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Question:

Do I have other options than a lung transplant?  

I am a 25 year old female. I attend college and I do not smoke, do drugs or drink alcohol. I have a decent diet. I've had a PDA (patent ductus arteriosus) closure at 4 years old and my pulmonary pressure was 45 until I turned 21. At 21, it increased to 90. I was placed on IV remonulin also I take sildenafil, coumadin, diuretics and potassium. Recently during a cardiac cath, I had an arrhythmia and was placed on amiodarone. My tricuspid valve has been leaking since 2011 after I was place on IV remonulin (165mg).  My pressures now are 60 on echo and cath, but the tricuspid is still leaking so my cardiologist suggests that I get a lung transplant and to fix/replace my tricuspid valve. My question is should I follow my cardiologist's suggestion or do I have other options if I do not believe in getting a lung transplant? Thank you.

submitted by Sonia from Boston, Massachusetts on 12/17/2013

Answer:

Deborah Meyers, MDby Texas Heart Institute cardiologist, Deborah E. Meyers, MD  

Dear Sonia,

From your email I understand that you have pulmonary hypertension as a result of the PDA that was repaired at 4 years of age. This is a result of the persistent exposure of the blood supply to the lungs to increased blood flow through the PDA (which is now closed). This resulted in changes in the pulmonary blood vessels called obstructive arteriopathy and that caused the blood pressures to rise in your lungs. This high blood pressure in your lungs is called pulmonary hypertension. The type of pulmonary hypertension that you have (due to congenital heart disease) seems to have a far better prognosis than something called idiopathic pulmonary hypertension.

It sounds like you are on good medication that has lowered the pressures in your lungs and this is great.

What you do not say is how this impacts you day to day? How limited are you? How far can you walk? Do you get fluid collecting in your feet, legs or abdomen? Are you short of breath? What are your limits in your daily life? These are very important clinical issues and would be central to decision making regarding the need for lung transplant.

Because survival after any transplant is limited, and because this therapy is complex and poses risks to patients- decisions regarding timing of transplant are critically important. This is something that any excellent lung transplant center would be expert at assessing. Transplant doctors would not do patients the best service by either transplanting too early or too late.

If transplant has been put forward to you as an option, you could certainly have an assessment at a lung transplant center where they would be expert at these assessments and could give you a great opinion as to whether or not your case is severe enough to merit transplantation. There also may be clinical trials to consider.

As a heart transplant doctor, I commonly see patients that are referred to me for heart transplants and I am often able to tell them that it is far too soon to consider transplant as an option.

I hope that answers your question a bit- the good news is that you are in a great city with excellent academic medical centers.

All the best to you.

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Updated January 2014
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Texas Heart Institute Heart Information Center
Through this community outreach program, staff members of the Texas Heart Institute (THI) provide educational information related to the prevention, diagnosis, and treatment of cardiovascular disease. It is not the intention of THI to provide specific medical advice, but rather to provide users with information to better understand their health and their diagnosed disorders. Specific medical advice will not be provided and THI urges you to visit a qualified physician for diagnosis and for answers to your questions.
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