With advances in diagnosis and treatment options, pulmonary hypertension is more treatable today than ever before. The biggest challenge, says Ronald Zolty, MD, cardiologist at Nebraska Medicine, is getting an early diagnosis.
"Pulmonary hypertension in its earliest stages is difficult to diagnose and often gets overlooked because it doesn’t show up in standard cardiac diagnostic tests," says Dr. Zolty.
In its earliest stages, patients often complain of shortness of breath, especially with exertion. If an echocardiogram is ordered, it too will typically come out normal unless the right side of the heart is evaluated. Even then, the test may still appear normal if the patient is in his or her earliest stages.
"At Nebraska Medicine, we perform a comprehensive evaluation to determine if the patient has pulmonary hypertension and to find the source or cause of the condition," says Dr. Zolty. "Knowing the cause of this condition is extremely important as this will guide us in how to appropriately treat the patient.
"The earlier we diagnose this disease, the more successful we will be at stopping its progression. If nothing is done to treat it, it will continue to progress. As the disease progresses, the patient will begin to develop resting pulmonary hypertension and eventually, right side heart failure. However, when the disease is caught early and treated appropriately, many patients can go on to live a normal lifespan."
Early symptoms of pulmonary hypertension include shortness of breath with exertion followed by fatigue, lower extremity edema and abdominal distention. In more advanced stages of the disease, the patient may experience dizziness and syncope.
"We like to see patients when they are having shortness of breath with exertion," says Dr. Zolty. All patients will undergo a comprehensive evaluation process that starts with a left and right side echocardiogram. This is followed by a B-type Natriuretic Peptide (BNP) blood test to measure the level of fluid retention and heart stretch. These two tests may be negative if the patient is in the earlier stages of the disease. The next test performed is a right side cardiac catheterization – the gold standard test for pulmonary hypertension. The catheter is placed in the patient’s jugular vein to assess resting cardiac and pulmonary pressures. The patient is then asked to exercise for three minutes to see if exercise induces elevation of the pulmonary pressures which may explain why the patient is experiencing shortness of breath with exertion. If pulmonary hypertension is confirmed, a host of other tests are performed to determine the cause. These may include a sleep study, pulmonary function tests, perfusion ventilation nuclear tests, blood work, liver function test, connective tissue tests, HIV test and a cardiac MRI.
"We like to follow up with patients about six weeks later with an echocardiogram to make sure their medications are working properly," says Dr. Zolty. "Once we have feel like we have them on the right treatment plan, we recommend follow up every three months to ensure the medications are keeping the condition under control."
"Eighty percent of patients have very good results," says Dr. Zolty. "About 5 to 10 percent, of patients may not respond to oral therapy and may need to be placed on continuous parenteral (intravenous or subcutaneous) therapy or may even be considered for lung or combined heart and lung transplantation. Nebraska Medicine has both a heart and lung transplant program, which can evaluate the patient for a transplant.
There are a number of conditions that can cause pulmonary hypertension. In some cases, it may be idiopathic or familial. The most common cause of pulmonary hypertension is heart failure or other types of heart disease like diastolic heart disease or valvular disease. This is followed by lung diseases like emphysema, bronchitis or pulmonary fibrosis; autoimmune diseases like lupus, scleroderma and rheumatoid arthritis; cirrhosis of the liver, HIV, illegal drug use, congenital heart defects; pulmonary embolism; and diseases like sickle cell anemia and sarcoidosis.
Pulmonary hypertension is more common in men ages 50 and over but it is also seen in young women as early as their 20s or 30s due to genetics, congenital heart defects and connective tissue diseases like scleroderma.
"This is a disease that affects about 3 percent of the population, but its prevalence is increasing as we see an increase in heart disease," says Dr. Zolty.