Mod VI: Congenital Heart Disease Part2

Question 1

_{Cyanotic Heart Defects}

Predominantly Right-to-Left Shunts/Mixing lesions include:

Answer 1

Tetralogy of Fallot

Transposition of the Great Arteries

Hypoplastic Left Heart Syndrome (HLHS)

Tricuspid valve abnormalities (Ebstein’s anomaly)

Truncus arteriosus

Total anomalous pulmonary venous connection

Question 2

_{Cyanotic Heart Defects}

Pathophysiologic changes a/w predominantly Right-to-Left Shunts/Mixing Lesions include:

Answer 2

Decreased pulmonary blood flow

_{leading to:}

Hypoxemia

LV volume overload

LV dysfunction

Question 3

_{Cyanotic Heart Defects}

Hemodynamic goals for predominantly Right-to-Left Shunts/Mixing Lesions include:

Answer 3

Maintain SVR

^(Squatting)

Decrease PVR

^{(via Hyperoxia - Hyperventilation - Avoiding lung hyperinflation)}

Question 4

_{Cyanotic Heart Defects}

Cyanotic Heart Defects are complex lesions that produce

Answer 4

Ventricular outflow obstruction

^{Obstruction favors shunt towards unobstructed side}

Intracardiac shunting

^{Affected by ratio SVR:PVR with mild obstruction}

^{Direction and magnitude fixed with large obstructions}

_{^{Atresia extreme form obstruction}}

_{^{Shunting occurs proximal to atretic valve}}

_{^{Survival depends on distal shunt (PDA, PFO, VSD) where blood flows in opposite direction}}

Question 5

_{Cyanotic Heart Defects - Predominantly Right-to-Left Shunts/Mixing Lesions}

Which Cyanotic Heart Defects are a/w decreased pulmonary blood flow?

Answer 5

TOF

Pulmonary atresia

Tricuspid atresia

Question 6

_{Cyanotic Heart Defects - Predominantly Right-to-Left Shunts/Mixing Lesions}

Which Cyanotic Heart Defects are a/w increased pulmonary blood flow?

Answer 6

Transposition of the great vessels

Truncus arteriosus

Hypoplastic left heart

Question 7

_{Cyanotic (R→L) Heart Defects}

The congenital heart condition that involves four abnormalities occurring together, including a defective septum between the ventricles and narrowing of the pulmonary artery, leading to cyanosis is also known as:

Answer 7

Tetralogy of Fallot

Question 8

_{Cyanotic (R→L) Heart Defects}

Most common CHD producing R to L shunt

Answer 8

Tetralogy of Fallot

Question 9

_{Cyanotic (R→L) Heart Defects - Tetralogy of Fallot}

What’s the prevalence of Tetralogy of Fallot in neonate?

Answer 9

3rd most prevalent CHD in the neonate

Question 10

_{Cyanotic (R→L) Heart Defects - Tetralogy of Fallot}

Anatomic defects associated with Tetralogy of Fallot:

Answer 10

VSD (R-to-L)

Aorta that overrides the pulmonary tract

Obstruction of pulmonary outflow tract

Right ventricular hypertrophy

Question 11

_{Cyanotic (R→L) Heart Defects - Tetralogy of Fallot}

Pathophysiologic characteristics seen with Tetralogy of Fallot:

Answer 11

R-to-L shunting

↓ Pulmonary blood flow

Polycythemia (>65%)

^{D/t body attempt to compensate for lack of O2 by producing more RBCs}

Ductal dependent pulmonary blood flow (L-R shunt) in neonate with severe obstruction (PGE1)

Question 12

_{Cyanotic (R→L) Heart Defects - Tetralogy of Fallot}

Blood flow in Tetralogy of Fallot

Answer 12

Note:

VSD in TOF is with R=>L shunt

Misplaced aorta that overrides pulmonary tract

Stenosis of the pulmonary valve and pulm artery out of the RV

Thickening of the RV wall

Question 13

_{Cyanotic (R→L) Heart Defects - Tetralogy of Fallot}

Blood flow in Tetralogy of Fallot

Answer 13

See picture

Note “pulmonary atresia”

Question 14

_{Cyanotic (R→L) Heart Defects - Tetralogy of Fallot}

Manifestations Tetralogy of Fallot:

