Cardio Flashcards
What is the hyperoxia / nitrogen wash out test?
Used to differentiate cardiac from non-cardiac causes of cyanosis
Process:
- Infant is given 100% O2 for 10 minutes
- ABG is taken
- A pO2 of <15 kPa = cyanotic congenital heart disease
Is cyanosis always pathological?
Peripheral cyanosis is very common in the first 24 hours of life and may occur when the child is crying or unwell from any cause
What type of CHD is tricuspid atresia?
RIGHT-TO-LEFT SHUNT
CYANOTIC HEART DISEASE
What is tricuspid atresia?
Absent tricuspid valve
- No connection between R atrium and R ventricle
- Small, non-functional RV (LAD)
- Only L ventricle effective
- Commonly have ASD + VSD
What are the symptoms of tricuspid atresia?
- Central cyanosis (presents very early ~10mins)
- SOB
What are the signs O/E of tricuspid atresia?
Murmur depends on associated abnormalities:
- Pan systolic - VSD
- ESM at left sternal edge - pulmonary stenosis
- Continuous murmur - PDA
What is the management of tricuspid atresia?
1. ASAP PGE1 infusion
2. Surgery <2 weeks
(complete corrective surgery not possible in most cases bcos only one functioning ventricle)
1. Glenn operation (connect SVC to pulmonary artery)
2. Fontan operation (connect IVC to pulmonary artery)
What type of CHD is TGA?
RIGHT-TO-LEFT SHUNT
CYANOTIC HEART DISEASE
What is TGA?
Anatomical reversal of the aorta and pulmonary artery
2 closed-loop systems (PA sends oxygenated blood to lungs and A sends deoxygenated blood to body)
Usually fatal immediately
What is a RF for TGA?
Maternal diabetes
What are the symptoms of TGA?
Postnatal cyanosis within a few hours (at rest)
Unaffected by supplemental O2
Tachypnoea
What are the signs of TGA?
- Single loud second heart sound (S2)
- Prominent R ventricular impulse
- No murmur (ES murmur if VSD + pulmonary stenosis)
What are the investigations for TGA?
CXR
- Egg-on-side / egg on a string (narrow upper mediastinum)
- Increased pulmonary markings
Echo
What is the management of TGA?
- ASAP prostaglandin infusion e.g. alprostadil (maintain PDA)
- Balloon atrial septoplasty (tears atrial septum down to allow mixing)
- Arterial switch surgery (within 2wks)
What type of CHD is AVSD?
RIGHT-TO-LEFT SHUNT
CYANOTIC HEART DISEASE
What is AVSD?
Single large defect connecting all 4 chambers (all blood mixes)
What is AVSD commonly associated with?
Down Syndrome
What are the S/S of AVSD?
Cyanosis at 2-3w of life
No murmur
Found on routine echo of Down’s
What is the management of AVSD?
- Treat HF medically
- Surgery at 3m
What type of CHD is ToF?
RIGHT-TO-LEFT SHUNT
CYANOTIC HEART DISEASE
Most common cause of cyanotic heart disease
What is the pathophysiology of ToF?
Simultaneous occurrence of 4 defects
- VSD (large)
- Overriding aorta
- R ventricular outflow tract obstruction (pulmonary stenosis)
- R ventricular hypertrophy
What are the RFs for ToF?
Maternal diabetes
Maternal alcohol consumption
What syndromes are associated with ToF?
DiGeorge Syndrome
Down Syndrome
What are the symptoms of ToF?
Mild to severe cyanosis (depending on RVOTO extent)
Tet spells (crying > increased pulmonary resistance > R-L shunt > cyanosis)
Squatting relieves symptoms
Clubbing
What murmur is heard in ToF?
Loud ESM at LUSB
(from pulmonary stenosis)
What are the investigations for ToF?
CXR = large boot-shaped (due to RVH)
ECG = RVH / right axis deviation
Echo
What is the management of ToF?
Mild cyanosis (<15 mins) = self-limiting
Initial Management
1. Knee-chest position and high-flow O2
2. ASAP Prostaglandin e.g. alprostadil
If prolonged cyanosis:
3. Morphine (sedation and pain relief)
4. IV propranolol (peripheral vasoconstriction and relive sub-pulmonary muscle contraction)
5. IV fluids and bicarbonate (severe acidosis)
6. Muscle paralysis and artificial ventilation to reduce metabolic O2 demand
Temporary Repairs:
1. Shunt
2. Balloon dilation / stent
Complete Repair:
Surgery 6m later (within 1st year)
What type of CHD is Ebstein’s Anomaly?
RIGHT-TO-LEFT SHUNT
CYANOTIC HEART DISEASE
What is Ebstein’s Anomaly?
Tricuspid Valve is displaced downwards
= small and hypocontractile RV
What is a RF for Ebstein’s Anomaly?
Associated with in-utero lithium exposure (maternal BPD)
What are the S/S of Ebstein’s Anomaly?
(Presentation depends on the degree of displacement)
Severe = in-utero hydrops fetalis
Mild = cyanosis
What murmur is heard in Ebstein’s Anomaly?
Pansystolic (tricuspid regurg)
Mid-diastolic (tricuspid stenosis)
What are the investigations for Ebstein’s Anomaly?
Echo = confirmatory
CXR = cardiomegaly (RA enlargement)
What is the management for Ebstein’s Anomaly?
Surgery
What type of CHD is ASD?
LEFT-TO-RIGHT SHUNT
ACYANOTIC HEART DISEASE
What are the 2 types of ASD?
Secundum (80%)
- Defect in middle of atrial septum (foramen ovale does not close)
- Usually small
- Associated with Holt-Oram syndrome (tri-phalangeal thumbs)
Partial/Primum (20%)
- Defect of AV septum (lower)
- Usually large / presents earlier
- Associated with abnormal AV valves
What are the RFs for ASD?
Down Syndrome
Maternal alcohol consumption
What are the symptoms of ASD?
Usually asymptomatic (until Eisenmenger Syndrome)
- Shortness of breath, especially when exercising
- Fatigue
- Oedema
- Arrhythmias (from 40+)
- Palpitations
- Recurrent chest infections / wheeze
What are the signs of ASD?
- Ejection systolic murmur (loudest at LUSE)
- Fixed wide splitting of S2
Not until a few weeks old
What are the investigations for ASD?
ECG
Secundum = RBBB with RAD
Partial = RBBB with LAD, prolonged PR interval, ‘superior’ QRS axis
Echo = diagnostic
CXR
What is the management of ASD?
Usually at 3yrs
Small = heal spontaneously (monitor)
Secundum = cardiac catheterisation and insertion of occlusive device (percutaneous/endovascular closure)
Partial = surgical correction