Congential Heart Disease Flashcards

1
Q

What is the ductus venosus?

A

Umbilical vein to inferior vena cava

Oxygen risk blood from the placenta bypasses liver

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2
Q

What is the foramen ovale?!

A

Right atrium to left atrium

Oxygen-rich blood bypasses lungs and is preferentially delivered to the heart and developing brain

***Takes severe days to close

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3
Q

What is the ductus arteriosus?

A

Pulmonary artery to descending aorta

***takes several weeks to close

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4
Q

What does the umbilical vein do?

A

Carries oxygen RICH blood from mother to fetus

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5
Q

What do the umbilical arteries do?

A

Carries oxygen poor blood from fetus to mother

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6
Q

What are the six different fetal circ differs from adult circ?

A

Placenta = lungs (organ of resp)
Circulation is in parallel (adult = series)
Right-to-left shunting
SVR is LOW ~ placenta provides wide vascular bed
PVR is HIGH ~ lungs are filled with fluid ~little pulmonary flow
Left atrium pressure is low

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7
Q

What happens when a baby takes it’s first breath?

A

Lung expansion > increase PaO2 & decrease in PaCO2 > decrease in PVR

***this decrease in PVR > reverses blood flow through ductus arteriosus > exposes the DA to ^ PO2 > DA closure

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8
Q

What happens when the placenta separates from the uterine wall?

A

Increase in SVR!

Decrease in PVR and increase in SVR > LA pressure > RA pressure ~ the flap valve of foramen ovale closes

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9
Q

How can the ductus arteriosus be closed if needed?

A

Indomethacine (a prostaglandin synthases inhibitor)

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10
Q

How can’t he ductus arteriosus remain open if need be?

A

Prostaglandin E1 (PGE1)

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11
Q

How does the ligamentum arteriosus play a role in trauma?

A

If a rapid deceleration tears the ligament, it could result in partial or complete Aortic dissection

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12
Q

Can the ligamentum venosum be re-opened?

A

No!

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13
Q

What are conditions that increase PVR?!

A

Hypothermia
Hypoxia
Acidosis
Light anesthesia
T berg
Pain
Vasoconstrictors
Hypercarbia
Atelectasis

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14
Q

When does a R > L shunt occur?

A

THIS IS A CYANOTIC SHUNT

When PVR > SVR

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15
Q

When does a L > R shunt occur?

A

THIS IS AN ACYANOTIC SHUNT

When SVR is > than PVR

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16
Q

What are some things that decrease PVR?

A

Alkalosis
Nitric oxide
Oxygenation
Hypocarbia
Hemodilution
Vasodilators

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17
Q

What is the pathophysiology behind a blue baby?

A

Decreased pulmonary blood flow

Hypoxemia
LV volume overload
LV dysfunction

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18
Q

What are the goal for a blue baby (R > L shunt)

A

Maintain SVR
Decrease PVR
>decrease hypoxia
> hyperventilate
> avoid lung hyperinflation

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19
Q

What are the 5 most common R>L shunts?

A

5 Ts!!!

Tetralogy of Fallot
Transposition of the great arteries
Tricuspid valve abnormality (Ebstein’s anomaly)
Truncus arteriosus
Total anomalous pulmonary venous connection

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20
Q

How is the rate of rise affected in a R>L shunt?

A

Slowed! ~ slower inhalation induction

***especially with less sociable agents (Des and nitrous)

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21
Q

How does a R>L shunt effect IV induction?

A

Bypasses the lungs and enters systemic circ FASTER!! FASTER ONSET

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22
Q

What is a Aycyanotic shunt?

A

L>R shunt

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23
Q

What is the pathophysiology behind a L>R shunt? (Pink baby)

A

Decreased systemic blood flow
> low CO
> hypotension
Increased PVR
> pulmonary HTN
> RVH

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24
Q

What are some of the Hemodynamic goals of a L> R shunt?

A

Avoid increases in SVR
Avoid decreases in PVR
> avoid alkalosis
> hypocapnia
> high FiO2
> vasodilators

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25
Q

What are the 4 most common L to R cardiac shunts?

A

Ventricular septal defect (most common)
Atrial septal defect
Patent ductus arteriosus
Coarctation of the aorta

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26
Q

How does a L>R shunt affect inhalation induction?

A

Negligible

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27
Q

How does a L> LR shunt affect IV induction?

A

Prolonged

28
Q

What is Eisenmenger Syndrome?

A

Can occur when a patient with a L>R shunt develops pulmonary HTN. This increased right heart pressure causes a flow reversal through defect ~ leading to a R>L shunt! ***hypoxemia and cyanosis

29
Q

What are the 4 defects of tetrology of Fallot?

A

Ventricular septal defect
Aorta that overrides the RV and LV
Pulmonic stenosis (obstruction for RV ejection)
RV hypertrophy

30
Q

What are the goals in a patient with tetralogy of Fallot?

A

Increase SVR
Decrease PVR
Maintain contractility and HR
Increase preload

31
Q

What is a “tet spell”?

A

Precipitated by increased sympathetic activity (crying, agitation, pain, defamation, fright, trauma) > increase myocardial contractility which can cause spasm of the infra-valvular region of the RVOT.

This increased resistance causes blood to flow through VSD > increased R>L shunting

32
Q

What will a child do during a “tet spell?”

A

Child will hyperventilate and squat.

Squatting increases intraabdominal pressure > compresses abd arteries and increases preload, SVR and blood flow through RVOT

33
Q

How can you treat a tet spell in the perioperative realm?