Answer 14

Hypoxemia/cyanosis

Clubbing

Squatting

^{(↑ SVR by reducing blood flow to femoral arteries)}

Ejection murmur

Hypercyanotic attacks (“tet spells”), _{as evidenced by:}

^{Infundibular “spasm” => worsen RV outflow tract obstruction}

^{↓ SVR}

^{Can occur w/o provocation but often associated with crying or exercise}

^{Accompanied by hyperventilation & syncope}

Question 15

_{Cyanotic (R→L) Heart Defects - Tetralogy of Fallot}

Treatment of Tetralogy of Fallot includes:

Answer 15

IV fluids

Knee-to-chest

Phenylephrine (↑ SVR)

Esmolol

MSO4

^{(caution w/ ↓ venous return and CO a/w MSO4 )}

Question 16

_{Cyanotic (R→L) Heart Defects - Tetralogy of Fallot}

The surgical palliation to Tetralogy of Fallot in which the Left subclavian artery is shunted to the left pulmonary artery to increase pulmonary blood flow is known as:

Answer 16

Blalock-Taussig shunt

Question 17

_{Cyanotic (R→L) Heart Defects - Tetralogy of Fallot}

What’s the difference btw the Traditional and the Modified Blalock-Taussig shunts?

Answer 17

The traditional Blalock-Taussig shunt uses the actual subclavian artery, whereas

The Modified Blalock-Taussig shunt uses a graft to to divert some of the subclavian artery blood flow to the PA

Schematic drawing of original Blalock-Taussig shunt on patient’s right side and modified Blalock-Taussig shunt on left side

Question 18

_{Cyanotic (R→L) Heart Defects - Tetralogy of Fallot}

Complete correction of Tetralogy of Fallot involves:

Answer 18

Closure VSD

Removal obstructing infundibular muscle

Pulmonic valvulotomy

Question 19

_{Cyanotic (R→L) Heart Defects - Tetralogy of Fallot}

The main goal of Anesthetic management of Tetralogy of Fallot is to lessen the R-to-L shunt. How can this be accomplished?

Answer 19

Maintain intravascular volume

Maintain SVR/Avoid decreasing

Avoid ↑ PVR

^{(By avoiding acidosis, hypoxemia, excessive PIP)}

Maintain higher FiO2 and lower ETCO2 to prevent PVR increase

Question 20

_{Cyanotic (R→L) Heart Defects - Tetralogy of Fallot}

Options for inducing Anesthesia in Tetralogy of Fallot

Answer 20

Inhalation with pink patients

Ketamine IV/IM with cyanotic patients

Question 21

_{Cyanotic (R→L) Heart Defects - Tetralogy of Fallot}

How does R-to-L shunting in Tetralogy of Fallot effect on rate of inhalational induction?

Answer 21

Slows inhalational induction

Slows uptake

^{(D/t to less blood flow to the lungs in general; Opposite of L-to-R shunts)}

Dilutional effect

Question 22

_{Cyanotic (R→L) Heart Defects - Tetralogy of Fallot}

How does R-to-L shunting in Tetralogy of Fallot effect on rate of IV induction?

Answer 22

Accelerates onset in IV agents

Question 23

_{Cyanotic (R→L) Heart Defects}

A form of congenital heart disease whereby there is a complete absence of the tricuspid valve. Therefore, there is an absence of right atrioventricular connection. This leads to a hypoplastic (undersized) or absent right ventricle. This condition is also known as:

Answer 23

Tricuspid Atresia

Question 24

_{Cyanotic (R→L) Heart Defects - Tricuspid Atresia}

Anatomical defects/physiologic characteristics of Tricuspid Atresia:

Answer 24

Complete absence of right atrioventricular connection

Severe hypoplasia or absent RV

Pulmonary blood flow dependent on PDA (L-R shunting)

LA & LV handle both systemic and pulmonary circulations

^{Systemic venous return shunted from RA => LA via ASD or PFO}

^{Mixing of O2 and deO2 in LA → LV → Aorta = CYANOSIS}

90% associated with VSD allowing some blood to enter RV

Normal related great arteries or with transposition

Question 25

_{Cyanotic (R→L) Heart Defects - Tricuspid Atresia} Clinical manifestations of Tricuspid Atresia:

Answer 25

Progressive cyanosis Poor feeding Tachypnea CHF

Question 26

_{Cyanotic (R→L) Heart Defects - Tricuspid Atresia} Treatment of Tricuspid Atresia:

Answer 26

PGE1 to maintain pulmonary flow Balloon atrial septostomy if atrial defect not sufficient Surgical interventions

Question 27

_{Cyanotic (R→L) Heart Defects - Tricuspid Atresia} Which Surgical interventions are used in the Treatment of Tricuspid Atresia?

