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

Pulmonary Hypertension 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 pulmonary hypertension. 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 pulmonary hypertension 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 a number of medications available to treat pulmonary hypertension. Choice of therapy will depend on the type of pulmonary hypertension, 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 pulmonary hypertension 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 (Lasixâ), toersemide (Demedexâ), spironolactone (Aldactoneâ) these medications help remove excess fluid from the body and improves swelling, bloating and breathing.
  • Anticoagulants: Some patients with pulmonary hypertension are at high risk for the formation of blood clots. Blood thinners like warfarin (Coumadinâ) 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 catheteriziation, drugs like nifedipine (Procardiaâ) or diltiazem (Cardizemâ) can lower the blood pressure in the lungs.
  • Endothelin Receptor Antagonists: Bosentan (Tracleerâ), ambrisentan (Letairisâ), macitentan (Opsumitâ) are drugs that block the endothelin pathway and help prevent narrowing of blood vessels in pulmonary hypertension.
  • Phospohodiesterase Inhibitors: Sildenafil (Revatioâ) and tadalafil (Adcircaâ, Cialisâ) help dilate blood vessels in the lungs by relaxing the smooth muscle that lines the vessels walls.
  • Prostanoids: Epoprostenol (Flolanâ, Veletriâ), treprostinil sodium (Remodulinâ, Tyvasoâ, Orenitramâ), iloprost (Ventavisâ), seleixpag (Uptraviâ) 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 (Adempasâ) helps relax pulmonary arteries by increasing the release and action of nitric oxide in the body.

Surgical Options

  • Pulmonary Endarterectomy: A surgery that “cleans out” blocked pulmonary arteries
  • 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 pulmonary hypertension 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

PH Care at Columbia

The Center for Advanced Cardiac Care offers patients a team-based approach with multidisciplinary specialists providing comprehensive care for PH. Our team includes:

  • Cardiologists
  • Pulmonologists
  • Rheumatologists
  • Interventional cardiologists
  • Cardiothoracic surgeons
  • Psychiatrists
  • Social workers
  • Physical therapists

Our specialists work together to create the best possible treatment plan for every individual patient. Patients are followed closely by their primary pulmonary hypertension doctor and should admission to the hospital be required, close care is provided by an inpatient team of doctors and nurse practitioners. Visiting nurse services and pulmonary hypertension support groups are an integral part of care providing physical and emotional support to our patients in their homes.