Pulmonary hypertension, pulmonary embolism, cor pulmonale

Question 1

pulmonary hypertension (PH)

Answer 1

Elevated pulmonary artery pressure
Definition:
a median pulmonary artery pressure of >25 mm Hg at rest
measured by invasive monitoring (R heart cath)
Leads to RVH
New nomenclature
Formerly termed primary and secondary
Now 5 subtypes depending on precipitating cause

Question 2

PH patients

Answer 2

USA prevalence - 50,000-100,000
But only 15,000 to 25,000 are appropriately diagnosed and treated
2:1 female to male ratio
Mean age- 50 years
Group 1 PAH the mean age and sex in 35 y/o female
Mean duration of symptoms prior to diagnosis is 27 months

Question 3

Group 1 PAH

Answer 3

pulmonary arterial hypertension includes idiopathic pulmonary HTN, drug and toxin induced, portal HTN, HIV, connective tissue d/o

Question 4

Group 2 PH

Answer 4

Pulmonary HTN owing to left heart disease

Question 5

Group 3 PH

Answer 5

Pulmonary HTN owing to lung diseases or hypoxemia

Question 6

Group 4 PH

Answer 6

chronic thromboembolic pulmonary HTN

Question 7

Group 5 PH

Answer 7

pulmonary HTN with unclear multifactorial mechanisms

Question 8

PH hx

Answer 8

symptoms are often present for 2 yrs before diagnosis
Exertional dyspnea
Weakness
Fatigue

Question 9

PH PE

Answer 9

Wide splitting of the 2nd heart sound
Accentuation of 2nd heart sound in pulmonic area
Pulmonic regurgitation
murmur of tricuspid regurgitation, and a right ventricular lift (heave) may be noted (advanced cases)
elevated jugular venous pressure

Question 10

PH complications

Answer 10

Advanced R-sided heart failure with hepatic congestion
Pedal edema
Pleural effusions
Ascites
Worsening DOE (dyspnea on exertion)

Question 11

PH work up

Answer 11

ECG
CXR
transthoracic echocardiography (TTE)
Pulmonary function tests 
Overnight oximetry
Polysomnography
VQ scan (ventilation–perfusion studies)
Lab studies
6 Minute walk test
Diagnostic Gold Standard**
Right heart catheterization

Question 12

PH Tx

Answer 12

Goal:
decrease in clinical symptoms
improvement in exercise tolerance
longer survival
Therapy:
Begins with baseline assessment
Primary therapy 
Treat underlying condition
Advanced therapy:
improve arterial vasodilation
improve control of cellular growth and remodeling

Question 13

PH Therapy

Answer 13

Diuretics
Oxygen therapy
Goal is oxygenation saturation > 90%
Anticoagulation
Group 1 PAH and Group 4 PH
Warfarin – INR target of 2
Digoxin 
Group 3 PH
Those with tachyarrythmias
Exercise Training
Beneficial for all patients