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Week 5 - Cardiology > Adult Congenital Heart Disease > Flashcards

Adult Congenital Heart Disease Flashcards

1
Q

Define congenital heart disease [1]

A

an abnormality of foetal heart development

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2
Q

Describe the normal anatomy of the heart

A
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3
Q

Describe the normal physiology of the heart [4]

A
  1. Oxygen poor blood passes into the right atrium through the superior and inferior vena cava (SVC & IVC)
  2. This passes through the right atrioventricular valve called the tricuspid valve into the right atrium and is pumped through the pulmonary valve into the pulmonary arteries and passes through the lungs.
  3. This blood is oxygenated and passes back into the heart through the pulmonary veins into the left atrium.
  4. This then passes through the left atrioventricular valve i.e. the mitral valve into the left ventricle and is pumped through the aortic valve into the aorta and the remainder of the systemic system.
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4
Q

How does oxygenation occur in utero? [1]

A

in utero, oxygenation is by the maternal placenta

  • pulmonary circulation is minimal and at high resistance
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5
Q

Describe the physiology of foetal circulation [3]

A
  1. Oxygenated blood returns to right atrium via inferior vena cava (IVC)
  2. It then bypasses the right ventricle & pulmonary artery via the foramen ovale
  3. Of the blood that is pumped to the pulmonary artery via the right ventricle, most passes to the aorta via the ductus arteriosus
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6
Q

In the foetal circulation, there are 2 shunts which allow mixing of oxygenated and deoxygenated blood. What are these 2 shunts called? [2]

A
  1. foramen ovale
  2. ductus venosus
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7
Q

Define levocardia [1]

A

the heart is on the left side of the body (normal)

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8
Q

Define situs solitus [1]

A

the normal arrangement of abdominal organs with levocardia

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9
Q

What are the morphological characteristics of the right atrium? [2]

A
  1. a broad appendage
  2. sinoatrial node
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10
Q

What are the morphological characteristics of the left atrium? [1]

A

a narrow sausage shaped appendage

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11
Q

What are the morphological characteristics of the right ventricle? [3]

A
  1. heavily trabeculated endocardium
  2. contains a muscular band called the moderator band
  3. the chordae tendineae insert into the interventricular septum
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12
Q

What are the morphological characteristics of the left ventricle? [3]

A
  1. smooth endocardium
  2. two papillary muscles
  3. an ellipsoid cavity
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13
Q

Name the acyanotic conditions that result in left to right shunts [3]

A
  1. ventricular septal defect (VSD)
  2. atrial septal defects (ASD)
  3. persistent ductus arteriosus
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14
Q

Name the acyanotic conditions that result in outflow obstruction [3]

A
  1. Pulmonary stenosis
  2. Aortic stenosis
  3. Coarctation of the aorta
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15
Q

Name the cyanotic conditions that can affect the fetal heart

A
  1. Tetralogy of Fallot
  2. Transposition of the Great Arteries
  3. Atrioventricular septal Defect (complete)
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16
Q

Define an atrial septal defect [1]

A

hole in the wall between the atria

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17
Q

What are the 2 types of atrial septal defect? [2]

A
  1. secundum ASD
  2. primum ASD
    • also known as partial atrioventricular septal defect (AVSD)
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18
Q

Define secundum ASD [2]

A
  1. defect is present in the central part of the atrial septum (the partition separating the atriums).
  2. this allows oxygenated blood to pass through into the right side of the heart, leading to enlargement of the right ventricle and excessive flow in the lung circulation
19
Q

What are the typical signs of secundum ASD that can be picked up on examination? [2]

A
  1. pulmonary flow murmur
  2. fixed, split second heart sound
20
Q

What are the potential complications of secundum ASD? [5]

A
  1. RV failure
  2. Tricuspid regurgitation
  3. Atrial arrhythmias
  4. Pulmonary hypertension
  5. Eisenmenger syndrome
21
Q

Define primum ASD [4]

A
  1. defect that is present in the lower part of the “atrial septum” (the partition separating the atriums).
  2. this allows oxygenated blood to pass through into the right side of the heart, leading to enlargement of the right ventricle and excessive flow in the lung circulation
  3. the primum variant occurs close to the atrioventricular (AV) valve and as such can distort the valve and lead to it becoming regurgitant or leaky
  4. In addition to the primum ASD hole can extend down into the ventricle and cause a VSD, this would then be called a partial atrioventricular septal defect
22
Q

What are the treatment options for ASDs? [2]

A

ASDs can be fixed either surgically with a sternotomy or through the groin vessels with a transcatheter device if small enough

23
Q

Define coarctation of the aorta [1]

A

narrowing of the aorta

24
Q

Where does coarctation of the aorta tend to form? [1]

A

Tends to form distal to the left subclavian artery in a ‘juxta-ductal’ position, at the insertion point of the ductus arteriosus

25
Q

What are the potential complications of coarctation of the aorta? [4]

A
  1. upper body hypertension
  2. berry aneurysms
  3. claudication
  4. renal insufficiency
26
Q

