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Residência Pediatria > Cardio 2 > Flashcards

Cardio 2 Flashcards

Tetralogy of Fallot

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1
Q

Primary defect in Tetralogy of Fallot:

A

Anterior deviation of the infundibular septum

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2
Q

Muscular septum that separates the aortic and pulmonary outflows:

A

Infundibular septum

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3
Q

Cite the 4 componentes of Tetralogy of Fallot:

A

Pulmonary stenosis; ventricular septal defect; dextroposition of the aorta; RV hypertrophy

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4
Q

Constitutes an extreme form of ToF:

A

Complete obstruction of RV outflow

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5
Q

Determinants of patient’s cyanosis and age of presentation: (2)

A

Degree of pulmonary outflow obstruction; ductus arteriosus patency

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6
Q

In ToF; Pulmonary valve usually mantains its three flaps T/F

A

False; usually bicuspid or unicuspid; ocasionally can be the only site of stenosis

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7
Q

In ToF; In the absence of pulmonary artery patency…

A

pulmonary flow may be provided by PDA or by “aortopulmonary collateral arteries”

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8
Q

In ToF; the aortic arch is right sided in ___ of cases.

A

20%; the aortic root is large and “overrides the VSD to varying degrees

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9
Q

In ToF; an aorta overriding the VSD by >50% can be classified as…

A

A form of double-outlet right ventricle; dynamics and method of repair are the same

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10
Q

In ToF; Systemic venous return to the right atrium and ventricle are diminished T/F

A

False; venous return is normal

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11
Q

In ToF; Pulmonary artery pressure is…

A

Either normal or lower than normal

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12
Q

In ToF; a mild to moderate RV outflow obstruction and a balanced shunt presents as…

A

An acyanotic or “pink” tetralogy of Fallot

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13
Q

In ToF; a severe RV outflow obstruction and important shunt presents as…

A

Severe cyanosis from birth and worsens with DA clossure

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14
Q

In ToF; Mild degrees of RV outflow obstruction develop cyanosis…

A

in the 1st few months of life

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15
Q

In ToF; Severe degrees of RV outflow obstruction develop cyanosis…

A

At birth; pulmonary bloodflow may depend solely on PDA

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16
Q

In ToF; Severe degrees of RV outflow obstruction may develop circulatory collapse if…

A

PDA begins to close in the 1st few hrs or days of life

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17
Q

In ToF; Older children with long-standing cyanosis who have not undergone surgery may present with… (3)

A

Dusky blue skin; gray sclerae with engorged blood vessels; finger clubbing

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18
Q

In ToF; Dyspneia occurs…

A

On exertion; the may play actively for a short time.

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19
Q

In ToF; Paroxismal hypercyanotic attaks (blue spells) are a problem during…

A

the first year of life

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20
Q

In ToF; Paroxismal hypercyanotic attacks (blue spells) symptoms: (5)

A

Infant becomes hyperpneic; restless; cyanosis increases; gasping and syncope may follow

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21
Q

In ToF; Paroxismal hypercyanotic attaks (blue spells) occur most frequently:

A

In the morning on initially awakening or after vigorous crying

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22
Q

In ToF; Paroxismal hypercyanotic attaks (blue spells) systolic murmur may…

A

Temporarily disappear or decrease in intensity as flow acros the RVOT “diminishes”

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23
Q

In ToF; Paroxismal hypercyanotic attaks (blue spells) may last…

A

From a few minutes to a few hours

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24
Q

In ToF; Paroxismal hypercyanotic attaks (blue spells) are followed by…

A

Weakness and sleep

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25
Q

In ToF; Paroxismal hypercyanotic attaks (blue spells); when severe; may lead to…

A

Unconsciousness; convulsions or hemiparesis

26
Q

In ToF; Paroxismal hypercyanotic attaks (blue spells) can result in ___ hypoxemia and metabolic…

A

Severe; acidosis

27
Q

In ToF; One procedure for blue spells consist of placing the infant on ___ in the ___ position.

