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Pathophysiology- Cardio > Congenital heart > Flashcards

Congenital heart Flashcards

1
Q

Shunt

A

Abnormal communication between chambers or blood vessels. Abnormal if it is present after birth. Permits blood flow Down the pressure gradient and can flow LEFT to RIGHT or vice versa.

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2
Q

Left blood flow

A

meaning systemic

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3
Q

Right blood flow

A

meaning pulmonary

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4
Q

Right to left shunts

A

bypass pulmonary circulation, and will often have hypoxemia and cyanosis because poorly oxygenated blood goes directly to the systemic circulation.

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5
Q

Cyanosis

A

blue discoloration of the skin & mucous membranes;in congenital heart disease, due to hypoxemia from mixing of pulmonary and systemic blood and/or due to decreased cardiac output

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6
Q

acrocyanosis

A

cyanosis in the tips of digits, tip of nose

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7
Q

central cyanosis

A

cyanosis in other parts of the body- mucous membranes and lips for example.

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8
Q

Decreased CO and Hypoxemia =

A

cyanosis. Not always Congenital heart disease

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9
Q

Hyperoxia test

A

can be used to distinguish pulmonary from cardiac etiology of hypoxia. Place pt in 100% O2 for 5-10 minutes, and then sample the arterial blood. If no increase in blood O2 content after being on forced O2, you need to think that there is a shunt/cardiac disorder. If you do see an increase in PO2 then the cyanosis/hypoxia may be pulmonary in origin.

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10
Q

Paradoxical embolism

A

emboli from peripheral veins that can bypass the lungs and move directly to systemic circulation. In a patient with a right to left shunt, this can happen and you are at risk of CVA. Can be an initial diagnostic clue in older patients with patent foramen ovale.

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11
Q

Digital Clubbing

A

caused by severe, long standing cyanosis. Fingers and toes can both be affected.

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12
Q

polycythemia

A

very high red blood cell count. can be caused by right to left shunt. Can be evident in complexure of face, hands, feet, will be flushed.

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13
Q

Defects leading to right to left shunt:

A
  1. TOF
  2. TGA
  3. persistant truncus arteriosus
  4. tricuspid atresia
  5. total anomalous pulmonary venous connection
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14
Q

Defects leading to left to right shunt:

A
  1. atrial septal defect
  2. ventricular septal defect
  3. patent ductus arteriosus
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15
Q

Left to right shunt

A

there is increased pulmonary blood flow. Initially there is no cyanosis- you have extra oxygenation really. Chronic right to left shunt will elevate the volume and pressure in pulmonary circulation, and you have remodeling effects that are equivalent to chronic HTN–> end up with pulmonary vascular vasoconstriction, fibrosis and medial hypertrophy (of the ventricular septum)

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16
Q

Eisenmenger syndrome

A

Pulmonary hypertension with a reversed central shunt. The phenomena that happens when you have progression of a large left to right shunt–> you get pulmonary hypertension–> pulmonary vascular resistance –> systemic vascular resistance –> original L to R shunt becomes a R to L shunt and you get cyanosis, hypoxia and erythrocytosis.

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17
Q

Obstructive congenital heart disease

A

the abnormal narrowing of the chambers, valves and blood vessels

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18
Q

atresia

A

complete heart obstructionA

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19
Q

Acyanotic heart lesions

A

Left to right shunt lesions: atrial septal defect, ventricular septal defect, atrioventricular septal defect (AV canal), and patent ductus arteriosus.

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20
Q

Atrial septal defect

A

Most common form of congenital heart disease seen in ADULTS. It is the abnormal, fixed opening in the atrial septum due to incomplete tissue formation. Usually asymptomatic until adulthood. It is not the same as PFO. Seen in 10% of all CHD.

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21
Q

Secundum ASD

A

at the fossa ovals, the most common type of ASD

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22
Q

Primum ASD

A

lower in position and is a form of atrioventricular septal defect. Can be associated with a cleft mitral valve

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23
Q

Sinus Venosus ASD

A

high in the atrial septum, associated with partial anomalous venous return and is the least common of all the ASD’s.

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24
Q

ASD signs and symptoms

A

rarely, there are signs of CHF or other CV issues. Most are asymptomatic, but may have easy fatigability or mild growth failure. Cyanosis does not occur unless pulmonary HTN is present. Can have hyperactive precordium, RV heave and fixed, widely split S2. Can have II-III/VI systolic ejection murmur at the left sternal border, and a mid-diastolic murmur heard over the LLSB.

