Pulmonary Hypertension Myths and Realities

This is a cautionary tale about a fairly rare disease, pulmonary hypertension (PH), and the fact that no physician can make the diagnosis based simply on an echocardiogram. As a medical transcriptionist and a medical editor, I have noticed a disturbing trend: Most physicians simply take the suggestion of PH from the echocardiogram and run with it, offering no treatment to their patients and no further diagnosis. I ran across this diagnosis purely based on an echocardiogram during one shift alone at least 10 times, and it was never borne out by other diagnostic procedures, nor was there ever any mention made of treatment for these patients.

If one truly has PH, then this is deadly. Otherwise, this is a misdiagnosis that follows the unsuspecting patient around. In short, it is nothing short of medical malpractice! I suspect the patients involved were never told they had "pulmonary hypertension." This "misdiagnosis" is widely spread across all spectrums of the physician community. Are they not trained to view right heart pressures as an indicator only for pulmonary hypertension and not diagnostic? If so, all should read this article and follow the Mayo Clinic guidelines for the diagnosis of such. Pulmonary hypertension is deadly without treatment, and this diagnosis should not be undertaken lightly.

It is my urging that all physicians consider the serious implication of including this diagnosis when only suggested on the echo and not confirmed by a right heart catheterization and stop dictating the diagnosis.

Every person should expect his or her medical record to be accurate. I also advise that each and every person periodically review his or her medical record (yes, you have the right to do so) and question any diagnoses with which you are not familiar. Mistakes are made by physicians and transcriptionists alike. It is your duty to review your records.

I reviewed my own bone scan report a few years back and found 10 glaring errors. Yes, some of these I would not have recognized but for my training in the medical field, but even the most untrained patient would recognize if the report dictated a broken bone that has never existed. Mine specifically stated words to the effect that the patient noted a history of a broken right hip but the scan showed no evidence of such. Now, I know for a fact that I never had a broken hip nor have I ever claimed to have such. Also in my scan were 5 spinal compression fractures but the report read such that there were 10 listed. I do truly have 5 spinal compression fractures, not 10. Now I know the doctor did not dictate these findings but the transcriptionist just used an existing template for such reports and did not delete the extraneous material. To make matters worse, the person who was supposed to do the editing on these outsourced reports did not do his or her job, so the mistakes slipped through. I circled all the errors and took them to the appropriate radiology department. They presented this report to the radiologist who came into the room I was in and apologized. He stated he never dictated such things, and I knew he did not, and he had the report corrected right away!

I have learned several lesions from my experience with the misdiagnosis of pulmonary hypertension, outlined below.

One cannot believe a cardiologic interpretation of an echocardiogram to be diagnostic of PH, especially when 2 cardiologists have the same numbers and come up with different conclusions. The most respected clinic possibly in the world says definitive diagnosis must be made by the right heart catheterization (RHC). I don't like misdiagnoses to be in my medical record, and now that I know for sure it is not PH, I will work to get this incorrect diagnosis expunged. My cardiologist (whom I really like) may or may not want to see me again after I went around him and got to the PH specialist in time, but that is okay. Ultimately we have to take charge of our own health issues and cannot leave it to people who only spend a very short amount of time with us and then go on to the next patient. I feel like it is like conveyor belt medicine.

The following is taken verbatim from the Pulmonary Hypertension Association website, http://www.phassociation.org/ and I could not state it any better than the experts. Pay attention to these symptoms if you ever hear your doctor say that you have PH:

The symptoms for all types of pulmonary hypertension may be similar, but the severity of the symptoms usually correlates with the progression of the disease.

• Breathlessness on exertion
• Chest pain (also called angina pectoris)
• Dizziness upon standing, climbing stairs, straightening up from a bent position, or even while just sitting
• Fainting (also called syncope)
• Loss of energy
• Swollen ankles and legs (also called edema)
• Depression
• Dry cough
• Raynaud's Phenomenon (chalky white and/or dusky blue fingers that may be painful and can sometimes be provoked by the cold)

In advanced stages of pulmonary hypertension, minimal activity may produce some or all of these symptoms. Patients in advanced stages may experience irregular heartbeat, a racing pulse, passing out, and difficulty breathing at rest.

Pulmonary hypertension is not necessarily related to high blood pressure, although it can be. There are several reasons for pulmonary hypertension, including genetics, and sometimes no reason for PH can be discerned. No matter, this disease can progress rapidly from mild to severe, and it is not a diagnosis to be toyed with. This is another reason for physicians not to make this diagnosis lightly or flippantly, and to be sure to follow up on any high pressure readings in the right heart with the RHC.

Last, but not the least, I would be remiss not to mention, again, the excellent website, Pulmonary Hypertension Association, http://www.phassociation.org/. Please do not hesitate to visit this excellent site. Their members have a wealth of experiences and advice to share, and they are most caring. This site also maintains a list of PH physicians. This is where I found my most excellent PH specialist, Dr. Steven Trombold, in Wichita, KS. Further information directly quoted from this website explains about physicians who specialize in this area:

"Doctors who treat pulmonary hypertension are PH specialists, and they have undergone extensive study and special training to treat PH. Pulmonary hypertension is an uncommon but serious disease, and most generalists have little training or experience in PH treatment. Therefore, patients are encouraged to see a PH specialist. These doctors have had extensive training and will provide you with the best care possible."

If this article even saves one person from a misdiagnosis and causes even one physician to investigate my claims and correct their rampant misuse of the term pulmonary hypertension, then it will all be worth it.