Pulmonary Hypertension

Pulmonary hypertension is a rare and serious disease in which the pressure in the pulmonary (lung) circulation is above normal levels. Left untreated, pulmonary hypertension can cause permanent damage to the lungs, resulting in life-threatening right heart failure and death.

Pulmonary Hypertension Association

The Pulmonary Hypertension Center is now accredited by the Pulmonary Hypertension Association as a designated Tier 1 Pulmonary Hypertension Comprehensive Care Center. Learn more here.

Pulmonary hypertension is currently classified into five main groups (1-5) with most treatments directed toward patients with group 1 pulmonary arterial hypertension. When there is no known cause, it is called idiopathic (primary) pulmonary arterial hypertension and pulmonary hypertension that occurs as a result of other disorders is called secondary pulmonary hypertension. One example of a common type of secondary pulmonary arterial hypertension, caused by abnormal development and defects in the heart and great vessels, is called Eisenmenger's syndrome.

At the Pulmonary Hypertension Comprehensive Care Center of NYP/Columbia, we treat all forms of pulmonary hypertension and associated right heart failure, including idiopathic pulmonary arterial hypertension and secondary forms of pulmonary hypertension associated with a variety of conditions:

  • congenital heart disease including Eisenmenger’s syndrome
  • Lupus, scleroderma and other connective tissue diseases
  • chronic thromboembolic pulmonary hypertension — CTEPH (including surgery for clot removal – pulmonary thromboendarterectomy)
  • chronic lung disease and advanced lung failure including mechanical bridging with ECMO when indicated
  • liver disease
  • HIV disease
  • genetic (familial/inherited) pulmonary hypertension
  • HHT (hereditary hemorrhagic telangiectasia)
  • pulmonary hypertension of the newborn
  • sickle cell disease
  • toxin (methamphetamines) exposure

While there have been many advances in the treatment of pulmonary arterial hypertension over the past two decades, there is still no cure aside from lung transplantation if medical therapy fails.

About Us

The Pulmonary Hypertension Comprehensive Care Center at Columbia University Medical Center, serving NewYork-Presbyterian and Morgan Stanley Children's Hospital of NewYork-Presbyterian, is one of the largest in the world. Established over 25 years ago, our center is committed to the medical and surgical treatment of both adults and children with pulmonary hypertension. With our commitment to providing all aspects of care to all patients with pulmonary hypertension, we have joined expert medical and surgical teams from pediatrics and adult medicine to create the Pulmonary Hypertension Comprehensive Care Center of NYP/Columbia, which was recently accredited by the Pulmonary Hypertension Association as a designated PHCC (Pulmonary Hypertension Care Center).

Pulmonary hypertension is a rare and serious disease which can occur at any age and affects the smallest blood vessels in the lungs leading to right heart failure if left untreated. While there have been many advances in the treatment of pulmonary hypertension over the past two decades, there is still no cure aside from lung transplantation and in cases of CTEPH (chronic thromboembolic pulmonary hypertension), a pulmonary thromboendarterectomy. Our center treats all forms of pulmonary hypertension including idiopathic (primary) pulmonary hypertension and pulmonary hypertension associated with a variety of conditions:

  • genetic (familial or  inherited forms of pulmonary hypertension)
  • congenital heart disease
  • scleroderma, lupus, and other connective tissue diseases
  • chronic thromboembolic pulmonary hypertension — CTEPH (including surgery for clot removal)
  • HIV disease
  • HHT (hereditary hemorrhagic telangiectasia)
  • Liver disease
  • pulmonary hypertension of the newborn
  • sickle cell disease
  • toxin (methamphetamines) exposure
  • chronic lung disease
  • advanced lung failure including those requiring ECMO bridge to recovery or lung transplantation

Patients and their families who are facing this challenging condition can be assured of receiving state-of-the-art care at our center. Patients with signs or symptoms of pulmonary hypertension (PH) will undergo an extensive evaluation by our team of medical and surgical experts. The initial visit involves a series of diagnostic tests to confirm the diagnosis, assess disease severity, and rule out other secondary causes. Most patients then require a cardiac catheterization with acute vasodilator testing by the specialized PH team to formulate an individualized treatment plan.

At the Pulmonary Hypertension Comprehensive Care Center, we remain focused on our ultimate mission to find a cure for pulmonary arterial hypertension and are committed to providing comprehensive care to adults and children facing pulmonary hypertension. Based on our years of experience in the field, we believe that patients with symptoms suggestive of pulmonary hypertension require a thorough evaluation at a specialized Pulmonary Hypertension Center where a comprehensive evaluation and medical and surgical options for all aspects of their disease can be provided.

