ana 1 Flashcards

(51 cards)

1
Q

What are congenital heart diseases (CHDs)?

A

Abnormalities of the heart or great vessels present at birth.

Most CHDs are well tolerated during fetal life but become apparent at birth.

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2
Q

What is a shunt in the context of congenital heart disease?

A

An abnormal communication or link between heart chambers or blood vessels that typically produces defects in circulation.

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3
Q

What are left to right shunts classified as?

A

Acyanotic group of heart diseases.

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4
Q

Name two examples of left to right shunts.

A
  • Ventricular septal defects (VSD)
  • Atrial septal defects (ASD)
  • Patent ductus arteriosus (PDA)
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5
Q

What complications arise from long-standing severe cyanosis?

A
  • Clubbing of fingers and toes
  • Polycythaemia
  • Cerebral thromboembolism
  • Abscesses
  • Scoliosis
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6
Q

What are obstructive congenital heart diseases?

A

Conditions like coarctation of the aorta and stenosis/atresia of aorta or pulmonary artery.

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7
Q

When does the cardiovascular system begin to develop?

A

At the end of the third week of gestation.

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8
Q

What is the critical period of heart development?

A

From day 20 to day 50 after fertilization.

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9
Q

What is the most common congenital cardiac anomaly in infants and children?

A

Ventricular septal defect (VSD).

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10
Q

What is the incidence of congenital heart disease in live births?

A

4-50 per 1000 live births.

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11
Q

What are some genetic factors associated with congenital heart disease?

A
  • Single gene defects
  • Chromosomal abnormalities (e.g., Down syndrome)
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12
Q

What is Eisenmenger’s syndrome?

A

A condition where long-standing left-to-right shunts lead to pulmonary hypertension and reversed shunting.

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13
Q

What characterizes Tetralogy of Fallot?

A
  • Large VSD
  • Pulmonary stenosis
  • Right ventricular hypertrophy
  • Overriding aorta
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14
Q

What is the most common cause of cyanotic congenital heart disease in adults?

A

Tetralogy of Fallot (TOF) and Eisenmenger’s syndrome.

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15
Q

What is the difference between acyanotic and cyanotic heart defects?

A

Acyanotic defects do not cause cyanosis, while cyanotic defects lead to arterial oxygen desaturation.

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16
Q

Fill in the blank: The ductus arteriosus connects the descending aorta to the _______.

A

left pulmonary artery.

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17
Q

What is the potential outcome of untreated patent ductus arteriosus (PDA)?

A

Heart failure, pulmonary hypertension, or endarteritis.

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18
Q

What are the three systems of paired veins that drain into the primordial heart?

A
  • Vitelline system
  • Cardinal veins
  • Umbilical vein
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19
Q

What is the prevalence of congenital heart disease in stillborns?

A

3-4 per 100 stillborns.

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20
Q

True or False: Most congenital defects are well tolerated during fetal life.

A

True.

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21
Q

What is the most common congenital heart anomaly that remains asymptomatic until adulthood?

A

Atrial septal defect (ASD).

22
Q

What does the term ‘dextrocardia’ refer to?

A

A condition where the heart lies on the right side of the thorax instead of the left.

23
Q

What is a significant risk associated with large ventricular septal defects (VSD)?

A

Left ventricular failure or pulmonary hypertension.

24
Q

What may cause a reversal of shunt from right to left in congenital heart defects?

A

Increased pressure in the right ventricle due to pulmonary overload.

