Congenital heart disease Flashcards
What is the most common form of congenital heart disease?
Ventricular septal defects
Risk factors for congenital heart disease
- Down syndrome
- Maternal alcoholism and smoking
- Preterm infants
- Family history of congenital heart disease in a first degree relative increases the risk from 1% to 4%
- Maternal factors: poorly controlled diabetes, hypertension, rubella infection or systemic lupus erythematosus
- Marfan syndrome, DiGeorge syndrome
- Teratogenc drugs eg lithium, certain anticonvulsants, ACE inhibitors
Pathophysiology of Eisenmenger syndrome
An atrial or ventricular septal defect causes a left to right shunt which results in increased pulmonary blood flow that can progressively lead to increased pulmonary artery pressure. This leads to pulmonary hypertension. When the pulmonary pressure exceeds the systemic pressure, this causes a right to left shunt. This causes deoxygenated blood to bypass the lungs resulting in cyanosis.
# Define patent ductus arteriosis Epidemiology
A patent ductus arteriosis refers to a congenital heart defect caused by a failure of closure of the ductus arteriosis soon after birth. This condition accounts for 10% of all congenital heart disease and is more common in premature infants.
Clinical features of patent ductus arteriosis
- Usually asymptomatic if small, and is characterised by a continuous machinery like murmur on examination
- A larger shunt may produce a bounding pulse and symptoms of heart failure (poor feeding and faltering growth in children)
Investigations and management of patent ductus arteriosus
Echocardiography is the investigation of choice.
Premature neonates (<37 weeks) may have their PDA closed using an NSAID such as indomethacin or ibuprofen.
Term neonates should receive transcatheter closure of the PDA, or surgical repair if the defect is too large.
Definition of coarctation of the aorta
A narrowing of the aorta. It is almost always congenital.
Key associations with coarctation of the aorta
- Bicuspid aortic valve
- Cerebral berry aneurysms
- Turner syndrome
Clinical features of coarctation of the aorta
- Patients may present with symptoms of heart failure
- May have radio-femoral or radio-radial pulse delays or an absent femoral pulse in children
- Differences in BP and oxygen saturation may also be seen
Complications of coarctation of the aorta if not repaired
- Aortic dissection
- Left ventricular failure
Investigations and management of coarctation of the aorta
Echocardiography is the first line investigation.
Management is balloon angioplasty, stenting or surgical repair depending on the presentation of the disease in the context of symptoms and the size of the defect.
Definition of an atrial septal defect
An atrial septal defect refers to a communication between the left and the right atria.
Epidemiology of atrial septal defect
- Accounts for up to 10% of all congenital heart defects
- Ostium secundum defects are the most prevalent
Clinical features of an atrial septal defect
- ASDs are usually asymptomatic
- A systolic murmur may be heard on the upper left sternal edge, with characteristic fixed splitting of the second heart sound as a result of pulmonary valve delay closure
- Atrial fibrillation may occur later on in life, as might heart failure secondary to progressive right heart dilation
Investigation and management of atrial septal defect
Echocardiography
Transcatheter closure may be used for most defects, but very large ASDs and primum ASDs require surgical closure