Congenital/Inherited Heart Disease Flashcards
In the foetal heart what connects the umbilical vein (coming from the placenta) to the IVC?
Ductus venosus
What is the importance of the ductus venosus?
Allows most of the blood from the placenta to bypass the liver and go to the heart
In the foetal heart what connects the right atrium to the left atrium?
Foramen ovale
What is the importance of the foremen ovale?
Allows oxygenated blood in the RA (from IVC from umbilical vein from placenta) to enter the LA i.e. Oxygenated blood is not sent to the lungs
Once oxygenated blood has gone through the foremen ovale where does it go?
Enters the LV and is pumped up the aorta to the body and importantly the brain
In the foetal circulation what connects the pulmonary artery to the aorta?
Ductus Arteriosus
What is the importance of the ductus arteriosus?
Allows deoxygenated blood from the pulmonary artery to enter the descending aorta to go the placenta to be oxygenated
Do they lungs take part in gas exchange in the foetus?
No
Hence why pulmonary system is cleverly bypassed in foetal circulation
Hat results in the closure of the foremen ovale?
Baby’s first breath after delivery (usually)
How does the baby’s first breath close the foremen ovale?
Pulmonary resistance falls (due to increased oxygen)
Causing more blood flow from RA to RV and PA
Decrease in RA plus an increase in LA pressure causes closure of the foreman ovale
Hats the remnant of the umbilical vein in adults?
Ligamentum teres
What is the remnant of the ductus venosus in adults?
Ligamentum venosus
What acquired heart disease complications can patients be genetically predisposed to?
VT/VF in heart failure/ischaemia
AF in structural heart disease
Drug acquired long QT syndrome
SCD in left ventricular hypertrophy
What are the classifications of cardiomyopathy?
Hypertrophic cardiomyopathy (HCM)
Dilated (idiopathic) cardiomyopathy (DCM)
Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)
Restrictive Cardiomyopathy (RCM)
Symptoms of cardiomyopathy
Heart failure (dyspnoea, fatigue, oedema, orthopnoea)
Arrthymias (palpitations, dizziness, syncope)
Outflow tract obstruction (syncope, angina)
Signs of cardiomyopathy
HCM
- jerky pulse
- forceful apex
- 4th heart sound
- mid systole murmur (valsalva manoeuvre)
RCM
- rapid descent of JVP
- loud 3rd & 4th heart sound
Investigations for cardiomyopathy
TFT, iron studies ECG Echo Cardiac MRI Genetic testing
Effects of hypertrophic cardiomyopathy on the heart?
LVOT obstruction
Reduced ejection fraction
Mitral regurgitation
Rhythm distances
What is the pathology of ARVC?
Heart muscle is replaced by fibrous and fatty tissue
Usually right ventricle
Clinical presentation of ARVC
Ventricular arrthymias of RV origin
ECG changes
Dysfunction of the RV
ECG characteristics of ARVC
T-wave inversion beyond lead 1
Increased QRS duration
What’s is restrictive cardiomyopathy?
Walls of ventricles become stiff but not necessarily thickened
Resist normal filling with blood
What is long QT syndrome?
Congenital disorder that leads to unexplained syncope, seizures and sudden death
What is brugada syndrome?
Cardiac sodium channel mutation
What cardiac problems does brugada syndrome cause?
VT VF AF ST elevation RBBB V1-3
How is HCM thought to be passed on genetically?
Autosomal dominant
What is the mutation in HCM?
Mutation in sarcomeric genes
