Pulmonary Hypertension and Pulmonary Vascular Disease

patient undergoing tests at Pulmonary Hypertension Clinic

The Johns Hopkins Pulmonary Hypertension Program (JHPHP) was established in 1999 and is among the largest pulmonary hypertension (PH) programs in the country. The JHPHP is formally accredited as a Pulmonary Hypertension Comprehensive Care Center (CCC) by the Pulmonary Hypertension Association. The program is made up of a multi-disciplinary team of pulmonary vascular disease experts with advanced training in pulmonary, rheumatology, cardiology, radiology and anesthesiology along with dedicated nurses and support staff. We evaluate and treat all forms of pulmonary hypertension, including idiopathic pulmonary arterial hypertension (IPAH) and diseases associated with pulmonary arterial hypertension (PAH) such as connective tissue diseases, liver disease, congenital heart disease, and hereditary forms of the PAH. In addition, we treat conditions such as pulmonary emboli (PE) and unexplained shortness of breath. We have specific expertise in the medical management of PH, including initiation and management of advanced therapies for PAH (inhaled, intravenous and subcutaneous infusions of prostacyclin analogues). The JHPHP evaluates patients at the Johns Hopkins Outpatient Center (JHOC) at the East Baltimore campus of the Johns Hopkins Hospital.

The JHPHP sponsors a PH Patient support group. The support group meets quarterly and provides an opportunity for PH patients to interact and participate in educational lectures and discussions about PH treatment, research and experiences. The support group is open to any patients, caregivers or families impacted by PH, regardless of whether they get their PH care at Johns Hopkins. For the schedule and more information, please contact Laura Camponeschi at 410-614-6311 or [email protected].

Scheduling an Appointment

Prior to scheduling a new patient evaluation, we ask that all pertinent medical records be sent to our office for review. This review helps to identify testing that may need to be scheduled at the same time as the new patient appointment. Medical records can be faxed to 410-614-7451.

Studies Requested by Our Providers

The standard evaluation of Pulmonary Hypertension includes the following tests and procedures. These tests are not required to be seen as a new patient at Johns Hopkins.

  1. Echocardiogram: An ultrasound of your heart that shows the size, structure, and function of your heart. This test can often, but not always, estimate the pressures in your heart and lungs. These can be done either at Hopkins or outside of Hopkins. Occasionally, specialized views or maneuvers are requested that are more easily obtained at Hopkins in JHOC.
  2. Cardiac Magnetic Resonance Imaging (cardiac MRI): An imaging study of your heart that gives a more detailed image of your heart that can better quantify heart function than echo. This test is usually performed at Hopkins with a specific protocol for PH.
  3. CT of the Chest: An imaging study of your lungs. This can be performed with or without intravenous contrast to help visualize the pulmonary blood vessels and look for blockages. These can be done either at Hopkins or outside of Hopkins.
  4. Ventilation/Perfusion Scan (VQ scan): A radiographic study to look for blood clots in the lung. These can be done either at Hopkins or outside of Hopkins, however, many times these tests are done at Hopkins.
  5. Pulmonary Function Tests (PFTS): Breathing tests that quantify your lung function.
  6. Six-Minute Walk Test: A submaximal exercise test that measures the distance you can walk in six minutes while checking your oxygen levels, heart rate, and degree of breathlessness. We prefer that this test be performed at Hopkins in JHOC.
  7. Blood Work: Basic blood work looking at blood counts, kidney and liver function, and screening tests for viral and autoimmune diseases.
  8. PH cannot be properly diagnosed without a RHC. Most commonly, we request that this test be performed at Hopkins. This test is typically done in our catheterization laboratory in the Zayed Building on the East Baltimore campus of Johns Hopkins Hospital. This is an outpatient procedure that takes, on average, about 2 hours.

In most instances, patients should be seen in the JHPHP clinic prior to undergoing RHC, particularly if all of the tests outlined above have not been completed.

Our Physicians and Experts

Pulmonary and Critical Care Medicine

  • Paul M Hassoun, M.D.

