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CARDIOLOGY > CONGENITAL > Flashcards

CONGENITAL Flashcards

1
Q

What are the 2 categories of congenital heart diseases?

A

Acyanotic
Cyanotic

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2
Q

What are examples of congenital heart defects that may present for the first time in adulthood?

A

Atrial septal defect
Ventricular septal defect
Coarctation of the aorta
Bicuspid aortic valve

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3
Q

What are examples of congenital heart defects that usually present and need management in infancy/childhood?

A

Patent ductus arteriosus
Tetralogy of Fallot
Ebstein’s anomaly
Transposition of the great arteries

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4
Q

What is cyanotic heart disease?

A

When blood can bypass the pulmonary circulation and the lungs due to a right-to-left shunt

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5
Q

What are heart defects that can cause cyanotic heart disease?

A

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

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6
Q

Why are pt with ventricular septal defect, atrial septal defect and patent ductus arteriosus not typically cyanotic?

A

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

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7
Q

What is Eisenmenger syndrome?

A

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.

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8
Q

What are heart defects that can cause acyanotic heart disease?

A

ventricular septal defects (VSD)
atrial septal defect (ASD)
patent ductus arteriosus (PDA)
coarctation of the aorta
aortic valve stenosis
Pulmonary stenosis

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9
Q

Whats the most common acyanotic congenital heart disease?

A

Ventricular septal defects - 30%

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10
Q

Are ventricular or atrial septal defects more common?

A

Ventricular
(However in adults ASDs are more common as they generally present later)

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11
Q

What are the complications of congenital heart disease?

A

Heart failure
Arrhythmias
Endocarditis
Stroke
Pulmonary hypertension
Eisenmenger Syndrome

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12
Q

What is an atrial septal defect?

A

A defect in the septum between the 2 atria that connect them and allow blood to flow between

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13
Q

What are the types of atrial septal defects?

A

Patent foramen ovals (most common)
Ostium secondum
Ostrium primum

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14
Q

What is ostium secondum?

A

where the septum secondum fails to fully close, leaving a hole in the wall

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15
Q

What is ostium primum?

A

where the septum primum fails to fully close, leaving a hole in the wall (this tends to lead to a atrioventricular septal defect)

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16
Q

Outline the pathophysiology behind atrial septal defects?

A

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.

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17
Q

How do atrial septal defects present?

A

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

18
Q

Why are atrial septal defects a cause of stroke with pt with a DVT?

A

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.

19
Q

How common is a patent foramen ovale?

A

1 in 4

20
Q

What murmur do atrial septal defects cause?

A

mid-systolic, crescendo-decrescendo murmur loudest at the upper left sternal border, with a fixed split second heart sound

21
Q

Why does a fixed split second heart sound occur in an atrial septal defect>

A

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.

22
Q

What conditions is patent foramen ovale associated with?

A

Migraine with aura
Stroke and TIA

23
Q

How are atrial septal defects managed?

A

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.

24
Q

What are ventricular septal defects?

A

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.

25
Q

What causes ventricular septal defects?

A

Congenital
After MI

26
Q

What are congenital ventricular septal defects associated with?

A

Underlying genetic conditions e.g. Down’s syndrome and Turner’s syndrome

27
Q

Why can an MI cause a ventricular septal defect?

A

It can damage the ventricular septum due to ischaemia

28
Q

Outline the pathophysiology of ventricular septal defects?

A

VSDs usually feature a left-to-right shunt. Over time this causes right-sided overload, right heart failure and increased flow into the pulmonary vessels. Pulmonary hypertension may progress to a right-to-left shunt, resulting in cyanosis (Eisenmenger syndrome).

29
Q

How do ventricular septal defects present?

A

Often VSDs are initially asymptomatic and patients can present as late as adulthood. They may be picked up on antenatal scans or when a murmur is heard during the newborn baby check.

30
Q

What can cause a pan-systolic murmur?

A

ventricular septal defect, mitral regurgitation and tricuspid regurgitation.

31
Q

What murmur is associated with ventricular septal defects?

A

Pan-systolic murmur best heard at left lower eternal border in 3rd and 4th intercostal space

32
Q

What is there an increased risk of in pt with ventricular septal defects?

A

Infective endocarditis

33
Q

How are ventricular septal defects managed?

A

transvenous catheter closure via the femoral vein or open-heart surgery.
Prophylactic antibiotics to reduce the rusk of infective endocarditis should be given before surgical procedures

34
Q

What is coarctation of the aorta?

A

Congenital narrowing of the aortic arch usually around the ductus arteriosus

35
Q

What is coarctation of the aorta most typically associated with?

A

Turner’s syndrome

Others - bicuspid aortic valve, berry aneurysms, neurofibromatosis

36
Q

Whats the effects of coarctation of the aorta?

A

Narrowing of the aorta reduces the pressure of blood flowing to the arteries that are distal to the narrowing. It increases the pressure in areas proximal to the narrowing, such as the heart and the three branches of the aorta arch

37
Q

How does coarctation of the aorta present?

A

Coarctation may go undiagnosed until adulthood.

hypertension is often the first sign
Murmu
High bp in limbs supplied from arteries that branch of the aorta before the narrowing and lower bp in limbs that branch off the aorta after narrowing
Radio-femoral delay
Left ventricular heave
Underdeveloped left arm
Underdevelopment of the legs

38
Q

What murmur is associated with coarctation of the aorta?

A

Systolic murmur in left infraclavicular area and below the left scapula

39
Q

Why may coarctation of the aorta present with an underdeveloped left arm?

A

Due to reduced blood flow to the left subclavian artery

40
Q

How do you diagnose coarctation of the aorta?

A

4 limb blood pressure
CT angiography

41
Q

How do you manage coarctation of the aorta?

A

In mild cases, patients can live symptom-free until adulthood without requiring surgical input. In severe cases, patients will require emergency surgery shortly after birth.

In adulthood it can be treated with:
Percutaneous balloon angioplasty (stretching the stenosis), potentially with a stent inserted
Open surgical repair

Pt also need medical management of hypertension

42
Q

Who is patent ductus arteriosus more common in?

A

Premature babies
Babies born at high altitudes
Maternal rubella infections in first trimester

CARDIOLOGY (19 decks)

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