25
What is the risk of infective endocarditis in patients with atrial septal defect (ASD)?
No need for antibiotic prophylaxis unless there is a concomitant valvular abnormality.
26
What is the role of the mesoderm in cardiovascular development?
Forms all muscle types, blood, and the cardiovascular system.
27
What is a common characteristic of patients with large VSD who survive to adulthood?
They usually have left ventricular failure or pulmonary hypertension.
28
What are the main causes of congenital heart disease?
* Autosomal dominant mutations * Sporadic genetic abnormalities
29
What is ventricular hypertrophy?
Enlargement of the ventricle due to increased workload ## Footnote Often occurs in response to conditions like hypertension or outflow obstruction.
30
What structural abnormality occurs in Tetralogy of Fallot (TOF)?
Overriding aorta ## Footnote The aorta is positioned directly above the ventricular septal defect (VSD).
31
What happens to pulmonary blood flow in TOF?
Reduced or decreased ## Footnote Due to the right ventricle being enlarged and the presence of right to left shunt.
32
What are some abnormalities associated with Tetralogy of Fallot?
* Right aortic arch * Atrial septal defect * Coronary artery anomalies ## Footnote Known collectively as the pentalogy of Fallot.
33
What is the primary cause of cyanosis in TOF patients?
Substantial right to left shunt ## Footnote Resulting from equal ventricular pressures due to large VSD.
34
How does systemic vascular resistance affect right to left shunting in TOF?
A decrease increases shunting; an increase decreases shunting ## Footnote This is due to the relatively fixed resistance in the right ventricular outflow tract.
35
What is the outcome for most patients with TOF without surgical intervention?
Most patients die in childhood ## Footnote Surgical repair can improve survival rates.
36
What are obstructive congenital heart anomalies characterized by?
Stenosis of ventricular outflow without re-routing of blood ## Footnote Examples include coarctation of the aorta, aortic stenosis, and pulmonary stenosis.
37
What is pulmonary stenosis?
Narrowing of the right ventricle outflow ## Footnote Can occur at the valvar, subvalvar, supravalvar, or branch levels.
38
What percentage of all congenital heart defects does pulmonary stenosis account for?
8-12% ## Footnote It is one of the more common defects.
39
What is D-Transposition of the great arteries?
Abnormal neural crest cell migration leading to non-spiral development of the aortico-pulmonary septum ## Footnote Results in aorta arising from RV and pulmonary trunk from LV.
40
What happens to systemic and pulmonary circulations in D-Transposition of the great arteries?
They are completely separated ## Footnote This condition is incompatible with life without mixing through defects.
41
What clinical signs are associated with D-Transposition of the great arteries?
Marked cyanosis and right-to-left shunting ## Footnote This occurs due to separation of blood flow systems.
42
What is L-Transposition of the great arteries?
Aorta and pulmonary trunk are transposed with inverted ventricles ## Footnote The anatomical RV lies on the left and the LV on the right.
43
What is ectopia cordis?
A rare condition where the heart lies exposed on the surface of the thorax ## Footnote Caused by non-union of the sternal plates.
44
What is Eisenmenger's syndrome?
A large left to right shunt causing severe pulmonary vascular disease and pulmonary hypertension ## Footnote This leads to reversal of shunting direction.
45
What are the initial morphologic alterations in Eisenmenger's syndrome?
* Medial hypertrophy of pulmonary arterioles * Intimal proliferation and fibrosis * Occlusion of capillaries/small arterioles ## Footnote These changes are potentially reversible.
46
What happens as Eisenmenger's syndrome progresses?
Advanced morphologic changes become irreversible ## Footnote Includes plexiform lesions and necrotizing arteritis.
47
What occurs when pulmonary vascular resistance exceeds systemic resistance in Eisenmenger's syndrome?
The shunt is reversed ## Footnote This typically occurs as the disease advances.
48
What is the most common congenital anomaly of the heart?
Ventricular septal defect (VSD) ## Footnote VSD can vary in size and type, with membranous being the most common.
49
What is the most significant type of atrial septal defect (ASD)?
Ostium secundum defect ## Footnote This type of ASD is the most prevalent and clinically significant.
50
What is the most common congenital cyanotic heart disease?
Fallot's tetralogy ## Footnote This condition is characterized by a combination of four heart defects.
51
What is the most common positional anomaly of the heart?
Dextrocardia ## Footnote This condition involves the heart being situated on the right side of the thorax.