Week 3 Flashcards
Define congenital heart disease?
Heart disease that patient is born with
Name 6 causes for congenital heart disease?
- Genetic defects
- Chromosomal abnormalities (Downs, Turners etc)
- Intrauterine infection (Rubella)
- Drugs
- Maternal alcohol
- Maternal diabetes
What are the 2 main great vessels that are derived from the 4th pharyngeal arch?
- Arch of Aorta (four rhymes with aOR)
2. Right subclavian artery (fouRS= Right Subclavian)
For the following stages in heart formation from the cardiac tube, name the day on occurrence and the milestones associated with stage:
- Cardiac crescent
- Linear heart tube
- Looping heart
- Chamber formation
- Cardiac crescent
- Day 15
- Cardiac differentiation
- Migration to midline - Linear heart tube
- Day 20
- Heart tube formation
- First heartbeat
- Anterior-posterior and dorsal-ventral patterning - Looping heart
- Day 28
- Early chamber formation (FHF = ventricles, SHF = atria)
- Looping to the right - Chamber formation
- Day 32
- Chamber formation
- Trabeculation
- Cushion formation
- Outflow tract septation
- Early conduction system formation
What are the sources of congenital cardiovascular diseases? (5)
- Failure of septation
- Failure of development
- Failure of/or incorrect rotation
- Abnormalities of the great vessels
- Failure of closure
How does oxygenated blood from umbilical vein by pass the lungs (uninflated, high resistance)?
- Crossing the fossa ovalis
- Passing from PT to aorta via ductus arteriosus.
Ductus arteriosus opening is maintained by prostaglandins
What conditions are caused by the following:
- Failure of septation
- Failure of development
- Failure of/or incorrect rotation
- Abnormalities of the great vessels
- Failure of closure
- Failure of septation: VSD, ASD
- Failure of development:
Obstruction= Tricuspid/pulmonary atresia, pulmonary stenosis, coarctation of the aorta
Hypoplasia= Hypoplastic left heart - Failure of/or incorrect rotation =TGA, congenitally corrected transposition of the great vessels, dextrocardia
- Abnormalities of the great vessels= Wrong connections/embryology
- Failure of closure=Persistent ductus arteriosus
What do the following abbreviations for congenital heart diseases mean: ASV VSD TGA PDA PAH
ASD= Atrial septal defect VSD= Venticular septal defect TGA= transposition of great arteries PDA= Patent ductus arteriosus PAH= Pulmonary arterial hypertension
What are 4 issues of congenital cardiovascular disease and how do they present?
Hint: CHIP
- Incidental: Murmur, echo finding
- Heart failure: Pulmonary pressure (Qp) > Systemic pressure (Qs)
- Central cyanosis: Result of venous mixing with systemic blood
- Pulmonary hypertension: Increased pulmonary pressure (Qp)
Name 6 presenting problems for congenital cardiovascular disease?
- Heart failure: Difficulty feeding, failure to thrive, tachypnea, cyanosis
- Cyanosis
- Clubbing
- Murmur
- Squatting (Fallot’s)
- Syncope
Name 3 acyanotic congenital CV diseases that are:
a) with shunts
b) without shunts
WITH SHUNTS
- ASD
- VSD
- PDA
WITHOUT SHUNTS
- Pulmonary stenosis
- Coarctation of the aorta
- Aortic/left heart obstruction
Name 3 cyanotic congenital CV diseases that are:
a) with shunts
b) without shunts
WITH SHUNTS
- Transposition of great vessels
- Fallots tetralogy
WITHOUT SHUNTS
- Hypoplastic left heart
- V severe pulmonary stenosis
- Pulmonary/tricuspid atresia
Discuss the management of congenital CV diseases?
Prevention and palliative
Prevention: Foetal ECG
Palliative:
1.To allow growth for definitive treatment
2. As long term treatment: Creation of systemic to pulmonary shunt
3. Definitive treatment: Correction of TGA, closure devices for septal defects
4.Transplantion
What are the 7 complications of congenital CV diseases?
- Failure to thrive
- Paradoxical embolus
- Endocarditis
- Pulmonary hypertension
- Polycythaemia
- Haemoptysis
- Artythmias
What is a paradoxical embolus?
When a venous thrombi enters the systemic circulation via an atrial septal defect
Pulmonary hypertension:
Cause:
Consequences:
Investigations:
Cause:Arises as a result of “high flow” through the pulmonary bed
Consequences:
Left to right shunts reverse to become right to left = cyanosis
Very high maternal mortality usually means that pregnancy is best avoided
Investigations:
- ECG and Doppler
- Cardiac catheterization
- Genetic
ASD: If uncomplicated, presents as? Heart sound abnormality? Secundum vs primum? Management?
If uncomplicated, presents as: Heart failure, failure to thrive or incidental finding
Heart sound abnormality: Fixed splitting of the second heart sound
Secundum vs primum: Secundum is isolated, primum often complicated by other lesions
Management: Nothing/ percutaneous closure (dependent on size of shunt)
What are the clinical differences between small and large VSDs?
Small VSDs:
- Restrict shunt to trivial levels (termed as a restrictive VSD)
- Safe ie never causes PAH
- Loud murmur
Large VSD:
- High Qp:Qs present in early infancy
- Palliated until definitive treatment
- Loud holosystolic murmur
What is PDA and how does it present itself clinically with large and small?
Patent ductus arteriosus
Larger PDA: will present as heart failure with continuous murmur and wide pulse pressure
Can cause PAH but this will only show lower body cyanosis
Smaller PDA: Continuous murmur, normal pulse pressure. In infancy, prostaglandin inhibition may close duct
Coarctation: Define? Men/women? Leads to.. Associations? Indications? Treatment?
Definition: Narrowing of the aorta: from complete interruption to small low gradient stenosis More common in men Causes: Systemic hypertension in adults Associations: with intracranial aneurysms Indications: - Absent/delayed femoral pulses -Lower bp in legs -Scapula collaterals -Rib notching on CXR Treatment: -Balloon dilatations -Surgery
What difference does the septum being intact make to the presentation of the TGA?
If the septum is intact, TGA presents rapidly as a blue and failing baby. This is because there is no mixing of oxy and de oxy blood, hence there is complete incorrect supply to the pulmonary artery and aortic.
The symptoms appear later is septum is not intact
Name 4 palliative approaching to TGA?
- Pharmacologic maintenance of arterial duct
- Atrial septostomy
- Radical switch procedure
- Pallative surgery- mustard procedure
Tetralogy of Fallot involves four heart defects, name them.
- A large ventricular septal defect (VSD)
- Pulmonary stenosis
- Right ventricular hypertrophy
- An overriding aorta
Tetralogy of Fallot:
Definite cause of?
Characteristic behavior?
Cyanosis
BUT one which is reversible as they never have PAH
Characteristic: Squatting as it raise systemic resistance