Pulmonary hypertension (PHN) is rare condition of the blood vessels that results in high blood pressure due to the narrowing of the pulmonary arteries that carry blood from heart to the lungs. Pulmonary hypertension causes the cells of the arteries to overgrow, thicken and become stiff. This raises blood pressure between the heart and lung and places and increased demand on the right ventricle of the heart causing it to weaken. Pulmonary hypertension leads to right-sided heart failure as the muscle works to pump blood to the lungs against resistance.
There is no cure for pulmonary hypertension and the condition can be fatal in some cases. For patients who must live with the disease, doctors are developing new ways to manage symptoms and improve quality of life.
Pulmonary hypertension starts when the cells of the pulmonary arteries experience changes that can include injury and inflammation.
Some patients develop pulmonary hypertension without any clear cause. Doctors believe that these patients may carry a genetic predisposition for the condition, but no clear answers have been found.
Secondary PHN is more common that idiopathic and results after another illness or injury has occurred in the body. Secondary PHN can be caused by:
- Blood clots in the lungs.
- Left sided heart failure.
- Chronic liver disease.
- Living or climbing in high altitudes (higher than 8,000 feet).
- Some stimulant drugs like cocaine, and other factors.
PVOD is a less common type of PHN, occurring in about 5-20 percent of cases. PVOD may have a variety of causes but all result in the constriction or complete occlusion of the pulmonary veins. The outlook for PVOD is grim without proper treatment. The condition is rapid and progressive and patients may need advanced surgery like a lung transplant or heart-lung transplant much sooner than other patients with PHN.
Eisenmenger syndrome is a congenital heart defect that can also lead to PHN. This less common trigger causes blood to flow through an abnormal hole between the bottom two chambers of the heart. Improper circulation and mixing of oxygenated and deoxygenated blood backs up in the lungs, increasing pressure and triggering PHN.
Symptoms of this condition include shortness of breath with daily activities, chest pain, racing heartbeat, upper abdominal pain, poor appetite, as well as fatigue and dizziness. As the condition progresses, symptoms may include fainting, blueness around the lips, and swelling of the legs and ankles.
Pulmonary hypertension may start out with symptoms similar to many other heart and lung problems. Doctors may not be able to distinguish it from other conditions without extensive testing. Patients may need a battery of exams and diagnostics to determine the cause of symptoms, beginning with basic procedures and advancing to more invasive techniques as needed.
- Chest X-ray
- Blood work
- Right-sided heart catheterization
- Transesophageal echocardiogram
- Pulmonary function tests
- Perfusion lung scans
- CT scan
- Open lung biopsy
- Genetic testing
Treating PHN may require a variety of approaches. Many of the techniques used for treatment are complex and will require adjustment if they stop working. For patients with secondary PHN, doctors will treat the underlying condition first. Medications that focus on treating swelling of the arteries, blood thinners, diuretics for fluid management, blood vessel dilating medications and oxygen are all standard. Surgical intervention may include open heart surgery to make a hole between the bottom two chambers of the heart to release pressure on the right side (atrial septostomy) and advanced cases may need a lung transplant or heart-lung transplant.