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School of Medicine Pulmonary and Critical Care
Scott Hall

Pulmonary Hypertension program

Pulmonary hypertension (PH) is a chronic and progressive condition that, if left untreated, can lead to right-sided heart failure, significant complications, reduced quality of life, and shortened survival. Although relatively rare, PH can be familial, idiopathic (without an identifiable cause), or associated with other underlying medical conditions.

In recent years, significant advancements in the understanding and treatment of PH have led to the development of new therapies that improve symptoms, enhance quality of life, and extend survival. (For more information, please refer to our "Patient Education" resources.)

At the Wayne State University Pulmonary Hypertension Program, we are dedicated to delivering state-of-the-art, patient-centered care. Our program is led by board-certified physicians in Pulmonary and Critical Care Medicine with specialized expertise in pulmonary hypertension. They are supported by a multidisciplinary team of skilled healthcare providers.

We offer a comprehensive range of services, including:

  • Detailed clinical evaluations
  • On-site diagnostic testing (echocardiography, right heart catheterization, pulmonary function testing, and more)
  • Individualized treatment plans and ongoing follow-up
  • Assistance with medication approvals and access through patient assistance programs
  • Opportunities to participate in clinical research and clinical trials

Our team works in close collaboration with specialists in cardiology, rheumatology, infectious disease, hematology, sleep medicine, and other fields to ensure coordinated, holistic care for every patient.

Understanding pulmonary hypertension

Pulmonary Hypertension (PH) refers to a group of conditions characterized by elevated pressure in the blood vessels of the lungs. Over time, this increased pressure puts strain on the right side of the heart, which may eventually lead to right-sided heart failure. It is different from regular (systemic) high blood pressure, which affects the left side.

WHO classification of pulmonary hypertension

Group 1: Pulmonary arterial hypertension (PAH)

  • Causes: Idiopathic, heritable, connective tissue diseases, HIV, congenital heart disease, portal hypertension, drug/toxin exposure
  • Treated with targeted PAH therapies

Group 2: PH due to left heart disease

  • Causes: Left-sided heart failure, valvular disease
  • Manage underlying heart condition

Group 3: PH due to lung diseases/hypoxia

  • Causes: COPD, interstitial lung disease, sleep apnea
  • Treat lung disease, use oxygen if needed

Group 4: PH Due to pulmonary artery obstruction

  • Cause: Chronic thromboembolic pulmonary hypertension (CTEPH)
  • Treat with anticoagulation, surgery, or angioplasty

Group 5: PH with multifactorial or unclear mechanisms

  • Causes: Sarcoidosis, sickle cell disease, kidney disease, metabolic disorders
  • Treatment individualized based on cause

Symptoms

The symptoms of PH are non-specific, which often leads to delays in diagnosis. Common symptoms include:

  • Shortness of breath, especially with activity
  • Fatigue or reduced exercise tolerance
  • Chest discomfort
  • Swelling in the feet or abdomen (due to fluid retention)
  • Lightheadedness or fainting in more advanced cases

Diagnosis

Timely referral to a PH specialist is crucial. Early diagnosis and treatment can significantly improve outcomes.

A comprehensive work-up typically includes:

  • Echocardiogram (initial screening test)
  • Right heart catheterization (gold standard to confirm diagnosis and measure pressures)
  • Pulmonary function testing
  • Chest X-ray
  • Electrocardiogram (EKG)
  • CT scan of the chest or V/Q scan (to evaluate for lung disease or chronic blood clots)
  • Six-minute walk test (to assess functional capacity and need for oxygen)
  • Blood tests to evaluate for contributing conditions and monitor response to therapy

The right heart catheterization is essential to confirm PH, determine its severity, and assess responsiveness to certain medications. It involves placing a catheter into the heart to directly measure blood pressure in the pulmonary arteries.

Treatment

Over the past decade, significant advances have been made in the treatment of PH, particularly Pulmonary Arterial Hypertension (PAH). There are currently Five classes of FDA-approved medications for PAH, aimed at improving symptoms, quality of life, and survival.

Approved treatments include:

  1. Calcium channel blockers (only for select patients who respond during catheterization)
  2. Prostacyclin analogs
    • Epoprostenol (IV via continuous infusion)
    • Treprostinil (IV, subcutaneous, inhaled, or oral)
    • Iloprost (inhaled)
  3. Endothelin receptor antagonists
    • Bosentan (oral)
    • Ambrisentan (oral)
    • Macitentan (oral)
  4. Phosphodiesterase-5 inhibitors
    • Sildenafil (oral)
    • Tadalafil (oral)
  5. Activin Signaling inhibitor
    • Sotatercept (a novel subcutaneous therapy given every 3 weeks)

Supportive therapies may include:

  • Diuretics to manage fluid overload
  • Oxygen therapy for those with low oxygen levels
  • Anticoagulation in specific types of PH
  • Pulmonary Rehab

In advanced or treatment-refractory cases, referral for lung transplantation may be considered.

