Question

Case 1:

A 57-year-old female with systemic sclerosis presents with progressive breathlessness. Her spirometry is well preserved but the diffusion capacity of the lungs for carbon monoxide (DLCO) is 45% of predicted. There is no fibrosis or thromboembolic disease on computed tomography (CT) scanning of her lungs. Right heart catheterization reveals a mean pulmonary arterial pressure of 42 mm Hg (normal values <25 mm Hg) and a pulmonary arterial wedge pressure of 12 mm Hg (normal values ≤15 mm Hg) together with a reduced cardiac output. The likely form of pulmonary hypertension is:  

1. Pulmonary arterial hypertension (PAH) (group 1).
2. Pulmonary hypertension associated with left heart disease (group 2).
3. Pulmonary hypertension associated with lung disease (group 3).
4. Chronic thromboembolic pulmonary hypertension (group 4).
5. Pulmonary hypertension associated with multifactorial mechanisms (group 5).

Case 2:

A 42-year-old with idiopathic pulmonary arterial hypertension (PAH) who is normally treated with sildenafil and who is anticoagulated with warfarin is admitted with a 24-h history of marked deterioration in exercise capacity. His blood pressure is 95/60 mm Hg, heart rate 130 beats min−1 and saturation 95% on room air. Chest X-ray shows clear lung fields and his C-reactive protein is 3 mg litre−1 (normal range is <8 mg litre−1) ECG demonstrates new-onset atrial flutter with 2:1 atrioventricular block. The most appropriate management is:

1. Addition of an endothelin receptor antagonist.
2. Commencement of intravenous iloprost.
3. Commencement of intravenous dobutamine.
4. DC cardioversion.
5. Increase in sildenafil dose.

Case 3:

A 63-year-old female with pulmonary arterial hypertension (PAH) associated with systemic sclerosis is admitted with increased breathlessness. She is currently treated with sildenafil and ambrisentan. Her blood pressure is 110/65 mm Hg, heart rate 95 beats min−1 and saturation 94% on room air. Her ECG shows sinus rhythm and a chest X-ray shows a new small right-sided pleural effusion. Her C-reactive protein is 4 mg litre−1 (normal range is <8 mg litre−1) and her creatinine is 115 μmol litre−1 (normal range is 49–90 μmol litre−1). She has a raised jugular venous pressure and pitting oedema to her thigh. The most appropriate initial treatment is:

1. Further reduction of right ventricular afterload with the addition of intravenous prostanoid.
2. Improvement in right ventricular contractility with the addition of intravenous dobutamine.
3. Improvement in systemic perfusion pressures with the addition of intravenous norepinephrine.
4. Optimization of right ventricular preload with the addition of intravenous loop diuretic.
5. Optimization of right ventricular preload with a fluid challenge

Answer 1

1

Pulmonary hypertension with lung disease

When the tiny blood vessels in your lungs become thickened, narrowed, blocked or destroyed, it's harder for blood to flow through the lungs. As a result, blood pressure increases in the lungs,then to pulmonary artery a condition called pulmonary hypertension

There are several conditions that can decrease the DLCO. These include cigarette smoking, emphysema, interstitial lung disease, anemia, decreased lung volume, heart failure, pulmonary vascular disease (pulmonary emboli and pulmonary hypertension), and others.

So pressure in pulmonary artery is also increased.

2

D - dc cardioversion

A DC Cardioversion (Direct Current Cardioversion) is a procedure to convert an abnormal heart rhythm to a normal heart rhythm.

Atrial Flutter(AF) is the most common cardiac arrhythmia (abnormal rhythm). Patients in AF are often not aware of any symptoms and the condition is not, in itself life threatening. However, patients are usually given anti-coagulants (blood thinning) medication – most commonly Warfarin, to protect them from having a stroke.
The procedure is carried out under a general anaesthetic and takes just a few minutes.

Patients who are referred for a DC Cardioversion are generally on Warfarin.  

3

B- Improvement in right ventricular contractibility with iv dobutamine.

This decrease the preload on right heart and venous pressure and edema will reduce