Congenital Heart Disease Flashcards
List the 5 acyanotic heart diseases
Ventricular septal defect (most common)
Atrial septal defect
Patent ductus arteriosus
Coarctation of aorta
Aortic valve stenosis
List the 3 cyanotic heart diseases
Tetralogy of fallot (most common)
Transposition of the great arteries
Tricuspid atresia
Which cyanotic heart disease tends to present first?
TGA presents at birth
ToF presents at 1-2 months
Define congenital heart disease.
Heart disease present at birth caused by a malformation.
Explain the aetiology of left-to-right shunts (“Acyanotic”) congenital heart disease.
- Atrial septal defects: Secundum ASD; Partial AVSD
- Atria-ventricular septal defect
- Ventricular septal defects
Blood flows from L to R as pressure is greater on LHS. Thus less oxygenated blood flows around the body. More blood flows through the pulmonary artery which can lead to increased resistance + ultimately pulmonary stenosis.
- PDA: blood flows from aorta into the pulmonary artery as pressure is greater in the aorta. Similar consequences as in ASDs, VSDs, + AVSDs
What are the 4 components of ToF?
Pulmonary valve stenosis
RV hypertrophy
VSD
Over-riding aorta
What is tricuspid atresia?
Lack of tricuspid valve, blood can’t flow from RA to RV
Blood pushed through ASD to LA, through mitral valve to LV, some through VSD to RV + lungs, some to body (deoxygenated)
Explain the aetiology of right-to-left shunts (“Cyanotic”) congenital heart disease.
Blood is flowing from R to L– effectively bypassing the lungs.
What is true ToGA? What is the consequence of this?
Pulmonary artery connected to LV + aorta to RV
which means no oxygenation at all= fatal
Thus ToGA must also present with a VSD to be compatible with life.
Explain the aetiology of obstructive congenital heart disease.
- Coarctation of aorta
- Arterial duct tissue encircling the aorta at the point of insertion of the duct. When the PDA closes so does the artery.
- Pulmonary stenosis : Fusing of the pulmonary artery valve leaflets.
Summarise the epidemiology of congenital heart disease.
1% of live births.
TOF is the most common cyanotic disorder.
What are the presenting symptoms for left-to-right congenital heart disease?
None, except for large L-to-R congenital heart disease which presents with heart failure.
What are the signs for secundum ASD left-to-right congenital heart disease?
ESM at ULSE
Fixed wide split S2
What are the signs for partial AVSD left-to-right congenital heart disease?
ESM at ULSE
Fixed split S2
Pansystolic murmur at apex
What are signs of VSD?
Pansystolic/ early systolic murmur at LLSE
What are 5 signs of an AVSD?
Hyperactive precordium
Pansystolic murmur
Systolic thrill
Tachypnoea
Hepatomegaly
What are 5 signs for PDA left-to-right congenital heart disease?
Continuous “machinery” mumur at ULSE + subclavicular
Bounding pulses
Left subclavicular thrill
Wide pulse pressure
Heaving apex beat
What are 5 signs and symptoms of Tetralogy of Fallot right-to-left congenital heart disease?
Loud ESM at 2nd ICS, ULSE
Clubbing of fingers + toes (older)
Hypercyanotic spells
“Boot shaped” heart of CXR
RV Hypertrophy of ECG
What are the signs and symptoms of transposition of the great arteries right-to-left congenital heart disease?
Neonatal cyanosis
No murmur
Tachypnoea
Loud single S2
Prominent RV impulse
“Egg on side” on CXR
What are 4 signs and symptoms of Eisenmenger’s syndrome right-to-left congenital heart disease?
No murmur
Right heart failure (late)
Cyanosis
Clubbing
What are the signs and symptoms of pulmonary stenosis obstructive congenital heart disease?
Asymptomatic
ESM at ULSE, +/- thrill.
Left parasternal heave.
Cyanosis (if a/w septal defect)
What are 4 signs and symptoms of duct-dependent coarctation of aorta obstructive congenital heart disease?
Acute circulatory collapse 2 days of age when duct closes.
Severe heart failure signs.
Absent femoral pulses.
Severe metabolic acidosis.
What are some appropriate investigations for congenital heart disease?
- Bloods
- ECG: May show axis deviation, ventricular hypertrophy.
- Echocardiography
- CXR
- Nitrogen washout test- To diagnose R-to-L shunts.
- Patient is placed on a ventilator with 100% O2 for 10 mins. If PaO2 remains low after 10 mins = diagnostic of cyanotic heart disease.
What is the management plan for acute cyanosis in congenital heart disease? (ToF, TGA, Tricuspid atresia)
- A-E approach
- Start prostaglandin E1 infusion (5 ng/kg per min) to promote mixing blood + keep the ductus arteriosus open.
- Consider balloon atrial septostomy (TGA)
- Consider Blalock-Taussig shunt (ToF + TA)
List 5 side effects of prostaglandins, precautions and what should be monitored
SE: apnoea, hypoglycaemia, seizures, hypotension, fever
Intubate
Monitor BMs
What is the management plan for Tetralogy of Fallot?
Prostaglandin E1 infusion
Blalock-Taussig shunt: tube between subclavian artery + pulmonary artery
Definitive surgery at 4-6 months to repair the pulmonary valve, closing the VSD + relieving RV outflow tract obstruction.
What is the management plan for transposition of the great arteries?
Correct acidosis + hypoglycaemia if present
Prostaglandin E1 infusion
Balloon artery septostomy
Arterial switch procedure in first 2w of life
What are potential complications associated with congenital heart disease?
In L-to-R there is an increased risk of Eisenmenger syndrome.
Cyanotic complications.
Death if not identified early.
Shorter life expectancy.
What is the prognosis of congenital heart disease?
Dependent on early detection but due to a/w other syndromes life expectancy is usually reduced.
Which congenital heart disease presents with ‘tet spells’?
ToF
Paroxysmal hyperpnoea + increased cyanosis that occur spontaneously/ after feeding/ prolonged crying
What is balloon artery septostomy?
Breaks the flap valve of the foramen oval + encourages mixing of blood
Bolus heparin given before BAS
Name a prostaglandin used in cyanotic heart disease, what is the MOA
Alprostadil
Maintains patency of ductus arteriosus
How does Eisenmenger syndrome develop?
Untreated L-R disease
In L-R shunt, increased pulmonary blood flow leads to pulmonary vascular remodelling
Leads to pulmonary vascular resistance
Causes RV hypertrophy
RV pressure >. LV pressure
Shunt reverses
Blood bypasses lungs causing cyanosis