    • Director, Pulmonary Hypertension Program
    • Professor of Medicine

    Expertise: Pulmonary and Critical Care Medicine, Pulmonary Hypertension

  • Todd Matthew Kolb, M.D., Ph.D.

    • Assistant Professor of Medicine

    Expertise: Pulmonary and Critical Care Medicine, Pulmonary Hypertension

  • Stephen Carl Mathai, M.D., M.H.S.

    • Director, Inpatient Pulmonary Service, Johns Hopkins Hospital
    • Co-Director, Ann Dana Kusch Multidisciplinary Program for Interstitial Lung Disease and Pulmonary Hypertension Research
    • Associate Professor of Medicine

    Expertise: Pulmonary and Critical Care Medicine

Cardiology

  • Rani Khalil Hasan, M.D., M.H.S.

    • Director, Interventional Cardiology Fellowship Program
    • Regional Medical Director, Structural Heart Disease Program at Suburban Hospital
    • Assistant Professor of Medicine

    Expertise: Adult Congenital Heart Disease, Aortic Stenosis, Aortic Valve Repair, Atrial Septal Defects (ASD), Cardiac Catheterization

  • Steven Hsu, M.D.

    • Medical Director, Mechanical Circulatory Support
    • Assistant Professor of Medicine

    Expertise: Cardiac Transplant, Cardiomyopathy, Heart Failure, Heart Transplant, Internal Medicine

  • Robert George Weiss, M.D.

    • Clarence Doodeman Professor of Cardiology
    • Professor of Medicine
    • Joint Appointment in Radiology and Radiological Science

    Expertise: Cardiomyopathy, Cardiovascular Disease, Coronary Endothelial Function, Heart Disease, Heart Failure

  • Ilan Shor Wittstein, M.D.

    • Director, Advanced Heart Failure Fellowship
    • Assistant Professor of Medicine

    Expertise: Acute Coronary Care, Acute Coronary Syndrome (ACS), Acute Myocardial Infarction (AMI), Cardiac Sarcoidosis, Cardiac Transplant

Rheumatology

  • Laura Kathleen Hummers, M.D.

    • Clinical Director, Division of Rheumatology
    • Co-Director, Johns Hopkins Scleroderma Center
    • Associate Professor of Medicine

    Expertise: Rheumatology, Scleroderma

  • Christopher Anthony Mecoli, M.D., M.H.S.

    • Assistant Professor of Medicine

    Expertise: Rheumatology

  • Julie Jisun Paik, M.D., M.H.S.

    • Director of Clinical Trials at the Johns Hopkins Myositis Center
    • Co-Director, Johns Hopkins Myositis Center
    • Associate Professor of Medicine

    Expertise: Myositis, Rheumatology, Scleroderma

  • Ami Aalok Shah, M.D.

    • Director, Division of Rheumatology, Department of Medicine
    • Co-Director, Johns Hopkins Scleroderma Center
    • Deputy Director, Rheumatology Precision Medicine Centers of Excellence Clinical Programs
    • Associate Professor of Medicine

    Expertise: Rheumatology, Scleroderma

  • Fredrick M Wigley, M.D.

    • Director, The Johns Hopkins Scleroderma Center
    • Martha McCrory Professor of Medicine
    • Professor of Medicine

    Expertise: Rheumatology, Scleroderma

Cardiac Anesthesia

Jochen Steppan, M.D.

  • Director of Perioperative Medicine, High Risk Cardiovascular Disease
  • Associate Professor of Anesthesiology and Critical Care Medicine

Expertise: Adult Congenital Heart Disease, Anesthesiology, Cardiothoracic Anesthesiology, Pediatric Cardiac Anesthesiology, Pulmonary Hypertension

Contact Information and Locations


Directions to the Pulmonary Hypertension Clinic at the Johns Hopkins Outpatient Center

Division of Pulmonary and Critical Care Medicine
Seventh Floor
601 N. Caroline Street
Baltimore, MD 21287

Phone: 410-614-6311
Fax: 410-614-7451

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