1) The document discusses a rare cardiac tumor known as an angiosarcoma that arises in the right atrium and can invade surrounding structures like the vena cava or tricuspid valve. 2) It also discusses Marfan syndrome, a connective tissue disorder associated with floppy mitral valves and aortic dissection, caused by a genetic mutation that leads to excessive TGF-β and weakening of heart and lung tissues. 3) Several questions are presented regarding various cardiac conditions, complications, diagnoses and treatment options.

1. Cardiology Questions
University of UTAH
Dr Ihab Suliman

3. • (D) This is a rare neoplasm (all cardiac
neoplasms are rare) but the size alone
suggests a malignancy.

4. • Nearly 80% of cardiac angiosarcomas
arise as mural masses in the right atrium.
Typically, they completely replace the
atrial wall and fill the entire cardiac
chamber.
• They may invade adjacent structures (eg,
vena cava, tricuspid valve).

6. • C) After about 4 days following infarction,
the muscle will still be necrotic and many
neutrophils will persist, while macrophage
infiltration will be beginning

8. • C). Mitral valve stenosis leads to left atrial
enlargement, but the left ventricle is
usually small.
• THere is typically a 'fishmouth' shaped
mitral valve that has stenosis as well as
insufficiency, since it does not close
completely. Most mitral valvular disease in
adults results from rheumatic valvulitis.

10. • C) CORRECT. A stab wound into heart
can lead to hemopericardium with
tamponade

12. • (D) Marfan syndrome is a connective
tissue disorder that is associated with
floppy mitral valve and also with cystic
medial necrosis that predisposes to aortic
dissection.

13. • Marfan syndrome (It is inherited as a
dominant trait. It is carried by a gene
called FBN1, which encodes a connective
protein called fibrillin-1.
• People have a pair of FBN1 genes.
Because it is dominant, people who have
inherited one affected FBN1 gene from
either parent will have Marfan's.

14. • The normal fibrillin-1 protein binds to another
protein, transforming growth factor beta (TGF-β).
TGF-β has deleterious effects on vascular
smooth muscle development and the integrity of
the extracellular matrix. Researchers now
believe that secondary to mutated fibrillin there
is excessive TGF-β at the lungs, heart valves,
and aorta, and this weakens the tissues and
causes the features of Marfan Syndrome.

15. • Since angiotensin II receptor blockers
(ARBs) also reduce TGF-β, they have
tested this by giving ARBs (losartan, etc.)
to a small sample of young, severely
affected Marfan syndrome patients. In
some patients, the growth of the aorta was
indeed reduced.

17. • (B) She has coarctation of the aorta, and
the constriction is postductal, allowing
prolonged survival. Her physical
characteristics also suggest Turner
syndrome (monosomy X).

19. • D) The small arteries of the kidney are
affected by hyperplastic arteriolosclerosis.
Malignant hypertension is often preceded
by chronic hypertension that leads to left
ventricular hypertrophy. Hypertension is a
risk for CNS hemorrhage

21. • D) The bioprosthesis has the advantage of
not requiring anticoagulation, but it does
not wear well with time, and typically must
be replaced within 5 to 10 years when its
leaflets undergo progressive calcification
leading to stenosis.

23. • A) The most common cause for a primary
myocarditis is a virus (such as Coxsackie
virus).
• Viral myocarditis can be a cause for
sudden death in a young person.

25. • (B) The history points to infectious
endocarditis and acute congestive heart
failure. Staphylococcus aureus and
Pseudomonas aeruginosa are the most
likely organisms to be found with a history
of injection drug use.

27. • Biatrial enlargement
• Left anterior fascicular block
• Left ventricular hypertrophy

29. • AF