Cutting-Edge Care Brings Specialists Together

Sometimes it is not technology or a new medication that makes the greatest difference in medical care but something far simpler. A new approach to treating pulmonary hypertension — a particularly dangerous type of high blood pressure that affects the arteries leading from the heart to the lungs — puts top specialists together as a “team” to work collaboratively with patients, with the goal of delivering a far better quality of care.

While specialists have always discussed their patients with one another, this approach takes the concept much further, I learned from James Calvin, MD, director of the section of cardiology at Rush University Medical Center and a cardiologist at the new Rush Pulmonary Hypertension Clinic in Chicago, which offers this type of multidisciplinary care. Dr. Calvin told me that teamwork is built into every aspect of a patient’s care to comprehensively address the many different causes of this complex disease. He said that the doctors’ combined specialized training and clinical experience leads to more accurate diagnoses and more successful treatment.

Patients can even schedule appointments with several different specialists on the same day in the same office, and the doctors can conveniently consult with one another to, as Dr. Calvin puts it, “put our heads together to come up with a solution.”

Hard to Diagnose

Pulmonary hypertension is not uncommon, and doctors are well aware of its signs and causes, but it can nonetheless be tricky to diagnose because its symptoms mimic those of so many other heart and lung diseases.

This condition develops in people whose hearts have had to pump especially hard to push blood through increasingly stiff and narrow arteries. It has many causes, including heart, lung and liver problems… living at altitudes higher than 8,000 feet… and heredity in some cases can contribute, too. When the body must expend intense effort to bring blood through the lungs to the left side of the heart the eventual result is weakening of the heart muscle on the right side leading to heart failure or other potentially fatal complications. Shortness of breath is usually the first warning sign of pulmonary hypertension, but other common symptoms include fatigue… dizziness… fainting… chest pain… leg and ankle swelling… palpitations (fast heartbeat)… and bluish lips and skin.

How pulmonary hypertension is diagnosed: An echocardiogram, a form of ultrasound that enables your doctor to get a good look at your heart and pulmonary arteries, is usually the first step toward diagnosis. If “problems” are found, the next step is right heart catheterization,to measure how well blood moves through and to look for blockages or other abnormalities. Doctors often order other tests to obtain additional information on the extent of the patient’s problem, possibly a chest X-ray, CT scan, MRI and/or pulmonary function assessment (a measure of how well the lungs take in and release air).

Life with Pulmonary Hypertension

While treatment is complex, inroads have been made. A variety of medications may be used, and together, they can allow many patients to continue to live their lives fairly normally. These may include:

  • Vasodilators. These drugs — including epoprostenol (Flolan) and iloprost (Ventavis) — widen blood vessels and reduce scarring in them. Patients used to have to come to the hospital or a doctor’s office for intravenous (IV) administration of this therapy, but now new drugs can be taken orally by patients at home.
  • Endothelin Receptor Agonists. ERAs — such as bosentan (Tracleer) or ambrisentan (Letairis) — block endothelin, a substance in blood vessel walls that causes them to narrow.
  • Calcium channel blockers. Though these were once the first line of treatment, they work only 5% of the time.
  • Other drugs. Your physician may also prescribe an anticoagulant such as warfarin (Coumadin)… a diuretic to prevent fluid accumulation… sildenafil (Revatio) to relax smooth muscle in pulmonary arteries… digoxin to help the heart pump blood… and/or oxygen to help you breathe.
  • Surgical treatments. If drugs alone cannot control your condition, options include open-heart surgery to create an opening between the right and left chambers of the heart and relieve pressure on the right side… and, in very severe cases, a lung or heart-lung transplant (in patients with a diseased lung).

Put Yourself on the Team

When you have pulmonary hypertension, it’s vital to control any other underlying conditions (for example, religiously take your blood pressure medications).

Dr. Calvin also says that for the best quality of life, keep your focus on making sensible, healthful daily lifestyle choices…

  • Get lots of rest. Listen to your body. When you are tired, take a nap.
  • Follow a healthy diet. In particular, avoid salt — which can increase swelling in your legs. Eat more whole foods and fewer salt-laden processed products.
  • Stay active. Ask your doctor what level of exercise is safe for you.
  • Don’t smoke. Smoking severely damages your arteries, heart and lungs.
  • Avoid stress. Improve your quality of life by meditating or practicing yoga or tai chi.
  • Eliminate saunas and hot baths. These can cause your blood pressure to drop dangerously low.
  • Avoid high altitudes (including air travel). Low oxygen levels worsen symptoms such as shortness of breath.