Pulmonary Hypertension

Pulmonary hypertension (PH) is high blood pressure in the blood vessels of the lungs. It occurs when the small blood vessels that go through the lungs become thicker, constrict or become plugged. This leads to increased pressure in those vessels, making the right side of the heart work harder to pump blood through the lungs. If high pressure remains, the heart will become enlarged and weaker, pumping blood less efficiently into the lungs and eventually throughout the body. Patients at this stage develop progressive fatigue and shortness of breath.

An estimated 1,000 or more new cases of PH are diagnosed each year in the United States, but the actual number of cases is unknown. It is most common in women, but anyone can develop the condition. 

The Pulmonary Vascular Disease Program — a unique multidisciplinary program between the Brigham and Women’s Hospital Lung Center and the Heart & Vascular Center — brings together pulmonary and critical care medicine physicians, cardiothoracic surgeons, interventional cardiologists and cardiovascular medicine clinicians who have extensive experience diagnosing and treating all forms of pulmonary vascular disease, pulmonary arterial hypertension, and chronic thromboembolic pulmonary hypertension.

Pulmonary Hypertension Topics

Risk Factors for Pulmonary Hypertension

While the exact cause of pulmonary hypertension is unknown, there are a number of factors that may contribute to the development of PH, including:

  • Connective tissue disease, particularly scleroderma
  • Liver disease
  • HIV
  • Valvular or hypertensive (caused by high blood pressure) heart disease
  • Lung disease
  • Family history
Symptoms of Pulmonary Hypertension

Early in the disease, symptoms of pulmonary hypertension may be nonspecific and can mimic symptoms of other medical conditions. It is also possible that patients may experience only limited symptoms. 

The most common symptoms of PH are:

  • Unexplained shortness of breath (dyspnea)
  • Fatigue or tiredness

Other common symptoms include:

  • Chest pain or pressure during activity (angina)
  • Dizzy spells that may occur during activity or exercise
  • Fainting
  • Ankle or leg swelling (edema)
  • Increased heart rate (tachycardia)
  • Heart throbbing (palpitations)
  • Cough
Diagnosis of Pulmonary Hypertension

Pulmonary hypertension is rarely discovered during a routine medical examination, and in its later stages, the signs of the disease can be confused with other conditions that affect the heart and lungs.

Diagnosing PH is typically a process of exclusion. Diagnostic procedures may include:

Treatment for Pulmonary Hypertension

Our team of clinicians collaborates to treat each patient's pulmonary hypertension and associated medical conditions. Treatment goals for PH include lowering a patients' pulmonary pressure, reducing symptoms, increasing exercise capacity and prolonging life expectancy. Options ranging from medications to transplant surgery are available for treating pulmonary hypertension, and their selection depends on the type of pulmonary hypertension present.

Medication

The following medications are used to treat PH:

View video about anticoagulant therapy featuring Dr. Gregory Piazza

Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Specialists within the Pulmonary Vascular Disease Program provide advanced, focused, and comprehensive evaluation and treatment approaches for a subset of patients with pulmonary hypertension who experience recurring emboli (blood clots) in their lungs – Chronic Thromboembolic Pulmonary Hypertension (CTEPH). Without effective treatment, CTEPH can lead to life-threatening heart failure. Brigham and Women’s Hospital is one of only a handful of hospitals nationwide that offer a comprehensive collaboration of medical, interventional and surgical care for CTEPH.

Surgery for Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Thromboendarterectomy

Read the article Pulmonary Vascular Disease Specialists Add Balloon Angioplasty as a Treatment Option for Patients Not Eligible for Surgery.

Read the article Improving Outcomes for Patients with Pulmonary Embolism and Chronic Thromboembolic Pulmonary Hypertension

Follow-Up Care

The Pulmonary Vascular Disease Program team provides long-term care and support to help patients manage their condition. Our team of physicians and nurses develops individualized treatment plans and regularly monitors each patient. Patients also can access information about research opportunities and continuing education. Close follow-up care helps physicians detect early changes that may require further treatment.

What You Should Expect

The Heart & Vascular Center is located in the Shapiro Cardiovascular Center, across the street from BWH’s main 75 Francis Street entrance. The Heart & Vascular Center brings together the full range of services in one location, fostering seamless and coordinated care for all cardiovascular patients.

If you are having surgery or a procedure, you will likely be scheduled for a visit to the Watkins Clinic for pre-operative information and tests.

The day of surgery, you care will be provided by surgeons, anesthesiologists and nurses who specialize in surgery for patients with pulmonary hypertension. After surgery, you will go to the post-surgical care unit where you will receive comprehensive care by an experienced surgical and nursing staff.

During your surgery, family and friends can wait in the Shapiro Family Center. Staff members will provide surgery updates and caregivers who leave the hospital will be contacted by cell phone.

Multidisciplinary Care

The Pulmonary Vascular Disease Program—a collaborative venture between the Brigham and Women’s Hospital Lung Center and the Heart & Vascular Center—brings together pulmonary and critical care medicine physicians, cardiothoracic surgeons, interventional cardiologists and cardiovascular medicine clinicians who have extensive experience diagnosing and treating all forms of pulmonary vascular disease including pulmonary hypertension.

Resources

Learn more about pulmonary hypertension in our health library.

Visit the Kessler Health Education Library in the Bretholtz Center where patients and families can access computers and knowledgeable staff.

Access a complete directory of patient and family services.

Visit the Pulmonary Hypertension Association website.

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