Mod 1 Congenital Heart Diseases (CHD)

Question 1

How early can congenital heart diseases develop?

Answer 1

Prior to 8 weeks.
- family Hx and Genetics play a factor

Question 2

What factors contribute to the development of congenital heart disease?

Answer 2

• Drugs/alcohol abuse
• Maternal diabetes, infection, exposure to solvents
• previous miscarriages
• Genetics (chromosomal defects i.e downs)

Question 3

What is the difference between acyanotic disease and cyanotic heart disease?

Answer 3

• Acyanotic disease shunts oxygenated blood from the left side of the heart to the right
  (L->R)
• Cyanotic heart disease shunts deoxygenated blood from the right to the left (R->L)

Question 4

What are hallmarks of Acyanotic heart disease?

Answer 4

• L->R
• Increased pulmonary blood flow
• Increases workload on right ventricle

Question 5

What are hallmarks of Cyanotic heart disease

Answer 5

• All congenital that start w/”T” are cyanotic
• Decreased pulmonary blood flow
• Requires a PDA for survival (ductal depedent)
• hypoxemia & central cyanosis

Question 6

Why are cyanotic heart diseases a problem?

Answer 6

• Decreased oxygenated blood is shunted from the right to the left side resulting in severe hypoxemia and central cyanosis.

Question 7

What are the 5 cyanotic heart defects we focus on?

Answer 7

1. Truncus arteriosus
2. Transposition of Great Arteries
3. Tricuspid Atresia
4. Tetralogy of Fallot
5. Total Anomalous Pulmonary venous Return

Question 8

What is the “snowball” effect of high pressures in the pulmonary vasculature?

Answer 8

Lead to R -> L Shunt

• PDA/PFO are kept open by high pressures in pulmonary vasculature

Question 9

What is the order of structures in the Ascending Aorta?

Answer 9

Think ABCS
1. Aorta
2. Brachiocephalic
3. Left Common Carotid
4. Left Subclavian

• Note The right subclavian and right common carotid artery branches of the brachiocephalic artery

Question 10

What is Hypoplastic Left Heart Syndrome (HLHS)?

Answer 10

A heart condition where the left ventricle is underdeveloped and there is severe narrowing in the ascending aorta.

• Mitral valve and aorta may also be missing.
• A Ductus arteriosus is necessary for systemic blood flow.

Question 11

Pathophysiology: What are the latent implications of Hypoplastic Left Heart Syndrome (HLHS)?

Answer 11

Restricted blood flow to systemic circulation

• All blood flow comes (R -> L) via PDA/FO
• PDA dependent to have ANY systemic circulation through the RV and a ASD is necessary for pulmonary blood flow to get in into the RA.

Question 12

Clinical Manifestations of Hypoplastic Left Heart Syndrome (HLHS)

Answer 12

• Poor feeder w/SOB and is weak
• Typically pale w/Poor perfusion
• peripheral pulses are poor to absent.
• A loud murmur (midsystolic gallop), hepatomegaly, and cardiomegaly will also be present.

Question 13

Management of Hypoplastic Left Heart Syndrome (HLHS)

Answer 13

Prostaglandin (PGE1) to maintain an open ductus

• No handling or suctioning at all
• Intubate and ventilate, high CO2 with lowest PO2’s (Keep SpO2s @75-80) to keep PVR –query–

Question 14

What are the oxygen goals for babes with Hypoplastic Left Heart Syndrome (HLHS) when ventialted??

Answer 14

High CO2’s w/pH of 7.20

• Target lower end of PO2 to keep PVR high
• my need subatmospheric O2 w/CO2 or NO2 mixtures to get 17-20% (don’t worry about this last point)

Question 15

Why are O2 targets low for Hypoplastic Left Heart Syndrome (HLHS)?

Answer 15

To maintain a balanced pulmonary and systemic circulations and optimize oxygen delivery to the body. Think of it as permissive hypoventilation.

• High CO2s (or low O2’s) -> vasoconstriction increases (or keeps) PVR to shunt blood where it needs to go.
• Left heart is underdeveloped, meaning the right side has to work harder. Elevated pulmonary pressures can make it difficult for blood to flow through the lungs and receive oxygen.

Question 16

What is Ventricular Septal Defect (VSD)?

Answer 16

When there is a opening between the right and left ventricles via the septum.

• Pressure are the same on both sides.
• So the size of the septal defect is important in this defect

Question 17

Pathophysiology of Ventricular Septal Defect (VSD)?

• snowball effect?

Answer 17

Increase in pulmonary blood flow. Acyanotic => (L->R) Shunt.

• Small VSD is asymptomatic
• Large VSD = increased pulmonary blood flow + CHF
• Increased pulmonary blood flow causes increased PVR and hypertrophy of the pulmonary arteries and L atrium.

Question 18

Management of Ventricular Septal Defect (VSD)?

Answer 18

Most heal spontaneously.

• Large VSD cause CHF which may require O2 and/or Vasodilators (to decrease load), and surgical repair.

Question 19

Clinical Manifestations of Ventricular Septal Defect (VSD)?

Answer 19

Depends on size, but, a large VSD would cause CHF.

CHF includes:
- tachypnea
- tachycardia
- feeding difficulties, - diaphoresis
- failure to thrive
- cardiomegaly

Question 20

What is a Atrial Septal Defect (ASD)?

Answer 20

Opening between the right and left atria via septum.

• ASD and PFO are not the same thing.

Question 21

Pathophysiology of Atrial Septal Defect (ASD)?

Answer 21

Increase in pulmonary blood flow.