How can coarctation of the aorta be treated? [3]

A
  • surgical repair via thoracotomy, where the aorta is either:
    • cut out or
    • the narrowed area is reconstructed via the subclavian artery
  • non-surgical options:
    • balloon angioplasty via a transcatheter through femoral artery
27
Q

What are the features of transposition of the great arteries (TGA)? [3]

A
  1. cyanosis
  2. usually no murmur
  3. clubbing
28
Q

Describe the pathophysiology of transposition of the great arteries [5]

A
  1. the aorta and pulmonary artery switch places, so that the aorta is now connected to the right ventricle and the pulmonary artery is connected to the left ventricle.
  2. this creates 2 separate circulation systems
  3. in the systemic circulation deoxygenated blood returns to the RA via the SVC & IVC, passes through the tricuspid valve into the RV and is then pumped through the AORTIC valve into the AORTA and around the body without being oxygenated.
  4. In the pulmonary circulation, the oxygenated blood passes back into the LA via the pulmonary veins, through the mitral valve into the LV, and is then pumped back into the PULMONARY artery to the lungs via the PULMONARY valve.
  5. Without mixing of the oxygenated & deoxygenated blood there is profound cyanosis and therefore transposition of the great arteries is an example of a cyanotic lesion.
29
Q

Why would you give a child with TGA prostaglandins? [2]

A
  1. When a baby takes it’s first breath at birth, the changes in pressure in the lungs and in the heart mean that the foramen ovale and ductus arteriosus closes within a few hours.
  2. This loss of the 2 shunts is catastrophic for TGA therefore we delay closing by given the baby prostaglandins. This buys us time to perform surgery.
30
Q

How do you treat TGA? [3]

A
  • treated by the arterial switch procedure
    • in this procedure we disconnect the aorta and pulmonary artery, switch them around and sew them back on.
    • So the aorta is now above the LV and the pulmonary artery is now above the RV
31
Q

What are the 4 components of Tetralogy of Fallot? [4]

A
  1. Ventricular Septal Defect
  2. Right Ventricular Outflow Tract (RVOT) obstruction
  3. Overriding aorta
  4. Right ventricular hypertrophy
32
Q

Define overriding aorta [1]

A

the aortic valve is enlarged and appears to arise from both the left and right ventricles instead of just the left ventricle

33
Q

How is Tetralogy of Fallot treated? [1]

A

BT shunt = connecting the carotid or subclavian artery directly on to the pulmonary artery

34
Q

What conditions does Tertalogy of Fallot put you at increased risk of, even after repair? [5]

A
  1. significant pulmonary regurgitation
    • RV dilatation +/- dysfunction
  2. arrhythmia
    • ventricular tachycardia
    • SVT with RBBB
  3. pulmonary arterial stenosis
35
Q

Define a univentricular heart [1]

A

a congenital heart condition which results in only one effective pumping ventricle

36
Q

Define single ventricle anomalies [1]

A

Common feature that only one of the two ventricles is of adequate functional size

37
Q

Name the anomalies that are described as single ventricle defects [5]

A
  1. Tricuspid atresia
  2. Hypoplastic left heart syndrome
  3. Double inlet left ventricle
  4. Many of the heterotaxic defects
  5. Some variations of double outlet right ventricle
38
Q

What are the corrective operations used for single ventricle anomalies? [4]

A
  1. Blalock-Taussig (BT) Shunts
  2. Norwood Procedure
  3. Bi-directional Glenn
  4. Fontan Circulation Operation
39
Q

What is involved in BT shunts? [1]

A

a tube graft is placed from an artery (usually the left subclavian or left innominate artery) to the pulmonary artery

40
Q

What is involved in Norwood Procedure? [3]

A
  1. the right ventricle becomes the systemic or main ventricle pumping to the body.
  2. A “neo” aorta is made from part of the pulmonary artery and the original, tiny aorta is reconstructed/enlarged to provide blood flow to the body.
  3. Finally, to provide blood flow to the lungs, a small tube graft is placed either from an artery to the lung vessels (called a modified Blalock-Taussig shunt) or from the right ventricle to the lung vessels (called a Sano modification)
41
Q

What is involved in Bi-directional Glenn procedure? [1]

A

superior vena cava is taken off the heart and sewn directly to the pulmonary artery

42
Q

What is involved in Fontan circulation operation? [2]

A

inferior vena cava is connected directly to the pulmonary arteries. Until now this blood has bypassed the lungs and has been pumped directly to the body resulting in oxygen levels lower than normal.

43
Q

What are the potential adverse complications of Fontan circulation and why? [5]

A
  • Pulmonary circulation dependent on high systemic venous pressure and low pulmonary vascular resistance
  • Anything that causes an imbalance can cause catastrophic haemodynamic compromise:
    • Pulmonary embolism (PE) - these patients need to be on constant anticoagulants
    • Arrhythmia
    • Dehydration
    • Bleeding

Week 5 - Cardiology (12 decks)

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