ATRIAL FLUTTER AND ABLATION
I believe my history of a rather unusual type of heart disease and its subsequent course presents an interesting picture of how medicine has evolved in recent years, certainly a far cry from when I first started practicing medicine in 1956.
In 2004 I was diagnosed with a genetic form of heart disease, Myxomatous Degeneration of the Mitral Valve. The event leading to the diagnosis was the sudden onset of shortness of breath while on a snow shoeing trip. An echocardiogram at that time showed an enlarged heart caused by severe Mitral regurgitation related to Myxomatous degeneration of the posterior leaflet of the Mitral valve and the rupture of a chordae tendonae, a tendon connecting the leaflet to the heart muscle which helps open the valve on filling of the left ventricle. Actually, it was the tendon rupture which accentuated the Mitral regurgitation that precipitated the acute episode
Open heart surgery followed shortly thereafter at the Texas Heart Institute in Houston where they repaired the Mitral Valve and inserted rings into both the Mitral and Tricuspid valve orifices to accommodate the smaller leaflet on the left side.
Post surgery, Dick was left with an abnormal rhythm, atrial flutter, for which he had a cardioversion several months later in Durango, CO. Then followed a quiescent period of two years but with a reduced capacity for exercise and with a normal heart rhythm but with frequent extra-systoles (extra heart beats) which were treated with medication.
In 2006 atrial flutter recurred after a short stint of running (my last attempt to return to running). One cardiologist actually thought I was in ventricular tachycardia (I didn’t think so as I felt pretty good) so she called for an ambulance and I was rushed to the OHSU hospital ER. There one of my cardiologists thought it was atrial flutter and he massaged my carotid sinus and the flutter stopped for a short time (but confirmed that it was a. flutter) but it recurred. At this time they decided to put in an ICD (intracardiac device, pacemaker/defibrillator) and they did a right heart catheterization to ablate the source of the a. flutter in the right artrium which was successful.
Now to the present time some two years later in the fall of 2008. The atrial flutter recurred two weeks ago for unknown reasons. My primary cardiologist, who is a well known electrophysiologist, Dr. Jack Kron, recommended that I have another ablation to stop the flutter. He warned me ahead of time that he thought the flutter might be originating from the left atrium but they were going in on the right, safer side anyway. On doing the procedure they were able to map out the electrical pathway that occurs in the heart that instigates the contraction of the several chambers of the heart. He was correct in that the mapping showed the locus for the a. flutter originated in the left atrium. A point of interest that I didn’t know before, and related by another cardiologist later, is that a. flutter originates in the atria of the heart while atrial fibrillation originates from the area of the pulmonary vein which empties blood from the lung into the left atrium.
During the procedure, they could have penetrated the septum between the two atria from the right side to enter the left heart with the catheter but decided not to do so because of the danger involved. So they did the next best thing. The a. flutter begins in the left atrium and then follows a circuitous path over to the right side. They ablated or cauterized (burned) several areas in the right atrium to break the circuit and then they cardioverted my heart to break the rhythm. So, now I am back in a normal sinus rhythm; however, they are not sure how long it will hold. When, and if, the a. flutter recurs, we will then decide whether or not to ablate the locus of the rhythm by catheterization from the left side. In any case, I am now feeling better.
Unfortunately, they had another bit of news from the procedure. They indicated that I had a lot of scarring of the electrical pathways on the right side of the heart. Dr. Kron left on vacation right after the procedure so I haven’t had a chance to talk to him yet but got the above information from one of his partners. They don’t know the source of the scarring but thought it might have been from my prior open heart surgery in 2004. I think that is unlikely as all they did on the right side of my heart at that time was to put in a Duran ring around the Tricuspid valve, as they had placed one around the Mitral valve, too, making both openings smaller to accomodate the smaller posterior leaflet after removing the middle degenerated portion. In any case, they should have seen this scarring when they did the ablation on the right side back in 2006. I await the return of Dr. Kron to get a better explanation of the source of this scarring.
So, what is turning out to be a saga, continues, so stay tuned for the next episode.
Thursday, December 4, 2008
Monday, November 10, 2008
Peripheral Neuropathy Associated with Gammopathy
Peripheral neuropathies are not that rare, the most common cause being Diabetes Mellitus. I have had a neuropathy for 25 years and until recently its cause to me was unknown. The fact that it appeared to be progressing since the beginning of this year prompted me to have it investigated further.
In 1983 I noticed slight numbness on the sole of my left foot. I had been running, using just my forefeet, not the usual heel-to-toe landing, because of a painful knee subsequent to a leg fracture. This running style cushioned my landing and muted pain in the knee. I attributed the numbness to the pounding from running 40 plus miles every week. Then in 1984 numbness appeared in my right sole, too, which made it unlikely that running was the cause.
I saw a neurologist at that time and he did the usual EMG and nerve conduction studies (NCS) which were completely negative. Blood studies were also unrevealing. I didn’t have diabetes, I had no family history to suggest a genetic basis for the disease, I drank little that might suggest an alcoholic basis and the blood studies ruled out heavy metal and other poisonings. I was diagnosed with a neuropathy of unknown etiology. I had no further investigation for some time thereafter. I continued to run high mileage for the next 6 years and I noticed no significant progression of the numbness which was my only symptom
In 1999 I developed Polymyalgia Rheumatica (PMR), a muscular disease, which in my case involved pain in the muscles of the shoulder girdle. This was quite severe but fortunately it responded to a course of steroids (Prednisone). It persisted to a low degree for a year and then cleared completely. However, during that year I noted onset of slight numbness in the fingers of both hands. I checked the literature and discovered that this could occur with PMR so I did not have it investigated. Now in 2008, some 25 years from first onset, I noticed that the numbness in my fingers was progressing which finally prompted me to see a neurologist.
