Lecture 5 (Part 2)-Congenital Disease Processes Affiliated With Congenital Heart Defects Flashcards
What disease is this?—Mucocutaneous lymph node syndrome; affects infants and young children; vasculitis; dilation of coronary arteries; aneurysm formation; MI; recently affiliated with COVID-19
Kawasaki Disease
This disease occurs every 1 in 800 live births; results from balanced, unbalanced chromosome translocation or mosaicism
Trisomy 21 (Down Syndrome)
Patients with Down Syndrome may experience—obstructive ___; mental ___; ___ spine disorders, vertebral ___; ___ disease; subglottic ___; ___ (small/large) tongue; difficult ___ access
Obstructive sleep apnea; mental retardation; cervical spine disorders, vertebral instability; thyroid disease; subglottic stenosis; large tongue; difficult vascular access
Down syndrome—CV defect in ___-___%—___ septal defects, ___ septal defects, ___, patent ___; ___cardia under anesthesia; pulmonary ___tension
CV defect in 40-50%—AV septal defects, ventricular septal defects, tetralogy of fallot, patent ductus arteriosus; bradycardia under anesthesia; pulmonary hypertension
Turner syndrome is ___ (X, Y) linked
X
Turner syndrome—___ neck; low set ___; pigmented ___; ___gnathia (micro/macro); ___ (tall/short) stature; ___edema; ___ failure; aortic ___; ___cuspid aortic valve; ___tension; ___ disease; obesity; diabetes; ___thyroidism
Webbed neck; low set ears; pigmented nevi; micrognathia; short stature; lymphedema; ovarian failure; aortic coarctation; bicuspid aortic valve; hypertension; liver disease; obesity; diabetes; hypothyroidism
___ syndrome—chromosome 7, deletion in the elastin gene
Williams syndrome
Williams syndrome—___ facies; ___ abnormalities—___calcemia, ___thyroidism; mental ___; growth ___; altered ___ development
Elfin facies; endocrine abnormalities—hypercalcemia, hypothyroidism; mental retardation; growth deficiency; altered neuro development
Williams syndrome cardiac anomalies—valvular and supravalvular aortic ___; aortic ___; can involve the origin of the ___ arteries; narrowing of the abdominal ___ and ___ arteries; coronary artery stenosis leading to myocardial ___ and severe biventricular outflow tract ___
Valvular and supravalvular aortic stenosis; aortic coarctation; can involve the origin of the coronary arteries; narrowing of the abdominal aorta and renal arteries; coronary artery stenosis leading to myocardial ischemia and severe biventricular outflow tract obstruction
Patients with Williams syndrome need ___ evaluation before anesthesia is administered
Cardiac
Williams syndrome is the leading cause of cardiac arrest in the perioperative cardiac arrest registry—T/F?
True
Patients with Williams syndrome also may have ___ weakness, so caution with ___ (what medication class?)
Muscle weakness, so caution with muscle relaxants
Patients with Williams syndrome may also exhibit ___
Autism
DiGeorge/Velocardiofacial syndrome—22q11.2 deletion syndrome; catch 22; ___ defects; thymic ___plasia; ___ palate; ___calcemia
Cardiac defects; thymic hypoplasia; cleft palate; hypocalcemia
DiGeorge/velocardiofacial syndrome—conotruncal abnormalities; ___ tract problems too; ___deficiency, so give ___ blood products
Outflow tract problems too; immunodeficiency, so give irradiated blood products
Noonan syndrome—___ webbing; low set ___; chest ___; ___ (tall/short) stature; over 50% have ___; pulmonary valve ___; pulmonary ___; bleeding diathesis (aka bleeding disorders)
Neck webbing; low set ears; chest deformities; short stature; over 50% have CHD; pulmonary valve dysplasia; pulmonary stenosis
___ syndrome—mutation of the fibrillin gene; connective tissue protein; CV—MVP, MVR, ascending aortic dilation, main pulmonary artery dilation; aortic dissection likely at any size; cardiac arrhythmias; treat with beta blockers and BP control
Marfan syndrome
Marfan syndrome—ventricular ___; abnormal ___ (systolic/diastolic) function; pulmonary disease—chest wall deformities leading to progressive ___, ___ lung disease
