Congenital Heart Disease

What is different between adult and pediatric PT in terms of discharge plan?

Pediatrics do not have the options of short-term rehab or LTACH, will be in the hospital or receiving therapy at home or school

What is the #1 birth defect?

CHD

In how many of every 1,000 live births will CHD be seen?

6-8

What fraction of children born with CHD require surgical intervention within the first year?

1/3

What is congenital heart disease (CHD)?

Problems with the heart's structure that are present at birth and may change the normal flow of blood through the heart

What percentage of children born with CHD survive into adulthood?

85-90%

What is the cause of CHD?

No known cause

What are five factors that might contribute to a child being born with CHD?

- Might be genetic: Trisomy-21 (50%)

- Smoking

- Medication (e.g. lithium)

- Drug use during pregnancy

- Maternal diabetes

When does the gestational period begin?

From the moment of conception

How many weeks of pregnancy are there typically?

About 40

When do the 40 weeks of pregnancy begin?

From the woman's last period (e.g. six weeks pregnant means it has been around four weeks since conception)

What is an embryo?

Developing human through the 8th week of gestation (about the 10th week of pregnancy)

What is a fetus?

Developing human from the 9th week of gestation (about the 11th week of pregnancy) until birth

When is the critical period for development of the heart? When is it highly sensitive and less sensitive?

- Highly sensitive period: halfway through week three to about 3/4 of the way through week six

- Less sensitive period: about 3/4 of the way through week six to the end of week eight

What are three major congenital anomalies that can be seen during the critical period in heart development?

- Truncus arteriosus (TA)

- Atrial septic defect (ASD)

- Ventricular septal defect (VSD)

Why is it important to understand how early in pregnancy important structures are developing?

Women may not even know they are pregnant at this point and can still be engaging in behaviors that are risk factors (e.g. smoking)

On what day do heart tubes develop?

Day 21

On what day do heart beats develop?

Day 22-23

On what day can a primitive atrium/ventricle and TA be seen?

Day 23

On what day does the heart begin circulating blood to the body?

Day 27

During which week does the atrium divide into right and left atria?

Week 4

On what day does cardiac (dextral) looping occur?

Day 23-35

What is cardiac (dextral) looping?

Heart twists on itself and the bottom comes to the top, taking on the orientation it will have through adulthood

During which week do the ventricles divide?

Week 7

During which week is cardiac development completed?

Week 10

How does blood flow through the fetal circulatory system?

- Oxygenated blood arrives via the umbilical vein

- Ductus venosus

- IVC

- RA

- Foramen ovale

- LA

- LV

- Aorta

- Body/brain (smaller portion of blood comes from RA)

- RV

- Pulmonary artery

- Ductus arteriosus

- Aorta

Which structure allows blood to bypass the lungs?

Ductus arteriosus (blood coming from the pulmonary trunk)

Why does some of the blood bypass the lungs in fetal circulation?

Blood is not being oxygenated as the fetus is not yet breathing

What causes blood to move through the fetal heart circulation?

Moves from areas of high to low pressure

Can CHD be diagnosed prenatally?

May be seen on US but often not discovered until after birth

What are five signs of CHD in infants?

- SOB/atypical breathing pattern

- Motor delay

- Feeding difficulty

- Poor weight gain and growth

- Impaired state control

What are four signs of CHD in toddlers/adolescents?

- Poor activity tolerance

- Decreased coordination

- Decreased strength ROM

- Self-shunting (squatting)

What is self-shunting seen in toddlers and adolescents with CHD?

May squat down, which increases pressure to pump blood back up to the heart

What percentage of CHD survivors will have a neurodevelopmental disability?

50%

What percentage of CHD survivors will have physical or cognitive delay?

20-30%

What percentage of CHD survivors will have a mood/behavioral disorder or anxiety?

10-15%

What are two other neurodevelopmental impacts that may be seen in CHD survivors?

