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CardioPulm > Congenital Cardiac Disorders > Flashcards

Congenital Cardiac Disorders Flashcards

1
Q

What does the mesodermal germ layer give rise to?

A

CV system

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2
Q

Embryology week 2

A

heart develops from 2 simple epithelial tubes

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3
Q

Week 3-4 embryology

A

tubes fuse to form single chambered heart

  • elongates and bends on itself
  • endo, myo and epicardium differentiated
  • Heart beats
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4
Q

Week 4 embryology

A

primititve heart

- atrial segment assumes cranial position

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5
Q

Week 5 embryology

A

endocardial cushions grow towards each other and fuse

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6
Q

Week 8 embryology

A

partitioning into 4 chambered heart completes

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7
Q

Two openings between the left and right sides of the heart when in the womb

A

•Ductus arteriosus(DA), Foramen ovale. (FO)
•In utero Only 8% flow goes through non-functioning lung, rest flows through DA
•Shunting protects the developing lungs.
- close a few days after birth

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8
Q

Flood flow through the fetus

A
  • Fetus receives oxygenated blood from mother via placenta travels back via the umbilical vein
  • 50% of Oxygenated blood passes through liver, 50% to inferior vena cava then to right atrium
  • Blood then passes through FO to the left atrium and then to the left ventricle and out the aorta.
  • Most oxygenated blood goes to brain
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9
Q

Congenital defects

A
  • Occurring at birth or failure of normal development of cardiovascular system•At least 15 types of defects identified
  • Usually abnormal opening between adjacent heart chambers
  • Cardiovascular defects are some of the most common congenital malformations
  • Present in ~1/100-125 births
  • Infant death rate
  • 38 per 100,000 Caucasian
  • 56 per 100,000 African American
  • Causes
  • Viral infection (German measles), hereditary, Down Syndrome, Tera
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10
Q

Association with developmental disorders

A
  • Children with CHD are at increased risk of developmental disorders, disabilities or delay.
  • Neurodevelopmental disability, affects as many as 50% of infants undergoing interventions for congenital heart lesion.
  • Patients with complex cardiac disease are more likely to have social functioning issues because of their increased risk for severe neurocognitive impairment
  • Children with down syndrome have impaired tolerance to exercise, altered sympathetic response to exercise and are at risk for aneurysm
  • An increasing number of patients with CHD are surviving to adulthood (85%)
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11
Q

Intrinsic Risks

A
  • Genetic (altered regulation of all organ development)
  • Poor perfusion during prenatal period & birth*
  • Pediatric Stroke (10% of patients) - High reoccurrence rate too
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12
Q

Extrinsic Risks

A
  • Surgery (Bypass machine)
  • Impaired socialization
  • Environmental Stressors of NNICU
  • Impaired capacity to explore environment
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13
Q

Acyanotic defects

A
  • Atrial Septal Defect (ASD)
  • Patent Ductus Arteriosum (PDA)
  • Ventricular Septal Defect (VSD)
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14
Q

Cyanotic Defects

A
  • Transposition of the Great Vessels
  • Pulmonary Valve Atresia
  • Tetralogy of Fallot
  • Hypoplasticleft heart syndrome
  • Shone’sSyndrome
  • TAVPR
  • Coarctationof the Aorta*
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15
Q

Shunting in acyanotic

A

left to right

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16
Q

shunting in cyanotic

A 
right to left
•Transposition of great vessels
•Tricuspid atresia
•Tetralogy of Fallot
•Total anomalous pulmonary venous return
17
Q

Patent ductus arteriosus

A
  • The Ductus arteriosus normally closes at birth within hours
  • PDA creates Left to right shunt
  • (Aorta to Pulmonary Artery)
  • Creates high pressure in pulm. art.
  • May require surgical intervention
  • Clinical presentation: infant might fatigue quickly, susceptible to pneumonia
18
Q

atrial septal defects

A
  • Blood flow between the atria
  • Forms a left to right shunt
  • Volume overload: right heart and pulmonary vasculature damage
  • May result in R heart failure
  • Shortened life span
  • Usually repaired at 4-6 years
19
Q

Ventral septal defects

A
  • Most common congenital heart defect is ventral septal defect (VSD) - Small defects may close spontaneously, some require surgery
  • Forms left to right shunt
  • If pressures in right ventricle become too high, blood can shunt right to left, a condition called - Eisenmenger’s syndrome (cyanotic problem)
  • Large defects can result in increased pressure in pulmartery, - Can become permanent even with repair to VSD
20
Q

Coarctation of the Aorta

A
  • “Pinching” or aorta
  • Usually distal to subclavian artery
  • May be due to abnormal involution of Ductus Arteriosus
  • Severity dependent on degree of pinching and location
  • Present in 15-20% of CHD cases
  • May not be detected until later in childhood
  • Kidneys see low BP and release substances to increase BP
  • BP may be normal or elevated in arms, lower in legs
  • ABI? Pulse Differential
21
Q

Triscuspid Atresia

A
  • Triscuspid valve fails to develop
  • Limited blood flow from RA to RV, Underdeveloped RV
  • Filling of left ventricle and Survival depends on ASD & VSD
  • Right to left shunt
  • Surgery Required
22
Q

