Clinician-to-Clinician Update Clinician-to-Clinician Update

Treating Pulmonary Hypertension

November 2016

Forty years ago, a patient with pulmonary hypertension could not hope to live more than a few years beyond the date of diagnosis.1 Since then, advancements in our understanding of the disease and its classification into subtypes have enabled physicians to better recognize and treat pulmonary hypertension in its various forms.2 Although no known cures currently exist, surgical interventions and the emergence of at least 15 new drugs have helped control symptoms and greatly extended quality of life for patients.3

Pulmonary hypertension—high blood pressure in the pulmonary artery, vein, and capillaries—produces exercise intolerance, shortness of breath, fatigue, and edema. Under the condition, the right heart, accustomed to pumping blood to a low-pressure lung, eventually fails. While pulmonary hypertension in its primary form is a deadly disease, its occurrence is relatively uncommon, contributing to 7 of 100,000 deaths annually in in the United States.4 Pulmonary hypertension, however, complicates the course of many diseases and determines both functional capacity and mortality risk of a much larger group of patients commonly seen by primary care physicians as well as specialists in pediatrics, rheumatology, pulmonary medicine, hematology/oncology, anesthesia, and surgery.

Nov-2016-Heart-Care-Main-Article-ImagePAH-RV crop
— Figure 1. Pulmonary arterial hypertension is characterized by remodeling of the small resistance pulmonary arteries (left image), a condition leading to right ventricle dilatation (image on right).

Pulmonary hypertension is currently classified into 5 main groups, according to presentation and shared therapeutic approach. Class I encompasses pulmonary arterial hypertension, which is characterized by remodeling of the small resistance pulmonary arteries, leading to increased pulmonary vascular resistance and right heart failure (Figure 1). This can be a spontaneous or heritable condition. It can also be induced by drugs or toxins and is associated with connective tissue diseases, such as lupus, among other factors. The second and third subtypes are pulmonary hypertension due to left heart failure and due to lung disease, respectively. Pulmonary hypertension is also associated with chronic thromboembolic disorders, and it can arise from multiple factors stemming from hematologic, systemic, or metabolic disorders. The presence of an associated or causative condition guides diagnosis.2

Because symptoms of pulmonary hypertension can resemble those of more prevalent cardiovascular diseases, physicians are encouraged to first consider whether a more common disease might be the cause of symptoms before making a diagnosis.3 Once pulmonary hypertension is suspected, an echocardiogram must be performed to estimate pressures within the pulmonary vascular system. If measured pressures are high or if they seem to be too low in the face of symptoms and disease context, right heart catheterization is mandatory. This step is taken as echocardiograms may misclassify the pulmonary artery pressure in 25% of patients. A mean pulmonary artery pressure of 25 mmHg or above confirms a diagnosis. Catheterization allows local deployment of drugs to see which one causes vasodilation, a tactic that can also help indicate the disease subtype.3 Right heart catheterization is also needed to obtain insurance approval for coverage of drug treatments specifically targeting pulmonary hypertension.

A chronic disease, pulmonary hypertension is optimally managed through collaborative care. As one of 36 accredited Pulmonary Hypertension Care Centers in the United States5, University of Minnesota Health pulmonary circulation specialists offer comprehensive, collaborative care to patients with pulmonary hypertension and/or right heart failure. The M Health pulmonary circulation team currently follows more than 1,000 patients. Its research has resulted in the repurposing of sildenafil for the treatment of pulmonary hypertension, the first use of immunosuppressive agents to treat advanced disease, and the creation of the first implanted pulmonary artery mechanical device to treat pulmonary hypertension and associated right heart failure.


  1. Hatano S, Strasser T, editors. Primary pulmonary hypertension. Report on a WHO meeting. Geneva: World Health Organization, 1975: 7-45.
  2. Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013; 62(25 Suppl.): D34-41.
  3. Hoeper MM, Bogaard HJ, Condliffe R, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013; 62(25 Suppl.): D42-50.
  4. George MG, Schieb LJ, Ayala C, et al. Pulmonary hypertension surveillance—United States, 2001 to 2010. Chest. 2014; 146(2): 476-495.
  5. Accredited Adult Pulmonary Hypertension Programs. Pulmonary Hypertension Association. Accessed Sept. 27, 2016.

When to refer

University of Minnesota Health Heart Care specialists are dedicated to the treatment of patients with all forms of pulmonary hypertension and right heart disease. Our team includes cardiologists and nurses who specialize in pulmonary hypertension, a nurse practitioner, and specialists who can assist with scheduling and therapy acquisition. 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 24 hours a day.

To speak to the nurse care coordinator, call 612-625-2191.

Multidisciplinary, Collaborative Care

Pulmonary hypertension encompasses a complex spectrum of diseases whose treatment requires consultation across a range of medical specialties. Often patients with pulmonary hypertension have other co-morbid conditions as well. To assist patients, we routinely collaborate with cardiothoracic surgeons, pulmonologists, immunologists, rheumatologists, hepatologists, and transplant specialists. Our physicians are experts in the multi-drug approach to treating pulmonary hypertension and have some of the most extensive experience in the world in the use of atrial septostomy as a surgical approach to right heart failure, a complication of pulmonary hypertension.

The pulmonary circulation specialists at M Health Heart Care emphasize treatment planning in collaboration with patients and their physicians in the community. Our team currently follows more than 1,000 people with pulmonary hypertension, many of whom have come from around the world for treatment. Working with the primary care physician and the patient, we continually assess a patient’s progress and recalibrate medications or add treatments as necessary. Our goals are to relieve patients’ symptoms and extend their health and longevity.

To find current clinical trials available through M Health providers:

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