Ventricular dilation; abnormal systolic function; pulmonary disease—chest wall deformities leading to progressive scoliosis, restrictive lung disease
VACTERL
Non random anomalies
Vertebral Anal Cardiovascular Tracheoesophageal Renal Limb defects
VACTERL—___ and ___ anomalies can make airway management and regional difficult; ___(hypo/hyper)plastic vertebrae can cause scoliosis; anal ___ or imperforate anus; ductal dependent lesions; limb defects can affect ___ access
Vertebral and tracheal anomalies can make airway management and regional difficult; hypoplastic vertebrae can cause scoliosis; anal atresia or imperforate anus; limb defects can affect vascular access
CHARGE association
Coloboma Heart defects Choanal atresia Retardation of growth and development Genitourinary problems Ear abnormalities Conotruncal and aortic arch anomalies Upper airway abnormalities
Congenital diaphragmatic hernia occurs at ___-___ weeks of fetal life
5-10 weeks
Congenital diaphragmatic hernia—gut herniates into the ___
Thorax
Mortality of congenital diaphragmatic hernia is ___%
50%
Congenital diaphragmatic hernia—___ abdomen (bowel is in the thorax);
Scaphoid abdomen
Causes of mortality with congenital diaphragmatic hernia—___ia; ___ insufficiency; persistent ___
Hypoxia; respiratory insufficiency; persistent pulmonary hypertension of newborn
Treatment of CDH—immediate stabilization with sedation, paralysis, and moderate ___ventilation; ___O; ___ (inhalation agent) for PPH
Immediate stabilization with sedation, paralysis, and moderate hyperventilation; ECMO; nitric oxide for PPH
CDH anesthesia concerns—decrease ___ distention; ___ (low/high) oxygen delivery; ___ (right/left) sided pneumothorax concern; ___ intubation; no ___ (inhalation agent); peak inspiratory pressures should be < ___ mm H2O
Decrease gastric distention; low oxygen delivery; right sided pneumothorax concern; awake intubation; no nitrous oxide; peak inspiratory pressure should be < 30 mm H2O
Most common variation of TEF is the form that ends in a blind ___ and ___ (upper/lower) esophagus that connects to the trachea
Ends in a blind pouch and lower esophagus connects to the trachea
TEF—gastric ___ with respirations; ___ leads to choking, coughing, and cyanosis, ultimately leading to ___ia and ___cardia
Gastric distention with respirations; feeding leads to choking, coughing, and cyanosis, ultimately leading to hypoxia and bradycardia
TEF is diagnosed by a failure to pass a catheter into the ___ and is confirmed by visualization of the catheter coiled in a blind ___
Into the stomach and is confirmed by visualization of the catheter coiled in a blind pouch
___ is common with TEF
Aspiration pneumonia
___ may also occur with TEF
VACTERL
TEF anesthesia concerns—copious pharyngeal secretions requiring frequent ___; no ___ ventilation prior to intubation; ___ intubation without ___; neonates with TEF are ___ and ___nourished; do not ___ neck or ___ esophagus for fear of disruption of repair
Copious pharyngeal secretions requiring frequent suctioning; no positive pressure ventilation prior to intubation; awake intubation without muscle relaxants; neonates with TEF are dehydrated and malnourished; do not extend neck or instrument esophagus for fear of disruption of repair
Complications of ___ include pneumonia, congenital anomalies, atelectasis, subcutaneous emphysema, recurrent laryngeal nerve injury, esophageal stricture, anastomotic leaks
TEF
Principle causes of death from TEF: ___ complications, associated ___, ___ leaks
Pulmonary complications, associated anomalies, anastomotic leaks
The most common type of TE fistula is: A. Type II B. Type IIIA C. Type IIIB D. Type IIIC
C. Type IIIB (Gross type C)
Upper esophagus ends in a pouch, lower esophagus connects to the trachea
In a patient with CDH, what should the peak inspiratory pressure be (cm H2O): A. 20 B. 30 C. 40 D. 50
B. 30
Which is not a manifestation of CDH: A. Scaphoid abdomen B. Decreased breath sounds C. Arterial hypoxemia D. Lab tests
D. Lab tests
An appropriate blood pressure for a neonate should be: A. 110/60 B. 100/70 C. 95/65 D. 65/40
D. 65/40
