Cardiology - Congenital heart defects Flashcards

(53 cards)

1
Q

A group of cardiac defects characterized by left-to-right sound which causes pulmonary hypertension and RA and PV hypertrophy

A

acyanotic congenital heart defects

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2
Q

Common acyanotic congenital heart defects

A
  • ASD
  • VSD
  • AVSD
  • PFO
  • PDA
  • Coarctation of the aorta
  • Pulmonary valve stenosis
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3
Q

Defect in the inter-atrial septum causing a left to right shunt and RA and RV volume overload

A

atrial septal defect (ASD)

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4
Q

Conditions associated with ASD

A
  • Down syndrome
  • Fetal alcohol syndrome
  • TORCH infections
  • Holt-Oram syndrome (hand-heart syndrome)
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5
Q

Auscultation findings of ASD

A
  • mid-systolic ejection murmur
  • WIDE split second heart sound (S2) over the second left intercostal space. The split does not change with respiration (fixed)
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6
Q

Patients with ASD are at increased risk for what complication?

A
  • Paradoxical embolism (increased risk of ischemic stroke) - embolus from the venous circulation passes from the RA through the ASD to the LA and enters the arterial circulation to cause a stroke
  • heart failure
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7
Q

Defect in the inter-ventricular septum causing a ventricular left to right shunt, RV and LV volume overload and pulmonary hypertension.

A

ventricular septal defect (VSD)

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8
Q

Conditions associated with VSD

A
  • maternal diabetes
  • Down syndrome
  • Edward syndrome
  • Patau syndrome
  • TORCH infection
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9
Q

Auscultation findings for VSD

A
  • harsh holosystolic murmur over the left lower sternal border
  • mid-systolic murmur over the cardiac apex
  • loud pulmonic S2 (pulmonary hypertension)
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10
Q

Variable deficiency in atrioventricular (AV) septa that can cause AV left to right shunt, AV valve regurgitation and pulmonary hypertension

A

atrioventricular septal defect (AVSD)

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11
Q

Condition associated with AVSD

A

Down syndrome

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12
Q

Persistence of the ductus arteriosus postnatally causing a left to right shunt from the aorta to the pulmonary artery

A

patent ductus arteriosus (PDA)

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13
Q

Conditions associated with PDA

A
  • Fetal alcohol syndrome
  • Congenital rubella
  • Down syndrome
  • prematurity
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14
Q

A complication of acyanotic heart disease in which the shunt direction reverses and cyanotic heart disease develops.

A

Eisenmenger syndrome

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15
Q

Findings in PDA

A
  • Bounding peripheral pulses
  • wide pulse pressure
  • laterally displaced heave
  • loud, continuous murmur heard best at the left infraclavicular region and loudest at S2 (machinery murmur)
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16
Q

Narrowing of the aortic arch causing LV outflow tract obstruction, collateral circulation, difference in blood pressures and distal systemic hypo-perfusion

A

Coarctation of the aorta

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17
Q

Conditions associated with Coarctation of the aorta

A

Turner syndrome

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18
Q

Coarctation of the aorta is often accompanied by

A
  • bicuspid aortic valve
  • VSD
  • PDA
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19
Q

Findings in Coarctation of the aorta

A
  • cyanosis of the lower extremities
  • brachial-femoral delay (weak femoral pulses)
  • increased BP in upper extremities and decreased BP in the lower extremities
  • cold feet and lower extremity claudication upon physical exertion
  • systolic ejection murmur over the left posterior hemithorax
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20
Q

Findings of Coarctation of aorta on CXR

A
  • Figure of 3 sign
  • rib notching (sign of chronic disease not usually visible before age 5)
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21
Q

Dilation of the aorta and left subclavian artery causing an hourglass-like narrowing of the aorta and a 3-shaped outline on CXR.

