Pathophysiology of Pulmonary Atrial Hypertension? Sigh and symptoms of PAH and as a medical provider when should we be  concern?

 What is Pulmonary Atrial Hypertension?

Pulmonary arterial hypertension (PAH) is a disease of small pulmonary Artery which is a rare disorder of the blood vessels in the lungs. The pulmonary arteries become narrowed and the pressure in the arteries rises above the normal limits. The normal PAH is <25 and when the pressure is >25 that’s when it is considered pulmonary arterial hypertension. This causes strain on the right side of the heart and may become life-threatening. PAH may be either associated with other disease states or exist alone for no known reason.

Cases:

Could be idiopathic, Left-sided heart failure can cause it, Scleroderma, HIV

Pulmonary Atrial Hypertension: what type of patient do I expect

Individuals with PAH may go years without a diagnosis, either because their symptoms are mild, nonspecific, or only present during demanding exercise.

Common S/SX:

• Severe shortness of Breath
• tiredness
• felling faintness or dizzy
• chest pain
• racing heartbeat
• leg and ankle swelling.

However, it is important to treat PAH because without treatment, high blood pressure in the lungs causes the right heart to work much harder, and over time, this heart muscle may weaken or fail and can lead to right-sided heart failure.

When it is an emergency

• High or prolonged fever, especially if you have a central line catheter
• Fainting or near fainting
• Coughing up blood or colored mucus
• Chest pain
• Rapid or irregular heartbeats
• Unusual (for you) shortness of breath
• Unusual (for you) fluid retention
• Dark, tarry stool if you are on blood thinners such as warfarin (Coumadin)

Treatment

Flolan®, Veletri® and Remodulin® Emergencies

Intravenous Flolan® and Veletri® are given by continuous infusion through a central venous catheter. The half-life of these medications is expected to be no greater than six minutes. They are delivered by a CADD Legacy pump. Intravenous Remodulin® is given by continuous infusion through a central venous catheter. A CADD Legacy, CRONO 5 or CADD MS-3 pump is used to deliver this medication.