Adult Pulmonary Hypertension Program at Columbia University Irving Medical Center (CUIMC)

The Pulmonary Hypertension Program at Columbia University Irving Medical Center (CUIMC) offers expert, team-based care for all forms of pulmonary hypertension. As a Pulmonary Hypertension Comprehensive Care Center designated by the Pulmonary Hypertension Association (PHA), we provide advanced diagnostic and treatment options grounded in the latest research.

Pulmonary hypertension is a complex condition with many possible causes. Accurate diagnosis and personalized care are essential. Our multidisciplinary team—including cardiologists, pulmonologists, rheumatologists, nephrologists, hematologists, cardiothoracic surgeons, nurse practitioners, mental health professionals, and physical therapists—works together to develop tailored treatment plans for each patient.

Understanding Pulmonary Hypertension

Pulmonary Hypertension (PH) is high blood pressure in the blood vessels of the lungs. This can cause difficulties for the right side of the heart, particularly the right ventricle, because of the excess resistance in pumping blood through the lungs.

Symptoms

PH is a progressive disease, and signs and symptoms can develop gradually. In the beginning patients may have no symptoms or mild shortness of breath with activities like climbing stairs or exercise. As the disease progresses, symptoms will become more significant and can range in severity. Common signs and symptoms include:

  • Shortness of breath
  • Chest pressure
  • Dizziness
  • Fatigue
  • Inability to exercise
  • Chronic cough
  • Palpitations or racing heart
  • Abdominal bloating (ascites)
  • Swollen legs (edema)
  • Blue lips or skin (cyanosis)

Diagnosis

Patients with prior symptoms may have a “screening” echocardiogram that suggests elevated pulmonary pressures and possibly right heart enlargement. Sometimes a chest CT scan done for other reasons may suggest an enlarged pulmonary artery.

For some diseases, like the connective tissue diseases and portal hypertension, patients should be screened for PH. This may lead to further evaluation of heart and lung function.

The gold standard for diagnosis of PH is the right heart catheterization, which measures the pressures in the heart. Since some patients may have more mild symptoms at rest, a noninvasive cardiopulmonary stress test can help determine if PH is a possible cause for their difficulty with exercise, along with a comprehensive evaluation that includes screening for other potential causes.

Treatment

While PH does not have a cure, your doctor can prescribe lifestyle changes, medications, and treatments that will help stabilize your condition and alleviate symptoms. Your doctor will make treatment recommendations after a comprehensive evaluation is complete.

Exercise and Diet

Carefully supervised exercise can improve the flow of oxygen to your body. So as part of their care, some patients may participate in pulmonary rehab programs to improve exercise capacity. Certainly, heart healthy diets and low sodium are also strongly encouraged.

Medications

There are many medications now available to treat pulmonary hypertension. Choice of therapy will depend on the type of PH, any underlying diseases, the severity of illness, and ability to tolerate a complex drug regimen. Medications are available in many forms including inhaled, oral, intravenous, and subcutaneous.

Oxygen: Some patients with PH have low oxygen levels and require oxygen. Oxygen may be required only at night, only with exertion, or continuously throughout the day and night.

Diuretics: Also known as “water pills” such as furosemide (Lasix), torsemide (Demedex), spironolactone (Aldactone) these medications help remove excess fluid from the body and improves swelling, bloating and breathing.

Anticoagulants: Some patients with PH are at high risk for the formation of blood clots. Blood thinners like warfarin (Coumadin) prevent the formation of these clots and can be taken orally or by injection.

Inotropes: Digoxin (Lanoxin) is a medicine that help the heart pump harder by releasing calcium. It can also be helpful in treating abnormal ad fast heart rates.

Calcium Channel Blockers: For a small number of patients who are still “vasoresponsive” meaning that their pressures decrease in response to a medicine given during catheterization, drugs like nifedipine (Procardia) or diltiazem (Cardizem) can lower the blood pressure in the lungs.

Endothelin Receptor Antagonists: Bosentan (Tracleer), ambrisentan (Letairis), macitentan (Opsumit) are drugs that block the endothelin pathway and help prevent narrowing of blood vessels in pulmonary hypertension. Phosphodiesterase Inhibitors: Sildenafil (Revatio) and tadalafil (Adcirca, Cialis) help dilate blood vessels in the lungs by relaxing the smooth muscle that lines the vessels walls.

Prostanoids: Epoprostenol (Flolan, Veletri), treprostinil sodium (Remodulin, Tyvaso, Orenitram), iloprost (Ventavis), seleixpag (Uptravi) are potent vasodilators that open up the pulmonary arteries and lower the pressures in the lungs. These drugs are available in different formulations: inhaled, oral, intravenous, and subcutaneous.

Guanylate Cyclase Stimulators: Riciguat (Adempas) helps relax pulmonary arteries by increasing the release and action of nitric oxide in the body.

Fusion Protein: Sotatercept (Winrevair) works by inhibiting the overproduction of cells that can narrow and block blood vessels in the lungs, a key factor in PAH. It is delivered by a self-administered sub-cutaneous injection every three weeks.

Surgical Options

Pulmonary Endarterectomy: A surgery that “cleans out” blocked pulmonary arteries in patients diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH)

Pulmonary artery angioplasty: A catheterization-based procedure for patients with blood clot disease

Atrial septostomy: A surgeon or interventional cardiologist can create a hole between the left and right sides of the heart to release pressure on the right side

Pulmonary artery to Aorta (Potts) shunt: A surgeon may create a connection from the left pulmonary artery to the descending aorta to reduce the load on the right heart

Lung Transplant: Patients with advanced lung disease from PH may be a candidate for a lung transplant

Heart/Lung Transplant: although this type of transplant is rare, patients with combined heart and lung failure, may be considered for a combined organ transplant

ECMO: Occasionally very sick patients will need to be placed on an extracorporeal membrane oxygenation (ECMO) machine while awaiting transplant. This device mimics the role of the lungs by adding oxygen and removing carbon dioxide from the blood

 

Our Team

  • Jennifer Haythe

    • Director Adult Pulmonary Hypertension Program
  • Estefania Oliveros-Soles

    • Associate Director Adult Pulmonary Hypertension Program, Director, Chronic Thromboembolic Disease Program
  • Koji Takeda

    • Surgical Director Adult Pulmonary Hypertension Program, Director of Pulmonary Endarterectomy
  • Lori Reyes, MFNP

    • Chief Coordinator, Pulmonary Hypertension Program
  • Sofia Diaz

    • Chief Administrator, Pulmonary Hypertension Program

For more information or to make an appointment call 212-305-9268.

Useful Links:

CTEPH Program:

Pulmonary Embolectomy and Pulmonary Thromboendarterectomy Program

Chronic Thromboelic Pulmonary Hypertension

Pulmonary Embolism Response Team (PERT):

PERT

Lung Transplant Program:

Center for Advanced Lung Disease and Transplantation

 

Our program collaborates closely with hospitals across the New York-Presbyterian Hospital (NYPH) system to ensure access to high-quality pulmonary hypertension care throughout the region. To learn more about services available at other NYP locations, please visit:

Pulmonary Hyptertension