Pulmonary Hyptertension Program
Frequently Asked Questions
Is pulmonary hypertension an inherited disease?The predisposition to develop pulmonary hypertension is inherited in a very small percentage of cases. Relatives of patients who have been diagnosed with idiopathic (“primary”) PH should consider seeking medical attention if they develop unexplained shortness of breath, given the increased risk they carry. Learn more about pulmonary hypertension.
When is the pulmonary hypertension severe enough for a lung transplant?Lung transplantation is recommended for patients with advanced PH who are not responding to medical treatments. While the use of the available therapies has led to a decline in the need for transplantation, it is possible that your physician will discuss this with you during the initial visit(s). Learn more about treatments for pulmonary hypertension.
What should I expect of my appointment at the Froedtert & The Medical College of Wisconsin Pulmonary Hypertension program when I am referred there?At the time of your appointment, you will be introduced to our pulmonary hypertension nurse, who will be your contact person for future questions and pulmonary hypertension-related needs.
You will then be evaluated by one of our physicians with expertise in PH. Our advanced practice providers may also see you in conjunction with the physician. At the end of this visit, the physician will explain the diagnostic and treatment plan and discuss all relevant issues with you. Tests will be performed after the initial visit(s) to confirm if PH is present and to better define the type of PH you may have. Learn more about diagnosing pulmonary hypertension.
Once the testing and evaluation are completed, your physician will discuss various treatment options and will determine the option(s) most appropriate for your specific case. Follow-up office visits and testing are initially scheduled every two to four months to monitor treatment effectives and disease progression.
Depending on the type and severity of your pulmonary hypertension and the presence of other conditions (e.g., sleep apnea, emphysema or heart failure) the PH team may recommend additional interventions as part of a multidisciplinary approach, such as referral to:
- One of our cardiologists to arrange for a diagnostic heart catheterization. The cardiologist may also address any specific cardiac issues such as heart arrhythmias (abnormal heart beats) or blockage of coronary vessels (for those patients who don’t already have a primary cardiologist).
- A rheumatologist, a specialist with expertise in managing conditions in which the immune system is overactive, leading to inflammation of organs such as the heart, lungs, muscles and joints. Conditions that fit in this category include lupus, scleroderma and rheumatoid arthritis.
- Pulmonary rehabilitation, a special type of physical therapy that concentrates on improving the strength of your breathing muscles and overall endurance and stamina. Many patients who go through pulmonary rehabilitation sessions enjoy a noticeable improvement in exercise tolerance.
What should I expect of my follow up visits?Once the diagnostic phase has been completed, your pulmonary hypertension team will discuss the findings in detail with you and your family. Your primary and referring physicians will also receive a detailed summary so they can help you understand some of the recommendations and future plans.
Depending on the results of the tests performed (including heart catheterization, as needed), a treatment plan will be created. Patients who have the more severe types of PH or who are significantly affected by the disease will likely require more aggressive treatment regimens that could include medication. Other therapies such as oxygen administration, inhalers for lung disease, devices to help with sleep apnea (if present) to prevent the “windpipe” from collapsing during sleep, diuretics (“water pills”) and drugs to achieve an optimal blood pressure will likely also be used.
During the follow-up visits, your physician will order some routine tests to evaluate progression of the disease and response to the therapies. Such diagnostic tests include:
- Routine examination and labs
- Echocardiogram (performed at three- to six-month intervals)
- Six-minute walk test to measure the distance covered during a six-minute period
- Repeat heart catheterization, typically performed four to six months after initiation of treatment
Date: July 18, 2012
Online Editor(s): Richard Petre