Size dependent
- (mostly asymptomatic). (L->R) Shunt.
- Can cause volume overloading of the R heart -> exercise intolerance (CHF,SOB)

Question 22

Atrial Septal Defect (ASD)

• what are different types of septal defects that can be causal agents?

Answer 22

• Ostium secundum is NOT a foramen ovale either
• Sinus venosus : (chamber upstream of R atrium that houses the cardiac pacemaker and becomes incorporated into the R atrium during development)
• Ostium primum: (defect in the atrial septum at the level of the tricuspid and mitral valves)
• Ostium secundum: (hole in the center of the atrial septum)

Question 23

Management of Atrial Septal Defect (ASD)

Answer 23

Surgical repair on bypass if severe

Question 24

To do list

Answer 24

Add Croup, and pre and post ductal issues

Question 25

What is Intraventricular Hemorrhage (IVH)?

Answer 25

TLDR: Bleeding of vessels in the brain bc of immature vasculature = can't regulate cerebral blood flow = **compression of brain parenchyma**. - Blood fills the ventricles bc of poor cerebral blood flow management -> compression of brain parenchyma - *not laymen defintion* Intracranial hemorrhage arising in the germinal matrix and periventricular regions of the brain in preterm infants.

Question 26

Risk factors of Intraventricular Hemorrhage (IVH)

Answer 26

Prematurity, birth asphyxia, hypoxia, hypercapnia, acidosis, RDS, pneumothorax, changes in hemodynamic status, rapid blood volume expansion, PDA.

Question 27

Pathophysiology of Intraventricular Hemorrhage (IVH)

Answer 27

Bleeding starts in the germinal matrix (a highly vascular network that matures and gets smaller w/gestational age)

Question 28

Why is Intraventricular Hemorrhage (IVH) especially problematic in preemies?

Answer 28

Preemies can't tolerate flucations in hemodynamics bc the fragile blood vessels wouldn't be able to handle them.

Question 29

How many ventricles are affected by Intraventricular Hemorrhage (IVH)?

Answer 29

4 Cerebral ventricles. - Lateral Ventricles (1&2) - 3rd ventricle - 4th ventricle

Question 30

Clinical Manifestations of Intraventricular Hemorrhage (IVH)?

Answer 30

subtle to catastrophic Asymptomatic to bulging fontanels, sudden drop in hematocrit, apnea, hypotension, hypotonia, bradycardia, acidosis, seizures, and loss of consciousness. - Best way to identify and monitor IVH is w/ultrasound or CT scan

Question 31

Management of Intraventricular Hemorrhage (IVH)

Answer 31

Give supportive treatments that avoid risk factors, So... - Maintain CPP - Suppress seizures - manage resp. status - Shunts & punctures to drain excess fluid

Question 32

What is Tricuspid Atresia?

Answer 32

The tricuspid valve doesn't exist, so blood can't go from the RA to the RV. (R->L) Shunt. Needs 1 for survival: - Atrial septal defect - Ventricular septal defect - Ductus Arteriosus

Question 33

Clinical manifestations of Tricuspid Atresia?

Answer 33

- Cyanosis & SOB - weak feeder/weak in general - blue baby - may or may not have a murmur. - Probably CHF when the above close.

Question 34

Management of Tricuspid Atresia

Answer 34

- Prostaglandin E1 to keep the ductus open - Immediate catheterization to improve atrial flow by enlarging the PFO - Surgical connection of PA to RA with septal defect closure

Question 35

What is Tetralogy of Fallot (TOF)

Answer 35

Cyanotic heart with 4 defects: 1. **Overriding aorta**, aortic valve is too wide and is open to both ventricles (RV and LV). 2. Pulmonary artery stenosis 3. R ventricular hypertrophy 4. VSD (hole in wall between RV and LV)

Question 36

What needs to be present for a babe w/Tetralogy of Fallot (TOF)

Answer 36

A patent ductus arteriosus (PDA) to allow pulmonary blood flow if pulmonary stenosis is severe.

Question 37

Is Tetralogy of Fallot (TOF) acanoytic or cyanotic?

Answer 37

Cyanotic (but can be both bc of pressure gradients) - Decreased pulmonary blood flow - severe if pressure gradients cause it to be a (R->L) shunt.

Question 38

Pathophysiology of Tetralogy of Fallot (TOF)?

Answer 38

- PDA must be present. - Causes an outflow obstruction to the right ventricle causing increased right ventricular pressures, decreased pulmonary blood flow, shunting through the VSD.

Question 39

Clinical manifestations of Tetralogy of Fallot (TOF)?

Answer 39

- Severe cyanosis at birth & tachypneic - SpO2s in the 60s & SOB - failure to thrive - clubbing - syncope (loses conscious freq) - **TET Spells**

Question 40

What are TET spells?

Answer 40

Any Stress/activity that affects blood flow w/in heart by increasing (R->L) shunts. - leads to hyperpnea, decreased heard sounds, and fainting.

Question 41

How do you correct TET spells?

Answer 41

Knees to chest & O2. - older kids can squat

Question 42

What does squatting do for older kids w/a potential heart defect?

Answer 42

Increases PVR and decreases severity of (R->L) shunt across VSD.

Question 43

Pathophysiology of TET spells?

Answer 43

1. Tet spells decrease SVR 2. lead to (R->L) shunt 3. Lead to decreased blood flow to lungs

Question 44

CxR of Tetralogy of Fallot (TOF) would have what sign?

Answer 44

Boot

Question 45

What is the difference between ASD and Ostium secundum

Answer 45

ASD’s are PFO’s that failed to close. Ostium Secondum (and others) are defects like secondary holes/failure of tissue to form properly

Question 46

Reference this as you review aka download image