CONGENITAL HEART DISEASE Flashcards
What are the common forms of acyanotic congenital heart disease?
Ventricular septal defect
Patent ductus arteriosus
Pulmonary stenosis (remember that critical PS will cause cyanotic disease)
Atrial septal defect
Coarctation of the aorta
Aortic stenosis
What the common forms of cyanotic congenital heart disease?
Tetralogy of Fallot
Transposition of the great arteries
Critical pulmonary stenosis
Pulmonary atresia
What are the different types of atrial septal defect and what are the differences?
Ostium primum
Ostium secundum
The less common type ostium primum develops as a result of incomplete fusion of the septum primum to the ventricular septum during heart development. It is therefore located lower in the atria. The atrial septal defect is said to exist when the two atrial septums try to fuse at birth but fail to due to this opening. It usually presents as symptomatic.
The more common ostium secundum is naturally created as part of the system that allows a right to left atrial shunt with the foramen ovale in the womb. The atrial septal defect is said to exist when the two atrial septums of the heart fail to fuse properly at birth. It is usually asymptomatic.
https://www.youtube.com/watch?v=ed6__8FaSOU
What is the difference between atrial septal defect and patent foramen ovale?
Both the ostium primum and ostium secundum atrial defects occur in the septum primum (associated with the left side of the heart in development). If they stay open at birth, fusion of the two atrial septums creates a large hole that remains open.
The foramen ovale is a hole in the septum secundum (associated with the right side of the heart in development). This only opens in conditions of raised left atrial pressure or volume.
What are the possible clinical features of atrial septal defects?
Widely split and fixed second heart sound (S2)
Pulmonary flow murmur - increased blood volume means that there is often be a soft, ejection systolic murmur in pulmonary area. Not to do with a structural defect of the valve.
A small proportion have a tricuspid flow murmur - rumbling mid diastolic murmur at the left sternal edge
Abnormal right ventricular electrical impulse
Paradoxical venous emboli if valsalva manoeuvre is performed
What are the differences in ECG changes between ostium primum and ostium secundum atrial septal defects?
Ostium secundum (70% of ASDs): RBBB with RAD
Ostium primum: RBBB with LAD
What is the most common type of congenital heart defect to be found in adulthood?
Atrial septal defect - usually ostium secundum
What is the syndrome associated with ostium secundum atrial septal defects?
Holt-Oram syndrome - autosomal dominant disorder that affects bones in the arms and hands
Fetal alcohol syndrome
What is the syndrome associated with ostium primum?
Down syndrome
What is the treatment and the purpose of the treatment of atrial septal defects?
Surgical (transcatheter)
ASD can cause heart failure in later life. Best to treat at 3-5 years old
What is the most common type of congenital heart defect?
Ventricular septal defect
What are the risk factors for developing ventricular septal defects?
Down syndrome
Fetal alcohol syndrome
Gestational diabetes
Maternal SLE
Rubella infection
Drugs:
Lithium
How do we classify ventricular septal defects?
By size:
Small
Medium
Large
By location:
Type 1 - infundibular (related to the aortic valve)
Type 2 - perimembranous (accounts for 70% of VSDs)
Type 3 - Inlet (atrioventricular septal defect)
Type 4 - muscular (accounts for 20% of VSDs)
Type 5 - Gerbode (absence of atrioventricular septum)
How long after birth does a ventricular septal defect usually manifest?
A few weeks
What are the clinical features of small ventricular septal defects and what is the other name given to such defects?
Also called maladie of Roger
Child is normally asymptomatic and a pansystolic murmur with a possible palpable thrill is heard at the left lower sternal border.
What are the clinical features of medium ventricular septal defects?
Symptoms:
Slow weight gain
Difficulty feeding - due to tachypnoea
Recurrent chest infections secondary to pulmonary congestion
Signs:
Increased cardiac electrical impulse
Palpable thrill
Harsh pansystolic murmur, loudest in third and fourth left intercostal spaces
Heart failure will eventually develop.
What are the signs of heart failure in an infant?
Tachycardia
Tachypnoea
Hepatomegaly
Pallor and sweating
What is the major paradoxical clinical difference between a medium and a large ventricular septal defect?
The murmur in large VSDs might actually be quieter than medium VSDs
How do you treat heart failure in children?
Diuretics
ACE inhibitors
What is patent ductus arteriosus?
The ductus arteriosus connects the aorta to the left pulmonary artery and usually closes by the fourth day of life. PDA is diagnosed if it does not close 3 weeks to one month after birth.
What are the risk factors for patent ductus arteriosus?
Preterm infants
Down syndrome
High altitudes
What are the clinical features of patent ductus arteriosus?
Bounding pulse - wide pulse pressure
Murmur - initially systolic develops into a continuous machinery murmur
Failure to thrive