What Is Pulmonary Hypertension?

Pulmonary hypertension is an acute or chronic pressure elevation located in the pulmonary vasculature, which is a mysterious phenomenon that may occur as an unexplained condition (primary pulmonary hypertension) or from diverse known causes (secondary pulmonary hypertension). Common causes of secondary hypertension are diseases of the left side of the heart, such as ischemic heart disease, aortic stenosis, or mitral stenosis. Through time, pulmonary hypertension can lead to pulmonary heart disease (cor pulmonale), which is characterized by right ventricular hypertrophy, dilation, and failure.

Illustration looks at mitochondrial gene mutation (represented by cross section of cell in foreground) effect on sodium transport and how that can become a contributing factor for hypertension (kidney glomerulus in background).

How Does Pulmonary Hypertension Impact the Body?

In most all forms of pulmonary hypertension, the pulmonary artery pressure rises, developing an impedance to right ventricular emptying. Initially, cardiac output is unchanged. However, as pulmonary hypertension progresses, the right ventricle begins to fail, cardiac output drops, and the patient develops shortness of breath and other symptoms at rest as well as during exercise.The following are the five categories that fall under pulmonary hypertension:

• diseases of the left side of the heart (most common)

• large left-to-right shunts

• thromboembolic hypoxia parenchymal

• lung disease

• idiopathic (primary) pulmonary hypertension (rare)

Signs of Pulmonary Hypertension

The typical signs and symptoms of pulmonary hypertension include exertional dyspnea, angina (from right ventricular ischemia), syncope (from low cardiac output), hypoxemia at rest, edema, S3 ventricular gallop, and unusual fatigue.

The Prognosis

Prognosis for pulmonary hypertension patients depend on the type and cause of the condition. If pulmonary causes are recognized and treated early, the pulmonary hypertension may go into remission. However, the condition may be rapidly progressive and fatal if the primary etiology is idiopathic or if the patient develops acute, severe right ventricular failure (as in patients with chronic obstructive pulmonary disease [COPD] complicated by pneumonia). Death could be imminent for the patient if a right-sided heart failure develops, or ischemia, or a pulmonary embolus, hemorrhage, or ventricular tachycardia/fibrillation results from hypoxemia and acidosis.

Lung transplant may be the only hope for survival for many patients with advanced pulmonary hypertension who no longer respond to medical management. Furthermore, patients with severe cardiomyopathy and pulmonary hypertension will need a heart and lung transplant.