A

Figure of 3 sign

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22
Q

Collateral circulation between the internal thoracic and intercostal arteries on CXR usually in children > 5 yo

23
Q

Narrowing at the pulmonary valve causing RV outflow tract obstruction and RV hypertrophy

A

Pulmonary valve stenosis

24
Q

Conditions associated with pulmonary valve stenosis

A

Congenital rubella

25
Systolic murmur heard best over the second left intercostal space and wide split S2
Pulmonary valve stenosis
26
This condition is caused by oxygenated blood from the lungs being shunted back into the pulmonary circulation via and ASD, VSD or PDA.
pulmonary hypertension
27
Left-to-Right shunts cause
LateR cyanosis
28
Right-to-Left shunts cause
eaRLy cyanosis
29
Why is there cyanosis in right-to-left shunt?
Deoxygenated blood from the right heart is shunted to the left heart and enters the systemic circulation causing cyanosis
30
Clinical features of acyanotic heart defects
- failure to thrive - recurrent bronchopulmonary infections - exercise intolerance - right heart failure - left heart failure
31
A group of heart defects characterized by right-to-left cardiac shunt which leads to deoxygenated blood entering the systemic circulation causing hypoxemia and cyanosis.
Cyanotic heart defects
32
What are the cyanotic heart defects?
- tetralogy of Fallot - transposition of the great vessels - tricuspid valve atresia - Ebstein anomaly - persistent truncus arteriosus - hypoplastic left heart syndrome
33
5 Ts of cyanotic heart defects
- Tetralogy of Fallot - Transposition of the great vessels - Tricuspid valve atresia - Total anomalous pulmonary venous return - Truncus arteriosus
34
What are the 4 defects of Tetralogy of Fallot?
P - pulmonary infundibular stenosis causing RV outflow tract obstruction R - RV hypertrophy O - Overriding aorta (displaced above the VSD) V - VSD
35
Intermittent hyper-cyanotic, hypoxic episodes with peak incidence at 2-4 months after birth associated with psychological/physical stress (crying, feeding, defecation)
Tet spells
36
Tetralogy of Fallot is most commonly associated with
- DiGeorge syndrome - Down syndrome - maternal alcohol consumption - maternal phenylketonuria - maternal diabetes
37
Cardiac exam findings for Tetralogy of Fallot
- harsh systolic ejection murmur at the left upper sternal border - single S2 - RV heave and systolic thrill
38
Why do Tet babies squat?
squatting increases SVR which decreases the right to left shunt and increases blood flow to the pulmonary circulation
39
An EKG finding characterized by narrow-peaked P waves in inferior leads II, III, aVF as a result of RA enlargement, indicating pulmonary diseases.
P pulmonale
40
Transposition of great vessels is commonly associated with
- maternal diabetes
41
Postnatal cyanosis not affected by exertion or supplemental oxygen, tachypnea and a single, loud S2, no murmur
Transposition of the great vessels
42
Findings for Transposition of the great vessels on CXR
"egg on a string" appearance of he heart - enlarged globular heart resembles an egg lying on its side and the string represents the superior mediastinum that appears narrow due to stress-induced thymus atrophy
43
Central cyanosis within days after birth, tachypnea, single S2 and a holosystolic murmur at the lower left sternal border.
44
Patient survival for tricuspid valve atresia depends on
Interatrial and interventricular communications (ASD, VSD)
45
Tricuspid valve atresia is commonly associated with
- Down syndrome
46
Malformed tricuspid valve leaflets that are displaced into the RV with subsequent tricuspid valve regurgitation and right heart enlargement known as RV atrialization.
Ebstein anomaly
47
Ebstein anomaly is commonly associated with
- prenatal lithium exposure - Wolff-Parkinson-White syndrome - SVT
48
Total anomalous pulmonary venous return (TAPVR) is commonly associated with
- heterotaxy syndromes (incorrect left-right axis arrangement of the thoracic and abdominal organs)
49
Persistent truncus arteriosus is commonly associated with
- DiGeorge syndrome
50
Hypoplastic left heart syndrome is commonly associated with
- Patau syndrome - Edwards syndrome - Turner syndrome - Jacobsen syndrome
51
General clinical features of cyanotic heart defects
- "blue babies" (pale gray or blue skin color caused by cyanosis) - feeding problems, failure to thrive - exertion dyspnea - hypoxemia - heart failure
52
Which congenital heart defects require the PDA to stay open until surgery?
- Transposition of the great arteries - Hypoplastic left heart syndrome - Coarctation of the aorta - Tricuspid valve atresia
53
How do you keep the PDA open?
Prostaglandin E1 infusion