Pulmonary Hypertension

In common practice, some doctors diagnose this condition based on tests that only estimate lung blood pressure. This is why it’s so important to have your test results evaluated and explained to you by true experts. URMC is the only program outside New York City that is accredited by the national Pulmonary Hypertension Association, and we were one of the first in the nation to achieve accreditation.

The UR Medicine Pulmonary Hypertension Clinic evaluates and manages many diseases, including:

• Pulmonary arterial hypertension (PAH)
• Heart failure preserved ejection fraction (HFpEF)
• Interstitial lung disease with Pulmonary Hypertension (ILD-PH)
• Chronic thromboembolic pulmonary hypertension (CTEPH)
• Pulmonary embolus, especially for those who were in the hospital
• Hereditary hemorrhagic telangiectasia (also known as Osler-Weber-Rendu syndrome), an unusual genetic disorder leading to “tangles” of tiny blood vessels on the lips and tongue
• Digital ulcers (usually from scleroderma)
• Pulmonary Hypertension complicating any other lung disease (COPD, sleep apnea, etc.)

We work with colleagues in cardiology, radiology, rheumatology, orthopedic surgery, and referring providers. Together we provide personalized care, treatment alternatives, risk assessments, opportunities for clinical research, and collaborations with other accredited center.

Numerous tests may be used in diagnosis, including:

• Walking test to evaluate exercise capacity
• Echocardiogram (ultrasound of the heart)
• CT scan
• Pulmonary function test
• Blood tests
• Sleep study
• V/Q scan to look for chronic blood clots
• Tests to measure the pressures in the heart and lung blood vessels or to visualize the lung blood vessels themselves.

We are experts in the many drugs available to treat pulmonary hypertension. Treatments help patients feel better, walk farther, and live longer. Many patients improve after we optimize medications that were started by less experienced physicians.

Our team also treats patients who have pulmonary hypertension and interstitial lung disease—a promising new opportunity for many patients who haven't had options before.

We often improve quality of life for people who have pulmonary hypertension associated with heart or lung disease. Many of these patients benefit from changes in their maintenance drug therapy, exercise instruction, and/or evaluation for obstructive sleep apnea.

Because sleep apnea is a major symptom, we help patients use CPAP or BiPAP devices even if they have had trouble previously. Good therapy for sleep apnea can improve patients’ lives greatly.