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Cardio Exam 1 > Congenital Heart Defects -Junkins > Flashcards

Congenital Heart Defects -Junkins Flashcards

1
Q

What are the 4 phases of heart development?

A

cardiac tube formation

cardiac tube folding

cardiac tube division

vascular system development

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2
Q

What are the 3 shunts in fetal circulation?

A

Ductus Venosus- hepatic system

Foramen Ovale- between right & left atrium

Ductus Arteriosus- vessel connecting pulmonary artery to descending aorta

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3
Q

Does the fetal IVC contain oxygenated blood or deoxygenated blood? The SVC?

A

IVC=mixed

  • oxygenated blood shunted from mother through ductus venosus (50%) (other 50% from umbilical vein goes to the portal vein to the liver)
  • low oxygenated blood from the systemic veins of the fetus

SVC=deoxygenated blood from the head and UE

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4
Q

Which has a higher oxygen tension in the fetus, IVC or SVC?

A

IVC

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5
Q

What is the distribution of fetal well-oxygenated blood?

A

9% enters coronary arteries and perfuses the myocardium

62% perfuses the brain and upper body

29% perfuses the rest of the fetal body

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6
Q

When does a fetus become a newborn?

A

when the cord is clamped

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7
Q

What happens when the umbilical cord is clamped?

A
  • placenta is separated
  • sudden increase in systemic BP
  • 3 main shunts close

***lungs fill with air and O2 tension increases while systemic vascular resistance increases

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8
Q

*What causes the closure of the fetal structures?

A

baby breathes–> decrease resistance of blood flow through the lungs and increase in blood flow from pulmonary arteries–> increase in oxygen tension –> decrease in PGE1 (high in fetal life due to relative hypoxia) –> ductus arterioles close

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9
Q

Why are many common congenital heart defects well tolerated before birth?

A

because the blood is shunted through the ductus arteriosus and the foramen ovale –> still get enough oxygenated blood

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10
Q

**What are the differences between acyanotic and cyanotic congenital heart lesions?

A

acyanotic:

  • left to right shunts
  • late cyanosis “blue kids”
  • can get blue babies with increase in pulmonary HTN –> increase pressure in the right side > left

cyanotic:
- right to left
- early cyanosis –> blue babies
- O2 sats around 80-85%

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11
Q

What are the 6 acyanotic lesions?

A

ASD- Atrial Septal Defect

VSD-Ventricular Septal Defect

Patent Ductus Arteriosus

Congenital Aortic Stenosis

Pulmonic Stenosis

Coarctation of the Aorta

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12
Q

What happens in ASD?How is an ASD normally diagnosed?

A

ASD: left flows from left atrium to right atrium

CXR: RA and RV dilatation
ECG: RV hypertrophy often with RA enlargement and RBBB
ECHO: RA and RV enlargement
Magnitude and direction of shunt can be detected

Treatment: Direct suture closure or patch

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13
Q

What are the symptoms of ASD?

A

Most are asymptomatic
Dyspnea on exertion
Fatigue
Recurrent lower respiratory tract infections

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14
Q

What is the treatment for ASD?

A

Direct suture closure or patch

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15
Q

*What is a possible complication of a patent foramen ovale? How does this happen?

A

paradoxical embolism

increase in pulmonary trunk pressure can cause an increase in right atrial pressure» left atrial pressure –> can lead to right to left shunting

if get an embolism–> can enter right atria and travel through the patent foramen ovale and then out the left ventricle to enter systemic circulation

increased chances of stroke

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16
Q

*How can a patent foramen ovale be diagnosed?

A

Bubble contrast transthoracic echocardiogram

inject IV full of air and watch on echo as moves from right atria–> left

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17
Q

What are some symptoms of VSD?

A

10%: early congestive heart failure

  • Tachypnea
  • Poor feeding, failure to thrive
  • Frequent lower respiratory tract infections

With Pulmonary vascular disease:
-Dyspnea, cyanosis (from reverse shunting–> right to left flow with increase right ventricular P)

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18
Q

How can a VSD be diagnosed?

A

CXR: Cardiomegaly with large defects

ECG: LA enlargement and RV hypertrophy

ECHO: Magnitude and direction of shunt

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19
Q

What is the treatment for VSD?

A

By age 2 years, 50% of small/moderate sized VSDs undergo spontaneous closure

Surgical correction with congestive heart failure or pulmonary vascular disease

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20
Q

*What are some risk factors for a patent ductus arteriosus?

A

First trimester maternal rubella infection, prematurity, and birth at high altitude

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21
Q

*What are some symptoms of patent ductus arteriosus?

A

If large, early congestive heart failure

If moderate, fatigue, dyspnea, and palpitations

Continuous, machine-like murmur heard best at the left subclavian region

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22
Q

How can a patent ductus arteriosus be diagnosed?

A

If large, CXR shows an enlarged cardiac sillouette

ECG: LA enlargement and LV hypertrophy

ECHO with Doppler flow can visualize defect

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23
Q

What are some symptoms of congenital aortic stenosis?

