Pulmonary Hypertension

Question 1

Pulm HTN =

Answer 1

PAP > or = to 25 mm Hg with normal pulmonary wedge pressure

Question 2

Pulmonary Arterial HTN causes

Answer 2

Idiopathic pulmonary hypertension
Heritable gene mutations
Drug and toxin induced
Connective tissue disease
HIV infection
Portal hypertension
Congenital heart defects
Schistosomiasis
Chronic hemolytic anemia

Question 3

Pulm HTN d/t Left Heart Disease causes

Answer 3

Systolic dysfunction

Diastolic dysfunction

Question 4

PHTN d/t Lung dz or hypoxia Causes

Answer 4

COPD
Interstitial lung disease
Other pulmonary disease with mixed restrictive and obstructive patterns

Question 5

Unclear Multifactorial Mechanisms

Answer 5

Hematologic disorders
Systemic disorders such as vasculitis, sarcoidosis
Metabolic disorders such as thyroid and glycogen storage disorders
Chronic renal failure on dialysis

Question 6

Signs and Symptoms

Answer 6

Early symptoms- dyspnea, chest pain, fatigue and lightheadedness
Later symptoms- syncope, abdominal distention, ascites and peripheral edema
Young women with evidence of progressive right-sided heart failure can have idiopathic primary pulmonary hypertension
Signs of COPD or obstructive sleep apnea

Question 7

Dx Studies

Answer 7

Must exclude a secondary cause
Hypercoaguable work-up
R/O chronic pulmonary emboli
Sleep study
ECG- right ventricular hypertrophy and right atrial enlargement
Echocardiogram - gold standard for dx pulm HTN, then need to r/o secondary causes
CT of chest

Question 8

Tx

Answer 8

Warfarin in all patients unless counterindicated
Diuretics in patients with right sided heart failure
Salt restricted diets
Referral to pulmonary hypertension specialist for treatment with vasodilators and other treatment strategies
Most patients die of right ventricular failure and required specialized care by pulmonary hypertension experts