CONGENITAL Flashcards
What are the 2 categories of congenital heart diseases?
Acyanotic
Cyanotic
What are examples of congenital heart defects that may present for the first time in adulthood?
Atrial septal defect
Ventricular septal defect
Coarctation of the aorta
Bicuspid aortic valve
What are examples of congenital heart defects that usually present and need management in infancy/childhood?
Patent ductus arteriosus
Tetralogy of Fallot
Ebstein’s anomaly
Transposition of the great arteries
What is cyanotic heart disease?
When blood can bypass the pulmonary circulation and the lungs due to a right-to-left shunt
What are heart defects that can cause cyanotic heart disease?
Transposition of the great arteries
Tricuspid atresia
Tetralogy of Fallot
Others:
Total anomalous pulmonary venous connection
Hypoplastic left heart syndrome
Truncus arteriosus persistant
Interrupted aortic arch
Pulmonary atresia
Pulmonary stenosis
Eisenmenger syndrome
Why are pt with ventricular septal defect, atrial septal defect and patent ductus arteriosus not typically cyanotic?
This is because the pressure in the left side of the heart is much greater than the right side, and blood will flow from the area of high pressure to the area of low pressure (left to right). This prevents a right-to-left shunt
What is Eisenmenger syndrome?
Cyanotic heart diseases dont typically cause cyanosis because the pressure in the L side of the heart is greater than the right
But …
If the pulmonary pressure increases beyond the systemic pressure, blood will start to flow from right to left across the defect, causing cyanosis.
What are heart defects that can cause acyanotic heart disease?
ventricular septal defects (VSD)
atrial septal defect (ASD)
patent ductus arteriosus (PDA)
coarctation of the aorta
aortic valve stenosis
Pulmonary stenosis
Whats the most common acyanotic congenital heart disease?
Ventricular septal defects - 30%
Are ventricular or atrial septal defects more common?
Ventricular
(However in adults ASDs are more common as they generally present later)
What are the complications of congenital heart disease?
Heart failure
Arrhythmias
Endocarditis
Stroke
Pulmonary hypertension
Eisenmenger Syndrome
What is an atrial septal defect?
A defect in the septum between the 2 atria that connect them and allow blood to flow between
What are the types of atrial septal defects?
Patent foramen ovals (most common)
Ostium secondum
Ostrium primum
What is ostium secondum?
where the septum secondum fails to fully close, leaving a hole in the wall
What is ostium primum?
where the septum primum fails to fully close, leaving a hole in the wall (this tends to lead to a atrioventricular septal defect)
Outline the pathophysiology behind atrial septal defects?
An atrial septal defect leads to a shunt. Blood moves from the left to right atrium because the pressure in the left atrium is higher. This means blood continues to flow to the pulmonary vessels and lungs to get oxygenated and the patient does not become cyanotic. However, the increased flow to the right side of the heart leads to right-sided overload and right heart strain. This can lead to right heart failure and pulmonary hypertension.
Eventually, pulmonary hypertension can lead to Eisenmenger syndrome. This occurs because the pulmonary pressure exceeds the systemic pressure, causing the shunt to reverse and become a right-to-left shunt across the ASD. This causes blood to bypass the lungs, resulting in the patient becoming cyanotic.
How do atrial septal defects present?
Atrial septal defects are often picked up on antenatal scans or newborn examinations.
It may present in adulthood with:
Dyspnoea (secondary to pulmonary hypertension and right-sided heart failure)
Stroke
Atrial fibrillation or atrial flutter
Why are atrial septal defects a cause of stroke with pt with a DVT?
Normally, when patients have a DVT and this becomes an embolus, the clot travels to the right side of the heart, enters the lungs and becomes a PE.
In patients with an ASD the clot can travel from the right atrium to the left atrium across the ASD. This means the clot can travel to the left ventricle, aorta and up to the brain, causing a large stroke.
How common is a patent foramen ovale?
1 in 4
What murmur do atrial septal defects cause?
mid-systolic, crescendo-decrescendo murmur loudest at the upper left sternal border, with a fixed split second heart sound
Why does a fixed split second heart sound occur in an atrial septal defect>
This occurs in an atrial septal defect because blood is flowing from the left atrium into the right atrium across the atrial septal defect, increasing the volume of blood that the right ventricle has to empty before the pulmonary valve can close. This doesn’t vary with respiration.
What conditions is patent foramen ovale associated with?
Migraine with aura
Stroke and TIA
How are atrial septal defects managed?
In cases where the ASD is small and asymptomatic, watching and waiting may be appropriate. ASDs can be corrected surgically using a percutaneous transvenous catheter closure (via the femoral vein) or open-heart surgery. Anticoagulants (such as aspirin, warfarin and DOACs) are used to reduce the risk of clots and stroke in adults.
What are ventricular septal defects?
a hole in the septum (wall) between the two ventricles. This can vary in size from tiny to the entire septum, forming one large ventricle.
