Paediatric Cardiology Flashcards
Give 4 CYANOTIC congenital heart defects
- Tetralogy of Fallot
- Transposition of the Great Arteries
- Tricuspid Atresia
- Truncus Arteriosus
Give 4 ACYANOTIC congenital heart defects
- Atrial Septal Defect
- Ventricular Septal Defect
- Patent Ductus Arteriosus
- Coarctation of the Aorta
When does TOF normally present?
Usually around 1-2 months. It is the most common CHD except at birth which is TGA.
What are the 4 cardiac defects involved in ToF?
- Pulmonary Stenosis (leads to) (functional due to overriding aorta)
- Right ventricular hypertrophy
- Ventricular Septal Defect
- Overriding aorta (onto VSD)
What is the direction of the shunt in ToF and why?
- Right to left
- Pressure in RV > LV
- Due to RV hypertrophy
- Results in mixing of oxygenated and deoxygenated blood = cyanosis
What are the cardiac presentations of ToF? (5)
- Ejection systolic murmur @ upper left sternal border
- Cyanosis +/- tet spells
- Breathing difficulty
- Clubbing at 1-2 months
- Polycythaemia? Chronic hypoxia
What is a tet spell?
- Period of cyanosis upon exertion or anxiety etc
- sudden marked increase in cyanosis followed by syncope
- child may instinctively squat to ↑peripheral vascular resistance (↑pressure in femoral arteries) = ↑ pressure in LV to reverse the shunt
Give 2 other, non-cardiac features of ToF
- Difficulty feeding
- Not growing properly/failure to thrive
What are the investigations for ToF?
- Echocardiogram + Doppler
- CXR - has a boot shaped heart
- Screen for DiGeorge Syndrome as conditions are often associated
What are 4 risk factors for ToF?
Non-modifable
- Maternal age >40
Modifiable
- Maternal alcohol use during pregnancy
- Maternal Diabetes
- Maternal Rubella infection during pregnancy
What is transposition of the great arteries?
- Pulmonary Artery and Aorta switch
- Aorta now onto RIGHT VENTRICLE via pulmonary valve
- Pulmonary artery now onto LEFT VENTRICLE via aortic valve
How is TGA compatible with life?
Another heart defect, usually a VSD (or ASD or PDA) is present so that oxygenated and deoxygenated blood can mix
How does TGA usually present?
- At birth
- Initially ok but then presents as cyanosis that may rapidly deteriorate as the PDA closes.
Give a risk factor for TGA
Maternal Diabetes
What is the immediate and long term management of TGA?
- Immediate = give prostaglandins as will keep the ductus arteriosus open
- Cardiac Repair Surgery
- Arterial Switch
May lead to:
Arrhythmias
Right ventricular hypertrophy
Tricupsid Regurgitation
What is tricuspid atresia?
Tricuspid valve is underdeveloped so doesn’t open/ is just some tissue.
Right Ventricle becomes hypoplastic too
RV is dysfunctional = ↓ blood to lungs
How is tricuspid atresia compatible with life?
- An atrial septal defect AND a ventricular septal defect are both needed for compatibility with life:
- ASD so that mixed blood can get into the LA to LV and onto the body
- VSD so that blood can get from LV to RV and into the lungs
What are the cardiac presentations of tricuspid atresia?
Progressive cyanosis
Pansystolic murmur in mitral area (valve closed when LV contracts = turbulent flow)
Left Ventricular Hypertrophy (palpable apex beat?)
Tachypnoea for first 2 weeks of life
What is the murmur associated with tricuspid atresia?
Pansystolic murmur in mitral area (valve closed when LV contracts = turbulent flow)
What is the treatment for tricuspid atresia?
Treatment
Prostaglandins to keep PDA open
Cardiac repair surgery
What is a ventricular septal defect?
There is a hole in the ventricular septum (usually the membranous part) allowing blood to flow from LEFT to RIGHT.
Forms a LEFT to RIGHT shunt as pressure is higher on the LEFT
What could be a complication of a VSD?
↑ blood flow to lungs may lead to pulmonary hypertension and therefore pulmonary oedema etc. AND may reverse the shunt (Eisenmengers Syndrome)
Which conditions are associated with a VSD?
- Foetal alcohol syndrome
- Down’s Syndrome
What are the symptoms of a VSD?
- Often asymptomatic but depends on severity
- Often ↓growth/ not growing as well as they should
- If more severe then symptoms of heart failure
What is the murmur associated with a VSD?
- Pansystolic murmur at lower left sternal edge
- Thrill here
What are the investigations for CHD?
Echocardiogram + doppler is diagnostic
What is the treatment for a VSD?
- Cardiac Repair Surgery between 3-6 months
- ↑ calorie intake (growth bit)
- Diuretics if HF
What is an atrial septal defect?
What are the two types?
- There is a gap in the septum between the atria
- 2 kinds:
- Secundum (more common) - involved Foramen Ovale
- Atrioventricular Septal Defect - communication between atria at bottom of septum + abnormal valves - Blood is shunted from LEFT to RIGHT
Is an ASD always pathological?
No
Opening within the septum is physiological in utero so that lungs can be bypassed
Oxygenated blood is received from the placenta via the umbilical vein →RA →LA →LV →Body
Foramen Ovale should close at birth
Which conditions are associated with ASDs?
- Foetal Alcohol Syndrome
- Down’s Syndrome
What is a potential complication of an ASD?
- Paradoxical embolism
- Embolism returns to R heart and moves into L
- Can potentially travel to the brain and cause a stroke etc
What is the murmur associated with an ASD?
- Ejection Systolic murmur at upper left sternal border
- Partial AVSD may also have a pansystolic murmur at the apex
- S2 heart sound splits
What are the symptoms of an ASD?
Most often is asymptomatic but can have recurrent chest infections
What are the treatment options for an ASD?
Occlusion Device
Cardiac Repair Surgery
What is the function of the ductus arteriosus?
Connects the pulmonary artery to the descending aorta in utero
Normally closes after birth if born at term
Blood can flow from aorta into pulmonary artery and to lungs so ↑ pulmonary blood flow
When is the ductus arteriosus counted as PATENT?
If it hasn’t closed one week after the estimated date of delivery
What are the symptoms of a PDA?
often asymptomatic but may have symptoms of RIGHT heart failure
What is the murmur associated with a PDA?
Continuous machinery murmur under the LEFT clavicle
Bounding pulse
What is the role of echo + doppler in PDA?
- Diagnosis
- Rules out duct dependent circulation before closure of the defect
How can a PDA be closed?
- Ibuprofen or endovascular surgery before 1 year old
Why is a PDA therapeutically closed?
- Close the duct to ↓risk of bacterial endocarditis/pulmonary vascular disease in later life
What is Ebstein’s Anomaly?
CHD due to the use of Lithium during pregnancy
What is the pathophysiology of Ebstein’s Anomaly?
Posterior leaflets of the tricuspid valve become anteriorly displaces towards the apex
causes tricuspid regurgitation AND tricuspid stenosis
AND right atrium enlargement
What are the murmurs associated with Ebstein’s anomaly?
- Pansystolic @ upper left sternal edge (tricuspid regurgitation)
- mid diastolic (tricuspid stenosis)
