637 Ch22 Pediatric CVP Implications

When does the respiratory system begin developing?

4th week of gestation

What are the four stages of maturation of the lungs and their key features? When do most of the alveoli develop?

1. Pseudoglandular period (6-16)

   1. All in place except those involved w/ gas exchange

2. Canalicular period (16-26)

   1. Lung tissue becomes vascularized

3. Terminal saccular period (26-birth)

   1. Surfactant starts being produced at 20 and reaches maturation at 34

4. Alveolar period (32wk-8years)

   1. After birth, surface area & lung tissue grow rapidly, 95% of alveoli develop AFTER birth

When does the heart beat start? When does circulation extend around the fetus’ body? When does the atria divide into R and L? When do all 4 form?

• Day 22-23, circulation extends around fetus’ body by day 27

• Middle of 4th gestational wk - atria divide

  • All 4 by 7th gestational wk

What is the process of fetal circulation and the structures involved with it? How is CO2 removed?

• Heart pushes most blood past the lungs to the LV w/ foramen ovale

  • O2 obtained and CO2 is removed through the placenta

  • Foramen ovale (shunt btwn LA&RA) fuses after 6mo, closes hours after birth

  • Ductus arteriosus (PA to aorta) fuses after weeks, closes 12-24hr after birth

  • Pulmonary blood vessel vasodilate after birth to allow enough blood for gas exchange

What does the foramen ovale and ductus arteriosis connect? What kind of blood do they deliver?

• Foramen ovale connects R atrium to L atrium

  • Oxygenated blood bypasses fetal lungs to L atrium then L ventricle and ascending aorta

• Ductus arteriosus connects pulmonary artery to aorta

  • Right ventricle deoxygenated blood bypasses fetal lungs to descending aorta, organs, and back to placenta

What is the etiology of congenital heart disease (CHD)?

• Genetic link

• Environmental factors - asbestos, lead exposure

• Maternal factors - diabetes, smoking, alcohol, folic acid (B9), obesity, diet, depression

What are acyanotic lesions? Sxs? Findings? Examples?

• O2SAT MAINTAINED; increase pulmonary blood flow (increased risk of pHTN) and fully oxygenated blood is brought back to lungs and body (L to R shunting)

• Sxs: sweating, increased RR, HF, skin well perfused

• Findings: reduced PaO2 to periphery, reduced systemic SV, increased CO

• EX: ventricular septal defect, atrial septal defect, patent ductus arteriosus, coarctation of the aorta, pulmonary stenosis, aortic stenosis

How is O2sat affected in acyanotic and cyanotic lesions?

• Acyanotic - O2sat maintained

• Cyanotic - O2sat NOT maintained, reduced

True or false: cyanotic and acyanotic lesions have signs of cyanosis

False, only cyanotic lesions

What are cyanotic lesions? Sxs? Findings? Examples?

• O2SAT REDUCED; most of the blood bypasses the lungs, deoxygenated blood flows back to body (R to L shunting). More RBCs produced → polycythemia → increased risk for CVA; more concerning, will need surgery immediately!

• Sxs: fatigue, fainting, SOB, chest pain, HF

• Findings: increased RBC count, cyanosis, SpO2 70-85%, capillary refill is delayed

• EX: hypoplastic left heart syndrome, tetralogy of fallot, transposition of the great arteries, total anomalous pulmonary venous return, tricuspid atresia, pulmonary atresia, truncus arteriosus, pediatric heart failure

What are typical sxs of acyanotic lesions?

Body working harder i.e. difficulty w/ feeding, fussing, crying, sweating

What is an atrial septal defect? Findings? Tx?

• ACYANOTIC LESION

• Hole btwn R & L atriums that doesn’t close after birth (such as foramen ovale)

• Findings: heart murmur & enlarged pulmonary A

• Closed w/ surgery if it doesn’t close on it’s own by 2-3y/o

*What is patent ductus arteriosus? What is normally supposed to happen? Findings? Tx?

