Congenital Heart Disease Flashcards
What is the incidence of congenital heart disease?
~1 in 125 live births
Describe how the survival rate in congenital heart disease has changed.
- Historic survival rates are low
- 1960 >50% died before 1st birthday
- 20% reached age 18
- Current survival rates
- >85% now reach age 18
What are the current challenges of the adult congenital heart disease population?
- Increased population size
- Services are trying to develop / keep up with demand.
- Increasing in complexity
- New interventional techniques
- No significant evidence base for guidelines
- Increasing age
- Increased incidence of acquired heart disease
- Increased complexity
What are the characteristics of the adult congenital heart disease population?
- Young
- Medically complex
- Congenital heart disease can be very complex
- Coupled with other congenital abnormalities
- Significant incidence of learning disabilities
- Syndromes
- Other congenital abnormalities
- Psychological issues
What are the 5 most common congenital heart defects?
- Bicuspid aortic valve
- Atrial septal defect
- Ventricular septal defect
- Tretralogy of Fallow
- Coarctation of the aorta
Describe the pathophysiology of a bicuspid aortic valve?
- Turbulent flow which leads to valve thickening and stiffening.
- Ultimately, valvular dysfunction (earlier than typical degenerative aortic valve disease).
- Can be associated with aortic dilatation.
- Can be associated with coarctation.
- Treatment - as for degenerative aortic valve disease (AVR +/_ aortic surgery).
Describe the progression of atrial septal defect.
- Often well-tolerated until 4th/5th decade
- Closure indicated if the right heart is dilated (and become breathless on exertion).
What interventions can be done in the event of atrial septal defect?
- Percutaneous closure
- Surgical closure
Describe the progression of a patient with ventricular septal defect.
- Small VSDs may be well-tolerated and may not require closure.
- Closure may be required if there is evidence of decompensation (dilated ventricles).
What interventions can be carried out to repair ventricular septal defect?
- Surgical closure
- Percutaneous closure
Describe Tetralogy of Fallot.
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Four components:
- Ventricular septal defect
- Pukmonary stenosis (subvalvular, valvular and/or supravalvular)
- An overriding aorta
- Right ventricular hypertrophy
Describe overriding aorta.
- The aorta sits just above the ventricular septal defect which means that there is a mixture of oxygenated and deoxygenated blood going into the systemic circulation.
- Patient can become cyanotic and pass out.
Describe the presentation of Tetralogy of Fallot.
- Infants present with cyanosis
- Children have ‘tetralogy spells’:
- Cyanotic
- Tired, groggy → unresponsive
- Irritable
- Spells relieved by squatting
Describe coarctation of the aorta.
- Significant narrowing of descending aorta.
- Can present dramatically in infancy.
- Can present later in life as hypertension with a murmur.
What are the long-term complications of congenital heart disease?
- Heart failure
- Valvular dysfunction
- Arrhythmias
Describe cardiac resynchronisation therapy.
Insertion of electrodes into the left and right ventricles of the heart, as well as on occasion the right atrium, to treat heart failure by coordinating the function of the left and right ventricles.
Describe left ventricular assist device?
Describe the haemodynamic changes in pregnancy.
- Cardiac output inceases the most.
- Heart rate increases, then plateaus.
- Stroke volume increases more slowly but overtakes heart rate and the HR plateua.
