Lecture 5 (Part 2)-Congenital Disease Processes Affiliated With Congenital Heart Defects

Question 1

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

Answer 1

Kawasaki Disease

Question 2

This disease occurs every 1 in 800 live births; results from balanced, unbalanced chromosome translocation or mosaicism

Answer 2

Trisomy 21 (Down Syndrome)

Question 3

Patients with Down Syndrome may experience—obstructive ___; mental ___; ___ spine disorders, vertebral ___; ___ disease; subglottic ___; ___ (small/large) tongue; difficult ___ access

Answer 3

Obstructive sleep apnea; mental retardation; cervical spine disorders, vertebral instability; thyroid disease; subglottic stenosis; large tongue; difficult vascular access

Question 4

Down syndrome—CV defect in ___-___%—___ septal defects, ___ septal defects, ___, patent ___; ___cardia under anesthesia; pulmonary ___tension

Answer 4

CV defect in 40-50%—AV septal defects, ventricular septal defects, tetralogy of fallot, patent ductus arteriosus; bradycardia under anesthesia; pulmonary hypertension

Question 5

Turner syndrome is ___ (X, Y) linked

Answer 5

X

Question 6

Turner syndrome—___ neck; low set ___; pigmented ___; ___gnathia (micro/macro); ___ (tall/short) stature; ___edema; ___ failure; aortic ___; ___cuspid aortic valve; ___tension; ___ disease; obesity; diabetes; ___thyroidism

Answer 6

Webbed neck; low set ears; pigmented nevi; micrognathia; short stature; lymphedema; ovarian failure; aortic coarctation; bicuspid aortic valve; hypertension; liver disease; obesity; diabetes; hypothyroidism

Question 7

___ syndrome—chromosome 7, deletion in the elastin gene

Answer 7

Williams syndrome

Question 8

Williams syndrome—___ facies; ___ abnormalities—___calcemia, ___thyroidism; mental ___; growth ___; altered ___ development

Answer 8

Elfin facies; endocrine abnormalities—hypercalcemia, hypothyroidism; mental retardation; growth deficiency; altered neuro development

Question 9

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 ___

Answer 9

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

Question 10

Patients with Williams syndrome need ___ evaluation before anesthesia is administered

Answer 10

Cardiac

Question 11

Williams syndrome is the leading cause of cardiac arrest in the perioperative cardiac arrest registry—T/F?

Answer 11

True

Question 12

Patients with Williams syndrome also may have ___ weakness, so caution with ___ (what medication class?)

Answer 12

Muscle weakness, so caution with muscle relaxants

Question 13

Patients with Williams syndrome may also exhibit ___

Answer 13

Autism

Question 14

DiGeorge/Velocardiofacial syndrome—22q11.2 deletion syndrome; catch 22; ___ defects; thymic ___plasia; ___ palate; ___calcemia

Answer 14

Cardiac defects; thymic hypoplasia; cleft palate; hypocalcemia

Question 15

DiGeorge/velocardiofacial syndrome—conotruncal abnormalities; ___ tract problems too; ___deficiency, so give ___ blood products

Answer 15

Outflow tract problems too; immunodeficiency, so give irradiated blood products

Question 16

Noonan syndrome—___ webbing; low set ___; chest ___; ___ (tall/short) stature; over 50% have ___; pulmonary valve ___; pulmonary ___; bleeding diathesis (aka bleeding disorders)

Answer 16

Neck webbing; low set ears; chest deformities; short stature; over 50% have CHD; pulmonary valve dysplasia; pulmonary stenosis

Question 17

___ 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

Answer 17

Marfan syndrome

Question 18

Marfan syndrome—ventricular ___; abnormal ___ (systolic/diastolic) function; pulmonary disease—chest wall deformities leading to progressive ___, ___ lung disease

Answer 18

Ventricular dilation; abnormal systolic function; pulmonary disease—chest wall deformities leading to progressive scoliosis, restrictive lung disease

Question 19

VACTERL

Answer 19

Non random anomalies

Vertebral
Anal
Cardiovascular
Tracheoesophageal
Renal
Limb defects

Question 20

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

Answer 20

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

Question 21

CHARGE association

Answer 21

Coloboma
Heart defects
Choanal atresia
Retardation of growth and development
Genitourinary problems
Ear abnormalities
Conotruncal and aortic arch anomalies
Upper airway abnormalities

Question 22

Congenital diaphragmatic hernia occurs at ___-___ weeks of fetal life

Answer 22

5-10 weeks

Question 23

Congenital diaphragmatic hernia—gut herniates into the ___

Answer 23

Thorax

Question 24

Mortality of congenital diaphragmatic hernia is ___%

Answer 24

50%

Question 25

Congenital diaphragmatic hernia—___ abdomen (bowel is in the thorax);

Answer 25

Scaphoid abdomen

Question 26

Causes of mortality with congenital diaphragmatic hernia—___ia; ___ insufficiency; persistent ___

Answer 26

Hypoxia; respiratory insufficiency; persistent pulmonary hypertension of newborn

Question 27

Treatment of CDH—immediate stabilization with sedation, paralysis, and moderate ___ventilation; ___O; ___ (inhalation agent) for PPH

Answer 27

Immediate stabilization with sedation, paralysis, and moderate hyperventilation; ECMO; nitric oxide for PPH

Question 28

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

Answer 28

Decrease gastric distention; low oxygen delivery; right sided pneumothorax concern; awake intubation; no nitrous oxide; peak inspiratory pressure should be < 30 mm H2O

Question 29

Most common variation of TEF is the form that ends in a blind ___ and ___ (upper/lower) esophagus that connects to the trachea

Answer 29

Ends in a blind pouch and lower esophagus connects to the trachea

Question 30

TEF—gastric ___ with respirations; ___ leads to choking, coughing, and cyanosis, ultimately leading to ___ia and ___cardia

Answer 30

Gastric distention with respirations; feeding leads to choking, coughing, and cyanosis, ultimately leading to hypoxia and bradycardia

Question 31

TEF is diagnosed by a failure to pass a catheter into the ___ and is confirmed by visualization of the catheter coiled in a blind ___

Answer 31

Into the stomach and is confirmed by visualization of the catheter coiled in a blind pouch

Question 32

___ is common with TEF

Answer 32

Aspiration pneumonia

Question 33

___ may also occur with TEF

Answer 33

VACTERL

Question 34

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

Answer 34

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

Question 35

Complications of ___ include pneumonia, congenital anomalies, atelectasis, subcutaneous emphysema, recurrent laryngeal nerve injury, esophageal stricture, anastomotic leaks

Answer 35

TEF

Question 36

Principle causes of death from TEF: ___ complications, associated ___, ___ leaks

Answer 36

Pulmonary complications, associated anomalies, anastomotic leaks

Question 37

The most common type of TE fistula is: 
A. Type II
B. Type IIIA
C. Type IIIB
D. Type IIIC

Answer 37

C. Type IIIB (Gross type C)

Upper esophagus ends in a pouch, lower esophagus connects to the trachea

Question 38

In a patient with CDH, what should the peak inspiratory pressure be (cm H2O):
A. 20
B. 30
C. 40
D. 50

Answer 38

B. 30

Question 39

Which is not a manifestation of CDH:
A. Scaphoid abdomen
B. Decreased breath sounds
C. Arterial hypoxemia
D. Lab tests

Answer 39

D. Lab tests

Question 40

An appropriate blood pressure for a neonate should be:
A. 110/60
B. 100/70
C. 95/65
D. 65/40

Answer 40

D. 65/40