• ACYANOTIC LESION

• Pathway of normal fetal circulation btwn descending aorta & pulmonary A doesn’t close after birth

  • Too much blood enters the lungs (risk of pHTN, destruction of pulmonary tissue, causes scarring → fibrosis)

  • High incidence in premies

• Normal: O2 level increases → reduces prostaglandin E1 → closes PDA (within 5-14 days)

• *Findings: tachycardia, respiratory distress, limited weight gain

• *Tx: surgery or Indomethacin to reduce prostaglandin prod.

What is ventricular septal defect? ? Findings? Tx?

• ACYANOTIC LESION

• Hole in septum separating ventricles: small deficits acyanotic, large deficits complications → bacterial endocarditis (fever), pulmonary vascular obstructive disease, aortic regurgitation, lower RTIs (risk of infection is HIGH), CHF (ventricular arrhythmias)

• Findings: feeding difficulties (hard to eat and breathe at the same time), rapid breathing, poor weight gain, irritability, restlessness, dehydration

• May close 5mo-6y/o w/o intervention; surgery for more complicating defects

*What is atrioventricular septal defect (AVSD)? Findings? Tx?

• ACYANOTIC LESION

• Failure of formation of embryonic atria/ventricles (one big chamber, no septum development)

  • Down syndrome

  • Impacts endocardial cushion development

• Findings: pHTN, lung congestion, HF

• *Tx: surgery REQUIRED within the first few months (BiPAP/CPAP to prolong if too young)

What is coarctation of aorta? Findings? Tx?

• Narrowing of aorta which obstructs LV outflow; >50% must be narrowed, diagnosed w/ echocardiogram NOT ultrasound

  • Pressure proximal to constriction of aorta is increased = HTN in upper body above level of coarctation, normal-low BP in LE

  • Many go undiagnosed until later, 50% rate of mortality under 30y/o if not tx

• Findings: secondary HTN, systemic congestion (increased HR), pulmonary edema (weight gain fluctuations)

• Tx: surgery to remove constriction of aorta, LIKELY TO REOCCUR even if tx

Differentiate the key findings between each of the acyanotic lesions.

• Atrial septal defect

  • Heart murmur

  • Enlarged pulmonary A.

  • Can close on it’s own or surgery

• Patent ductus arteriosus

  • Too much blood in lungs

  • Premies

  • Too much prostaglandin E1, doesn’t close PDA

  • Tachycardia, respiratory distress, limited weight gain

  • Indomethacin or surgery

• Ventricular septal defect

  • Large defect comp.

  • Fever, high risk of infections, feeding difficulties, tachypnea, poor weight gain, irritability, restlessness

  • Can close on it’s own or surgery

• AV septal defect

  • 1 chamber

  • Down syndrome

  • pHTN, lung congestion, HF

  • surgery REQUIRED

• Coarctation of aorta

  • HTN UE, normal/low LE, systemic congestion, pulm edema

  • High rate of mortality, surgery tx, may reoccur

What is hypoplastic left heart syndrome (HLHS)? Cause? Fx? Sxs? Tx?

• CYANOTIC LESION

• Idiopathic cause, underdeveloped LV, aortic & mitral valve stenosis (or atresia), and coarctation of aorta

  • Diagnosed during pregnancy or shortly after birth

• Fx: L side of heart doesn’t form correctly

  • Systemic blood flow is dependent upon PDA, once closed → severe CHF

• Sxs: tachycardia, tachypnea, poor feeding, cyanosis, weakness and cold extremities

• Tx: mech vent, surgery soon after birth → not permanent fix, 3 stage palliative surgery or heart transplant

What is tetralogy of fallot? Cause? Fx? Sxs? Tx?

• CYANOTIC LESION

• Tetrad of defects: pulmonary stenosis, overriding aorta, ventricular septal defect, RV hypertrophy

  • Degree of cyanosis dependent on severity of stenosis

• Idiopathic cause, RF: family hx, virus during pregnancy, alcohol, smoking, or eating poorly during pregnancy, mom >35y/o, down syndrome

• Fx

  • Pulmonary stenosis - obstruction to RV outflow → hypertrophy

  • VSD: oxy&deoxy blood mixing

  • Aorta above VSD on RA instead of LV → RV hypertrophy

• Sxs: “TET’ spells of cyanosis w eating or crying, SOB, fainting, difficulty w/ weight gain, tachypnea

• Tx: surgery 3-6mo

What is transposition of great arteries? Sxs? Tx?