A

FiO2 100%
Administer fluids to expand volume
Increase SVR (Neo)
Reduce SNS stimulation (deepen anesthetic, beta blockers)
Avoid inotropes
Avoid excess airway pressure
Place infant in knee-chest position

34
Q

What type of appearance is the heart of a patient with tetrology of Fallot on X-ray ?

A

Boot shaped!

35
Q

What type of axis deviation with occur with tetrology of Fallot

A
36
Q

What is the best induction agent with a patient with tetrology of Fallot?

A

Ketamine!

37
Q

What narcs should be avoided in a patient with tetrology of Fallot?

A

Morphine
Meperidine

(Due to histamine release!!)

38
Q

What happens to tetrology of Fallot patients who are consistently hypoxic?

A

They develop polycythemia

39
Q

What are the 3 types of atrial septal defects?

A

Secundum: ASD occurs in middle of the atrial septum and results when fossa ovalis fails to close (80% of all ASDs)

Primum: ASD occurs in the lower region of the atrial septum ~ just above tricuspid valve

Sinus venosus: ASDs are located just below the IVC or above IVC

40
Q

What is the most common type of ventricular septal defect?

A

Perimembranous
**located in the middle of the ventricular septum, just below the septal leaflet of the tricuspid valve

41
Q

What is the most common type of cardiac anomaly in children?

A

Ventricular septal defect (VSD)

42
Q

What is the most common congenital cardiac defect in adults?

A

Bicuspid aortic valve.

43
Q

What are some early signs of an ASD?
What are some later signs of an ASD?

A

Early: exercise intolerance
Late: afib, aflutter, CHF

44
Q

What is VSD associated with? (Congenital dx)

A

Trisomy 13, 18, 21
VACTERL
CHARGE

45
Q

Where is the best place to measure BP in a patient with coarctation of the aorta?

A

Right upper extremity

46
Q

What is coarctation if the aorta?

A

Narrowing of the thoracic aortic lumen ~ usually occurs before or after after ductus arteriosus, BUT occasionally it comes proximal to the subclavian 😳

47
Q

When does preductal coarctation usually present?

A

Less common ~ present in the neonate!

48
Q

When does postductal coarctarion usually present?

A

More common ~usually present in the adult!

49
Q

What syndrome is strongly associated with coarctation?

A

Turner Syndrome

50
Q

Due to the obstruction of blood flow at the level of the coarctation….?

A

LV afterload is increased!

SBP is elevated in upper extremities
SBP reduced in the lower extremities

51
Q

What might be visible on CXR for a patient with Coarctation of the aorta?

A

Rib notching (this is due to increased vessel diameter)

52
Q

What is epstein’s anomaly?

A

Downward (apical) displacement of the tricuspid valve ~ RV becomes part of the right atrium (atrialization)

**usually an ASD or PFO

53
Q

What is transposition of the great arteries?

A

Each great vessel arises from wrong ventricles. This produces circulation in parallel instead of series

54
Q

What are some traits about Epstein’s anomaly?

A

Most common defect of tricuspid valve
Tricuspid regurge can be intense
IV drug onset is prolonged (d/t pooling)
Risk of CHF
SVT is common
RV failure is common in postop

55
Q

In transposition of the great arteries, what does the RV give rise to?

A

The aorta (poorly oxygenated circuit)

**systemic venous blood > RV > aorta > repeat

Does not circulate to the pulmonary system

56
Q

In transposition of the great arteries, what does the LV give rise to?

A

Pulmonary artery (well-oxygenated circuit)

LV > lungs > repeat

Does not circulate to systemic

57
Q

What type of emergency is transposition of the great arteries?

A

Medical!! Without fetal communication (I.e. pda or ASD) death is eminent.

May need prostaglandin to keep PDA open.

58
Q

What is the definitely treatment for transposition of the great arteries?

A

Intra atrial baffle and arterial switch procedures

59
Q

What is hypoplastic left heart syndrome?

A

Hypoplastic LV
Hypoplastic aortic arch
Mitral and Aortic stenosis or atresia
Ductal-dependent circulation

60
Q

What is the surgical goal for hypoplastic left heart?

A

Separating the pulmonary and systemic circulations.

61
Q

What are the 3 stages of surgical correction for hypoplastic Left heart?

A

Norwood stage 1
Norwood stage 2
Norwood stage 3 (Fontan procedure)

62
Q

What is the Norwood stage 1?

A

Occurs in neonatal period

> aortic reconstruction ~ aortic arch rises from pulmonary trunk. Pulmonary arteries are disconnected from pulmonary trunk and are used to create a shunt from subclavian artery to RV

63
Q

What is the Norwood stage 2? (Glenwood)

A

3-6 months of age

Shunt from first stage is taken down and a new connection is made between the SVC and pulmonary arteries

64
Q

What is the Norwood 3 (fontan procedure)?

A

The IVC is connected to the pulmonary artery with a conduit

65
Q

How is the circulation system after Fontan completion?

A

Patient has single ventricle that pumps blood into systemic circ while pulmonary blood flow occurs passively from SVC/IVC to the pulmonary artery

66
Q

How does PVR affect blood flow in the hypoplastic heart?

A

Increased PVR is detrimental to pulmonary flow

> Spontaneous breathing is best!
positive-pressure vent is bad (avoided)
patients are PRELOAD dependent

67
Q

What is truncus arteriosus?

A

Characterized by a single artery that gives rise to the pulmonary, systemic, AND coronary circulations.