SBE & Syndromes with CHD Flashcards
Infective endocarditis is also called
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
IE is _____ but children with _______ are at an increased risk
uncommon; children with uncorrected congenital heart defects
Acute bacterial endocarditis is caused most commonly by
staphylococcus aureus
Antibiotics are required for patients with:
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
The antibiotic of choice for SBE prophylaxis is
Cefazolin 50 mg/kg IV or Ampicillin 50 mg/kg IV***
clindamycin 20 mg/kg IV (if allergy to penicllin)
Suggestions to prevent SBE include:
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
15-30% of children with congenital heart defects have an associated genetic syndrome or extra-cardiac anomaly
Down syndrome DiGeorge syndrome Williams syndrome Noon syndrome Marfan Syndrome VATER or VACTRL Charge association
Correct understanding of the genetic relation to specific defects helps improve
diagnosis, anesthetic approach, and overall outcome for patients
Down syndrome is a
Trisomy 21 chromosomal disease with distinct facies, significant airway & cardiac findings with varying degrees of intellectual disability
HEENT & Airway concerns with down syndrome include
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
Describe neuromuscular considerations of Down Syndrome.
hypotonia
dementia & parkinsonism in older adults
intellectual decline may occur with age
Describe CV considerations of Down Syndrome.
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
Describe issues with the lungs with Down syndrome.
may have chronic upper airway obstruction with hypoventilation and OSA. recurrent pulmonary infections
Describe orthopedic issues with Down syndrome.
joint laxity; atlantoaxial cervical instability*****, short stubby hands, single horizontal palmar crease
Describe GI/GU issues with Down syndrome.
congenital duodenal atresia or stenosis, increased incidences of Hirschsprung disease
Describe other issues with Down Syndrome.
increased incidence of leukemias, hypothyroidism, and anti-thyroid antibodies
Anesthesia for Down syndrome children includes
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
Additional anesthetic considerations for patients with DS include
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
DiGeorge syndrome is 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
DiGeorge syndrome has been associated with
prenatal exposure to alcohol & isotretinoin (accutane)
Morbidity & mortality for DiGeorge syndrome is associated with
cardiac defects, T-Cell immunodeficiency, and seizures related to hypocalcemia***
DiGeorge syndrome typically involves
congenital heart disease (75%)
otolaryngologic 50%
Hypocalcemia 60%
HEENT/Airway considerations for DiGeorge syndrome includes
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
CV considerations for DiGeorge syndrome includes
conotruncal cardiac defects (i.e truncus arteriosus, TOF, interrupted aortic arch, and right aortic arch)
Neuromuscular considerations for DiGeorge syndrome include
hypocalcemia*** tetany or seizures in neonates, occasional mental deficiency & about 20% of adults carry a bipolar diagnosis
Describe GI/GU considerations for DiGeorge syndrome.
Esophageal atresia, imperforated anus, diaphragmatic hernia
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
The treatment for DiGeorge syndrome is
thymus transplant*****
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
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
Williams syndrome occurs because of a
deletion on Chromosome 7
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
Neuromuscular considerations for Williams syndrome includes:
Mild mental disability, ADD, advanced language development, infantile hypotonia with hypertonicity later, cerebral artery stenosis with ischemic events
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*****
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
Describe orthopedic considerations with Williams syndrome.
short stature, puberty occurs earlier, hypoplastic nails, progressive joint limitation
Describe GI/GU considerations with Williams syndrome
chronic constipation, inguinal hernias, recurrent UTIs, renal structural abnormalities
Describe other considerations with Williams syndrome.
neonatal hypercalcemia, possible hypothyroidism, socially gregarious, friendly, “cocktail party” personality
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
Noonan syndrome is
autosomal dominant
Noon syndrome is characterized by
hypertelorism (increased distance btw orbits)
- micrognathia
- webbed neck
- short stature
- pectus excavatum/carinatum
- bleeding diathesis
- congenital heart disease
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
Describe pulmonary considerations for Noonan syndrome.
Sternal deformities, pectus excavatum or carinatum, thoracic kyphoscoliosis, restrictive lung disease, chylothorax
Describe the cardiovascular considerations for Noonan syndrome
pulmonic stenosis, septal defects, HOCM, LVH, PDA< aortic stenosis, and coarctation
Describe the neuromuscular considerations for Noonan syndrome.
intellectual disability, narrow spinal canal with normal spinal cord size, chiari malformations, cerebral AVMs
Describe the orthopedic considerations for Noonan syndrome.
short stature, abnormal vertebrae
Describe the GI/GU considerations for Noonan syndrome.
Decreased fertility in males, delayed menarche in females, renal dysfunction, hepatosplenomegaly, GERD, delayed gastric emptying
Describe other considerations for Noonan syndrome.
Coagulation or PLT defects, lymphatic vessel dysplasia, and subcutaneous edema of hands and feet
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
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)
Marfan syndrome involves the
CV, skeletal, and ocular systems
With Marfan syndrome, there is a high degree of
incomplete expressivity so there is much individual variation
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
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
Describe the CV considerations for Marfan syndrome.
aortic or pulmonary artery dilation; dissection, insufficiency and mitral valve prolapse**, possible coronary artery narrowing
Describe the neuromuscular considerations for Marfan syndrome.
wide lumbosacral canal and spinal arachnoid cysts
Describe the orthopedic considerations for Marfan syndrome.
Tall, winged scapula, long fingers, recurrent dislocations, scoliosis
Describe the GI/GU considerations for Marfan syndrome.
inguinal, umbilical, and femoral hernia
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
