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Cardiology Block 1 > Congenital Anomalies > Flashcards

Congenital Anomalies Flashcards

1
Q

What defects are associated with 22q11 deletions?

A

Truncus arteriosus

Teratology of Fallot

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2
Q

What congenital cardiac defects are associated with Down Syndrome?

A

ASD

VSD

AV septal defect (endocardial cushion defect)

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3
Q

What congenital cardiac defects are associated with Congenital rubella?

A

Septal defects

PDA

Pulmonary artery stenosis

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4
Q

What congenital cardiac defects are associated with Turner Syndrome?

A

Coarctation of the aorta

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5
Q

What congenital cardiac defects are associated with Marfan’s Syndrome?

A

Aortic insufficiency and dissection

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6
Q

What congenital cardiac defects are associated with being an infant with a diabetic mother?

A

Transposition of great vessels

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7
Q

Normally, the pressure in the ____ heart is stronger than in the ____ side of the heart.

A

Normally, the pressure in the left heart is stronger than in the right side of the heart.

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8
Q

Acyanotic heart defect characteristics and examples:

A

Intracardiac or vascular stenosis

Valvular regurgitations

Left to right shunts

Examples:

  • Atrial septal defect
  • Ventricular septal defect
  • Patent ductus arteriosus
  • Congenital aortic stenosis
  • Pulmonic stenosis
  • Coarctation of the aorta
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9
Q

Cyanotic heart defect characteristics and examples:

A

Poorly oxygenated blood shunted from right heart to left

Blood bypasses fetal lungs

O2 sat 80-85%

The 5 T’s!

  1. Truncus Arteriosus
  2. Transposition of the great vessels
  3. Tricuspid atresia
  4. Tetralology of Fallot
  5. TAPVR – Total anomalous pulmonary venous return
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10
Q

What is the incidence of atrial septal defects?

Which kind is the most common?

A

1 in 1,500 live births

Ostium secundum is the most common

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11
Q

What are the four common forms of atrial septal defects?

A

Ostium secundum (most common)

Ostium primum defect

Sinus venosus defect

Patent foramen ovale (PFO)

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12
Q

Ostium secundum

A

Most common form of ASD

Due to inadequate formation of spetum secundum or excessive resorption of septum primum

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13
Q

Ostium primum defect:

A

Type of ASD

Inferior portion of the septum fails to fuse with endocardial cushions.

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14
Q

Sinus venosus defect:

A

Type of ASD, BUT not actually a true ASD - defects don’t actually occur in intra-atrial septum.

Sinus venosus defect (IVC or SVC) – related to ASDs, but mophologically distinct! “Unroofing” of area between pulm veins and RA. May have opening between LA…pathopys is similar

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15
Q

Patent Foramen Ovale:

A

20% of population!

No missing tissue, just persistence of fetal anatomy…atrial septa fail to fuse (should have occurred by 6 months).

Only an issue if RA pressures >> LA pressures.

Can be source of paradoxical emboli.

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16
Q

ASD pathophysiology:

A

Left to right shunt

Flow limited by defect size and ventricle compliance

Volume overload of the right ventricle.

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17
Q

What is the incidence of VSD?

A

1.5-3.5 per 1,000 live births

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18
Q

What type of VSD is most common? least common?

A

Membranous (70%)

Muscular (30%)

Rarely adjacent to aortic or AV valves

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19
Q

VSD pathophysiology

A

Hemodynamic changes related to defect size, SVR, and PVR

Restrictive (size of defect is the “rate limited” step for flow) vs Nonrestrictive defect (BIG defect. Pressure gradients are what determine flow)

RV, pulmonary circulation, LA and LV may all experience volume overload with time

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20
Q

What is the difference between a restrictive vs. nonrestrictive VSD?

A

Restrictive – size of defect is the “rate limited” step for flow

Non-restriticve – BIG defect. Pressure gradients are what determine flow

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21
Q

What symptoms suggest congestive heart failure in the infant?

A

Pulmonary venous congestion (left heart failure)

  • Tachypnea
  • Respiratory distress
  • Difficulty feeding - cyanosis, sweating

Systemic Venous Congestion (Right heart failure)

  • Hepatosplenomegaly
  • Edema/ascites - less common
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22
Q

What is the incidence of PDA?

A

1 in 2500 - 5000 live births

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23
Q
A
24
Q

PDA is associated with what risk factors?

A

Maternal, fetal, and environmental risk factors:

  • First trimester rubella infection,
  • birth at high altitude (decrease oxygen tension),
  • prematurity (tissue less responsive to vasoactive substances for closure),
  • sepsis,
  • stress,
  • hypoxia/hypercarbia/acidosis.
25
Q

The ductus arteriosus usually closes due to increased _____ and decreased ____.

A

The ductus arteriosus usually closes due to increased oxygen tension and decreased circulating prostaglandin.

26
Q

PDA pathophysiology

A

LA and LV overload

Potential LV dysfunction

Decreased diastolic systemic perfusion

Flow across the PDA depends of size of ductus (width and length) and relative SVR and PVR. As PVR drops with age, more L –> R shunt = pulmonary and LA/LV volume overload. …can lead to LV dysfunction. Right heart unaffected unless pulm dz develops and flow reversal.

27
Q

Treatment options for PDA:

A

1st line - Indomethacin

Percutaneous closure

Surgical closure

28
Q

What is Eisenmenger’s Syndrome?

A

THIS IS A CYANOTIC CONDITION! Blue kids!

Uncorrected VSD/ASD/PDA causes compensatory pulmonary vascular hypertrophy

Progressive pulmonary HTN

Shunt reversal: now Right –> Left!

Cyanosis, clubbing, polycythemia

29
Q

What population does aortic stenosis occur in?

A

5 of 10,000 live births

Males more than Females

20% have additional cardiac abnormality

30
Q

Aortic stenosis is mosty commonly due to a ____ abnormality, such as a _____ valve.

A

Aortic stenosis is mosty commonly due to a structural abnormality, such as a bicuspid aortic valve.

31
Q

Is a bicuspid aortic valve alone problematic/dangerous?

A

Not really - the bicuspid valve is asymptomatic at birth, BUT will go on to fibrose and calcify with age - this is the most common cause of Aortic Stenosis in adulthood.

Can be critical and ductal dependent lesion.

32
Q

Aortic stenosis pathophysiology:

A

High LV systemic pressure required (LVH)

High velocity jet may impact proximal aorta

Narrowed valvular orifice..requires lots of LV systemic pressure to pump blood.

Causes LVH…high velocity jet may impact proximal aorta and causes dilation of proximal aorta!

33
Q

Pulmonic stenosis is associated with a ____ obstruction

A

Pulmonic stenosis is associated with a right ventricular outflow tract obstruction

34
Q

What causes pulmonic stenosis?

A

Abnormal formation/regression of subendocardial mesenchymal tissue

Multiple levels of RVOT obstruction possible – VALVAR is most common

Valves can be congenitally fused, RV body muscle hypertrophy, PA obstruction. Varying severity.

35
Q

Pathophysiology of Pulmonic Stenosis:

A

Impaired RV outflow

Right ventricular hypertrophy

May cause RV failure

Impaired RV outflow, increased RV pressures and chamber hypertrophy. Mild pulm stenosis usually doesn’t effect RV function, severe PS may result in Right heart failure

36
Q

What is the incidence of coarctation of the aorta?

A

1 in 6000 live births

37
Q

What is the etiology of coarctation of the aorta?

A

Etiology unclear - 2 theories:

1st theory – no flow, no grow (esp if aortic stenosis…decreased LV outflow during fetal period)

2nd theory – ectopic muscular tissue from ductus extends into aorta and constricts following birth (when ductus closes!)

38
Q

Where does coarctation typically occur?

A

Distal to the arch, proximal to the ductus arteriosus.

Typically Juxtaductal

Area and degree of narrowing are critical for presentation.

39
Q

Coarctation pathophysiology:

A

Distal blood flow may be compromised

May have discrepancy between upper extremity and lower extremity pressure (high in upper, lower in lower)

LVH

Collateral formation

Might be ductal dependent; based on severity

40
Q

Persistent truncus arteriosus:

A

Cyanotic!!

Failure of truncus arteriosus to divide into pulmonary trunk and aorta

41
Q

Most patients with a persistent truncus arteriosus have an accompanying ___.

A

ASD

42
Q

What is the incidence of Transposition of the Great Arteries?

A

40 in 10,000 live births

43
Q

What is the most common cause of cyanosis in neonates? Infants?

A

Neonates = Transposition of the Great Arteries

Infants = Teratology of Fallot

44
Q

How does Transposition of the Great Arteries arise?

A

Failure of aorticopulmonary septum to spiral in a normal fashion.

Have 2 parallel circuits

45
Q

Is TGA compatible with life? Why or why not?

A

Not compatible with life unless a shunt is present to allow adequate mxing of blood (eg VSD, ASD, PDA)

Most infants die early without intervention!

46
Q

Tricuspid atresia:

A

Absent tricuspid valve and hypoplastic RV

Requires both ASD and VSD for viability

47
Q

What is required for viability in patients with Tricuspid atresia?

A

ASD and VSD

48
Q

What is the most common form of cyanotic congenital heart disease?

A

Teratology of Fallot

49
Q

Incidence of Teratology of Fallot:

A

5 in 10,000 live births

50
Q

Teratology of Fallot is often associated with other abnormalities, such as ____, ____, and ____.

A

Teratology of Fallot is often associated with other abnormalities, such as right-sided arch, ASD, and left coronary anomalies.

51
Q

What chromosomal deletion is associated with Teratology of Fallot?

A

22q11

52
Q

What are the 4 criteria of Teratology of Fallot?

A
  1. VSD - anterior malalignment of the interventricular septum
  2. Pulmonic stenosis – obstruction
  3. Overriding aorta – receives blood from both ventricles
  4. Right Ventricular Hypertrophy – due to pressure load on RV
53
Q

Tetralogy of Fallot pathophysiology:

A
  • Pulmonic stenosis causes resistance to RVOT flow
  • R –> L shunt causes cyanosis
  • Magnitude of shunt depends on degree of stenosis and balance of PVR and SVR

(Isolated VSDs usually have left to right shunt. In TOF, Pulmonic stenosis causes right to left shunt)

54
Q

What is a Tet Spell?

A
  • Dypsnea on “exertion” (in children, exertion is eating and crying)
  • Alterations in PVR and SVR
  • Worsening R –> L shunt reversal causes cyanosis
  • Resolve by decreasing R –> L shunt…squatting!
55
Q

Total Anomalous Pulmonary Venous Return (TAPVR):

A

Pulmonary veins drain into the right heart circulation

Associated with ASD +/- PDA to allow for right to left shunt to maintain CO

56
Q

What cardiac defects are associated with TAPVR?

A

ASD and maybe PDA to allow for right to left shunt to maintain CO

Cardiology Block 1 (9 decks)

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