• CYANOTIC LESION

• Positions of aorta & pulmonary A. are reversed, does not support life, often diagnosed in utero or in first few hours of life

  • Severity is determined by the amount of blood that is mixing on both sides

• Sxs: severe cyanosis if PDA, VSD, or ASD is not available

• Tx: surgery, prostaglandin E1 to maintain PDA; may develop coronary stenosis, arrhythmias, or ventricular dysfunction later in life

What is atresia? Describe atresia of the tricuspid valve, pulmonary valve, and truncus arteriosus.

• CYANOTIC LESION

• Poor/missing development

• Tricuspid valve: obstruction of blood flow to RV which is typically underdeveloped w/ some shunting of blood → lungs (ASD and VSD may be present)

  • Sx: anoxia, R sided HF, dyspnea, severe cyanosis

  • Tx: surgery

• Pulmonary valve: fails to develop leading to an obstruction from R side of heart → lungs; blood flow to lungs is dependent on PDA (ASD and VSD would allow shunting to the body)

• Truncus arteriosus: aorta and pulmonary A. do not separate in development and both empty pulmonary and systemic blood flow into one large vessel, single ventricle (VSD is always present)

What is total anomalous pulmonary venous return? Sxs? Tx?

• CYANOTIC LESION

• Pulmonary veins attach to the R atrium or other veins that attach to the R atrium (ASD might be present which could aide in atrial decompression)

• Sxs: cyanosis, HF, pulmonary congestion

• Tx: surgery ASAP to join pulmonary veins to LA

What is pediatric heart failure? What are the 3 types? Sxs classification?

• Circulation doesn’t meet metabolic needs of body b/c reduced CO or reduced ventricular relaxation (diastolic HF)

  • Cardiomyopathy: categorized as dilated, hypertrophic, or restrictive conditions

  • Dilated: most common cause of heart transplant in children >1y/o, enlarged ventricles w/ reduced systolic ejection and CO

    • More common in males and in infants w/ muscular dystrophy

  • Hypertrophic: reduced ventricle filling & reduced CO; most common cause of SCD in children

  • Restrictive: poor ventricular diastolic relaxation but normal ventricular size

  • Failed palliative of CHDs

• Sxs: Ross Classification for Infants & Children

  • Diaphoresis, tachypnea, dyspnea, tachycardia, hepatomegaly

  • 0-2 (no HF), 3-6 (mild HF), 7-9 (moderate HF), 10-12 (severe HF)

What are the sxs classified in the Ross Classification for Infants & Children? Scoring?

• Diaphoresis, tachypnea, dyspnea, tachycardia, hepatomegaly

• 0-2 (no HF), 3-6 (mild HF), 7-9 (moderate HF), 10-12 (severe HF)

What is a ventricular assist device (VAD)? Goals? Conditions that may need a VAD?

• Mechanical device to help blood flow around the body

• Goals: increase candidacy for transplant, recovery from illness or bridge for designation tx

• 50% reduction in transplant waitlist mortality

• Used in correlation w/ PT: improvement in fx, exercise capacity, accelerated transplant recovery time

• Common conditions: myocarditis, CHD, transplant rejection, cardiomyopathy, heart dysfunction after chemo

What are 8 pediatric respiratory conditions?

• Prematurity

• Persistent pulmonary HTN

• Respiratory distress syndrome

• Sudden infant death syndrome

• Meconium aspiration syndrome

• Bronchopulmonary dysplasia

• Cystic fibrosis

• Primary ciliary dyskinesia

What is extracorporeal membranous oxygenation (ECMO)? Two types? Fx? Can you mobilize pts on ECMO?

