PH (Pulmonary Hypertension)

PH LogoPulmonary hypertension is a relatively rare heart and lung condition that is classified by an increased mean pulmonary arterial pressure, indicating that the arteries leading to the lungs have become narrowed or have increased in vascular resistance. This constriction leads to a reduced blood flow in the lungs, which increases the blood pressure in the pulmonary artery, veins, and/or capillaries. Most patients who present to the clinic have severe dyspnea, or difficulty breathing, because of the lack of blood flow to their lungs, which makes oxygen diffusion more difficult, and decreases the amount of oxygen that is transported to the rest of the body. Fatigue, chest pain, edema, and dizziness are also common symptoms of this condition.

There are five different groups of pulmonary hypertension (PH), and patients are classified to a certain World Health Organization (WHO) group based on the etiology of their disease. WHO group 1 patients have pulmonary arterial hypertension, which is often subcategorized into heritable, idiopathic, drug/toxin induced, associated with connective tissue disease, etc. WHO group 2 patients have elevated heart pressures because of systolic/diastolic dysfunction (in the heart muscle) or heart valve disease. WHO group 3 is characterized by lung diseases such as interstitial lung disease, COPD, or obstructive sleep apnea, to name a few. Chronic thromboembolic pulmonary hypertension (CTEPH) dominates WHO group 4. WHO group 5 consists of pulmonary hypertension secondary to multifactorial mechanisms, such as systemic, metabolic, or hematological disorders. Baseline diagnostic testing enables physicians to classify the patients into the respective WHO groups.

PH LogoDepending on the type and severity of pulmonary hypertension, patients may or may not be eligible for various treatments to manage their disease. Vasodilators are the most common type of therapy for WHO group 1 patients and managing the effects of underlying health conditions is standard for WHO groups 2 and 3. Those with group 4 CTEPH have the only form of pulmonary hypertension that is curable, and if eligible can undergo a pulmonary thromboendarterectomy surgery. Certain classifications of PH patients can undergo a lung transplantation to reduce symptoms.

A database has been established at MCORRP to track treatment and outcomes of PH patients admitted to Michigan Medicine. MCORRP staff, working closely with PH faculty and students, plan to analyze outcomes to determine best practices to achieve optimal outcomes in these patients..