Research

We have successfully completed the following clinical trials:

    • ATHENA-1 : A Randomized, Multicenter Study of Ambrisentan and Sildenafil Combination Therapy in Subjects With Pulmonary Arterial Hypertension Who Have Demonstrated a Sub-Optimal Response to Sildenafil
      Principal Investigator: Ghulam Saydain, M.D.
    • COMPASS-2: Effects of Combination of Bosentan and Sildenafil Versus Sildenafil Monotherapy on Morbidity and Mortality in Symptomatic Patients With Pulmonary Arterial Hypertension - A Multicenter, Double-Blind, Randomized, Placebo-Controlled, Parallel Group, Prospective, Event Driven Phase IV Study.
      Principal Investigator: Ghulam Saydain, M.D.
    • REVEAL Registry:Registry to Evaluate Early And Long-Term PAH Disease Management.
      Principal Investigator: Ghulam Saydain, M.D.
    • PAH QuERI: Pulmonary Arterial Hypertension Quality Enhancement Research Initiative.
      Principal Investigator: Ghulam Saydain, M.D.
    • SERAPHIN: A Multicenter, Double-Blind, Randomized, Placebo-Controlled, Parallel Group, Event-Driven, Phase III Study to Assess the Effects of ACT-064992 on Morbidity and Mortality in Patients With Symptomatic Pulmonary Arterial Hypertension.
      Principal Investigator: Ghulam Saydain, M.D.
    • PHIRST-2: Open-label, Extension Study to Evaluate the Long-term Safety and Efficacy of the Phosphodiesterase Type 5 (PDE5) Inhibitor Tadalafil in the Treatment of Patients with Pulmonary Arterial Hypertension.
      Principal Investigator: Ghulam Saydain, M.D./ Kamal Mubarak, M.D.
    • ARIES-3: Phase 3, Long-Term, Open-Label, Multicenter Safety and Efficacy Study of Ambrisentan in Subjects With Pulmonary Hypertension.
      Principal Investigator: Ghulam Saydain, M.D./ Kamal Mubarak, M.D.
    • STRIDE-III: Long-Term, Open-Label Study to Evaluate the Safety of Sitaxsentan Sodium Treatment in Patients with Pulmonary Arterial Hypertension.
      Principal Investigator: Kamal Mubarak, M.D.
    • ASSET-1 & ASSET-2: Randomized, Placebo-controlled, Double-blind, Multi-center, Parallel Group Study To Assess the Efficacy, Safety and Tolerability of Bosentan in Patients with Symptomatic Pulmonary Arterial Hypertension Associated with Sickle Cell Disease.
      Principal Investigator: Kamal Mubarak, M.D.
    • ASSET-3: Long-Term, Open-Label, Multi-center, Extension Study of Bosentan in Patients with Pulmonary Hypertension Associated with Sickle Cell Disease Completing a Double-blind ASSET study.
      Principal Investigator: Kamal Mubarak, M.D.
    • PHIRST-1: Randomized, Double-Blind, Placebo-Controlled Phase 3 Study of the Phosphodiesterase Type 5 (PDE5) Inhibitor Tadalafil in the Treatment of Patients With Pulmonary Arterial Hypertension.
      Principal Investigator: Kamal Mubarak, M.D.

Contact and referral information:

Ghulam Saydain, M.D., FCCP - Pulmonary & Critical Care and Sleep Medicine

Shefali Godara, M.D. - Pulmonary & Critical Care and Sleep Medicine

For appointments:

Pulmonary Hypertension Clinic
400 Mack Avenue,
2nd Floor Detroit, Michigan, 48201

Main appointment number 877-929-6342 (877-WAYNE-HC)

For your appointment, please be sure to bring any old medical records, list of medications you are taking, any x-ray films, and appropriate insurance information including referral.

When you arrive in the clinic, you may first be seen by a Pulmonary Fellow before being seen by a staff physician.

If you are on oxygen and have only a limited supply, please ask the receptionist to place you on oxygen available in the clinic so your supply does not run out while you are waiting to be seen.

For additional information regarding pulmonary hypertension, can visit:

https://phassociation.org/