Your Questions Answered
Who should be worried about CTEPH?
Chronic Thromboembolic Pulmonary Hypertension (CTEPH) develops when blood clots accumulate in the lungs (pulmonary arteries). Most patients that develop blood clots in the lungs are able to dissolve these clots with the help of blood thinners. However, some patients may never be aware that they have had a blood clot or their blood clots did not dissolve while on blood thinner therapy. These patients are at risk for developing increasing pressure in the lung blood vessels. This leads to progressive shortness of breath.
Anyone with a history of blood clots who has progressive shortness of breath should be evaluated to see if they might have CTEPH. There are many causes of shortness of breath and a lung doctor will consider all the appropriate possibilities. Two tests in particular are helpful when considering CTEPH—echocardiography (ultrasound of the heart) and a ventilation-perfusion lung scan (V/Q scan). These two tests will help point your doctor in the right direction.
We received several questions about surgery to remove chronic blood clots in CTEPH
All patients with suspected PAH should be evaluated to make sure that they don’t have CTEPH (chronic blood clots causing pulmonary hypertension). If chronic blood clots are found in the lungs then there may be a surgery that can dramatically improve the disease. This surgery is called pulmonary thromboendarterectomy (PTE). Not every patient with chronic blood clots is a candidate for surgery. There are only a handful of centers of excellence in the USA. The surgery is very complicated and requires a specialized team. The centers of excellence have a track record of good results and low complication rates. Surgeons/centers with less experience generally have higher complication rates.
The surgery involves stopping your heart and working on one side of the lungs then the other side. Your heart is then restarted and your chest is closed. Patients generally wake up within the first 24 hours after surgery and many patients will be sitting in a chair within 48 to 72 hours. The hospital stay is generally about a week. Patients are treated with blood thinners for life. Many patients will be able to stop their pulmonary hypertension medications, and others will be able to decrease their medications. Some patients will still require medication Click here to learn more about medication therapy for CTEPH . Patients who are not candidates for surgery are treated with medications.
PAH after corrective surgery for congenital heart defects
Some children are born with abnormal connections in their heart. There are many types of defects that can lead to PAH over time. In the United States, most of these defects are identified either before birth or shortly after birth. These defects are then corrected early in life. With early identification, many children can avoid developing PAH. Unfortunately, some children will still develop PAH. Once a child has developed PAH, medications are used to lower the pressure and improve blood flow. In certain circumstances, the heart defect may be able to be repaired after the development of PAH, but in most cases once PAH has developed surgery is no longer an option.
The good news is that PAH due to congenital heart disease has a much better prognosis than other causes of PAH. I have cared for more than a handful of eighty-year-old patients with PAH due to congenital heart disease. In severe cases, heart-lung transplantation may be an option.
We continue to receive frequent questions about where to get expert PAH care.
The PHA has an accreditation system that is a rigorous process for evaluating PAH centers. Click here to find a full list of Comprehensive Care Centers for PAH.