Research and Education: Meeting the Challenge

In addition to patient care, research and education are essential priorities at the Pulmonary Hypertension Comprehensive Care Center. We remain on the forefront of clinical research in the field of pulmonary hypertension. In fact, our center has participated in most of the clinical research drug trials to date for the development of new medical treatments for pulmonary hypertension, including the first drug approved for treatment of this condition, intravenous epoprostenol in 1995. We are currently participating in several more scientific trials to study novel mechanisms and medications for the treatment of pulmonary arterial hypertension. Thus, our patients have access to clinical trials of new medications, allowing our physicians to provide novel treatments when appropriate, to patients not responsive to currently available treatment options. We also work in close collaboration with the genetics team, under the direction of Dr. Wendy Chung at Columbia University Medical Center, to provide a unique service of genetic counseling and testing to patients and families with inherited forms of pulmonary hypertension.

For more information about clinical research trials at the Pulmonary Hypertension Comprehensive Care Center, please check our research trials page.

Education of patients and their families plays a critical role in ensuring that patients with pulmonary hypertension receive the best possible care. Our doctors and nurses are readily available to educate and support our patients and their needs. We also believe that close relationships with referring physicians are critical for optimizing the management of patients with pulmonary hypertension. Furthermore, with rapid advances in the management of adults and children, our team is highly committed to the local, national, and international pulmonary hypertension community to provide education and awareness about the condition. Our active involvement in the scientific community allows us to advocate for more support, education and research that directly impacts our patients.

New Treatments on the Horizon

Medical Therapies

The current treatment for pulmonary arterial hypertension includes the use of targeted pulmonary hypertension medications and other conventional treatments to support heart and lung function. Just 15 years ago, the only targeted medical treatment option for patients was a continuous intravenous medication, epoprostenol. Fortunately, medical treatment of pulmonary arterial hypertension has advanced significantly during the past decade with 12 FDA approved drugs including a number of oral and inhaled medications as well as alternate intravenous and subcutaneous infusions of prostacyclin medications. All of these treatments are available to our patients if indicated, and when a patient requires an infusion therapy, our nurses are there to assist with teaching and management. As our understanding of pulmonary hypertension increases, new treatment options are being developed and implemented at a rapid pace.

The Pulmonary Hypertension Comprehensive Care Center proudly remains on the forefront of these scientific advances and also offers the newest treatment options to our patients through clinical research trials when approved therapies are not adequate. Each patient with pulmonary hypertension is unique, with a different cause and clinical course of his or her disease, therefore each treatment plan developed at our center is specifically tailored to the individual patient. 

Surgical Options

Patients with a rare cause of pulmonary hypertension due to clotting in their lungs (CTEPH — chronic thromboembolic pulmonary hypertension) are offered curative surgery through the Pulmonary Thromboendarterectomy Program at Columbia. Under the directorship of Dr. Matthew Bacchetta, we are one of the few CTEPH/PTE programs in the United States to offer this lifesaving surgery along with complementary medical care.

In rare cases of PAH, when targeted medical therapy alone is not providing the patient with adequate support, our patients will be offered a lung transplant evaluation with the team of lung transplant experts  at NYP/Columbia.

Our medical and surgical teams work hand in hand to provide every treatment option to our patients with pulmonary arterial hypertension.

FDA approved Treatments Available:

intravenous epoprostenol (flolan, veletri), intravenous/subcutaneous treprostinil (remodulin), inhaled prostanoids (iloprost, tyvaso), phosphodiesterase-5 inhibitors (sildenafil, tadalafil), endothelin receptor antagonists (bosentan, ambrisentan, macitentan) and soluble guanylate cyclase stimulator, (riociguat).

Clinical Research Trials at Columbia (2012-2014)