• External heart and lung bypass machine

  • V-V (venovenous) lungs

  • V-A (venoatrial) lungs and heart

• Fx: removes blood, adds O2, removes CO2

• Yes, mobilizing pts on ECMO decreases ICU morbidity

What are 6 pediatric conditions with secondary CVP concerns?

1. Down syndrome - chromosomal disorder, endocardial cushion (AV) defect, VSD, ASD, TOF

   1. Cardiac defects & sleep apnea common

   2. Lower PA, reduced muscle strength, endurance, poor eating habits

2. VATER association - vertebral defects, imperforate anus, tracheoesophageal fistula, and radial and renal dysplasia

3. DiGeorge syndrome - immunodeficiency disease w/ significant cardiac changes, TOF, coarctation of aorta, ASD, pulmonary stenosis, hypoplasia left heart, PDA, VSD, truncus arteriosus

4. Marfan syndrome - connective tissue disease, high prevalence of aortic aneurysm & aortic/mitral insufficiency, increased height and decrease weight for age, scoliosis , excessively long extremities, stooped shoulders, depressed sternum, fragility of blood vessels

5. Williams syndrome - chromosomal condition associated w/ supravalvular aortic and pulmonary stenosis

6. Fetal alcohol syndrome - chronic alcohol exposure in utero, facial dysmorphia, poor coordination, hyperactivity, learning disabilities, delays in development

   1. VSD, TOF, pulmonary valve stenosis, PDA commonly seen in this population

What are some activity and breathing impairments associated with pediatric conditions with reduced acitvity and posture?

Muscle weakness, abnormal muscle tone (hypertonicity & hypotonicity), motor learning deficits, medical fragility, motor planning deficits

What are pediatric conditions with pulmonary system impairments?

Cerebral palsy, spina bifida, muscular dystrophy, spinal muscular atrophy, spinal cord injury, severe progressive scoliosis

What are associated impairments with an ineffective cough in pediatric conditions?

• Poor saliva control, poor gag reflexes, muscle spasms, altered breathing patterns

  • CP have increased O2 demands and fatigue faster - take more breaks

What are observations you want to make during a pediatric PT examination?

• Environment

• Resting position

• Vital signs

• Pattern of breathing

• Shape of rib cage

  • 0-3 months: triangular, ribs horizontal, diaphragm for inspiration

  • 3-6 months: rectangular, ribs horizontal, diaphragm and accessory muscles for inspiration

  • 6-12 months: rectangular, ribs angled downward, diaphragm and intercostals for inspiration

• Integumentary condition

• Resting posture

What are the typical shapes of rib cage from 0-1y/o?

• 0-3 months: triangular, ribs horizontal, diaphragm for inspiration

• 3-6 months: rectangular, ribs horizontal, diaphragm and accessory muscles for inspiration

• 6-12 months: rectangular, ribs angled downward, diaphragm and intercostals for inspiration

Describe some items on a medical chart review you would like to know for a pediatric patient.

What should you look at during a pediatric PT evaluation?

• Rib cage mobility

• Flexibility

• Dynamic posture

• Use of accessory muscles

• Breathing pattern: shallow, irregular breathing

• Thoracic expansion w/ tape measure (4th IC space 1”)

• Monitoring vital signs (norms w/ aging below)

  • Weight increases

  • HR decreases

  • RR decreases

  • BP increases

Is it ok for a baby to be using accessory muscles during breathing? Head turns?

• Accessory muscle use up to 6mo OK (2y/o?)

• No head turns w/ breathing

What are two prevention goals for the CVP pediatric population?

• Maximize O2 transport and aerobic activity

• Reduce impact of lifestyle conditions

Describe 12 PT interventions for the CVP pediatric population.

1. Rib cage mobility

2. Flexibility

3. Strength

4. Posture

5. Breathing pattern

6. Airway clearance techniques

7. Positive expiratory pressure (PEP) device

8. Blow toys

9. Huffing

10. Assisted cough

11. Functional mobility

12. CV/endurance training

Describe rib case mobility as a pediatric intervention.

• In supine, prone, S/L (special consideration for osteopenia)

  • Manual release techniques for Q/L and intercostals, scar tissue massage for thoracic incision

Describe flexibility as a pediatric intervention.

