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New Treatment for Pulmonary Arterial Hypertension

Pulmonary arterial hypertension (PAH) is perhaps the most dangerous form of pulmonary hypertension. It can be idiopathic, heritable, or associated with various other conditions such as connective tissue disorders. Characterized by narrowing or blockage of the small pulmonary arteries, the disorder produces increased pulmonary vascular resistance, leading to right-heart failure and eventually death in approximately 50% of patients within 5 years of diagnosis.1 The first line of treatment is medical therapy. The goals of medical therapy are halting progression of disease and maintaining a lower-risk health status for the patient. Patients with advanced PAH may eventually require double lung or heart-lung transplant.2

New therapies and treatment approaches, however, are improving the outlook for these patients. Upfront combination therapies for PAH3 have been shown to significantly reduce risk of death and disability by 50% when compared to stepwise treatment regimens that call for the addition of single drugs over time.4

PAH can present challenges in diagnosis and treatment. Symptoms of PAH include exercise intolerance, fatigue, shortness of breath, angina, and edema. PAH is suspected with the presence of right-heart enlargement on a transthoracic echocardiogram and can be confirmed by a cardiologist when patients meet specific hemodynamic criteria and once thromboembolic diseases and diseases of the lung and left heart are ruled out. PAH is rare, occurring in up to 26 in 1 million people, yet the high death rate has spurred a number of clinical trials leading to the identification of 14 different medical treatments for patients.1

Pulmonary arterial hypertension is
characterized by remodeling of the
small resistance pulmonary
arteries (left), a condition leading
to right ventricle dilatation (right).

Treatment guidelines have indicated the sequenced addition of single drug therapies over time with combination therapies being employed for patients with advanced PAH.4 The central therapies for PAH target 1 of 3 molecular pathways known to contribute to the pathology of PAH, including the nitric oxide, endothelin, and prostacyclin pathways.

New data, however, suggest that specific drug therapies given in combination as initial treatment produce outcomes superior to those obtained by the stepwise addition of monotherapies. In the AMBITION trial, patients given an upfront combination of ambrisentan and tadalafil could walk farther during a standard exercise test than could patients given each therapy individually.5 Patients in the trial also had a lower chance of death, hospitalization, and progression of PAH.5 (See figure 1 from reference 5.)The 2 drugs target the endothelin and nitric oxide pathways, respectively.

In patients with advanced PAH, such drug combinations may delay the need for lung transplant.3,2

University of Minnesota Health physicians are among the researchers leading investigations into new therapies and treatment approaches to PAH. (See Program Updates.) They also participate in a clinical program focused exclusively on the treatment of pulmonary hypertension and its different types, including PAH.


  1. Thenappan T, Ormiston ML, Ryan JJ, Archer, SL. Pulmonary arterial hypertension: pathogenesis and clinical management. BMJ. 2018;360:j5492.
  2. Keogh AM, Mayer E, Benza RL, et al. Interventional and surgical modalities of treatment in pulmonary hypertension. J Am Coll Cardiol. 2009;54(1 Suppl):S67-77.
  3. Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2016;37:67-119.
  4. Hill NS, Cawley MJ, Heggen-Peay CL. New therapeutic paradigms and guidelines in the management of pulmonary arterial hypertension. J Manag Care Spec Pharm. 2016;22(3 Suppl A):S3-21.
  5. Galiè N, Barberà JA, Frost AE, et al. Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension. N Engl J Med. 2015;373:834-44.

When to refer

University of Minnesota Health Heart Care cardiologists offer coordinated services focused solely on patients with pulmonary hypertension. Our pulmonary hypertension specialists are skilled in the diagnosis and treatment of the condition and its several subtypes. The specialist team works alongside primary care providers and other specialists in assisting patients and is dedicated to facilitating the optimal introduction of multi-drug therapy, rehabilitation, surgery, or mechanical devices for the long-term management of these diseases. A provider dedicated to pulmonary vascular disease services is available to physicians and patients by phone. To speak to the nurse care coordinator, call 612-625-2191.

Our cardiologists are experienced in the use of the latest combination therapies for the treatment of pulmonary hypertension. Patients may also be eligible to participate in one of several clinical trials evaluating treatments for the condition.

Multidisciplinary, Collaborative Care

Pulmonary hypertension is a complex syndrome that often co-occurs with other health conditions, including heart, lung, or liver disease or connective tissue disorders. Our clinical teams may include cardiologists, cardiac surgeons, transplant surgeons, rheumatologists, pulmonologists, hepatologists, and cardiac nurses to cover all aspects of patient care. Our goals are to relieve each patient’s symptoms and improve quality of life by providing multidisciplinary, collaborative care in close partnership with the referring provider. In cases where organ transplant may be necessary, cardiologists refer their patients to transplant surgeons for evaluation. The University of Minnesota Health lung transplant program is one of the largest in the nation, with years of experience in setting the standard for transplant care, including performing the first lung transplant in the state of Minnesota.

To find current clinical trials available through University of Minnesota Health providers, visit

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