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Pathophys II > SBE & Syndromes with CHD > Flashcards

SBE & Syndromes with CHD Flashcards

1
Q

Infective endocarditis is also called

A

subacute bacterial endocarditis (SBE)*** and is an infection caused by bacteria that enter the bloodstream and settle in the heart lining, a heart valve or a blood vessel

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2
Q

IE is _____ but children with _______ are at an increased risk

A

uncommon; children with uncorrected congenital heart defects

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3
Q

Acute bacterial endocarditis is caused most commonly by

A

staphylococcus aureus

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4
Q

Antibiotics are required for patients with:

A

prosthetic cardiac valves, history of infective endocarditis, unrepaired or incomplete repaired cyanotic heart disease (including shunts), complete repairs with prosthetic material during the 1st 6 months, cardiac transplant recipients with valve disease and other high risk factors

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5
Q

The antibiotic of choice for SBE prophylaxis is

A

Cefazolin 50 mg/kg IV or Ampicillin 50 mg/kg IV***

clindamycin 20 mg/kg IV (if allergy to penicllin)

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6
Q

Suggestions to prevent SBE include:

A

Abx for a dental procedure that involves manipulation of gingival tissues, periapical region of teeth, or perforation of the oral mucosa is suggested only for patients with highest risk of adverse outcome from VGS IE
-maintenance of good oral health & regular access to dental care are considered more important to prevent VGS IE than AP for a dental procedure

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7
Q

15-30% of children with congenital heart defects have an associated genetic syndrome or extra-cardiac anomaly

A 
Down syndrome
DiGeorge syndrome
Williams syndrome
Noon syndrome
Marfan Syndrome
VATER or VACTRL
Charge association
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8
Q

Correct understanding of the genetic relation to specific defects helps improve

A

diagnosis, anesthetic approach, and overall outcome for patients

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9
Q

Down syndrome is a

A

Trisomy 21 chromosomal disease with distinct facies, significant airway & cardiac findings with varying degrees of intellectual disability

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10
Q

HEENT & Airway concerns with down syndrome include

A 
Brushfield spots on the iris
up-slanting eyes
narrow nasopharynx
small external ears
macroglossia
pharyngeal hypotonia 
high-arched palate
tonsil adenoid hypertophy
micrognatia
short, broad neck
small trachea
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11
Q

Describe neuromuscular considerations of Down Syndrome.

A

hypotonia
dementia & parkinsonism in older adults
intellectual decline may occur with age

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12
Q

Describe CV considerations of Down Syndrome.

A

1/2 have CHD: ASD, VSD, PDA, A-V canal defect, TOF. Left to right shunting may lead to pulmonary HTN and pulmonary vascular disease

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13
Q

Describe issues with the lungs with Down syndrome.

A

may have chronic upper airway obstruction with hypoventilation and OSA. recurrent pulmonary infections

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14
Q

Describe orthopedic issues with Down syndrome.

A

joint laxity; atlantoaxial cervical instability*****, short stubby hands, single horizontal palmar crease

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15
Q

Describe GI/GU issues with Down syndrome.

A

congenital duodenal atresia or stenosis, increased incidences of Hirschsprung disease

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16
Q

Describe other issues with Down Syndrome.

A

increased incidence of leukemias, hypothyroidism, and anti-thyroid antibodies

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17
Q

Anesthesia for Down syndrome children includes

A 
assess airway, cardiac, & ROS 
-assess atlantoaxial instability****
-assess for OSA
-CHD- bacterial endocarditis prophylaxis
PRONE TO BRADYCARDIA ON INDUCTION***
downsize ETT due to SUBGLOTTIC STENOSIS***
challenging vascular access
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18
Q

Additional anesthetic considerations for patients with DS include

A

developmental delay present but varies in severity- premeds, caregiver present for induction

  • hypothyroidism- when present could result in delayed gastric emptying, alterations in drug metabolism and impaired temperature regulation
  • postoperative stridor and respiratory complications are common
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19
Q