Answer 27

Modified **Blalock-Taussig shunt** to maintain pulmonary blood flow Cavopulmonary anastomosis (**hemi-Fontan** or bi-directional Glenn)

Question 28

_{Cyanotic (R→L) Heart Defects - Tricuspid Atresia} What's the goal of surgical interventions in the Treatment of Tricuspid Atresia?

Answer 28

Redirection of IVC and hepatic vein flow into pulmonary circulation

Question 29

_{Cyanotic (R→L) Heart Defects} A form of heart disease in which the pulmonary valve does not form properly. It is present from birth (congenital heart disease). This heart defect is also known as:

Answer 29

**Pulmonary Atresia**

Question 30

_{Cyanotic (R→L) Heart Defects - Pulmonary Atresia} Anatomical defect and physiologic effects a/w Pulmonary Atresia:

Answer 30

Absent pulmonary valve RV hypoplasia Tricuspid hypoplasia Obligate atrial shunt from R to L Ductal dependent pulmonary blood flow Coronary artery-myocardial sinusoid communications ^{Myocardial infarction/death may occur with any palliative procedure that decompresses the RV if coronaries are RV dependent}

Question 31

_{Cyanotic (R→L) Heart Defects - Pulmonary Atresia} In Pulmonary Atresia, right heart is underdeveloped and cannot support the blood supply to the lungs. All the blood supply to the lungs is provided through which structure?

Answer 31

**Ductus arteriosus**

Question 32

_{Cyanotic (R→L) Heart Defects - Pulmonary Atresia} Manifestations of Pulmonary Atresia:

Answer 32

Severe cyanosis immediately after birth Tachypneic

Question 33

_{Cyanotic (R→L) Heart Defects - Pulmonary Atresia} Pharmacological Treatment of Pulmonary Atresia:

Answer 33

PGE1

Question 34

_{Cyanotic (R→L) Heart Defects - Pulmonary Atresia} Surgical Treatment of Pulmonary Atresia:

Answer 34

**RV to PA conduit** ^{(if coronary circulation is not RV dependent)} **Blalock-Taussig shunt** _Fontan palliation_ or _heart transplant_ ^{(for RV-dependent coronary circulation)}

Question 35

_{Cyanotic (R→L) Heart Defects} The congenital (present at birth) heart defect in which the aorta is connected to the right ventricle, and the pulmonary artery is connected to the left ventricle is known as:

Answer 35

**Transposition of the Great Vessels**

Question 36

_{Cyanotic (R→L) Heart Defects - Transposition of the Great Vessels} Prevalence of Transposition of the Great Vessels:

Answer 36

Accounts for 5% of all CHD

Question 37

_{Cyanotic (R→L) Heart Defects - Transposition of the Great Vessels} Anatomical defect in Transposition of the Great Vessels:

Answer 37

Aorta arises from the RV =\> Deoxygenated blood returns back to systemic circulation Pulmonary artery arises from the LV =\> Oxygenated blood returns back to lungs

Question 38

_{Cyanotic (R→L) Heart Defects - Transposition of the Great Vessels} Survival in Transposition of the Great Vessels is possible only if:

Answer 38

_Some form of intercirculatory mixing exists_ ASD/VSD Patent foramen ovale PDA (PGE1 required) *Variable degrees of pulmonary blood flow occur dependent on types of lesions present*

Question 39

_{Cyanotic (R→L) Heart Defects - Transposition of the Great Vessels} What's the immediate management of Transposition of the Great Vessels?

Answer 39

This is a **Surgical emergency** Delivery takes places in OR Neonate taken immediately for open heart surgery The quicker the surgery, the better the outcomes

Question 40

_{Cyanotic (R→L) Heart Defects - Transposition of the Great Vessels} Blood flow in Transposition of the Great Vessels

Answer 40

See picture

Question 41

_{Cyanotic (R→L) Heart Defects - Transposition of the Great Vessels} Corrective surgical treatment for Transposition of the Great Vessels include:

Answer 41

**Arterial switch** with coronary artery reanastomosis **Atrial switch** (Senning procedure)

Question 42

_{Cyanotic (R→L) Heart Defects - Transposition of the Great Vessels} Anesthetic considerations for Transposition of the Great Vessels (TGV):

Answer 42

​Maintain HR, contractility, preload, CO Special considerations based on PBF

Question 43

_{Cyanotic (R→L) Heart Defects - Transposition of the Great Vessels} What are Anesthetic considerations for TGV a/w ↓ PBF & minimal intracardiac mixing (ICM)?

