Pulmonary Hypertension

Clinical Services: Cardiology
Upper East Side
520 East 70th Street, Starr Pavilion, 4th Floor
New York, NY 10021
Call
(212) 746-2381

Pulmonary hypertension (PH) is “high blood pressure” in the lung vessels.  This  often leads to insufficient oxygen delivery and results in an excess load to the heart leading to “right sided heart failure.”  The smaller arterial blood vessels in the lung may be abnormally thick, scarred, inflamed, constricted or have clots.  Normally, the lung vasculature can easily adapt to exercise-induced marked increases in blood flow.  However in pulmonary hypertension, the vascular resistance and the pressure in the lungs is further elevated with activity and leads to decreased flow to the left side of the heart (which pumps to the rest of the body) .  Consequences of this are common symptoms of shortness of breath , exercise intolerance, right sided heart failure symptoms (edema, ascites), chest pain, and/or near or complete fainting (presyncope and syncope).

PH may be idiopathic (unknown cause), genetically or familial related, associated with connective tissue diseases such as lupus and scleroderma, congenital heart disease (VSD, PDA, ASD large shunts), liver disease (WHO Group 1).  In some patients, the pulmonary hypertension is due to left-sided heart disease (WHO 2 PH), lung disease or low oxygenation (WHO 3).  Importantly, a curable form of this disease is that which is caused by chronic blood clots, CTEPH (Group 4), which may be amenable to surgery, and WHO Group 5 which involve a multitude of miscellaneous diseases of a wide spectrum (including sarcoid, end stage renal disease, myeloproliferative disease).  We often see patients who have mixed etiologies, i.e. some left heart disease, some chronic lung disease.

Appropriate testing for patients referred for pulmonary hypertension will often include: special blood tests, ECG, special echos, cardiac MRI, cardiopulmonary stress testing (to distinguish cardiac vs. pulmonary shortness of breath), lung scan to assess for clots (V/Q scan), breathing test (PFTs), high resolution chest CT of the lungs and ultimately the diagnosis needs to made with a cardiac catheterization as the definitive gold standard and the means of differentiating multifactorial disease.

Treatments include: diet and appropriate exercise recommendations, diuretics, oxygen when necessary, often anticoagulation, rarely calcium antagonists, targeted pulmonary vasodilators which can include oral, inhaled, subcutaneous, intravenous medications, research studies, consideration for conventional or special surgical or catheter based interventions (i.e. atrial septostomy, percutaneous or surgical valvular interventions for WHO 2 PH, referral for pulmonary thromboendarectomy, lung transplantation).  Additional treatment for sleep apnea, interstitial lung disease will be addressed by our pulmonary colleagues and additional treatment for active scleroderma, lupus or related diseases by our rheumatology colleagues.

For all, early diagnosis, treatment, and long-term care of patients with pulmonary hypertension requires a team approach with collaborative physicians, nurse practitioners, dieticians, physical therapists and social workers working together to maximize patients’ functioning, know when it is appropriate to consider more advanced interventions, to empower patients to partner with the team to adjust medications and maximize their capabilities, and to know when we have exhausted therapies.

The Perkin Center for Heart Failure, Mechanical Circulatory Support and Pulmonary Vascular Disease at Weill Cornell Cardiology offers the following services:

  • Cardiopulmonary exercise testing to identify and follow patients with PH and heart failure.
  • Inpatient transfer and out-patient management of individuals with pulmonary hypertension. This includes intensive nutritional/dietary counseling, as well as telephone follow-up by a Nurse Practitioner trained in management of patients with pulmonary hypertension.
  • Specialty cardiac catheterization to diagnose and gauge response to treatment (done by our catheterization colleagues)
  • Opportunities for participation in multi-center clinical trials as well as ongoing original research.
  • End-of-life management and palliative care for patients with unresponsive symptoms and limited life expectancy.
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