Congenital Heart Defects

Question 1

Etiology

Answer 1

• Genetic component
• Associated with other conditions (Downs, Turner, Williams, Marfan, DiGeorge, Costello, VACTERL)
• Maternal medical problems (diabetes, obesity)
• Environmental conditions

Question 2

Defects- Acyanotic

Answer 2

• Atrial septal defects (ASD)
• Ventricular septal defects (VSD)
• Atrioventricular septal defects (AVSD)
• Patent ductus arteriosus (PDA)
• Coarctation of the aorta
• Aortic valve stenosis
• Pulmonary valve stenosis

–

• Child is pink with normal oxygen saturation
• Blood shunts from left side of heart to right
• Oxygenated blood goes to lungs and body

Question 3

Defects- Cyanotic

Answer 3

• Tetralogy of Fallot (TOF)
• Double-outlet right ventricle
• Transposition of the great arteries
• Tricuspid atresia
• Pulmonary atresia
• Truncus arteriosus
• Hypoplastic left heart

• Arterial oxygen saturation 15-30% below normal
• Blood shunts from the right side of heart to the left
• Unoxygenated blood is returned to body

Question 4

ASD

Answer 4

–Failure of septal tissue between atria to form (not PFO)

–Oxygen rich blood flows into chamber with oxygen poor

–Small defects spontaneously close

–Larger defects corrected surgically 2-5 yo

Question 5

VSD

Answer 5

–Most common CHD

–Often close spontaneously by preschool

–Surgical repair at 1yo for large VSD not closing

Question 6

AVSD

Answer 6

–Seen commonly in kids with Down syndrome

–Extra blood flows to lungs; causes pulmonary hypertension

–Heart overworked

and enlarges

–Surgery in early infancy

Question 7

PDA

Answer 7

–Seen in preemies or maternal rubella

–Communication between pulmonary artery and descending aorta

–Closes soon after birth except in response to hypoxia

–Treated with prostaglandins to encourage patency

–Treated with prostaglandin inhibitors to encourage closure (indomethacin)

Question 8

Coarctation of Aorta

Answer 8

–Narrowing of aorta

–Must keep PDA open with meds

–Requires surgical correction

Question 9

Pulmonary Stenosis:

Answer 9

• narrowing of the right ventricular outflow tract

Question 10

Aortic Stenosis:

Answer 10

•narrowing of the left ventricular outflow tract

Question 11

TOF

Answer 11

Most common complex cardiac defect

–VSD

–Pulmonary stenosis

–Overriding aorta (sits over VSD, not left ventricle)

–Hypertrophy of right ventricle

Must be surgically repaired – often in stages

Question 12

Transposition of Great Arteries

Answer 12

• Pulmonary artery arises from left ventricle
• Aorta arises from right ventricle

Systemic blood returns to body unoxygenated and…

Pulmonary blood returns to lungs fully oxygenated

—–Death unless ductus arteriosus remains open

—–Surgery (arterial switch in first month of life)

Question 13

Double-Outlet Right Ventricle

Answer 13

–Aorta and pulmonary artery come off of right ventricle

Question 14

Hypoplastic Left Heart Syndrome

Answer 14

–Small or absent left ventricle and hypoplasia of ascending aorta

–Meds used to keep PDA

–Surgical correction or heart transplant

Question 15

Cyanotic Episodes-what can we do?

Answer 15

•Squat or bring knees to chest

—–Increases systemic vascular resistance to help increase pulmonary blood flow

Question 16

Surgical Repair Required

Answer 16

• Tricuspid atresia: lack of communication between right atrium and right ventricle
  
  • Typically ASD and/or VSD exist to allow pulmonary blood flow
• Pulmonary atresia: obstruction of blood flow from right side of hear to lungs
  
  • Blood flow maintained by PDA
  • ASD and/or VSD may also be present to shunt blood RàL
• Truncus Arteriosus: aorta and pulmonary artery fail to separate leading to a common trunk arising from both ventricles

Question 17

Cardiac Transplant

Answer 17

• Due to heart failure, cardiac defects, cardiomyopathy, pulmonary hypertension —Most common due to CHD or cardiomyopathy
• Graft vs host disease (GVHD)
• Results in denervated heart so cannot assess exercise intensity via heart rate – must use Rate of Perceived Exertion Scale (RPE) such as the Borg (11-13 desired) or modified Borg (5 desired)

•

Question 18

PT Implications

Answer 18

• Fatigue
• Poor feeding/nutrition/growth – takes too much effort/calories to sustain eat/life – dx failure to thrive (FTT) and gross motor delay
• Low oxygen sats
• Medications

–Anticoagulants

–Immunosuppressants (transplant)

Question 19

Children at highest risk for Neurodevelopmental Impairments

Answer 19

• Neonate of infant requiring open heart sx
• Child with other cyanotic hear lesion not requiring surgery
• Congenital heart disease in combo with..

–Prematurity

–DD

–Suspected genetic abnormality or syndrome associated with DD

–H/O mechanical support

–Heart transplant

–CPR

–Hospital stay >2 weeks post surgery

–Perioperative seizures

–Abnormal finds on brain imaging or small head circumference