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Pulmonary Hypertension occurs when the pressure in the circulation rises to high with or without reason.

Hypertension is considered as an increase in blood pressure.

When blood flows through the Pulmonary Circulation it goes through the pulmonary artery, pulmonary vein, and/or the pulmonary capillaries.

Often the result is having a high blood flow but at the same time a low pressure.

This allows for a large increase in volume when exercising but does not actually cause a huge increase in blood pressure.

Smooth Muscle line the pulmonary circulation to help allow for constriction and dilation when necessary.

Once present, it often results in a “backing-up” problem leading to additional consequences

Can be classified as Primary or Secondary pulmonary hypertension depending on cause

It can be one of five types:

1.       Arterial
2.       Venous
3.       Hypoxic
4.       Thromboembolic
5.       Miscellaneous

The Venice 2003 Revised Classification system Illustrates that Five types

WHO group I         –   Pulmonary arterial hypertension (PAH)
WHO group II       -   Pulmonary hypertension associated with left sided heart disease
WHO group III     -   Pulmonary hypertension associated with lung diseases and/or hypoxemia
WHO group IV      -   Pulmonary hypertension due to chronic thrombotic and/or embolic disease
WHO group V        -   Miscellaneous

“Proceedings of the 3rd World Symposium on Pulmonary Arterial Hypertension. Venice, Italy, June 23-25, 2003” (2004). J. Am. Coll. Cardiol. 43 (12 Suppl S): 1S–90S. doi:10.1016/j.jacc.2004.02.037. PMID 15194171.

Symptoms

-    Difficulty breathing
-    Painful breathing
-    Breathing can be worse with exercise and better at rest
-    Fatigue
-    Dizziness
-    Syncope
-    Cough
-    Coughing of blood
-    Edema

Diagnosis

X-rays and CT scans can be helpful

Dilation of arteries may be seen

Echocardiography can evaluate the heart

Doppler Ultrasonography helps with the pressure

The exact pressure can be determined only by heart catherization

Causes:

·     Pulmonary Embolism
·     Sickle Cell disease
·     Pericarditis
·     Mitral stenosis
·     Left sided Heart Failure
·     Polycythemia
·     HIV
·     Hepatic Cirrhosis
·     Portal Hypertension
·     Emphysema
·     and others

Treatment:

The use of medications largely depends on condition and underlying cause – not all medications would be used in all populations;

Those suffering Heart Failure

Diuretics
Beta-Blockers
ACE Inhibitors

Pulmonary Arterial Hypertension

Anticoagulants
.         Digoxin

Diuretics

.         Adenosine
.         Epoprostenol
Calcium Channel Blockers

Vasoactive Substances are for patients that have abnormal constriction with smooth muscles

·         Endothelin receptor antagonists
·         Phosphodiesterase type 5 inhibitors
·         Prostacyclin derivates

Prostaglandins

·         Epoprostenol   [Flolan]
·         Treprostinil   [Remodulin]
·         Iloprost   [Ilomedin] IV
·         Iloprost   [Ventavis]

Endothelin receptor antagonists

Phosphodiesterase type 5 inhibitors