supportive
- O2
- Maintain volume
- Phlebotomy for hyperviscosity
Ebstein’s Anomaly
Septal leaflets of Tricuspid displaced into RV
80% ASD
Range from asymptomatic to severe symptoms
Assessment
Cyanosis proportional to degree of R-L shunt
TR - systolic murmur lower left sternal edge
ECG
P pulmonale, RBBB, 1HBm accessory pathway 20%
CXR
cardiomegaly, RA enlarged, dec pulmonary vascular markings
Mx
Endocarditis prophylaxis
Rx HF
Surgery
- Temporary shunt from systemic to pulmonary cir to inc pulmonary blood flow
- Fontan
- Repair tricuspid + ASD
Transposition Great Arteries
Not compatible with life
Large VSD or PDA decreases severity of symptoms
Cyanosis hours after birth
CXR - egg on string
Increased cardiac silhouette
Pulmonary artery and aorta lie in front of each other
Mx
Urgent balloon atrial septoplasty - create large ASD
Arterial switch
Fontan Procedure
Create univentricular heart so blood from IVC/SVC diverted from RA to pulmonary arteries
Indications
Single functional ventricle
Tricuspid or mitral atresia
Hypoplastic heart
CI in children with high Pulmonary vascular resistance
Blalog-Tausig shunt
Shunt between branch of aorta and pulmonary artery
O2 sats between 70-85% optimal
Incomplete Kawasaki Disease
Heart Disease
