Quiz 2- Pediatric Cardiology (add On)

Question 1

What other cardiac anomaly is coarctation of the aorta associated with?

Answer 1

Bicuspid aortic valve (seen in 70% of all cases)

Question 2

What genetic syndrome is coarctation of the aorta seen in?

Answer 2

Turner’s syndrome

Question 3

What are the hemodynamics of coarctation of the aorta?

Answer 3

LV outflow—> pressure hypertrophy of the LV

Question 4

What are clinical symptoms of coarctation of the aorta?

Answer 4

• ** CLASSIC SIGN IS DIMINUATION OR ABSENCE OF FEMORAL PULSES **
• cardiomegaly and rib notching on CXR
• higher BP in UE
• pulse discrepancy between left and right arms
• if ductus still open—> cyanosis
• with severe coarctation- LE hypoperfusion, acidosis, HF, shock

Question 5

What is the treatment or coarctation of the aorta?

Answer 5

Severe—> maintain patent DA with prostaglandin E1

• surgery to prevent LV dysfunction
• balloon angioplasty if re-coarctation occurs

Question 6

What is the treatment for obstruction to systemic flow, that may be caused by AS, coarctation, aortic arch interference, hypoplastic left heart syndrome?

Answer 6

• surgery or transcatheter approach in the first few days of life
• inotropes, mechanical ventilation, prostaglandin E
• with high Qp: Qs—> diuretics

Question 7

How is obstruction to pulmonary flow treated? (Caused by ductal dependent lesion, critical pulmonary valve stenosis, pulmonary atresia with intact ventricular septum)

Answer 7

• prostaglandin E1 to manage cyanosis

Question 8

What happens with D- transposition of the great arteries?

Answer 8

• RV ejects into the aorta while the LV ejects into the PAs

Question 9

How is D- transposition of the great arteries treated?

Answer 9

Prostaglandin E, and balloon angioplasty to enlarge PFO

Question 10

What are the various single ventricle lesions?

Answer 10

• tricuspid atresia
• double inlet left ventricle: both atria empt]y into LV, may have a right ventricle but is very tiny and non functional
• unbalanced AVSD—> complete mixing of systemic and pulmonary venous blood

Question 11

What are intrinsic myocardial disorders?

Answer 11

Cardiomyopathy and myocarditis

Question 12

T/F Shunts vary in direction and magnitude over time, even within the cardiac cycle.

Answer 12

True

Question 13

What is the equation for flow (Q)?

Answer 13

Q= (Pπr^4)/8nL

Question 14

When would you use endocarditis prophylaxis?

Answer 14

• prosthetic heart valve
• previous infective endocarditis
• heart transplant with valvulopathy
• CHD
  
  • unrepaired CHD
  • repaired CHD with prosthetic material during the 1st 6 months after procedure
  • repaired CHD it’s residual defects at or near the site of prosthetic patch/device

Question 15

Which antibiotics are used for endocarditis prophylaxis?

Answer 15

Amoxicillin
Ancef
Ampicillin
Clindamycin

Question 16

What is Kawasaki’s disease?

Answer 16

• mucocutaneous lymph node syndrome
• effects infants and young children
• vasculitis, coronary artery dilation, aneurysm formation, MI
• present as red hands, soles of feet, mouth and body rash

Question 17

How does trisomy 21 present?

Answer 17

• smaller for age
• short neck
• oblique palpebral fissures (opening between eyelids widened)
• small and low set ears
• macroglossia
• microdonia
• mandibular hypoplasia
• narrow nasopharynx
• hypertrophic lymphatic tissue
• generalized hypotonia

Question 18

Regarding Trisomy 21, what are anesthetic considerations?

Answer 18

*** BRADYCARDIA UNDER ANESTHESIA—> within 30-60 seconds, have atropine ready- VERY sensitive to volatile anesthetics **** (if you know nothing else, know this!!!)

- pulmonary HTN
 CV defects in 40-50%
- OSA
- MR
- C-spine disorders
- thyroid disease
- subglottic stenosis
- large tongue

Question 19

What is Turner Syndrome?

Answer 19

• x- linked
• webbed neck
• low set ears
• pigmented nevi (birthmark)
• short stature
• lymphadema
• HTN
• liver dz
• obesity
• DM
• hypothyroid
• • aortic coarctation and bicuspid aortic valve **

Question 20

What is most important regarding a patient with William’s Syndrome?

Answer 20

(Elfin faces)
*** with have narrowing of the aorta—> valvular and supravalvular aortic stenosis as well as aortic coarctation
- narrowing of abdominal and renal arteries
** can involve origin of coronary arteries as well as CA stenosis —> leading to myocardial ischemia and severe biventricular outflow obstruction
—> CV EVAL BEFORE ANESTHESIA IS A MUST !!!

Question 21

What is the leading cause of cardiac arrest in the perioperative cardiac arrest registry?

Answer 21

William’s syndrome MI

Question 22

What are the four characteristics of tetralogy of Fallot?

Answer 22

• pulmonary stenosis
• RVH
• VSD
• aorta overrides septal defect

Question 23

A concern for paradoxical air embolism May occur in the neonate because of:

Answer 23

Patent foramen ovale

Question 24

What are examples of a cyanotic shunt (right to left)?

Answer 24

• tetralogy of Fallot
• transposition of the great arteries
• tricuspid valve abnormality (ebsteins anomaly)
• truncus arteriosus
• total anomalous pulmonary venous connection

Question 25

Which anomaly is associated with a boot shaped heart on CXR?

Answer 25

Tetralogy of Fallot