Pulmonary Valve Atresia

A
  • Pulmonary valve fails to develop
  • No exit from the right ventricle
  • Blood regurgitates into the left atrium via the foramen ovale
  • The lungs get perfused retrograde flow via a wide PDA
  • Considered a Critical Congenital Heart Defect, requires intervention soon after birth, drugs to keep PDA patent
  • This cardiac abnormality is very rare and accounts for only 1-3%
23
Q

Tetralogy of Fallot

A
  • Blueness appears soon after birth, in infancy or childhood
  • Infants might have sudden episodes of cyanosis, unconsciousness (Tet Spells)
  • Early surgery indicated
24
Q

Four defects that Tetralogy of Fallot is made up of

A
  • VSD
  • Pulmonary Valve Stenosis
  • Overriding Aorta (usually lies over VSD)
  • RV Hypertophy(due to PV Stenosis)
25
Q

Transposition of the Great Vessels

A
  • Positions of Pulmonary Artery and aorta reversed
  • Deoxygenated blood from RV goes into systemic circuit
  • O2 from blood goes back into lung
  • Child only survives if AS, VSD or PDA present
  • Surgery Option - Arterial Switch
26
Q

Total Anomalous Pulmonary Venous Return TAPVR

A
  • Pulmonary veins don’t connect to the left atrium. - Supracardiac, Cardiac or Infracardiac
  • Instead they go to the right side of the heart, via an abnormal (anomalous) connection.
  • Usually child possesses an ASD, which is only way for oxygenated blood to get to Left side
  • Child will require surgery soonafter birth, considered a critical congenital heart defect
27
Q

Hypoplastic Left Heart Syndrome HLHS

A
  • Failure or inadequate development of the left ventricle. - Variable aortic & mitral involvement
  • Child is dependent upon a PDA for systemic perfusion
  • Without intervention, HLHS is fatal within the first weeks of life
28
Q

Medical and surgical management of congenital heart diseases

A
  • Surgical Intervention
  • Timing of surgery depends on type of defect
  • Some defects require multiple surgeries
  • Majority of surgeries performed in first 2 years of life
  • With the advent of technological and surgical advances in the care of the infant born with a congenital heart defect (CHD), mortality rates have decreased dramatically; however, morbidity remains a concern
29
Q

Extracorporeal Membrane Oxygenation (ECMO)

A
  • Support cardiac and respiratory systems until disease process resolves
  • ECMO is used for longer-term support ranging from 3-10 days
  • Disease process must be reversible
  • Reversible lung and cardiac disease
  • Bridge to transplant (heart or lung)
  • ARDS (both pediatric and adult)
30
Q

Norwood Procedure

A
  • Done within the first 2 weeks of a life•Surgeons create a “new” aorta and connect it to the right ventricle - Constructed utilizing the pulmonary root, ascending aorta, and homograft tissue.
  • A Bialock-Tussing Shunt is then placed from either the aorta or the right ventricle to the pulmonary arteries.
  • Thus the heart becomes a “single ventricle” structure capable of pumping mixed blood to lungs and periphery.
  • Arterial oxygen saturation following procedure 70-75%
  • Mixed venous oxygen saturation is usually forty-five to fifty-five percent.
31
Q

Bi-directional Glenn Shunt Procedure

A
  • This usually is performed when an infant is 4 to 6 months of age.
  • Creates a direct connection between the pulmonary artery and the superior vena cava.
  • Directly returns venous blood to lungs, from UE.
  • Right Ventricle still pumps mixed blood but this procedure reduces it’s work.
32
Q

Fontan Procedure

A
  • Done at 18months to 3yrs of age.
  • Doctors now connect the pulmonary artery to the inferior vena cava.
  • Now venous blood completely bypasses the right ventricle
  • 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.
  • Kids may still require heart transplantation
33
Q

Pediatric Heart Transplantation

A

•~10% of CHD cases uncorrectable. - Most common hypoplastic left heart syndrome (HLHS), changing though since surgical procedures and technology improving.
•Survival in excess of 20 years after pediatric heart transplantation (HTx) has been achievedin some cases - 70% of their recipients survive at least 5 years.
•HTx can provide excellent quality of life for pediatric patients, and progress has been made prolonging graft survival, however it will not last a normal lifespan.
•If this occurs later in life the only effective therapy is re-transplantation (re-HTx)
.•Re-HTxcurrently accounts for 5.6% of pediatric HTxin North America, and
•Nearly 10% of HTxin pediatric patients >11 years of age

34
Q

Effects on children with congenital heart disease

A
  • Labored breathing
  • Increased RR
  • Diffuse generalized edema
  • Decreased urine output
  • Eating problems
  • Impaired tolerance to activity
  • Irritable (Track Using NIPS scale)
35
Q

PT considerations for children with congenital heart disease

A
  • Post Op: prevent complications - Inactvity, pulmonary complications, etc
  • FAMILY EDUCATION!!!!!!!!!!!
  • Early mobilization
  • Ambulation: if appropriate team effort
  • Positioning: Prone> side-lying> supine for ventilation/perfusion matching.
  • Some activity and exercise restrictions may be present
  • Acceptable Pulse oximetry cutoffs may be lower depending on case - Communicate with RN, MD, RRT etc
  • Some patients may need guidance away from competitive sports

CardioPulm (14 decks)

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