Pulmonary Vascular Disease (Gerke)

Question 1

where does an embolus most often get lodged?

Answer 1

pulmonary arteries

Question 2

why do people get low oxygen with a pulmonary embolism?

Answer 2

v/q mismatch! (it’s baaaaaack)

Question 3

why do patients get hypoxemic with an arterio-venous malformation (ie, fistula)?

Answer 3

shunt (blood bypasses the oxygen beds of the lungs, considered an extreme form of v/q mismatch)

Question 4

condition that may or may not be caused by heart failure, in which blood backs up into the pulmonary arterial system and leaks out through the vessels because of hydrostatic pressure

Answer 4

pulmonary edema

Question 5

fibrin degradation product from crosslinked fibrin indicating recent coagulation

Answer 5

d-dimer

Question 6

in terms of the wells criteria, what is considered low probability for a PE?

Answer 6

less than 4, with negative d-dimer assay

Question 7

dilated right ventricle and flattened ventricular septum with a compressed left ventricle is indicative of what condition?

Answer 7

PE

Question 8

What are 3 imaging findings that are indicative of a PE?

Answer 8

1. dilated right ventricle
2. flattened ventricular septum
3. compressed left ventricle

Question 9

A 30 year old female who just got back from her honeymoon in Hawaii was walking off the plane when she had sudden onset of shortness of breath and a pain in her right side. Her husband drove her to ER where a physical exam revealed the following: BP 100/60, P 110, sats 89%, R20, T37.9. She was anxious appearing with crackles in right lung base with accentuation of second heart sound. Her right leg was slightly bigger in size than left and was mildly erythematous. What is the likely diagnosis?

Answer 9

PE

Question 10

A 46 yo female has a 1-2 year onset of progressive shortness of breath with exertion. She shows up to the ER where a physical exam revealed the following: P 90, R 12, BP 125/75, Sats 88% on RA. Chest was clear to auscultation (CTA) bilaterally, heart rate was tachycardic but regular, and there was mild cyanosis of fingernails. Imaging revealed a large mass on the right lower lobe, with pooling blood. What is the likely diagnosis?

Answer 10

Pulmonary arterio-venous malformation (AVM); common causes of mortalilty of this include stroke, cerebral abscess, hemoptysis and hemothorax.

Question 11

A 37 y.o. year old male with no previous medical problems was admitted to the hospital with 1-2 week history of significant night sweats, painful skin lesions.
fatigue and myalgias. Now also 2 days of abdominal pain, cough, dyspnea, and hemoptysis. The patient went to his doctor a few days before admission and was prescribed azithromycin which he took but his symptoms did not change. Laboratory values were remarkable
for very high CRP, WBC, ANCA and platelet counts. A CT scan of the chest showed multiple pulmonary cavitating nodules. What is the likely diagnosis?

Answer 11

granulomatosis with polyangitis (ANCA vasculitis)

Question 12

What test is essential to the diagnosis of vasculitis?

Answer 12

tissue biopsy

Question 13

early symptoms of this disease appear in the respiratory tract (e.g., nose, sinuses and lungs) and include nasal congestion, frequent nosebleeds, shortness of breath, and cough that may produce bloody phlegm; may eventually lead to kidney failure

Answer 13

granulomatosis with polyangitis (ANCA vasculitis)

Question 14

c-ANCA is associated with antibodies against this cytoplasmic protein, and presents with symptoms of what kind of disease?

Answer 14

anti-PR3; presents with symptoms of vasculitis (often with nodules present)

Question 15

p-ANCA is associated with antibodies against this perinuclear protein, and often presents with symptoms of what kind of disease?

Answer 15

anti-MPO; presents with symptoms of pulmonary fibrosis

Question 16

what is the primary treatment for ANCA associated vasculitis (AAV)?

Answer 16

corticosteroids, rituximab, cyclophosphamide

Question 17

A 40 yo female presents to clinic with 1 year of worsening shortness of breath. Now, she can barely make it around her house without stopping. She passed out while walking two times in the past week (full syncope). She is obese and smokes ½ pack/day. Physical exam revealed SpO2 88% RA, perioral cyanosis, loud P2 heart sound, midsystolic ejection murmur on the left sternal border. Her chest is clear to ausculatation and she has edema of bilateral lower extremities. Echo was significant for right ventricle overload as indicated by a flattening septum and D-shaped LV. Her V/Q scan and a CT for PE were both normal. What is the likely diagnosis?

Answer 17

idiopathic pulmonary hypertension (meaning it occurs pre-capillary in the pulmonary arteries, as opposed to post-capillary as in, for example, left sided heart failure and blood back up)

Question 18

Which of the following statements regarding the WHO classification of PH is false?
A. The BMPR2 gene mutation falls into group 2
B. Left heart disease is the most common cause of PH and is often treated with diuresis
C. COPD is a group 3 disease
D. The only type of PH that can be surgically treated is CTEPH, which is group 4
E. Idiopathic PH is a group 1 disease

Answer 18

A. The BMPR2 gene mutation falls under familial causes of Group 1 pulmonary hypertension.

Group 1: idiopathic and familial PAH
Group 2: Left heart disease
Group 3: Chronic lung disease
Group 4: Chronic thromboembolitic PH
Group 5: Unclear mechanism

Question 19

how should pulmonary hypertension be treated?

Answer 19

for every group except group 1, first treat the underlying cause

Question 20

how is idiopathic PH treated?

Answer 20

advaned therapies that focus on relaxing the pumonary arteries

Question 21

which of these is NOT a target of advanced therapy for idiopathic PH?
A. endothelin receptor antagonists
B. phosphodiester type 5 inhibitors
C. cGMP inhibitors
D. prostacyclin derivatives

Answer 21

C.

cGMP stimulation, not inhibition, is used to produce NO which is a vasodilator (ie, riociguat)

Question 22

A 32 year old female with no past medical history presents with 8 months of progressive dyspnea. Her echo shows high pulmonary artery pressures. Right heart catheterization shows a mean pulmonary artery pressure of 41 mmHg. Her wedge pressure is 8 mmHg. Her right ventricle is enlarged and has diminished function. She has no evidence of pulmonary embolism, connective tissue disease, chronic lung disease, hypoxia, or any other systemic disorder.

Which of the following treatments might you choose to improve symptoms in this patient?

a) rotating antibiotics with amoxicillin and doxycycline alternating every other month
b) low-dose corticosteroids
c) tiotropium inhaler daily
d) phosphodiesterase -5 inhibitor

Answer 22

D

Question 23

Which of the following diseases would be MOST LIKELY to present with lung granulomas?

a) Asthma
b) Mesothelioma
c) Sarcoidosis
d) Emphysema
e) Pulmonary embolus

Answer 23

C