A

The abdomen; knee-chest; also important to check for constrictive clothing

28
Q

In ToF; Administration of O2 during a blue spell can reverse cyanosis T/F

A

False; intracardiac shunting is unaffected by inspired O2; O2 should be provided nonetheless

29
Q

In ToF; Morphine should be administered via ___ to aid in calming the infant

A

Subcutaneous; not to exceed 0.2mg/kg

30
Q

In ToF; Blood samples should be obtained during spells T/F

A

False; may cause further agitation and be counterproductive

31
Q

In ToF; Rapid acidosis may develop if PaO2<40; prolonged or severe spells may need…

A

Rapid correction with IV BIC

32
Q

In TOF; Refractory spells may need…

A

Intubation and anesthetic sedation

33
Q

In ToF; Drugs that increase SVR (phenylephrine)…

A

Can improve RC outflow; decrease R-to-L shunt and improve symptoms

34
Q

In ToF; __-Adrenergic blockade has also been used to abort spells

A

Beta; Propanolol 0.1mg/kg given slowly to a max of 0.2/kg

35
Q

In ToF; Chronically SaO2<70 may lead to…

A

Growth and development delay

36
Q

In ToF; Pulse; venous and arterial pressures are…

A

Normal

37
Q

In ToF; The classical murmur is…

A

Systolic; loud; harsh; most intense at the left sternal border

38
Q

In ToF; Typical AP Rx shows:

A

Narrow base; concavity of the L heart border ocupied by pulmonary art; “Normal heart size”; rounded right border

39
Q

In ToF; The ECG shows:

A

Right axis deviation and RVH

40
Q

In ToF; Prefered method of Dx:

A

2D echocardiography with doppler; also useful in determining PDA role in pulmonary flow

41
Q

In ToF; During catheterization shows a SO2 of 75-85% in…

A

Moderately cyanotic patients; pink fallots can have normal SO2

42
Q

In ToF; Most children undergo repair (some cases palliation) during…

A

The first months of life

43
Q

In ToF; Polycythemia and dehydration can lead to…

A

Cerebral tromboses; usually in the cerebral veins or dural sinuses

44
Q

In ToF; Heat failure is more common in pink fallots T/F

A

True; undeveloped mechanism to tolerate acute hypoxemia

45
Q

In ToF; an concomitant atrioventricular septal defect is often associated with…

A

Down syndrome

46
Q

In ToF; If radiographic appearance of pulmonary vasculature differs on the 2 sides…

A

Absence of a branch pulmonary artery should be suspected; most often to the left

47
Q

In ToF; Can be associated with ___ syndrome

A

DiGeorge; CATCH 22 (Cardiac def; Abnormal facies; Thymic hypoplasia; Cleft palate; HypoCa; deletion of chrom 22

48
Q

In ToF; therapy is aimed at…

A

providing immediate increase in pulmonary blood flow

49
Q

In ToF; Normal body temp must be mantained due to…

A

increased o2 consumption during cold

50
Q

In ToF; DA patency can be prolonged with…

A

Prostaglandin E; 0.01-0.2 mcg/kg/min; can cause apnea

51
Q

In ToF; Acyanotic patiens can progress fairly quickly to cyanotic episodes T/F

A

True; they require careful observation

52
Q

In ToF; Propanolol can be used if Cx is expected to be delayed T/F

A

True; 0.5-1mg/kg 6/6h; reduces frequency and severity of spells

53
Q

In ToF; Infants with severe cyanosis have 2 Cx options:

A

Corrective open heart surgery; Palliative shunts (Blalock-Taussig shunt)

54
Q

In ToF; infants with mild to moderate cyanosis can perform Cx electively at…

A

4-6mo if adequate growth and lack of spells

55
Q

In ToF; Corrective Cx consist of…

A

Resecting obstructive muscle bundles and closure of VSD

56
Q

In ToF; Blalock-Taussig shunt is performed to…

A

Augment pulmonary artery blood flow by systemic-to-pulmonary artery shunt

57
Q

In ToF; Blalock-Taussig shunt consists of…

A

Conduit anastomosed side to side from the subclavian artery to the homolateral branch of the pulmonary artery

58
Q

In ToF; Blalock-Taussig shunt complications: (5)

A

Chylothorax; diaphragmatic paralysis; Horner syndrome; pulm overcirculation and HF

59
Q

In ToF; Blalock-Taussig shunt a ___ indicates functioning anastomosis.:

A

Continuous murmur over the lung fields; may not be heard until several days

60
Q

In ToF; Blalock-Taussig shunt; rapid cyanosis may develop after the Cx if…

A

Thrombosis of the shunt; emergency Cx

Residência Pediatria (12 decks)

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