Congenital Heart Disease Flashcards
What is congenital heart disease?
- Abnormality of the structure of the heart
- Present at birth
What are the three stages in the spectrum of severity of congenital heart disease and how are they defined?
- Mild - asymptomatic and may resolve spontaneously (can progress)
- Moderate - requires specialist intervention and monitoring in a cardiac centre
- Severe – present severely ill / die in newborn period or early infancy
What is major congential heart disease?
Heart disease that requires surgery within the first year of life
What is antenatal screening?
- Ultrasound at 18-22 weeks
- 4 chamber heart view and outflow tract view
Outline the management of an antenatally diagnosed CHD
- Disease dependant
- Expert team puts plan in place
- May decide to deliver in cardiac surgical centre
- Prostaglandin infusion if duct dependant lesion
What is newborn screening?
- Clinical examination at 24hrs of age
- Femoral pulses, heart sounds and presence of murmurs
What would happen if there was a murmur found on newborn screening pointinf to a small muscular VSD?
- Murmur early in life
- No haemodynamic consequences
- May close spontaneously
What is the differential of cyanosis in the newborn?
- Cardiac disease (blue with litte/no respiratory distress, may have pre/post ductal differential)
- Respiratory disease (increased WOB and xray changes)
- Persistent pulmonary hypertension of the newborn (PPHN) - (otherwise very unwell babies, large pre-post ductal differential)
What is transposition of the great vessels?
When does the duct close normally in transpostion of the great vessels and what symptoms present?
- 2-7 days
- Severe cyanosis or pallor
- Tachyponea, distress
- Rapid deterioration to death
What are the signs of duct closure with transposition of the great vessels?
- Pallor
- Prolonged CRT
- Poor or absent pulses
- Hepatomegaly
- Crepitations
- Acidosis
What is the treatment of duct closure with transposition of the great vessels?
- ABC
- Prostaglanding E2 to open duct
- Multisystem supportive treatment
- Transfer to cardiac surgical centre for definitive management
What are some duct dependant systemic circulation conditions?
- Hypoplastic left heart
- Critical aortic stenosis
- Interrupted aortic arch
- Critical coarctation of aorta
What are some duct dependant pulmonary circulation conditions?
- Tricuspid atresia
- Pulmonary atresia
What is hypoplastic left heart?
What is pulmonary atresia?
When do babies normally present with cardiac failure?
- Seen with moderate to large left to right shunts
- Increased pulmonary flow, decreased ventricular load
- Tend to present a few weeks as pulmonary pressures drop
What are the clinical signs of heart failure in babies?
- Failure to thrive
- Slow / reduced feeding
- Breathlessness (especially when feeding)
- Sweatiness
- Hepatomegaly
- Crepitations
When do moderate/large VSD murmurs present? Why?
- There is a big defect so less of a gradient
- Often no murmur at baby check
- Murmur develops as pulmonary pressures drop over first week
- Increased pulmonary circulation, congestive cardiac failure
What is the repair for patent ductus arteriosus?
- Catheter procedure
- Couple of follow ups
- Ensure flow stopped
- Device in correct position
- Discharged
What is the repair for VSD?
- Closure by patch
- Follow up during childhood/adolescence
- Rhythm or valve problems
- Generally expect to go on to a normal life
What is the repair for HLHS?
- 3 Stage complex surgery
- Significant mortality at each stage and between
- Ends with RV supplying systemic circulation
- Will fail over time
- Transplant
