Congenital Cardiac Disease

Question 1

How does the heart develop in the embryo at the 2 week mark?

Answer 1

heart develops from 2 simple epithelial tubes

Question 2

How does the heart develop in the embryo at the 3 week mark?

Answer 2

two simple epithelial tubes fuse to form a single chambered heart

• heart elongates and bends on itself
• endo, myo and epicardium differentiate
• heart begins to beat

Question 3

How does the heart develop in the embryo at the 4 week mark?

Answer 3

primitive heart, atrial segment assumes cranial position

Question 4

How does the heart develop in the embryo at the 5 week mark?

Answer 4

endocardial cushions grow towards each other and fuse

Question 5

How does the heart develop in the embryo at the 8 week mark?

Answer 5

partitioning into 4 chambered heart complete

Question 6

What are the two openings that connect the left and right side of the heart in the embryonic stage?

Answer 6

ductus arterious and foramen ovale

Question 7

True or False: Blood flows from the heart to the lungs in the embryonic heart in order to oxygenate the developing lungs

Answer 7

False-blood is shunted from the heart to the lungs for the most part and blood is pushed through the ductus arterious, this is done to protect the developing lungs

Question 8

How does the fetus recieve oxygenated blood?

Answer 8

from the mother via placenta and travels back via the umbilical vein

Question 9

When do the ductus arterious and foramen ovale usually close?

Answer 9

foramen ovale-a few days after birth

ductus arterious-within hours after birth

Question 10

What are the common acyanotic defects of CHD?

Answer 10

Atrial Septal Defect
Patent Ductus Arteriosum
Ventricular Septal Defect

Question 11

What is the difference between acyanotic and cyanotic defects?

Answer 11

Acyanotic is the absence of cyanosis which means it does not affect oxygen levels in the body

Cyanotic defects mean there is mixing of oxygenated and non-oxygenated blood, which leads to cyanosis

Question 12

What are common cyanotic defects of CHD?

Answer 12

• transposition of the great vessels
• pulmonary valve atresia
• tetralogy of fllot
• hypoplastic left heart syndrome
• shone’s syndrome
• TAVPR
• coarctation of the aorta

Question 13

What type of shunt does patent ductus arteriosum (PDA) create?

What is the clinical presentation of PDA?

Answer 13

left to right shunt (aorta to pulmonary artery) which creates high pressure in the pulmonary artery and may need surgical intervention

infant might fatigue quickly, susceptible to pneumonia

Question 14

What type of shunt do atrial septal defects cause?

Answer 14

left to right shunt which creates volume overload to the right heart and results in pulmonary vasculature damage, this may result in right heart failure and cause a shortened life span

Question 15

What is the most common congenital heart defect?

What type of shunt does this defect create?

What happens if pressures in the right ventricle become too high?

Answer 15

Ventral Septal Defects

Left to Right shunt

blood can shunt right to left which is a condition called Eisenmenger’s syndrome (a cyanotic problem)

Question 16

What is coarctation of the aorta?

Answer 16

pinching of the aorta which may be due to abnormal involution of ductus arteriosus

Question 17

What is Tricuspid Atresia?

What type of shunt does it create?

Answer 17

when tricuspid valve fails to develop which leads to limited blood flow from RA to RV, leading to an underdeveloped RV

right to left shunt

Question 18

What is pulmonary valve atresia?

Answer 18

pulmonary valve fails to develop which means no exit from the right ventricle and blood gets regurgitated into the left atrium via the foramen ovale

requires intervention soon after birth

Question 19

What four defects form tetralogy of fallot?

What are the signs of this defect?

Answer 19

• Ventricular Septal Defect (VSD)
• Pulmonary Valve Stenosis
• Overriding Aorta (usually lies over VSD)
• RV hypertrophy

blueness appears soon after birth, in infancy or childhood and infants might have sudden episodes of cyanosis or unconsciousness

Question 20

What is transposition of the great vessels?

Answer 20

positions of pulmonary artery and aorta reversed which means deoxygenated blood from RV goes into systemic circuit and O2 from blood goes back into the lung

child only survives if AS, VSD, or PDA present

Question 21

What is hydroplastic left heart syndrome?

Answer 21

failure or inadequate development of the left ventricle (variable aortic and mitral involvement)

• child is dependent upon a PDA for systemic perfusion
• without intervention, HLHS is fatal within the first weeks of life

Question 22

What is the Norwood procedure?

When is it usually performed?

Answer 22

surgeons create a new aorta and connect it to the right ventricle, a shunt is then placed from either the aorta or righ ventricle to the pulmonary arteries making the heart a “single ventricle” capable of pumping mixed blood to lungs and periphery

first 2 weeks of life

Question 23

What is the bi-directional glenn shunt procedure?

When is it usually performed?

Answer 23

creates a direct connection between the pulmonary artery and the superior vena cava which allow direct return of venous blood to lungs from the UEs, right ventricle still pumps mixed blood but this procedure reduces it’s work

4-6 months of age

Question 24

What is the fontan procedure?

Answer 24

continuation of bi-directional glenn shunt procedure where the inferior vena cava is also attached to the pulmonary arteries, venous blood now completely bypasses the right side of the heart

once this procedure is complete oxygen rich and oxygen poor blood no longer mix in the heart and the infant’s skin will be cyanotic and the child may still need a heart transplant

Question 25

What are the PT considerations when working with children who have CHD?

Answer 25

• labored breathing and increased RR
• Impaired tolerance to activity
• Irritable (Track Using NIPS scale)
• Post Op: prevent complications and educate the family
• early mobilization
• Positioning: prone->side lying->supine for ventilation/perfusion matching
• some pts. may need guidance away from competitive sports