- Impaired executive function

- Impaired health-related QoL

What is acyanotic CHD?

- Shunting of blood from L to R

- Little to no effect on oxygen level of body

What is cyanotic CHD?

- Shunting of blood from R to L, mixing of blood

- Blood with < oxygen than normal

- Skin, lips, and nail beds appear bluish

- Polycythemia and risk of CVA (connection to coagulation)

What are five defects seen in acyanotic CHD patients?

- Ventricular septal defect (VSD)

- Atrial septal defect (ASD)

- Complete atrioventricular septal defects (complete AV canal)

- Patent ductus arteriosus (PDA)

- Coarctation of the aorta

What are six signs/symptoms of acyanotic defects?

- Fatigue

- Tachypnea

- Difficulty feeding

- Higher HR/RR on inspection

- Accessory muscle use

- Murmur heard on auscultation

What are three surgeries performed for acyanotic defects and which defects are they performed for?

- Patch closure (usually around six months for VSD and 2-3 years for ASD)

- Valve repair

- IV indomethacin (PDA)

What is coarctation of the aorta?

Narrowing of the aorta that obstructs LV outflow

What are two symptoms of coarctation of the aorta in infancy?

- CHF

- Elevated UE BP but normal to low LE BP (one of the big differentiators)

Which symptoms are seen initially after coarctation of the aorta?

Initially asymptomatic

What are six signs/symptoms of coarctation of the aorta after infancy?

- HBP

- Headaches

- Muscle weakness

- Leg cramps or cold feet

- Nosebleeds

- Chest pain

What is the goal of surgery for coarctation of the aorta?

To remove coarctation

How is surgery for coarctation of the aorta typically performed?

Often cardiac catheterization

What are five defects seen in cyanotic CHD patients?

- Tetralogy of fallot

- Transposition of the great vessels

- Total anomalous pulmonary venous return (TAPVR): pulmonary veins connect to R atrium instead of L so all blood goes back to the R side of the heart

- Truncus arteriosus: aorta and pulmonary trunk are together

- Single ventricle defects: pulmonary or tricuspid atresia (small/no opening where valve is supposed to be), hypoplastic left heart syndrome (most severe CHD, LV does not develop at all)

What are the fatal fours of fallot?

- RV hypertrophy

- RV outflow tract obstruction (so blood cannot get through to the pulmonary artery)

- Ventricular septal defect

- Overriding aorta

What are four abnormalities seen in children with cyanotic heart defects?

- Cause severe cyanosis/hypoxemia

- Blood is pumped out to the body prior to being oxygenated by the lungs

- Blood shunts R to L through an ASV or VSD

- Blood vessels in the incorrect place

How is the tetralogy of fallot often medically managed?

Often require early and repeated surgeries

What is CHD the most common indication for?

Heart transplant

What percentage of total heart transplants performed are for patients with CHD?

12% (600-700 transplants worldwide/year)

What is the one-year survival rate for heart transplant?

80-90%

What percentage of children on the waiting list will not receive a heart?

30-50%

What is another outcome following heart transplant that is required by some CHD patients?

Second transplant

What four settings where a physical therapist might see patients with CHD?

- Acute care

- Early intervention

- School based services

- Across practice settings for adults

Which body structures/functions should be screened in children with CHD?

- CP

- GI

- Neurological

- Integumentary

- MSK

What are five elements of the PT evaluation, examination, and intervention for patients with CHD?

- Review medical record and gather social hx

- Assess vitals/hemodynamics

- Assessment of motor function, education for parents on back to sleep, supervised tummy time, position changes, Kangaroo care

- Assessment of state, education on avoiding increased agitation, and proper feeding as goal is for the baby to gain weight

- Discuss family dynamics and social implications of caring for this child

What is a normal neonatal HR in a patient with CHD when they are awake and sleeping?

- Awake: 100-250 bpm

- Sleeping: 90-160 bpm

What is a normal neonatal RR in a patient with CHD?