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25
Q

What causes the systolic & diastolic murmurs of ASD?

A

Systolic murmur is usually caused by increased flow across the pulmonary valve, NOT THE ASD.

 Diastolic murmur is caused by increased flow across the tricupsid valve.
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26
Q

ASD treatment

A

Surgical closure is recommended for Secundum ASD, and this is usually done between 2-5 years old. Surgical correction is done earlier if pt has CHF or significant Pulmonary HTN.

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27
Q

Ventricular septal defect

A

an abnormal opening in the ventricular septum, which allows free communication between the R and L ventricles. Accounts for 25% of all Congenital Heart disease

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28
Q

Perimembranous/membranous VSD

A

the most common

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29
Q

infundibular/subpulmonary/ supracristal VSD

A

involves the RV outflow tract. Spontaneous closure is not common.

30
Q

Muscular VSD

A

can be single or multiple. Often close on their own.

31
Q

AVSD

A

inlet VSD, almost always involves AV valvular abnormalities. Commonly correlated with Down Syndrome.

32
Q

VSD treatment indications

A
  1. large VSD with uncontrolled sxs and continued failure to thrive
  2. 6-12 mo with large VSD and pulmonary HTN
  3. older than 24 mos with a Qp:Qs> 2:1
  4. Supracristal VSD of any size
33
Q

Patent Ductus Arteriosus

A

persistence of the normal fetal vessel that joins the PA to the aorta. Normally closes in the 1st week of life and accounts for 10% of all CHD. Female:Male 2:1. often associated with coarctation of the Aorta and VSD.

34
Q

PDA hemodynamics

A

Blood shunts L to R from the Aorta to the pulmonary artery. In a large PDA, pulmonary artery pressures are equal to systemic pressures and in extreme cases, 70% of cardiac output is shunted through the ductus to pulmonary circulation. This leads to increased pulmonary volumes and pressures, and Pulmonary Vascular disease

35
Q

PDA signs and syxs

A

small PDAs are usually symptomatic. Large PDA’s have sxs of CHF and failure to thrive. Bounding arterial pulses. Widened pulse pressure. Enlarged heart, prominent apical impulse. “machinery” systolic murmur. Mid-diastolic murmur at the apex.

36
Q

Prostaglandins and PDA

A

prostaglandins can be used to the the DA open, or patent. Indomethacin can be used to help close it (inhibitor of prostaglandins), but not in term infants- only in prematures because their lack of lung development allows it to work.

37
Q

PDA tx

A

requires a surgical/catheter closure. Usually done by ligation and division or intravascular coil.

38
Q

AVSD- atrioventricular septal defect

A

results from incomplete fusion of the endocardial cushions, which help to form the lower portion of the atrial septum, the membranous portion of the ventricular septum and the septal leaflets of the tricuspid and mitral valves. Accounts for 4% of all congenital heart disease.

39
Q

complete AVSD

A

there is no real chambering off- open concept of all chambers in the heart.

40
Q

Signs and syxs of AVSD

A

Congestive HF in infancy, Failure to thrive, recurrent respiratory infections, exercise intolerance, late cyanosis from pulmonary vascular disease with R to L shunt, hyperactive precordium, normal-accentuated S1, wide fixed split of S2, pulmonary systolic ejection murmur w/thrill, holosystolic murmur @ apex with radiation to the axilla, Mid-diastolic rumbling murmur @ left sternal border and marked cardiac enlargement on CXR.

41
Q

AVSD treatment

A

Surgery is always required. Mortality increases as the ASVD becomes more “complete”.

42
Q

Types of Obstructive Heart Lesions

A

Pulmonary stenosis, Aortic stenosis and coarctation of the aorta.

43
Q

Coarctation of the Aorta

A

narrowing of the aorta at varying points anywhere along the arch. 98% are juxtaductal- next to the ductus arterioles. Male : female 2 or 3:1
Accounts for 7% of all congenital heart defects.

44
Q

With what other heart anomaly is coarctation associated?

A

Bicuspid aortic valve, seen in > 70% of cases.

45
Q

What genetic syndrome is coarctation seen in?

