2018 Board Review Pre-Test:
Pulmonary Hypertension

Pulmonary Hypertension - Jonathan D. Rich, MD, FHFSA

Please complete this brief Pre-Test to give a baseline for learning .

Question Title

* 1. A 48-year-old woman was diagnosed with idiopathic pulmonary arterial hypertension 6 years ago. She was initially treated with oral targeted PAH therapy but three years ago, she was transitioned to intravenous epoprostenol because of worsening symptoms. Unfortunately, despite initial improvements, her condition has again begun to deteriorate, and she is now seeking an opinion regarding additional treatment options. An echocardiogram reveals severe RV dysfunction. You repeat a right heart catheterization which reveals: RA pressure is 24 mmHg, PA pressure 92/40/58, PCWP 12, CO 3.8/1.9. HR is 110, BP 92/68, O2 saturation 94% on room air.

Which of the following is correct?

A. Intravenous epoprostenol is the only approved PAH therapy shown to have a survival benefit in PAH.

B. She is not a candidate for lung transplantation alone given her severe PAH and RV dysfunction and would thus require a combined heart-lung transplant.

C. Her hemodynamics suggest she should be referred for an atrial septostomy in experienced hands.

D. Approved PAH drugs improve six-minute walk distance and improvements in six-minute walk distance have been shown to be associated with improved survival in PAH.

E. A right heart catheterization is necessary in the evaluation of PAH unless there is a good tricuspid regurgitation envelope using Doppler Echo, which allows for the calculation of PA systolic pressure.

Question Title

* 2. A 32-year-old woman has had progressive dyspnea on exertion for the past 6 months and is seeking a diagnosis. On examination, she has mild jugular venous distention, slightly diminished carotid upstrokes, an RV lift, a right sided S4, and a 2/6 TR murmur. Her ECG reveals sinus rhythm with right ventricular hypertrophy. Echo shows severe RV dilatation and reduced function with normal LV function and no valvular abnormalities. The remainder of your evaluation is highly suggestive of pulmonary arterial hypertension. You proceed with a right heart catheterization which reveals:

1. Baseline: RA 12, PA 95/38/55, PCWP 10, CO 5.0 (CI 2.5), BP 110/74/85

2. After nitric oxide challenge: RA 10, PA 82/32/44, PCWP 10, CO 6.0 (CI 3.0), BP 115/70/85

After the procedure, you meet with the patient and discuss next steps in management. Which of the following would be appropriate?

A. You recommend starting high dose amlodipine

B. You determine that her pulmonary vascular resistance is 15 Wood units and thus recommend initiating her on targeted pulmonary hypertension treatment

C. You opt to treat her with the endothelin receptor antagonist, bosentan, but recommend monthly LFT testing and counsel her about birth control

D. In addition to starting her on PAH therapy, you recommend genetic testing for the Bone Morphogenetic Protein Receptor II (BMPR2) mutation.

Question Title

* 3. Please provide your first and last name.
Pre-test questions will not be scored, your name is required solely to confirm completion.

First Name *

Last Name

Question Title

Question Title

Question Title

* 3. Please provide your first and last name.Pre-test questions will not be scored, your name is required solely to confirm completion.

* 3. Please provide your first and last name.
Pre-test questions will not be scored, your name is required solely to confirm completion.