DiGeorge syndrome is a

A

22q11 micro deletion
this gene is involved in the developmental processes and includes defects in the development of the thymus, parathyroid, and great vessels

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20
Q

DiGeorge syndrome has been associated with

A

prenatal exposure to alcohol & isotretinoin (accutane)

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21
Q

Morbidity & mortality for DiGeorge syndrome is associated with

A

cardiac defects, T-Cell immunodeficiency, and seizures related to hypocalcemia***

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22
Q

DiGeorge syndrome typically involves

A

congenital heart disease (75%)
otolaryngologic 50%
Hypocalcemia 60%

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23
Q

HEENT/Airway considerations for DiGeorge syndrome includes

A

displacement of inner canthi and either upward or downward slant to eyes, low-set ears, bulbous nose, choanal atresia, small mouth opening, cleft palate, micrognathia, and short trachea

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24
Q

CV considerations for DiGeorge syndrome includes

A

conotruncal cardiac defects (i.e truncus arteriosus, TOF, interrupted aortic arch, and right aortic arch)

25
Q

Neuromuscular considerations for DiGeorge syndrome include

A

hypocalcemia*** tetany or seizures in neonates, occasional mental deficiency & about 20% of adults carry a bipolar diagnosis

26
Q

Describe GI/GU considerations for DiGeorge syndrome.

A

Esophageal atresia, imperforated anus, diaphragmatic hernia

27
Q

Describe other involvement with DiGeorge syndrome.

A

Hypocalcemia*, hypoplastic parathyroid glands, cell-mediated immune deficiency from decreased T-cell activity with aplastic thymus, short stature, and growth hormone deficiency

28
Q

The treatment for DiGeorge syndrome is

A

thymus transplant*****

29
Q

Anesthesia airway concerns for DiGeorge syndrome includes:

A

micgronathia may make for difficult intubation**
short trachea may lead to endobronchial intubation
choanal atresia precludes nasal trumpets or nasal intubations

30
Q

Additional anesthetic concerns for DiGeorge syndrome includes:

A

all blood products must be irradiated*** to kill donor leukocytes which can incite a serious graft versus host response

  • careful asepsis***
  • calcium levels** must be evaluated
  • PTH disfunction leads to significant hypocalcemia- Requirements of exogenous calcium may be very large
  • CHD requires SBE prophylaxis
31
Q

Williams syndrome occurs because of a

A

deletion on Chromosome 7

32
Q

HEENT/Airway considerations for Williams syndrome includes:

A

“Elfin” facies. Puffy eyes, lacey iris, depressed nasal bridge, enamel hypoplasia, small teeth, harsh voice, possible vocal cord paralysis

33
Q

Neuromuscular considerations for Williams syndrome includes:

A

Mild mental disability, ADD, advanced language development, infantile hypotonia with hypertonicity later, cerebral artery stenosis with ischemic events

34
Q

Describe cardiovascular considerations for Williams syndrome.

A

HTN, abdominal aortic coarctation, narrow celiac, mesenteric, and renal arteries
Stenotic lesions at multiple levels
-vavlar pulmonary stenosis
-branch pulmonary stenosis
-aorta stenosis
-Supravalvar aortic stenosis with coronary artery stenosis*****

35
Q

Patients with Williams syndrome have a risk of

A

sudden death**** due to severe myocardial ischemia, LV dysfunction & ventricular arrhythmias (similar to adults with atherosclerotic heart disease, however it is due to stenosis rather than atherosclerotic processes

36
Q

Describe orthopedic considerations with Williams syndrome.

A

short stature, puberty occurs earlier, hypoplastic nails, progressive joint limitation

37
Q

Describe GI/GU considerations with Williams syndrome

A

chronic constipation, inguinal hernias, recurrent UTIs, renal structural abnormalities

38
Q

Describe other considerations with Williams syndrome.

