There are two main forms of the disease. Pulmonary arterial hypertension is when the pressure of the arterial system of the lungs is elevated, creating more work for the right side of the heart. Pulmonary venous hypertension is caused by a disruption in the flow of blood to the left atrium. There are varying causes for both, some of which include left to right shunts, mitral valve stenosis, clots in the lung, drugs and medications, HIV, all forms of cardiomyopathy, COPD, and connective tissue disorders that might cause scarring in the lungs, or it can be idiopathic.
The patient may have a normal presentation in the early stages of the disease. The nursing assessment should focus on breathlessness, hypoxia, dizziness, JVD, extra heart sounds, and swelling of the extremities and organs, particularly on the right side of the body. The disease usually takes a slow course of progression, and the patient may have adaptive behaviors and not realize how sick they truly are until they experience chest pain or other more acute symptoms. It is important to note that angina can occur even though the coronary arteries are clean, and the mechanism for this is still unknown.
The nurse should be familiar with the medications and treatments for this still relatively rare disorder. These range from medications to surgery. The surgical treatments are lung transplant and pulmonary thromboendarterectomies. The medications vary from the well known, such as anticoagulants to prevent clots from forming to the less well known, such as prostacyclin. Prostacyclin is a short-acting vasodilator and platelet aggregate inhibitor. The administration of this drug is complex, but can be taught to patients and families. In pulmonary venous hypertension, it is important to reduce afterload. This can be achieved with diuretics and inotropics such as Digoxin. It is important to note that hypoxia causes constriction of the pulmonary vasculature, so it is important for the nurse to properly identify and advocate for oxygen therapy.
The nursing care of these patients focuses on medication management, oxygen therapy, reducing anxiety, clustering care to reduce hypoxia and stress, physical therapies to reduce deconditioning, and a low salt diet. Take the time to explain the physical mechanics of how the blood flows and what makes their disease FEEL the way that it does. This will go a long way to reducing anxiety, increasing compliance, and empowering the patient and family.
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