Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Pathophys II > SBE & Syndromes with CHD > Flashcards

SBE & Syndromes with CHD Flashcards

(59 cards)

Start Studying
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

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

A

uncommon; children with uncorrected congenital heart defects

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Acute bacterial endocarditis is caused most commonly by

A

staphylococcus aureus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Correct understanding of the genetic relation to specific defects helps improve

A

diagnosis, anesthetic approach, and overall outcome for patients

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Down syndrome is a

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Describe neuromuscular considerations of Down Syndrome.

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Describe issues with the lungs with Down syndrome.

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Describe orthopedic issues with Down syndrome.

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Describe GI/GU issues with Down syndrome.

A

congenital duodenal atresia or stenosis, increased incidences of Hirschsprung disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Describe other issues with Down Syndrome.

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

DiGeorge syndrome has been associated with

A

prenatal exposure to alcohol & isotretinoin (accutane)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Morbidity & mortality for DiGeorge syndrome is associated with

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

DiGeorge syndrome typically involves

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

CV considerations for DiGeorge syndrome includes

Study These Flashcards
A

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

25
Neuromuscular considerations for DiGeorge syndrome include
hypocalcemia*** tetany or seizures in neonates, occasional mental deficiency & about 20% of adults carry a bipolar diagnosis
26
Describe GI/GU considerations for DiGeorge syndrome.
Esophageal atresia, imperforated anus, diaphragmatic hernia
27
Describe other involvement with DiGeorge syndrome.
Hypocalcemia*, hypoplastic parathyroid glands, cell-mediated immune deficiency from decreased T-cell activity with aplastic thymus, short stature, and growth hormone deficiency
28
The treatment for DiGeorge syndrome is
thymus transplant*****
29
Anesthesia airway concerns for DiGeorge syndrome includes:
micgronathia may make for difficult intubation** short trachea may lead to endobronchial intubation choanal atresia precludes nasal trumpets or nasal intubations
30
Additional anesthetic concerns for DiGeorge syndrome includes:
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
Williams syndrome occurs because of a
deletion on Chromosome 7
32
HEENT/Airway considerations for Williams syndrome includes:
"Elfin" facies. Puffy eyes, lacey iris, depressed nasal bridge, enamel hypoplasia, small teeth, harsh voice, possible vocal cord paralysis
33
Neuromuscular considerations for Williams syndrome includes:
Mild mental disability, ADD, advanced language development, infantile hypotonia with hypertonicity later, cerebral artery stenosis with ischemic events
34
Describe cardiovascular considerations for Williams syndrome.
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
Patients with Williams syndrome have a risk of
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
Describe orthopedic considerations with Williams syndrome.
short stature, puberty occurs earlier, hypoplastic nails, progressive joint limitation
37
Describe GI/GU considerations with Williams syndrome
chronic constipation, inguinal hernias, recurrent UTIs, renal structural abnormalities
38
Describe other considerations with Williams syndrome.
neonatal hypercalcemia, possible hypothyroidism, socially gregarious, friendly, "cocktail party" personality
39
Anesthetic considerations for Williams syndrome include:
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
Noonan syndrome is
autosomal dominant
41
Noon syndrome is characterized by
hypertelorism (increased distance btw orbits) - micrognathia - webbed neck - short stature - pectus excavatum/carinatum - bleeding diathesis - congenital heart disease
42
Describe the HEENT/Airway considerations for Noonan syndrome.
Triangular facies, downward slanting eyes, ptosis, strabismus, low-set ears, hearing loss, wide mouth, high arched palate, micrognathias, short/webbed neck, dental malocclusion
43
Describe pulmonary considerations for Noonan syndrome.
Sternal deformities, pectus excavatum or carinatum, thoracic kyphoscoliosis, restrictive lung disease, chylothorax
44
Describe the cardiovascular considerations for Noonan syndrome
pulmonic stenosis, septal defects, HOCM, LVH, PDA< aortic stenosis, and coarctation
45
Describe the neuromuscular considerations for Noonan syndrome.
intellectual disability, narrow spinal canal with normal spinal cord size, chiari malformations, cerebral AVMs
46
Describe the orthopedic considerations for Noonan syndrome.
short stature, abnormal vertebrae
47
Describe the GI/GU considerations for Noonan syndrome.
Decreased fertility in males, delayed menarche in females, renal dysfunction, hepatosplenomegaly, GERD, delayed gastric emptying
48
Describe other considerations for Noonan syndrome.
Coagulation or PLT defects, lymphatic vessel dysplasia, and subcutaneous edema of hands and feet
49
Describe the anesthetic considerations for Noonan syndrome.
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
Marfan syndrome is 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
Marfan syndrome involves the
CV, skeletal, and ocular systems
52
With Marfan syndrome, there is a high degree of
incomplete expressivity so there is much individual variation
53
Describe the HEENT/airway considerations for Marfan syndrome.
long, narrow facies with high arched palate and crowded teeth, lens dislocation and cataract formation, increased risk of retinal detachment and glaucoma
54
Describe the chest considerations for Marfan syndrome.
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
Describe the CV considerations for Marfan syndrome.
aortic or pulmonary artery dilation****; dissection, insufficiency and mitral valve prolapse******, possible coronary artery narrowing
56
Describe the neuromuscular considerations for Marfan syndrome.
wide lumbosacral canal and spinal arachnoid cysts
57
Describe the orthopedic considerations for Marfan syndrome.
Tall, winged scapula, long fingers, recurrent dislocations, scoliosis
58
Describe the GI/GU considerations for Marfan syndrome.
inguinal, umbilical, and femoral hernia
59
Describe the anesthetic considerations for Marfan syndrome.
- 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)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions