Clinical Associate Professor of Medicine
Board Certifications: Cardiovascular Diseases, Internal Medicine.
What is Pulmonary hypertension (PH)?
Pulmonary hypertension (PH) is high blood pressure in the lungs. It results
from an abnormal condition in the heart and/or lungs. It can also be associated
with liver diseases or HIV-related disorders. The majority of PH patients are
female (9 females to every one male).
When the pressures are elevated in the lung arteries, there is an abnormal
"back pressure" into the right side of the heart. If not corrected,
the right heart enlarges and eventually fails. The right heart is not capable of
pumping against high pressures for long periods of time (months or years). When
it fails, the following symptoms appear:
the liver enlarges and becomes painful
the abdomen enlarges from excess fluid
appetite decreases
exercise is poorly tolerated
increased shortness of breath with exertion
These symptoms are often quite subtle at first, usually starting with mild
shortness of breath with minimal exertion. Rather than evaluating these symptoms
as a medical illness, they are frequently attributed to work or home situations,
stress, fatigue, chronic anemia, raising children, etc. The symptoms progress
over an average of 18-24 months before a diagnosis of PH is made.
Evaluation: What tests will I need to undergo?
It is important to undergo a thorough evaluation since up to 50% of patients
with pulmonary hypertension have inaccurate diagnoses and the treatment plan
that follows is based on a correct diagnosis of the cause of the PH.
Basic tests might include:
A complete history and physical examination, with copies of all records
you have up to this time (this is very important)
Lab studies:
Complete Blood Count (CBC)
Comprehensive Metabolic Panel (CMP)
Thyroid Stimulating Hormone (TSH)
Antinuclear Antibody (ANA)
Erythrocyte Sedimentation Rate (ESR)
Rheumatoid Factor (RF)
C-Reactive Protein (CRP)
Anticardiolipin Antibody
SCL-70
Urinalysis
HIV
PA & Lateral chest X-Ray
Electrocardiogram (ECG)
Echocardiogram
Lung Scan (Nuclear Medicine)
Pulmonary Function Tests
Overnight pulse oximetry or full sleep study
Treatment: What are my options?
The stage of your disease, how ill you are, what caused your disease and your
ability to participate in your care and treatment must all be taken into
consideration. Possible treatments could include any of the following:
Dobutamine - an intravenous drug infusion used to assist the
failing right ventricle. It also improves blood flow to the kidney.
Diuretics - also known as "water pills". This needs to be
given intravenously until your condition improves, at which time you can be
switched to a pill form of the medication.
Coumadin - a blood thinner taken daily to decrease blood clotting
in the lung arteries.
Calcium Channel Blockers - pill form medication to help the elastic
layer of the pulmonary arteries relax, thereby decreasing the pressure.
These are not helpfu with severely elevated pressures.
Flolan - a prostacyclin intravenous drug given continuously by a
pump worn in a "fanny pack." This is the first FDA approved drug
for treatment of pulmonary hypertension. It requires a surgically implanted
IV line.
Remodulin - (UT-15 or Treprostinil) - a drug similar to Flolan but
is administered under the skin of the abdomen, similar to insulin (SubQ).
Infusions are set for 72 hours per pump loading.
Nitric Oxide - an inhaled gas that is only used in critical cases
in a hospital. It is extremely expensive ($3,000/day) and the equipment is
very cumbersome. Patients cannot be mobile.
Tracleer - the first "pill" approved by the FDA for the
treatment of pulmonary hypertension. It is a new category of drug called
endothelin receptor blockers. This medication has many significant side
effects and needs careful monitoring.
Oxygen - oxygen is prescribed when your oxygen level drops below a
certain point. Low oxygen levels cause the pulmonary arteries to narrow
(constrict), which increases the pressure in the lung arteries, worsening
the effects of the PH.
PTE (pulmonary thromboendarterectomy) - is a surgical procedure to
remove old blood clots from the pulmonary arteries. In select individuals,
this surgical procedure is a cure. The procedure is performed at St. Luke's
Medical Center (Milwaukee, WI).
Lung transplantation - if medical therapy is not successful or if
you are severely ill at the time of starting treatment, you may be evaluated
for a lung transplant. Unlike the other organ transplant lists, you need to
be listed early as the ranking is by seniority (not severity) and it takes
18-24 months to match a lung.
Pulmonary hypertension clinics
A pulmonary hypertension clinic specializes in treatment of combined cardiac
and pulmonary diseases. They are staffed by PH specialists and have readily
available consultants as needed.
The PH clinic at Aurora St. Luke's Medical Center is held the 4th Monday of every
month, with other patients seen as needed throughout the month. Consultants in
rheumatology, pulmonary diseases, infectious diseases, transplantation,
gastroenterology, nephrology and social service/financial counseling are
available.
A support group meeting is held from noon to 1:00 p.m. on the day of the
clinic each month. Each patient is assigned a primary RN coordinator, who
assists with his or her care, appointments, testing, acute illnesses and
medication therapies. You can reach the Clinic by calling 414-649-3319.
by Dianne L. Zwicke, MD, FACC, FACP, FCCP