A

20% have an additional abnormality

Less than 10% show heart failure before age 1 year

When older, may show fatigue, exercise dyspnea, angina pectoris, and syncope

CXR: Enlarged LV
ECG: LV hypertrophy
ECHO: identifies the abnormal structure of the aortic valve

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24
Q

What is the treatment for congenital aortic stenosis?

A

Close follow-up if mild

Immediate repair if severe

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25
Q

What will diagnostic studies show in pulmonic stenosis? how is this treated?

A

CXR: There may be an enlarged RA and RV
ECG: RV hypertrophy
ECHO with Doppler assesses valve and measures the pressure gradient

Treatment
Dilatation of stenotic valve

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26
Q

What is coarctation of the aorta? What is this associated with?

A

pinching of the aorta

associated with Turner syndrome (45, XO)

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27
Q

What symptoms will be seen in coarctation of the aorta?

A
  • differential cyanosis if the ductus arteriosus remains open ==> upper half of the body is perfused well and bottom half appears cyanotic
  • A systolic pressure in the right arm that is 15-20 mmHg greater than that in a leg
  • Weak, delayed femoral pulses
  • Elevated upper extremity blood pressure
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28
Q

What will diagnostic studies show in coarctation of the aorta?

A

CXR: Notching of the inferior surface of the posterior ribs

ECG: LV hypertrophy

Doppler ECHO confirms the diagnosis

MRI demonstrates the length and severity

*imaging to see where the problem is!!

29
Q

Is a pre-ductal or post-ductal coarctation of the aorta better?

A

post-ductal

30
Q

What is the treatment for coarctation of the aorta?

A

In neonates with severe obstruction, give prostaglandin infusion to keep the ductus arteriosus patent until surgery (only helps in post-ductal**)

In children, elective repair may prevent systemic hypertension

31
Q

What are the 5 cyanotic heart lesions?

A

The 5 T’s

Tetralogy of Fallot

Transposition of the Great Arteries

Truncus arteriosus

Tricuspid atresia

TAPVR (Total Anomalous Pulmonary Venous Return)

32
Q

*What genetic defect is Tetralogy of Fallot associated with?

A

micro deletion of 22q11

DiGeorge Syndrome (Truncas Arteriosus, poor immune function, cleft palate, low Ca2+, delayed development)

33
Q

*What are the 4 features of tetralogy of fallot?

A
  1. VSD
  2. Subvalvular pulmonic stenosis
  3. Overriding aorta that receives blood from both ventricles
  4. Right ventricular hypertrophy
34
Q

*What are some symptoms of tetralogy of fallot?

A
  • “Spells” may occur following exertion, feeding or crying
  • Systemic vasodilitation results in an increased right-to-left shunt
  • Irritability, cyanosis, hyperventilation, syncope or convulsions
  • Squatting may increase systemic vascular resistance and decrease the right to left shunt
  • can see tachycardia or increase in RBCs to compensate for low O2 sat
35
Q

What physical findings will be seen in all cyanotic heart lesions?

A

clubbing of fingers and toes and cyanosis die to hypoxia

36
Q

*Which heart defect will have a “boot-shaped heart” on x-ray? Why?

A

tetralogy of fallot due to the right ventricular hypertrophy (with right axis deviation)

37
Q

What is transposition of the great arteries? Who is more likely to get the defect, boys or girls?

A

aorta is connected to the right ventricle and the pulmonary artery is connected to the left ventricle

boys=60-70%

38
Q

What does the x-ray in a kiddo with transposition of the great arteries look like? How do you make a definitive diagnosis?

A

“egg on a string”

do NOT diagnose based off his

diagnose based off ECHO

39
Q

*What should you do if a newborn comes in blue??

A

give them PGE

SE of PGE is apnea–> -intubate
can also give IV isotonic saline for increasing volume –> decrease hypotension

if the problem is transposition of the great arteries, it will keep the ductus arteriosus open until you can perform surgery

40
Q

What do the diagnostic studies in transposition of the great arteries show?

A

ECG: RV hypertrophy

ECHO: definitive diagnosis

41
Q

**What is the treatment of Transposition of the Great Arteries?

A

PGE!!!! to keep the ductus arteriosus open

then creation of an inter-atrial communication (create and ASD) using a balloon catheter (Rashkind procedure) (palliative–> baby is too small to fix the problem)

Definitive surgery “arterial switch” operation

42
Q

What is truncus arteriosus?

A

Lack of separation of the embryological truncus into a separate aorta and pulmonary trunk.

This results in a single arterial vessel that originates from the heart

43
Q

What are the finding on diagnostic studies in truncus arteriosus? what is the treatment?

A

CXR: cardiomegaly with pulmonary plethora
ECHO: Allows direct visualization of a single trunk
MRI

Treatment: Definitive surgical management

44
Q

What is Tricuspid Atresia?

