CHD Syndromes Flashcards
Syndromes associated w/ CHD:
- Down
- DiGeorge
- Williams
- Noonan
- Marfan
- VATER or VACTRL
- CHARGE
Down Syndrome
Trisomy 21
Chromosomal disease
Down Syndrome
Anesthetic Considerations
Assess atlantoaxial instability Bacterial endocarditis prophylaxis Severe bradycardia on induction - Sevoflurane only 6% - Pre-medicate w/ Atropine or Glycopyrrolate Challenging vascular access Downsize ETT d/t subglottic stenosis Varying developmental delays Hypothyroidism → delayed gastric emptying, drug metabolism alterations, & impaired temperature regulation
What are common complications associated w/ Down Syndrome
Postop stridor
Respiratory complications
DiGeorge Syndrome
122q11 micro deletion
Defects in the development of the thymus & parathyroid
Cardiac defects, T-cell immunodeficiency, & seizures r/t hypocalcemia
CHD 75%
Otolaryngologic 50%
Hypocalcemia 60%
DiGeorge Syndrome
Causes
Prenatal exposure to alcohol
Isotretinoin (Accutane)
Inherited/genetic component
DiGeorge Syndrome
Anesthetic Considerations
Strict asepsis w/ reverse isolation
Potential difficult intubation d/t micrognathia
Short trachea → endobronchial intubation
Avoid nasal trumpets or intubation d/t choanal atresia
Blood products must be irradiated to kill donor leukocytes (graft vs. host response)
Parathyroid dysfunction → significant hypocalcemia
Monitor Ca2+ levels
CHD requires SBE prophylaxis
Williams Syndrome
Chromosome 7 deletion
“Elf” appearance
Mild mental disability
Infantile hypotonia → hypertonic
CARDIOVASCULAR:
- HTN, abdominal aortic coarctation, narrow celiac, mesenteric, & renal arteries
- Stenotic lesions at valvar pulmonary, branch pulmonary, aorta, supravalvar aortic w/ coronary artery stenosis
Sudden death risk d/t severe myocardial ischemia, L ventricle dysfunction, & ventricular arrhythmias
Williams Syndrome
Anesthetic Considerations
Sudden death risk - valvular stenosis, obstructed outflow, ischemic cardiac disease; supply & demand impairment w/ L ventricle hypertrophy
Baseline EKG & echocardiogram
ECMO on standby (never an outpatient surgery)
SBE prophylaxis
Noonan Syndrome
Autosomal dominant
Hypertelorism, micrognathia, webbed neck, short stature, pectus excavatum/carinatum, bleeding diathesis, CHD
Noonan Syndrome
Anesthetic Considerations
Possible difficult intubation - micrognathia & short/webbed neck
Difficult vascular access when edema present
Restrictive lung disease w/ chest deformities
Impaired excretion impact on renally excreted drugs
Difficult epidural catheter placement d/t spinal abnormalities
SBE prophylaxis
Hypertrophic obstructive cardiomyopathy considerations
Marfan Syndrome
Multisystem disorder resulting from connective tissue protein disorder (fibrillin - extracellular microfibrils element & elastic/non-elastic connective tissues)
Involves cardiovascular, skeletal, & ocular systems
- Aortic or pulmonary artery dilation, dissection, insufficiency, & mitral valve prolapse
Individual variation
Marfan Syndrome
Anesthetic Considerations
Avoid HTN in patients at risk for aortic dissection
Preop echocardiogram
β blocker
SBE prophylaxis (especially w/ mitral valve prolapse & insufficiency)
Careful admin w/ +pressure ventilation d/t ↑risk pneumothorax
Positioning considerations (joint laxity)
↑epidural/spinal doses based on height & CSF
CHARGE Syndrome*
Colobomas of the eye Heart disease Atresia of the choanae Retarded growth or CNS anomalies Genital anomalies Ear anomalies or deafness
At least 4 present for diagnosis
All organ systems involved at critical development during 2nd month gestation
CHARGE Syndrome
Anesthetic Considerations
Interpreter
↑aspiration risk d/t GERD & impaired gag reflex
SBE prophylaxis
Difficult tracheal intubation & becomes more difficult w/ age - micrognathia
Laryngomalacia - difficult to provide ventilation via LMA or mask
Smaller ETT d/t subglottic stenosis
Choanal atresia → severe respiratory distress in newborn
Avoid nasal airways or NG tubes