Answer 43

Avoid ↑ PVR relative to SVR ^{(↑ PVR​ will further reduce PBF and intracardiac mixing)} Adjust vent settings to ↓ PVR ^{(By: ↑ FiO2 & RR - ↓ ETCO2 - Maintain slight alkalosis)}

Question 44

_{Cyanotic (R→L) Heart Defects - Transposition of the Great Vessels} What are anesthetic considerations for TGV aw ↑ PBF & large ICM?

Answer 44

Maintain normal PVR, ETCO2, low FiO2 Adjusting PVR will only modestly improve saturations at the expense of systemic circulation

Question 45

_{Cyanotic (R→L) Heart Defects} A birth defect that affects normal blood flow through the heart. As the baby develops during pregnancy, the left side of the heart does not form correctly. This condition is also known as:

Answer 45

**Hypoplastic Left Heart Syndrome**

Question 46

_{Cyanotic (R→L) Heart Defects - Hypoplastic Left Heart Syndrome} Anatomical defects seen in Hypoplastic Left Heart Syndrome

Answer 46

Underdeveloped LV Aortic valve stenosis or atresia Mitral valve stenosis or atresia Hypoplasia ascending aorta

Question 47

_{Cyanotic (R→L) Heart Defects - Hypoplastic Left Heart Syndrome} Pathophysiologic effects of Hypoplastic Left Heart Syndrome:

Answer 47

Blood flow to left side heart eliminated or reduced =\> obligatory L-to-R shunt Systemic blood flow completely ductal dependent (R-L shunt) ^{All blood entering aorta derived from PDA PGE1 essential to keep PDA open} RV main pumping chamber for both pulmonary & systemic circulation

Question 48

_{Cyanotic (R→L) Heart Defects - Hypoplastic Left Heart Syndrome} Surgical correction for Hypoplastic Left Heart Syndrome include:

Answer 48

Palliative ^{(Norwood, hemi-Fontan or bidirectional Glenn, completion Fontan)} Heart Transplantation

Question 49

_{Cyanotic (R→L) Heart Defects - Hypoplastic Left Heart Syndrome} Normal heart vs Hypoplastic Left Heart Syndrome

Answer 49

See picture

Question 50

_{Cyanotic (R→L) Heart Defects - Hypoplastic Left Heart Syndrome} Another view of Hypoplastic Left Heart Syndrome

Answer 50

See picture

Question 51

_{Cyanotic (R→L) Heart Defects - Hypoplastic Left Heart Syndrome} In hypoplastic left heart syndrome, the left heart is underdeveloped and cannot support the blood supply to the body. All the blood supply to the body is provided through which structure?

Answer 51

**Ductus arteriosus**

Question 52

_{Cyanotic (R→L) Heart Defects} Palliative surgical procedure used in children with univentricular hearts

Answer 52

**Fontan Procedure**

Question 53

_{Cyanotic (R→L) Heart Defects - Fontan Procedure} Fontan” Physiology

Answer 53

All venous blood returning to heart bypasses the right heart and flows passively into the lungs RV pumps oxygenated blood returning from lungs into systemic circulation

Question 54

_{Cyanotic (R→L) Heart Defects - Fontan Procedure} Why is it important to avoid ↑ PVR w/ Fontan Procedure?

Answer 54

Blood is passively flowing into the PA ↑ PVR =\> ↓ PBF =\> **hypoxemia**

Question 55

_{Cyanotic (R→L) Heart Defects - Fontan Procedure} Which Factors ↓ PVR/↑PBF and must be considered w/ Fontan Procedure?

Answer 55

Hyperoxia Alkalosis HTN/↑ SVR Low mean airway pressures

Question 56

_{Cyanotic (R→L) Heart Defects - Fontan Procedure} Which Factors↑ PVR/↓ PBF and must be considered w/ Fontan Procedure?

Answer 56

Hypoxemia Acidosis Hypotension/↓ SVR PEEP

Question 57

_{Congenital Heart Defects} Representation of the adult heart and incidence of congenital heart defects

Answer 57

_{See picture} Numbers in paranthesis represent incidence of heart defect for every 1,000 births

Question 58

_{Congenital Heart Defects} Anesthesia providers will encounter patients with CHD for which types of procedures?

Answer 58

**Elective** or **Emergent noncardiac surgery** and during **Pregnancy**

Question 59

_{Congenital Heart Defects} **Unpalliated** procedures are reserved for which Congenital Heart Defects?

Answer 59

**Cardiac anomaly** does _not cause hemodynamic compromise_ ^{(ASD/VSD/Mild valvular stenosis)} **Surgical emergencies** ^{(CDH, Intestinal instruction)} where **lesion** may be more **complex** ^(TOF) and **infant** waiting for _palliative surgery_ **These procedures do not cause hemodynamic compromise**

Question 60

_{Congenital Heart Defects} Partially palliated anomalies and procedures include:

Answer 60

**Staged repairs**

Question 61

_{Congenital Heart Defects} Completely palliated procedures include:

Answer 61

**Fontan** **Blalock-Taussig**

Question 62

_{Congenital Heart Defects} Complete familiarization with the anatomic and hemodynamic function of the CHD is essential to formulating and managing a safe anesthetic plan. How can this be achieved?

Answer 62

**Categorize** the anomaly ^{(Cyanotic vs acyanotic, direction of shunt, reduced blood flow vs obstruction, etc.)} Develop **ideal anesthetic plan** to keep pt safe during the procedure Be familiar with common sequelae after repair Management of anesthesia for patients with CHD requires thorough knowledge of the pathophysiology of each cardiac defect You may have a pt that had a Fontain procedure 10 years ago and you must be able to care for them This may help you care for that pt when they are undergoing other procedures if you are familiar w/ their blood flow as a result of their past cardiac hx Ideal to discuss case with child’s pediatric cardiologist prior to surgery

Question 63

_{Congenital Heart Defects} Understanding the impact of anesthesia management on the ratio of systemic to pulmonary blood flow is also vital when formulating the anesthetic plan. What questions must you be able to answer once you categorize where the Congenital Heart Defect sits?

Answer 63

What events ↑ SVR? What events ↓ SVR? What events ↑ PVR? What events ↑ SVR? _{Review M & M: page 521-524}

Question 64

_{Congenital Heart Defects - Atrial Septal Defect (ASD)} What are the three anatomical varieties of ASD?

Answer 64

See picture

Question 65

_{Congenital Heart Defects - Atrial Septal Defect (ASD)} What are the pathophysiological changes a/w ASD?

Answer 65

See picture

Question 66

_{Congenital Heart Defects - Atrial Septal Defect (ASD)} Which surgical corrections are used to correct ASD?

Answer 66

See picture

Question 67

_{Congenital Heart Defects - Atrial Septal Defect (ASD)} What are anesthetic considerations for ASD?

Answer 67

See picture

Question 68

_{Congenital Heart Defects - Ventricular Septal Defect (VSD)} What are anatomical findings in VSD?

Answer 68

See picture

Question 69

_{Congenital Heart Defects - Ventricular Septal Defect (VSD)} What are the pathophysiological changes a/w VSD?

Answer 69

See picture

Question 70

_{Congenital Heart Defects - Ventricular Septal Defect (VSD)} What are anatomical findings in VSD? What are the pathophysiological changes a/w VSD? What are the surgical correction for VSD? What are anesthetic considerations for VSD?

Answer 70

See Picture

Question 71

_{Congenital Heart Defects - Co-arctation of the Aorta} What are anatomical findings, pathophysiological changes, surgical correction, and anesthetic considerations in **Co-arctation of the Aorta**?

Answer 71

See picture

Question 72

_{Congenital Heart Defects - Co-arctation of the Aorta} What are anatomical findings, pathophysiological changes, surgical correction, and anesthetic considerations in **Patent Ductus Arteriosus** (PDA)?

Answer 72

See picture

Question 73

_{Congenital Heart Defects - Co-arctation of the Aorta} What are anatomical findings, pathophysiological changes, surgical correction, and anesthetic considerations in **Tetralogy of Fallot**?

Answer 73

See picture

Question 74

_{Congenital Heart Defects - Co-arctation of the Aorta} What are anatomical findings, pathophysiological changes, surgical correction, and anesthetic considerations in **Transposition of the Great arteries**?

Answer 74

See picture

Question 75

_{Congenital Heart Defects - Co-arctation of the Aorta} What are anatomical findings, pathophysiological changes, surgical correction, and anesthetic considerations in **Truncus Arteriosus**?

Answer 75

See Picture

Question 76

_{Congenital Heart Defects - Co-arctation of the Aorta} What are anatomical findings, pathophysiological changes, surgical correction, and anesthetic considerations in **Atrioventricular Canal Defect**?

Answer 76

See picture

Question 77

_{Congenital Heart Defects - Co-arctation of the Aorta} What are anatomical findings, pathophysiological changes, surgical correction, and anesthetic considerations in **Hypoplastic left heart syndrome**?

Answer 77

See picture