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The Latest in Diagnosis and Care for Pulmonary Arterial Hypertension

Pulmonary arterial hypertension can begin subtly, yet end fatally. The condition is treatable, but can be difficult to recognize, often causing irreversible damage before it is diagnosed.

Specialists of The University of Kansas Health System are here to help. We offer a Pulmonary Hypertension Association-accredited Center of Comprehensive care, one of fewer than 40 recognized programs in the nation. The distinction identifies highly organized, full-time pulmonary hypertension centers that proficiently evaluate patients based on published, evidence-based guidelines and deliver gold-standard treatment with all the FDA-approved therapies. In addition, such centers make important contributions to research and education in the field.

Defining the disease

Pulmonary arterial hypertension (PAH) is a condition in which blood pressure in the lungs is high, requiring the right ventricle of the heart to pump much harder than normal. This weakens the muscle over time until right-heart failure becomes a risk if the condition remains untreated.

The disease affects individuals regardless of gender, age or ethnicity. There are a number of risk factors that may increase the likelihood of PAH. These include:

  • Family history, particularly if at least 2 members of a family have PAH
  • Drug use, particularly methamphetamines and the diet drug fen-phen
  • Connective tissue disease, such as scleroderma, lupus and HIV
  • Lung scarring, such as from emphysema, cystic fibrosis or sleep apnea

Early symptoms of PAH include shortness of breath, fatigue and chest pains. As the disease progresses, patients may experience lightheadedness, swelling in the hands, legs or belly, or a blue tinge to the lips or fingers.

Caring for patients with PAH

Pulmonary arterial hypertension is treatable, but the treatment options are greater in number and less invasive in nature when the disease is identified early. However, as symptoms can be mild and easily associated with less dangerous conditions, and the disease itself is rare, affecting only about 30,000 patients in the United States, a PAH diagnosis can come too late to achieve a positive outcome. Patients frequently under-report symptoms they believe to be harmless, and community physicians less familiar with PAH cases may – understandably – treat patients for more commonly seen conditions that exhibit similar symptoms.

Our organization offers the highest levels of care, broadest scope of services and deepest range of experience for patients with pulmonary arterial hypertension. We are pleased to support our colleagues in general medicine and offer the following suggestions for identifying PAH early enough to provide the best options for patients. Consider PAH if:

  • Your patient has an underlying disease known to be associated with PAH, such as connective tissue disease, stimulant use, etc.
  • Your patient's overall condition appears to be declining rapidly
  • Initially prescribed therapy isn't working
  • Your patient becomes very sick, such as requiring a respiratory ventilator
  • If the results of a screening echocardiogram are concerning – but don't begin PAH treatment on an echocardiogram alone

If your patient exhibits any of the above, we would urge you to consider a referral to specialty care. As a care team, we have more opportunities to together help these patients when a conclusive diagnosis of PAH is determined early. Too often, patients in late-stage PAH at the time of referral pass away within 24-48 hours. We are here to support local physicians in getting more patients more care, earlier, to avoid this.

The leading approach

When we see a new patient suspected to have PAH, we will perform several diagnostic tests – blood tests, chest X-ray, echocardiogram – or review the results of tests performed before referral. But one definitive test is crucial to making an accurate diagnosis, and that is right-heart catheterization. This entails navigating a flexible catheter through the groin to the right ventricle to measure the blood pressure in the pulmonary arteries. This is integral to achieving a definitive diagnosis, and our experts will perform the test with nuances unique to our field. As such, we will perform the right-heart catheterization here. Primary care providers may want to avoid ordering these tests elsewhere to spare the duplicative cost and stress to the patient.

Once PAH is confirmed, we'll determine the most appropriate treatment by evaluating:

  • How sick is the patient?
  • How rapid is the patient's decline?
  • How limited is the patient's functionality?

Oral, inhaled, intravenous and subcutaneous medication therapies are among the treatment options. These are complex therapies that affect each patient uniquely depending upon the nuances of each person's disease, so experience is critical in determining personalized treatment plans.

Shaping the future

Our pulmonologists and critical care specialists serve both The University of Kansas Health System and the University of Kansas Medical Center. In addition to patient care, they are deeply immersed in teaching the next generation of physicians and researching new treatments and therapies that offer the potential to change the future of medicine. Patients who seek PAH care here benefit from the combined expertise of a highly experienced team as well as access to novel clinical trials and treatments not available elsewhere.

We take a holistic and multidisciplinary approach to patient care, addressing patients' medical, physical, emotional and psychosocial needs. This includes collaboration among highly trained specialists in pulmonology, cardiology, rheumatology, critical and palliative care. Our team is strengthened by excellent pharmacists and nurses experienced in PAH care as well as financial counselors available to assist patients in navigating insurance coverage for PAH medications that are, unfortunately, expensive. Our team brings together the many skills PAH patients need to control symptoms and achieve the best possible outcome in managing this complex condition.

To consult with a physician or refer a patient, call 913-588-5862.

Dr. Williamson's clinical practice focuses exclusively on PAH and other diseases of the pulmonary vascular system.

Timothy Williamson,

MD, vice president of quality and safety, pulmonary care specialist

We offer a variety of appointment types. Learn more or call 913-588-1227 to schedule now.

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