40-60

What is a normal neonatal BP in a patient with CHD?

60-75/31-45 (diastolic)

What is the neonatal infant pain scale (NIPS)?

Evaluates pain in neonatal infants based on facial expression and how they are acting

What is the NIPS indicated for?

Full- or pre-term infants that are less than one year old

What is the max score on the NIPS? What score indicates pain?

- Max score: 7

- Score of > 3 indicates pain

Why is swaddling helpful for getting the baby to rest?

- Important to maintain physiological flexion, especially for premature babies

- Helps with state control

- As they grow, ANS needs to be in more PNS mode

How will babies vitals change if they are not in a good state or have poor state control?

- HR/RR increase

- BP decreases

- SpO2 decreases as well

What are two things to remember when swaddling a baby?

- Neck and shoulders should be in the top of the blanket to support the neck/arms

- Arms should be inside the blanket, might have to hold them in yourself as the baby may fight this

What are four benefits of positioning/handling of children?

- Provides comfort and conserves energy (containment)

- Promotes midline orientation and symmetrical positioning to enhance neurobehavioral organization

- Facilitate development

- Skin breakdown

What is important to make sure of if positioning a child in prone?

Make sure head is turned to the side so they can breathe

What is kangaroo care?

A method of caring for premature babies in which the infants are held skin-to-skin with a parent, usually the mother, for as many hours as possible every day

What are five benefits of kangaroo care?

- Helps infant with state regulation

- Breathing pattern and O2 saturation

- Weight and growth

- Breastfeeding patterns

- Parent-baby bonding

What are six important points for education on family dynamics?

- Infection prevention

- Proper handling

- How to introduce siblings/family members

- Normal developmental milestones (how the baby acts/looks)

- Parental stress relief and/or referral to support groups

- Reassurance/education regarding lines, tubes, alarms, and roles of staff members

What are eight sternal precautions/considerations for children born prematurely with CHD?

- Initiate tummy time while lying on parent's chest in reclined position

- Allow baby to roll into prone, however change position with signs of pain or distress

- Burp in supported position or chest to chest

- Lift by supporting under the back, pelvis, and shoulders

- Support trunk in sitting

- Keep incision covered well during feedings

- Cannot return to daycare until after 2 week checkup

- Older children cannot lift > 10% body weight (backpack) and must avoid contact sports until full healing has occurred

What is the FLACC pain scale?

- Face, legs, activity, crying, consolability

- Can be used for patients > 2 months to 7 years old OR for patients who cannot communicate their pain

- Patient is observed for 1-5 minutes

What are four aspects of inspection during the CP evaluation for children born prematurely with CHD?

- Color

- Breathing pattern

- State

- Position

What are three aspects of palpation during the CP evaluation for children born prematurely with CHD?

- Pulses

- Chest expansion

- Tactile fremitus

What are two aspects of auscultation during the CP evaluation for children born prematurely with CHD?

- Lung sounds

- Heart sounds

What is the role of the PT for children born prematurely with CHD?

Assessment and intervention for:

- Positioning

- Promoting normal development

- Parent handling

What is the role of the PT in acute care s/p revision or new open heart surgery?

- Early mobilization

- Airway clearance

- D/c planning

- Patient/family education

What are three goals of PT in acute care s/p revision or new open heart surgery?

- Reduce LOS

- Decrease neurodevelopmental delay

- Improve functional independence

What is the role of the PT in outpatient rehabilitation s/p revision or new open heart surgery?

Cardiac rehabilitation:

- Individualized exercise to improve endurance and functional independence

- BP and EKG monitoring

- Weekly cardiac, medication, and healthy lifestyle education

- Work with multidisciplinary team

What are six complications seen with adult congenital heart disease?

- Arrhythmias

- Endocarditis

- Stroke

- Heart failure

- Pulmonary HTN

- Heart valve problems