Adult

  • PVDOMICS: Defining the future fingerprints of pulmonary vascular disease (NIH funded: Dr. Berman Rosenzweig, Co-Principal investigator)
  • CTEPH Registry: First National CTEPH registry (Dr. Berman Rosenzweig , Co-Principal Investigator, Dr. Matthew Bacchetta, Co-Principal Investigator); Approval pending, Enrolling soon.
  • AMBITION: A Randomized, Multicenter Study of First Line Ambrisentan and Tadalafil Combination Therapy in Subjects with Pulmonary Arterial Hypertension (Dr. Berman Rosenzweig, Principal investigator
  • GRIPHON: A multicenter, double-blind, placebo-controlled Phase 3 study to demonstrate the efficacy and safety of ACT293987 (selexipag) in patients with pulmonary arterial hypertension (Dr. Berman Rosenzweig, Principal investigator)
  • SERAPHIN OLE: Extension study with a novel Endothelin Receptor Antagonist (macitentan) in Pulmonary Arterial Hypertension to improve clinical outcomes. (Dr. Berman Rosenzweig, Principal investigator).
  • TDE-PH-304: An Open Label, Extension Trial of UT15C SR (oral Remodulin) in Subjects with Pulmonary Arterial Hypertension. (Dr. Berman Rosenzweig, Principal investigator)
  • RIOCIGUAT: An open-label phase IIIb study of riociguat in patients with in-operable CTEPH, or recurrent or persisting pulmonary hypertension (PH) after surgical treatment who are not satisfactorily treated and cannot participate in any other CTEPH trial. (Dr. Berman Rosenzweig, Prinicipal Investigator). 
  • Eisenmenger Quality Enhancement Research Initiative (QuERI): To compare the management of patients with Eisenmenger Syndrome and adherence to national guidelines. (Dr. Berman Rosenzweig, Prinicipal Investigator). 
  • NIH sponsored R24: National Biological Sample and Data Repository for Pulmonary Arterial Hypertension (Dr. Berman Rosenzweig, Co-investigator) Enrolling Now
  • ASPIRE: A post-marketing, observational study to assess respiratory tract adverse events in pulmonary arterial hypertension patients treated with Tyvaso (treprostinil) Inhalation Solution. (Dr. Berman Rosenzweig, Principal investigator)
  • PROSPECT: Registry to PROSPECTively Describe Use of Epoprostenol for Injection (Veletri) in Patients with Pulmonary Arterial Hypertension. (Dr. Berman Rosenzweig, Principal investigator)
  • REVEAL: Registry to Evaluate Early and Long-term PAH disease management. (Dr. Berman Rosenzweig, Principal investigator)

Pediatric

  • AMB 112529: A randomized, open label study comparing safety and efficacy parameters for a high and low dose ambrisentan (adjusted for body weight) for the treatment of pulmonary arterial hypertension in pediatric patients age 8 years up to 18 years. (Dr. Berman Rosenzweig, Principal investigator
  • FUTURE-3: An open-label, prospective, multicenter study to assess the pharmacokinetics, tolerability, safety, and efficacy of the pediatric formulation of bosentan two versus three times a day in children with pulmonary arterial hypertension. (Dr. Berman Rosenzweig, Principal investigator)
  • PPHNet Database: A multicenter database of Pediatric PH Centers for the study of pulmonary hypertension in children. (NIH: UO1: Dr. Berman Rosenzweig, Principal Investigator, Dr. Usha Krishnan co-investigator), Enrolling Now
  • Identification of Novel Genes for Congenital Diaphragmatic Hernia by Characterizing Genetic Copy Number Alterations (R01 HD057036;)(Wendy Cheung, Principal Investigator, Dr. Usha Krishnan co-investigator, Dr. Erika Berman Rosenzweig, Co-investigators).

Faculty

Erika Berman Rosenzweig, MD
Medical Director, The Pulmonary Hypertension Comprehensive Care Center
Phone: 212.305.4436
Fax: 212.342.1443

Matthew Bacchetta, MD, MBA, MA
Surgical Director, The Pulmonary Hypertension Comprehensive Care Center
Phone: 212.305.3408
Fax: 212.305.3474

Jennifer Haythe, MD
Associate Medical Director, Adult Pulmonary Hypertension
Phone: 212.305.7912
Fax: 212.305.8304

Usha Krishnan, MD
Associate Medical Director, Pediatric Pulmonary Hypertension
Phone: 212.305.4436
Fax: 212.342.1443

Diane Kerstein, MD
Pediatric Pulmonary Hypertension, Cardiac Catheterization, Atrial Septostomy
Phone: 212.305.4436
Fax: 212.342.1443

Lewis Rubin, MD
Volunteer Faculty
Adjunct Professor of Medicine, Division of Pulmonary, Allergy Critical Care Medicine at CUMC

Affiliated Faculty

NYP/Columbia University Medical Center Campus

Selim Arcasoy, MD
Medical Director, Lung Transplantation

Emile Bacha, MD
Cardiothoracic Surgeon – Congenital Heart Specialist

Daniel Brodie, MD
Pulmonary Critical Care

Elana Bernstein, MD
Rheumatology

Wendy Chung, MD, PhD
Genetic Screening – Hereditary Pulmonary Hypertension

Amy Dzierba, Pharm D
Inpatient Pharmacist

Andrew Eisenberger, MD
Hematology

Robert Garofano, PhD
Pediatric Exercise Physiologist

Susheel Kodali, MD
Adult, Interventional Cardiology

David Lederer, MD
Interstitial Lung Disease/Lung Transplantation

Nina Patel, MD
Interstitial Lung Disease

Joshua Sonett, MD
Thoracic Surgery, Lung Transplantation

NYP/Weill Cornell Medical Center Campus

Evelyn Horn, MD
Consultant, Adult Pulmonary Hypertension, Non PAH Pulmonary Hypertension, Device Therapy