S/L trunk rotation for intercostals; laying supine over a ball, corner pectoralis stretch, pelvic tilt, stretches for Q/L, lats, and abs

Describe strength as a pediatric intervention.

• Body weight exercises

  • Global - games that involve UE and LE body weight and dynamic stabilization (wheelbarrow, hopping and jumping, prone on therapy ball)

  • Spinal - extensors and retractors (good for hypotonicity

  • Inspiratory - inspiratory muscle trainer (ex program for CP 30% of their max inspiratory pressure for 10-15 breaths over 15 minutes, 2x/day, 6 wks)

Describe posture as a pediatric intervention.

• Mirror training, tapping to facilitate spinal extensors, consider position in stroller, car seat and chair, and amount of time spent in each (want to adjust posture for optimal positioning)

Describe breathing pattern as a pediatric intervention.

• After 2 y/o child should not demonstrate use of accessory muscles

  • If kids fatigue quickly, they may use accessory muscles to breathe shallow due to pain from an incision, train diaphragm and intercostals (can use stuffed animals for diaphragmatic breathing; can use inhibitory techniques as a last resort)

  • Once efficient breathing pattern is established should progress to harder positions like sitting

Describe airway clearance techniques as a pediatric intervention.

• PD, vibration, percussion

  • Trendelenburg position contraindicated in a child younger than 2 y/o, kids w/ CF b/c reflux → vomiting

  • Can modify for kids w/ CF → 20deg head down

  • Infants, young children in PT/caregiver’s arms (keep pt awake during PD)

Describe high-frequency chest wall oscillation as a pediatric intervention.

• Contraindications: bronchospasm, chest pain, lung contusion, burns or open wounds, recent pacemaker, skin grafts, recent spinal epidural, etc.

Describe positive expiratory pressure (PEP device) as a pediatric intervention.

• Provide resistance to exhalation

  • Bubble PEP: using suction tubing and a container of water

Describe blow toys as a pediatric intervention.

• Kid versions of incentive spirometer

  • Kazoo, noisemakers, pinwheels, bubbles, cotton ball

Describe huffing as a pediatric intervention.

Start w/ low volume and progress to larger force; less likely to risk dynamic airway collapse than strong coughing

Describe assisted cough as a pediatric intervention.

• NM weakness

  • Educate pt and caregiver on manual assisted cough techniques and positioning

  • Rhythmic rocking for increased tone, dissociating extremities, reducing external stimulation

Should you be getting a child out of bed after surgery? in the PICU?

• Yes, ASAP to reduce CP complications after surgery (DVT, respiratory infections, delirium)

• Early mobilization in PICU is safe and recommended

How can you help kids overcome fear? Infants?

• Dolls and toys, games that increase mobility i.e. scavenger hunts, assistive devices (be mindful of lifting, pushing, pulling restrictions after surgery)

• Infants - bring extremities to midline for calming, swaddling w/ baby’s hands near face, facilitate more milestone directed movement w/ support (can initiate infant in prone on parent’s chest w/ parent gradually reclining)

Describe CV/endurance training as a pediatric intervention.

• Monitor HR, BP, SpO2, RPE, dyspnea

• Goals can include social interactions: walking further to go on a vacation or do an activity w/ friends

  • Increasing length of time before a rest, increasing overall time of exercise, increasing intensity, decreasing amount of SpO2 needed, decreasing RPE

  • If child is interested in/excited by the activity → increase performance; HEP → bike riding, fitness video games, playing tag, jumping rope, obstacle course

• Educate pt and caregiver on what exercise should feel like and when to stop

*What are sxs to stop exercise?

• If the child has any of these sxs → stop exercising and REST. Attempt a lower intensity or duration during the next exercise session.

  • Chest pain or discomfort

  • RPE >17

  • Dyspnea of 4

  • Dizziness

  • Visual changes

  • Pale or ashen appearance

  • New-onset joint pain

  • HR >80% of maxHR

    • O2sat falls <90% (in some cases MD will say it’s OK)