A

Turner’s Syndrome

46
Q

Coarctation of the Aorta hemodynamics

A

Obstruction of the left ventricular outflow –> pressure induced hypertrophy of the LV

47
Q

Coarctation of the aorta signs and symptoms

A

Diminuation or absence of femoral pulses. Higher BP in upper extremities compared to lower extremities. 90% have systolic hypertension of the UE, and there is typically a pulse discrepancy between the right and left arms. II/VI systolic ejection murmur at the left sternal border, and cardiomegaly, rib notching on CXR (b/c of engorged collateral circulation)

48
Q

Coarctation of the aorta Tx

A

Maintain the ductus with prostaglandin E, if severe coarctation. Surgical intervtion usually takes place to prevent LV dysfunction. Re-coarctation can occur, and if this happens balloon angioplasty is usually done.

49
Q

Pulmonary stenosis

A

obstruction of the region of either the pulmonary valve or the sub pulmonary ventricular outflow tract. Accounts for 7-10% of all CHD. May be bicuspid or a fusion of 2+ leaflets, and may be in conjunction with an intact or not-intact ventricular septum.

50
Q

What syndrome is Pulmonary Stenosis associated with?

A

Noonan’s Syndrome, secondary to valve dysplasia.

51
Q

___ is indicative of Critical pulmonary stenosis

A

Cyanosis

52
Q

Mild pulmonary stenosis

A
53
Q

Moderate to severe pulmonary stenosis

A

30-60mmHg, will have prominent jugular a-wave and RV lift. Split S2 with a delay. Ejection click, followed by a systolic murmur.

54
Q

Pulmonary Stenosis Tx

A

need to relieve the stenosis in moderate-severe cases. Do this by a balloon valvuloplasty

55
Q

Aortic Stenosis

A

obstruction to the outflow from the left ventricle at or near the aortic valve that causes a systolic pressure gradient of more than 10mmHG. Get pressure hypertrophy of the LV and LA with obstruction to flow from the LV. There are 3 types.

56
Q

Valvular Aortic stenosis

A

most common type of aortic stenosis

57
Q

Subvalvular aortic stenosis

A

involves the left outflow tract

58
Q

Supravalvular aortic stenosis

A

involves the ascending aorta and is the least common

59
Q

Which syndrome is supravalvular stenosis found in?

A

Williams syndrome

60
Q

Mild Aortic stenosis

A

0-25mmHg. Present with exercise intolerance

61
Q

Moderate Aortic stenosis

A

25-50 mmHG. Present with chest pain, dyspnea on exertion, dizziness and syncope

62
Q

Severe Aortic stenosis

A

50-75 mmHg. Present with weak pulses, left sided HF and potentially sudden death

63
Q

Critical Aortic Stenosis

A

> 75mmHg

64
Q

Aortic stenosis general signs

A

LV thrust at Apex. Systolic thrill at the right base. Ejection click, III-IV/VI systolic murmur @ right sternal border/left sternal border with radiation to the carotids.

65
Q

Aortic Stenosis tx

A

surgery does not offer a cure, so it is only drone for symptomatic pts with a resting gradient of 60-80mmHg, or if subaortic stenosis with gradient of 40-50mmHg. Tx is balloon valvuloplasty. No competitive sports for moderate to severe cases.

66
Q

Cyanotic Diseases

A

Right to left shunts: Tetrology of Fallot, Transposition of the great arteries, Persistent truncus arterioles, tricuspid atresia, and total anomalous pulmonary venous connection.

67
Q

4 cardinal features of TOF

A
  1. Narrowing of the pulmonary valve
  2. thickening of the wall of the right ventricle (will have boot shaped heart)
  3. Displacement of the aorta over the ventricular septal defect.
  4. Ventricular septal defect- opening between the left and right ventricles.
68
Q

Pink TOF

A

A mild obstruction TOF, its more like an isolated Ventricular septal defect and the shunt may be Left to right. No cyanosis

69
Q

Tet spell

A

children with TOF exhibit bluish skin during episodes of crying after feeding.

70
Q

Squatting and Knee-chest position

A

Behavioral manifestations of TOF patients.

71
Q

Hypoplastic left heart syndrome

A

The left ventricle is underdeveloped and too small.
The mitral valves is not formed or is very small.
The aortic valve is not formed or is very small.
The ascending portion of the aorta is underdeveloped or is too small.
Often, babies with hypoplastic left heart syndrome have an ASD

72
Q

Significant mortality and risk of sudden cardiac death for adults in which Congenital heart defects?

A

Coarctation of the aorta, TOF, dTGA and Aortic stenosis

Pathophysiology- Cardio (3 decks)

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