In reality, it was far more serious. Two days after his oldest son's wedding, Matthies collapsed at home and was taken to Cass County Memorial Hospital in Atlantic, Iowa, for observation and treatment.

Matthies' family physician, Richard F. Coatney, DO, subsequently referred him to Iowa Methodist Medical Center in Des Moines, where cardiologist Frank Haugland, MD, diagnosed primary pulmonary hypertension (PPH). PPH is a potentially lethal lung condition characterized by sustained elevations of pulmonary artery pressure for no apparent cause.

"Shocked would not be strong enough to tell you my reaction," Matthies says. "We had a very scary couple of weeks."

While heart-lung, single lung, or double-lung transplants have until recently been the only treatment options for patients with severe PPH, patients can now be effectively treated with a relatively new vasodilator medication called prostacyclin (also known as epoprostenal). Prostacyclin therapy is so complex it requires special training to administer, so only a relatively small number of medical centers nationwide offer the treatment.

"Dr. Haugland referred me to the University of Iowa Hospitals and Clinics because they have the only prostacyclin program in the state," Matthies says.

Cardiologist Todd Noreuil, MD, directs the UI Hospitals and Clinics's Primary Pulmonary Hypertension Clinic, with support from cardiologist Ron Oren, MD The clinic specializes in treating patients with primary pulmonary hypertension. The PPH Clinic provides a long-term, multidisciplin-ary team (including physicians, nurses, pharmacists, and dietitians) approach for complete evaluation and treatment.

"Primary pulmonary hypertension was universally fatal until this medication became available a few years ago," Dr. Noreuil says. "The condition itself is pretty rare; about 500 new cases are diagnosed in the United States each year. My training in prostacyclin therapy occurred while I served a fellowship at the University of Alabama at Birmingham."

Dr. Noreuil joined the UI faculty on July 1, 1996, and brought with him the expertise he acquired at Alabama. "Prostacyclin has a half-life of only two to three minutes, so it must be administered continuously through a small, portable, battery-operated infusion pump worn on the belt," Dr. Noreuil says. "The pump pushes the medication into the body through a Hickman® catheter placed in the internal jugular vein."

Matthies required only five days of hospitalization to get started on the medication. He came in on a Monday for evaluation (doctors must carefully assess how much medication each patient should initially receive, a process called "dose ranging"). His catheter was placed on Tuesday, while Wednesday and Thursday were devoted to patient education (patients must learn to mix prostacyclin once a day and insert it into the pump). Friday, he went home.

"I was back at work the following Monday," says Matthies, who sells and merchandises feed and grain at the Farmers Cooperative in Anita, Iowa, west of Des Moines. "I haven't missed a day since. I have a few side-effects, including a little jaw pain when I eat, but otherwise I've been fine."

Dr. Noreuil says Matthies' experience differs dramatically from patients who developed PPH only a few years ago. "PPH has traditionally been a debilitating disease that results in death within two or three years," he says.

"Prostacyclin has changed all that by improving functional status and lengthening the patient's lifespan. In my experience, some patients responded so favorably to prostacyclin that they would be taken off the transplant waiting list."

While the cause of PPH is unknown, its effects are well-documented. The pulmonary arteries become thickened and scarred, and constrict. Little blood clots also form in the distal arteries. This combination of effects causes the patient's mean pulmonary pressure (normally about 15) to soar to 40 or above.

While PPH often affects women between the ages of 20 and 40, it can potentially affect anyone, from children to the elderly.

"The encouraging thing is that we now have an effective medical therapy for it, and we hope to improve our ability to treat the condition as more is learned about it," Dr. Noreuil says. "We'll be doing some research here in collaboration with Drs. Robert Weiss (UI Hospitals and Clinics cardiologist) and William Stanford (a UI Hospitals and Clinics radiologist) using cine CT for data collection on how it allows the heart to work better and longer."

Dr. Noreuil adds that the UI Hospitals and Clinics provides treatment in collaboration with the patient's family physician. "We need patients to return to the UI Hospitals and Clinics monthly so we can modify their medication levels," he says, "but all other care is locally managed. This includes warfarin (a blood thinner), which the patient must take in conjunction with PPH therapy."

More information about the Primary Pulmonary Hypertension Clinic is available from Dr. Noreuil at (319) 356-7173 or Nurse Coordinator Lori Panther at (319) 356-1028.