A

neonatal hypercalcemia, possible hypothyroidism, socially gregarious, friendly, “cocktail party” personality

39
Q

Anesthetic considerations for Williams syndrome include:

A

risk of sudden death outside of the hospital as well as during cardiac catheterization and during anesthesia

  • baseline EKG & echo
  • preparation for possible ECMO
  • SBE prophylaxis
40
Q

Noonan syndrome is

A

autosomal dominant

41
Q

Noon syndrome is characterized by

A

hypertelorism (increased distance btw orbits)

  • micrognathia
  • webbed neck
  • short stature
  • pectus excavatum/carinatum
  • bleeding diathesis
  • congenital heart disease
42
Q

Describe the HEENT/Airway considerations for Noonan syndrome.

A

Triangular facies, downward slanting eyes, ptosis, strabismus, low-set ears, hearing loss, wide mouth, high arched palate, micrognathias, short/webbed neck, dental malocclusion

43
Q

Describe pulmonary considerations for Noonan syndrome.

A

Sternal deformities, pectus excavatum or carinatum, thoracic kyphoscoliosis, restrictive lung disease, chylothorax

44
Q

Describe the cardiovascular considerations for Noonan syndrome

A

pulmonic stenosis, septal defects, HOCM, LVH, PDA< aortic stenosis, and coarctation

45
Q

Describe the neuromuscular considerations for Noonan syndrome.

A

intellectual disability, narrow spinal canal with normal spinal cord size, chiari malformations, cerebral AVMs

46
Q

Describe the orthopedic considerations for Noonan syndrome.

A

short stature, abnormal vertebrae

47
Q

Describe the GI/GU considerations for Noonan syndrome.

A

Decreased fertility in males, delayed menarche in females, renal dysfunction, hepatosplenomegaly, GERD, delayed gastric emptying

48
Q

Describe other considerations for Noonan syndrome.

A

Coagulation or PLT defects, lymphatic vessel dysplasia, and subcutaneous edema of hands and feet

49
Q

Describe the anesthetic considerations for Noonan syndrome.

A

possible difficult intubation, usually becomes less marked with age
difficult PIV placement if there is significant edema
chest deformities may lead to decreased lung function (restrictive)
bleeding diathesis may increase the amount of perioperative bleeding
renal impairment may affect metabolism of renally excreted drugs
-spinal abnormalities may make epidural catheter placement difficult
-SBE prophylaxis, & HOCM considerations

50
Q

Marfan syndrome is a

A

multisystem disorder resulting from a mutation in connective tissue protein (fibrillin which is a major element of extracellular microfibrils in the elastic and non elastic connective tissues)

51
Q

Marfan syndrome involves the

A

CV, skeletal, and ocular systems

52
Q

With Marfan syndrome, there is a high degree of

A

incomplete expressivity so there is much individual variation

53
Q

Describe the HEENT/airway considerations for Marfan syndrome.

A

long, narrow facies with high arched palate and crowded teeth, lens dislocation and cataract formation, increased risk of retinal detachment and glaucoma

54
Q

Describe the chest considerations for Marfan syndrome.

A

Pectus excavatum, lower than predicted forced vital capacity due to early airway closure from inadequate small airway elastic tissue, pulmonary blebs & OSA secondary to pharyngeal laxity

55
Q

Describe the CV considerations for Marfan syndrome.

A

aortic or pulmonary artery dilation; dissection, insufficiency and mitral valve prolapse**, possible coronary artery narrowing

56
Q

Describe the neuromuscular considerations for Marfan syndrome.

A

wide lumbosacral canal and spinal arachnoid cysts

57
Q

Describe the orthopedic considerations for Marfan syndrome.

A

Tall, winged scapula, long fingers, recurrent dislocations, scoliosis

58
Q

Describe the GI/GU considerations for Marfan syndrome.

A

inguinal, umbilical, and femoral hernia

59
Q

Describe the anesthetic considerations for Marfan syndrome.

A
  • avoid hypertension who are at risk for aortic dissection, preop echo, beta-blocker therapy, SBE prophylaxis especially with mitral valve prolapse & insufficiency
  • increased for pneumothorax, use careful positive pressure ventilation
  • careful positioning due to joint laxity
  • may require larger than normal doses of spinal or epidural medications due to increased length and increased CSF

Pathophys II (43 decks)

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