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Cardiovascular II > Congenital heart conditions > Flashcards

Congenital heart conditions Flashcards

1
Q

Congenital heart condition associated with single gene mutations

A

Tetralogy of Fallot

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2
Q

Congenital heart defect associated with down syndrome

A

Common AV canal –> large primum ASD, VSD, and common AV valve

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3
Q

Congenital heart defects associated with Turner syndrome

A

Bicuspid aortic valve
Coarctation of the aorta

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4
Q

Congenital heart defect associated with Williams syndrome

A

Supravalvular AS

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5
Q

Congenital heart defects associated with DiGeorge syndrome

A

Congenital abnormalities of the cardiac outflow tract –> tetralogy of Fallot, truncus arteriosus

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6
Q

Characteristics of Williams syndrome

A

Mental retardation
Hypercalcemia
Short stature
Facial abnormalities
HTN

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7
Q

Features of DiGeorge syndrome

A

Cardiac abnormalities
Abnormal facial appearance
Thymic aplasia
Cleft palate
Hypocalcemia

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8
Q

Pathogenesis of DiGeorge syndrome

A

Deletion in chromosome 22 affecting TBX1 transcriptional factor causes abnormal development of 4th pharyngeal arch, and 3rd-4th pharyngeal pouches

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9
Q

Congenital heart defects associated with poorly controlled GDM

A

Transposition of great vessels
VSD

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10
Q

Congenital heart defects associated with alcohol intake in pregnancy

A

PV stenosis
VSD

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11
Q

Congenital heart defects associated with maternal rubella infection during gestation

A

PDA
PV stenosis

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12
Q

Congenital heart defect associated with maternal lithium use

A

Ebstein anomaly

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13
Q

Complications of L to R shunts

A

Volume overload in R heart
Pulmonary HTN
LV hypertrophy
Shunt reversal

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14
Q

Types of R to L shunts

A

Tetralogy of Fallot
Transposition of the great arteries
Tricuspid atresia
Persistent truncus arteriosus
Total anomalous pulmonary venous return

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15
Q

Clinical signs of R to L shunt

A

Hypoxemia
Cyanosis
Hypertrophic osteoarthropathy
Polycythemia

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16
Q

Complications of R to L shunt

A

Secondary polycythemia
Digital clubbing
Increased risk of infective endocarditis before or after corrective surgery
Metastatic abscesses
Cyanosis

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17
Q

Most common ASD in down syndrome

A

Of ostium primum

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18
Q

Most common type of ASD

A

Of septum secundum

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19
Q

Clinical findings in adults with ASD

A

RV heave
Widened, fixed S2
Systolic murmur at upper-left sternal border
Decreased stamina and palpitations due to atrial tachyarrhythmias resulting from RA enlargement

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20
Q

Conditions when PFO becomes clinically significant

A

Pulmonary HTN
RHF

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21
Q

Risk factor with PFO

A

Paradoxical embolism

22
Q

Congenital diseases associated with VSD

A

Cri du chat syndrome
Fetal alcohol syndrome

23
Q

Early symptoms of HF in infants with large VSD

A

Tachypnea
Poor feeding
Failure to thrive
Frequent lower respiratory infections

24
Q

Harsh holosystolic murmur best heard at the L sternal border. Mid-diastolic rumbling murmur can be heard at the apex.

A

VSD

25
Q

Reasons for closure of ductus arteriosus after birth

A

Increased arterial oxygenation
Decreased pulmonary vascular resistance
Declining levels of prostaglandin E2

26
Q

Reasons for delayed or absent closure of ductus arteriosus

A

Hypoxia - respiratory distress or heart disease
When associated with other congenital defects

27
Q

Medication used to close PDA

A

Indomethacin

28
Q

Adult remnant of ductus arteriosus

A

Ligamentum arteriosum

29
Q

Eisenmenger syndrome

A

Reversal of a L to R shunt

30
Q

Results from anterosuperior displacement of the infundibular septum

A

Tetralogy of Fallot

31
Q

Four cardinal features of Tetralogy of Fallot

A

Pulmonary infundibular stenosis
RV hypertrophy
Overriding aorta
VSD

32
Q

Reason for RV hypertrophy in tetralogy of Fallot

A

Due to high pressure load placed on RV by pulmonary stenosis

33
Q

Manifestations of Tet spells

A

Irritability
Cyanosis
Hyperventilation
Occasionally syncope or convulsions

34
Q

Reason for Tet spells

A

Systemic vasodilation results in an increased R to L shunt in tetralogy of Fallot

35
Q

S2 in tetralogy of Fallot

A

Single, no split

36
Q

Murmur in tetralogy of Fallot

A

Systolic ejection murmur heard best at the upper L sternal border

37
Q

CXR findings in tetralogy of Fallot

A

Prominent RV and decreased size of main pulmonary A segment –> boot shaped heart

38
Q

ECG findings in tetralogy of Fallot

A

RV hypertrophy with R axis deviation

39
Q

Surgical treatment of transposition of great vessels

A

Creation of interatrial communication using balloon catheter

40
Q

Common trunk for aorta, pulmonary A, and coronary arteries. Associated with VSD.

A

Persistent truncus arteriosus

41
Q

Downward displacement of an abnormal tricuspid valve into an underdeveloped RV. Divides RV into atrialized dilated portion and functional reduced portion.

A

Ebstein anomaly

42
Q

Tubular hypoplasia of the aortic arch proximal to PDA

A

Infantile form of coarctation of the aorta

43
Q

Discrete ridge-like infolding of the aorta just opposite to the ligamentum arteriosum distal to the arch vessels

A

Adult form of coarctation of the aorta

44
Q

Compensatory alterations in uncorrected coarctation of the aorta

A

LV hypertrophy
Dilation of collaterals from intercostal arteries

45
Q

When will systolic BP in the R arm is greater than the L arm

A

Coarctation of aorta proximal to branching of L subclavian A

46
Q

Murmur of aortic coarctation

A

Midsystolic ejection murmur, may be audible over chest and/or back

47
Q

CXR findings in aortic coarctation

A

Notching of inferior surface of ribs

48
Q

Possible complications proximal to aortic coarctation

A

Increased UE SBP
Dilation of aortic root and dissection
Increased risk for developing berry aneurysms –> increased cerebral blood flow

49
Q

Possible complications distal to aortic coarctation

A

Decreased in SBP and pulse amplitude in LE
Leg claudication
HTN –> activation of RAAS
Collateral circulation

50
Q

Group of heart defects characterized by involvement of the atrial septum, ventricular septum, and 1 or both of the AV valves

A

Endocardial cushion defects

Cardiovascular II (29 decks)

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