Pulmonary Vascular Disease

Question 1

The most common causes of pulmonary vasculitides are the ANCA-associated vasculitis?

Answer 1

GME:
Granulomatosis with polyangiitis (GPA, formerly Wegener’s granulomatosis)

Microscopic polyangiitis (MPA)

Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome)

Question 2

Is DAH more commonly found in autologous or allogenic BMT?

Answer 2

Autologous BMT

Question 3

What is Lane-Hamilton syndrome?

Answer 3

Association of gluten- sensitive sprue (celiac sprue) with idiopathic pulmonary hemosiderosis. Only 18 cases are described in the literature. All IPH patients should be screened for sprue with serologies (antigliadin and antiendomysial antibodies). Gluten-free diet seems key to therapy and prevention of relapses.

Question 4

Question 5

Protamine should be given only when resuscitation techniques and treatment of anaphylactic and anaphylactoid shock are readily available. Reduce the rate of administration in cases of bradycardia, dyspnea, and hypotension. Administration that is too rapid may cause severe hypotension.

Question 6

For patients undergoing major orthopedic surgery, extend thromboprophylaxis in the outpatient period for up to 35 days (rather than 10–14 days).

Question 7

How is pulmonary vascular resistance calculated in Wood units?

Answer 7

(mPAP – PCWP) ÷ cardiac output (L/min) = PVR in Wood units

Question 8

What is the most common cause of pulmonary hypertension?

Answer 8

Left-sided systolic or diastolic heart disease (WHO Group 2), although this represents pulmonary venous hypertension rather than pulmonary arterial hypertension.

Question 9

What are common drugs/toxins implicated in pulmonary hypertension?

Answer 9

Aminorex,
fenfluramine,
dexfenfluramine, amphetamines, methamphetamines

Question 10

BMPR2 mutations are detected in ~70% of heritable PAH cases and 11–40% of IPAH patients (regardless of family history).

Question 11

What are the most common mutations seen in heritable PAH?

Answer 11

Bone morphogenetic protein mutation type 2 (BMPR2) and activin receptor-like kinase (ALK- 1)

Question 12

Question 13

As part of the evaluation of PAH, look for signs and symptoms of connective tissue disease and stigmata of liver disease.

Question 14

Which of the following cellular mechanisms is involved in the development of PAH?
A. Increased prostacyclin synthase
B. Increased NO synthase
C. Increased endothelin levels

Answer 14

C. Increased endothelin levels

Question 15

How is a positive vasoreactivity test defined during RHC?

Answer 15

A positive response to therapy with vasodilators is defined as a fall in mean PAP of > 10 mm Hg to reach a mean PAP of < 40 mm Hg with an increased or unchanged cardiac output.

Question 16

How does one establish a definitive diagnosis of PAH?

Answer 16

With RHC showing a mPAP ≥ 25 mm Hg with pulmonary capillary wedge pressure ≤ 15 mm Hg and pulmonary vascular resistance > 3 Wood units (240 dynes/sec/cm5).

Question 17

Patients with IPAH who respond to acute vasodilator testing should be treated initially with calcium channel blocker therapy.

Question 18

Which group of drugs is associated with hepatotoxicity?

Answer 18

Endothelin receptor antagonists (ERAs)

Question 19

Which group of drugs is contraindicated with the use of nitrates?

Answer 19

Phosphodiestrase-5 (PDE- 5) inhibitors

Question 20

Which group of drugs causes teratogenicity?

Answer 20

Endothelin receptor antagonists (ERAs)

Question 21

Riociguat, a soluble guanylate cyclase stimulator, was recently approved for the treatment of PAH after the PATENT- 1 trial showed improvement in exercise capacity/6MWD in patients with Group 1 PAH.

Question 22

Which of these categories of PAH has the best prognosis?
A. PAH associated with congenital heart disease
B. PAH associated with scleroderma
C. PAH associated with HIV
D. PAH associated with liver disease

Answer 22

A. PAH associated with congenital heart disease.

Question 23

What gene plays a role in the pathogenesis of PVOD?

Answer 23

BMPR-2

Question 24

Because of the significant risk of pulmonary edema, initiation of PAH-specific therapy in patients with PVOD needs to be carefully monitored, likely in an intensive care unit (ICU) setting, with a slow titration and aggressive use of diuretics.

Question 25

Acute chest syndrome is the leading cause of death in patients with sickle cell disease.

Question 26

What is the most common pathogen identified in patients with acute chest syndrome?

Answer 26

Chlamydia pneumoniae

Question 27

The most important predictor of chronic restrictive lung disease in sickle cell disease is the number of prior episodes of acute chest syndrome.

Question 28

What molecule is felt to be primarily responsible for the intrapulmonary vascular dilations seen in HPS?

Answer 28

Nitric oxide

Question 29

What is the most commonly used method for confirming IPVD in HPS patients?

Answer 29

Contrast enhanced transthoracic echo

Question 30

What intervention has been shown to improve oxygenation and survival in HPS patients?

Answer 30

Liver transplantation

Question 31

Answer 31

necrotizing granulomatous inflammationo f GPA (Wegner’s)

Question 32

Answer 32

masses and nodules seen in GPA (granulomatous manifestation)

Question 33

Answer 33

vasculitis and capillaritis, showing DAH 2/2 anca-associated vasculitis

Question 34