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1

A 40-year-old male, non-smoker, born to consanguineous parents presented to his PMD with recurrent productive cough, rhinorrhea, and headache for 20 years. He had been previously treated with antibiotics, antihistamines, bronchodilators, inhaled and oral corticosteroids, and anti- tuberculous drugs. He has no children, although he and his wife of 15 years would like to have children. His chest x-ray is shown. (heart is flipped) Which is the most likely diagnosis?
a. Cystic Fibrosis b. Kartegener syndrome c. Heart failure d. Atrial septal defect

Kartegener syndrome: the heart is on the right side - a ciliary dysfunction

Kartagener's syndrome (a type of primary ciliary dyskinesia) is a rare, autosomal recessive disorder comprising a triad of situs inversus, chronic sinusitis, and bronchiectasis. Defects in dynein (involved in L/R asymmetry) cause the ciliary dysfunction. Defective movement of cilia leads to recurrent chest, ear and sinus infections and infertility. Dextrocardia, which this patient has, is the most common positional defect of the heart and in most cases the CV system functions normally.

2

Paradoxical emboli are seen in ASD. What is the paradox?

An embolus originating in the venous circulation is in the aortic circulation.

If a clot is in the leg, it goes to right side of the heart, venous blood goes to lungs, that clot can never reach the left side.

3

A 36 year old woman ruptured her right Achilles tendon in a skiing accident. She smokes 15 cigarettes a day and takes oral contraceptives. She is admitted to a local hospital where surgical repair of the Achilles tendon is performed followed by fitting of a plaster cast. At one week, she develops increasing pain in her right leg. When the plaster is removed the right calf is found to be painful, warm, and swollen. The next day while straining on the toilet she collapses with right arm weakness. Clinical examination revealed dysarthria with weakness of the right arm. What mechanism(s) explain(s) her complete clinical findings?

A patent foramen ovale and straining on the toilet.

Straining increases venous pressure, push on abdomen, it pushes on all the viscera so it goes to the right heart, so when you strain right heart pressure goes up. You have a transient reversal allowing for opening of PFO.

4

What is the neural crest cells important for forming.

PNS (dorsal root ganglia, cranial nerves, autonomic ganglia, Schwann cells),
melanocytes,
chromaffin cells of adrenal medulla, -parafollicular (C) cells of thyroid,
-pia and arachnoid,
-bones of the skull,
-odontoblasts,
-aorticopulmonary septum,
-endocardial cushions

5

A 5-year-old child is brought to the physician by his parents because he develops cyanosis with minimal exertion. The parents tell you that he has experienced occasional cyanotic episodes that first began soon after he was born, and that they appear to be becoming more frequent. During the episodes, the child assumes a squatting position because it makes him "feel better.” On physical examination, he is found to have an enlarged right ventricle. Which of the following embryological events most likely underlies his condition?

This is early cyanosis -

classically squatting is associated with tetralogy of fallot, but really associate it with creating a right to left shunt.

6

a 2 year old child is seen in the pediatric cardiology unit for a congenital heart condition, which of the conditions occur the most often?

Muscular VSD

7

A 5-year-old male with a bounding pulse has a thrill best palpated over the upper left sternal edge. A continuous murmur is heard over the area on cardiac auscultation and caused by the lack of closure of fetal bypass channel. This channel is a derivative of which of the following embryologic structures?

Sixth aortic arch

Machinery murmur - note the location - open ductus arteriosis

8

Aortic arch derivatives

1st, 2nd, 3rd, 4th, 6th

.

9

Measurements of blood oxygen are taken in a fetus. The highest value is most likely recorded in which of the following vessels?

Inferior vena cava

what would dilute the blood in the aortic arch. PFO

10

Where does coronary artery occlusion usually happen?

Left anterior descending.

11

Which is the following statements is most accurate for ventricular cardiac muscle?
A. The T tubules of cardiac muscle store less calcium than the T tubules of skeletal muscle.
B. Repolarization is caused by opening of sodium channels.
C. The strength of contraction depends on extracellular calcium concentrations.
D. The initiation of the AP is caused by opening of voltage- gated calcium channels.

C.

That is the special property of cardiac muscle. Its contractility is dependent on concentration of extracellular calcium.

12

A man has an end-diastolic volume (EDV) of 140 mL, an end-systolic volume (ESV) of 70 mL, and a heart rate of 70 beats/min. What is his stroke volume and his cardiac output ?

70 ml, 4900 ml/min

13

Ejection fraction – left ventricular EF is an index of ventricular contractility. EF=SV/EDV. Normal EF is > 55%
A man has an end-diastolic volume (EDV) of 140 mL, an end-systolic volume (ESV) of 70 mL, and a heart rate of 70 beats/min. What is his ejection fraction and is it normal ?

50%, no

14

Contractility (and SV) increase with:

Contractility (and SV) increase with:
1) Increased intracellular Ca2+
2) Catecholamines
3) Decreased extracellular Na+
4) Digitalis (digoxin)

15

Contractility (and SV) decrease with:

Contractility (and SV) decrease with:
1) β-blockade
2) Heart failure
3) Acidosis
4) Hypoxia
5) Ca2+ channel blockers

16

Why do you think an increase in preload (reflected by SV) increases MVO2 less than an increase in afterload (reflected by MAP)?
A. No idea – get me out of here
B. Volume considerations are not part of the Laplace equation
C. An increase in afterload actually reduces wall tension D. A volume increase would not linearly increase chamber radius
E. A volume increase would not affect chamber radius

radius increased by 3rd root of volume.
Answer D.

17

Using Laplace’s equation which hypertrophy strategy or strategies would be most effective in reducing O2 consumption?
A. Concentric because wall tension would increase
B. Concentric because wall tension would decrease
C. Eccentric because wall tension would increase
D. Eccentric because wall tension would decrease
E. Both strategies because wall tension would increase with either
F. Both strategies because wall tension would decrease with either

decrease of radius and thickness increases, decreasing wall tension would decrease oxygen consumption.