A

agenesis of the tricuspid valve and right ventricular inlet –> blood does not go from right atria to right ventricle

There is almost always an obligatory intra-atrial connection through either anASDorpatent foramen ovale(PFO) in order for circulation to be complete

45
Q

What are the extra-cardiac associations of tricuspid atresia?

A

asplenia

right-sided aortic arch

46
Q

How is Tricuspid Atresia diagnosed?

A

ECHO

47
Q

How is Tricuspid Atresia treated?

A

Routinely initiate prophylaxis against bacterial endocarditis when any invasive or dental procedure is contemplated

Some form of surgical treatment during the first year of life

48
Q

What is Total Anomalous Pulmonary Venous Return (TAPVR)? What is the most common type?

A

the four pulmonary veins are connected somewhere besides the left atrium.

type I: supracardiac. pulmonary veins converge to form aleft vertical veinwhich then drains to eitherbrachiocephalic vein,SVCoraygous vein

49
Q

What is TAPVR associated with?

A

many have heterodoxy syndrome (things on opposite side), particularly asplenia

Type III (infracardiac) is also associated with thoracic lymphangiestasia

50
Q

What does the x-ray for TAPVR show?

A

figure 8 sign or snowman sign

51
Q

How is TAPVR diagnosed?

A

Cardiac catheterization can confirm the diagnosis by showing that the blood vessels are abnormally attached

ECG shows enlargement of the ventricles (ventricular hypertrophy)

52
Q

*What is Eisenmenger Syndrome?

A

the condition of severe pulmonary vascular obstruction that results from chronic left-to-right shunting through a congenital cardiac defect

Elevated pulmonary vascular resistance causes reversal of the original shunt (to the right to left direction) causing cyanosis

53
Q

What are some symptoms of Eisenmenger Syndrome?

A

Exertional dyspnea and fatigue

Hyperviscosity which may result in stroke (make more RBCs)

Hemoptysis due to rupture of the of the pulmonary vessels (too much pressure)

PE:

  • cyanosis
  • digital clubbing
  • prominent wave in jugular venous pulsation
  • absent murmur
54
Q

What is likely to happen if an Eisenmenger Syndrome woman gets pregnant?

A

45% maternal mortality

55
Q

What is the treatment for Eisenmenger Syndrome?

A

Avoid vigorous activity, high altitude and use of peripheral vasodilator drugs which may increase right-to-left shunt

Supportive care

Lung or Heart-lung transplant

56
Q

What congenital heart defect is 22q11 associated with?

A

Truncus arteriosis, Tetralogy of Fallot

57
Q

*What congenital heart defects is Down syndrome associated with?

A

ASD, VSD, PDAs

40% of Downs kids have congenital heart defect

58
Q

What congenital heart defects is congenital rubella associated with?

A

Septal defects, PDA, pulmonary artery stenosis

59
Q

*What congenital heart defects is turner syndrome associated with?

A

Coarctation of aorta (pre ducta), bicuspid aortic valve and left heart hypoplasia
–> left sided obstructive lesions

short stature, broad chest, low hairline, webbed neck

60
Q

What congenital heart defects is Marfan’s syndrome associated with?

A

Aortic insufficiency and dissection (late complication)

61
Q

What congenital heart defects is an infant of a diabetic mother associated with?

A

Transposition of great vessels

62
Q
  • (*=lg group) What is the defect that leads to all of the symptoms in Tetrology of Fallot?
A

Abnormal anterior and cephalad displacement of the infundibular (outflow tract) portion of the interventricular septum

63
Q

*What congenital heart defect is Williams Syndrome associated with?

A

Supravalvular Aortic Stenosis

Rare neurodevelopmental disorder
“Elfin” facial appearance
Developmental delay

7q11.23

64
Q

What are the 2 reasons that pulmonary vascular resistance falls when a baby takes its first breath?

A

Mechanical inflation of the lungs stretches the lung tissue, causing pulmonary artery expansion and wall thinning

Vasodilation of the pulmonary vasculature occurs in response to rise in oxygen tension due to lung aeration

65
Q

*How is PDA treated?

A

All PDAs should be closed –> many close spontaneously

If significant respiratory distress: Intravenous (IV) indomethacin is frequently effective in closing a PDA if it is administered in the first 10-14 days of life

66
Q

*Why would we use ibuprofen over indomethacin for PDA?

A

Indomethacin decreases the incidence of intracranial hemorrhage but ibuprofen has less renal toxicity (oliguria)

67
Q

***what is an important side effect of indomethacin????

A

cerebral vasoconstriction

–> this is how it decreases the chances of intracranial hemorrhage

68
Q

*When does a PFO become a significant concern? What comorbidity increases this risk?

A

When the right atrial Pressure becomes elevated –> embolisms

A. fib WITH increased right sided atrial P increases the risk ==> more likely to have a clot

69
Q

*When does a PFO need to be closed?

A

When the pt becomes symptomatic (elevated right sided atrial P)

Cardio Exam 1 (5 decks)

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