I had both an EMG and NCS and this time the latter was abnormal, showing damage to both the myelin sheath (covering of the nerve fiber or axon) as well as axonal damage, both in my feet and hands. My neurologist thought this unusual to see both types of damage so she referred me for a repeat NCS which showed the same findings.
At this time I related to my neurologist that I had noticed, particularly in the past year, unsteadiness in my gait. She related that the vibratory sense in my feet was reduced, and this, along with the numbness, was the likely cause of this new symptom; simply stated, I couldn’t tell where my feet were without looking.
I had laboratory studies which were unremarkable except for two findings; I had very elevated immunoglobulins, IgG and IgM, both being more than a thousand above normal limits. I was told that this signified that I had a Gammopathy. The incidence is 1% of the population over age 50 and 3% over the age of 70. The majority of these are termed macroglobhulinemia, unspecified, or MGUS, and in most instances this is benign. However 10% develop a peripheral neuropathy which is where I fall. What happens is that the anti-MAG (Myelin Associated Glycoprotein) antibodies in the IgM attach to the myelin sheath of the nerves and destroy it. I am not sure what destroys the axon, perhaps the antibody in the IgG. They can measure the anti-MAG antibody, itself, and my level was high as you would expect.
After I had digested all this information, the next logical question to ask was what about treatment? Initially I was told by my two neurologists that, inasmuch as my disease had been progressing slowly, I might be better off not having treatment as I would probably die before it got bad enough to really bother me. Ha, Ha (a little gallows humor)! But what bothers me most is having an unsteady gait and the fear of falling, such as on going up and down stairs, especially if there is no railing.
My hematologist, who had done the lab studies, called me to come in to discuss my situation. He had talked to my neurologists (the docs at OHSU are really good and caring). He recommended that I consider a new treatment that was just getting underway around the country; however, there were yet no completed studies but the results coming in were promising. Rituxan is a monoclonal antibody that is being used in the treatment of breast and colon cancer. It reduces the antibodies produced by the plasma cells in the bone marrow, including IgG and IgM which should then reduce their attack on the nerve cells. The effect is not permanent but so far it has been shown to last at least a year in most cases. If IgG and IgM go up in the future, I can have another series of treatments.
So, this Wednesday I go in for my first infusion of Rituxan which will take 5 – 6 hours I am told. I will then have 3 more infusions a week apart but these will be faster, taking only 2-3 hours. I told Patti to bring a book along to keep me company and I think I will bring a Sudoku with me
The Rituxan is not like the chemotherapies they use for cancer treatment. It has some side effects but not like the former. Incidentally, the cost of the treatment is $20,000. Thank goodness for Medicare.
In 1983 I noticed slight numbness on the sole of my left foot. I had been running, using just my forefeet, not the usual heel-to-toe landing, because of a painful knee subsequent to a leg fracture. This running style cushioned my landing and muted pain in the knee. I attributed the numbness to the pounding from running 40 plus miles every week. Then in 1984 numbness appeared in my right sole, too, which made it unlikely that running was the cause.
I saw a neurologist at that time and he did the usual EMG and nerve conduction studies (NCS) which were completely negative. Blood studies were also unrevealing. I didn’t have diabetes, I had no family history to suggest a genetic basis for the disease, I drank little that might suggest an alcoholic basis and the blood studies ruled out heavy metal and other poisonings. I was diagnosed with a neuropathy of unknown etiology. I had no further investigation for some time thereafter. I continued to run high mileage for the next 6 years and I noticed no significant progression of the numbness which was my only symptom
In 1999 I developed Polymyalgia Rheumatica (PMR), a muscular disease, which in my case involved pain in the muscles of the shoulder girdle. This was quite severe but fortunately it responded to a course of steroids (Prednisone). It persisted to a low degree for a year and then cleared completely. However, during that year I noted onset of slight numbness in the fingers of both hands. I checked the literature and discovered that this could occur with PMR so I did not have it investigated. Now in 2008, some 25 years from first onset, I noticed that the numbness in my fingers was progressing which finally prompted me to see a neurologist.
I had both an EMG and NCS and this time the latter was abnormal, showing damage to both the myelin sheath (covering of the nerve fiber or axon) as well as axonal damage, both in my feet and hands. My neurologist thought this unusual to see both types of damage so she referred me for a repeat NCS which showed the same findings.
At this time I related to my neurologist that I had noticed, particularly in the past year, unsteadiness in my gait. She related that the vibratory sense in my feet was reduced, and this, along with the numbness, was the likely cause of this new symptom; simply stated, I couldn’t tell where my feet were without looking.
I had laboratory studies which were unremarkable except for two findings; I had very elevated immunoglobulins, IgG and IgM, both being more than a thousand above normal limits. I was told that this signified that I had a Gammopathy. The incidence is 1% of the population over age 50 and 3% over the age of 70. The majority of these are termed macroglobhulinemia, unspecified, or MGUS, and in most instances this is benign. However 10% develop a peripheral neuropathy which is where I fall. What happens is that the anti-MAG (Myelin Associated Glycoprotein) antibodies in the IgM attach to the myelin sheath of the nerves and destroy it. I am not sure what destroys the axon, perhaps the antibody in the IgG. They can measure the anti-MAG antibody, itself, and my level was high as you would expect.
After I had digested all this information, the next logical question to ask was what about treatment? Initially I was told by my two neurologists that, inasmuch as my disease had been progressing slowly, I might be better off not having treatment as I would probably die before it got bad enough to really bother me. Ha, Ha (a little gallows humor)! But what bothers me most is having an unsteady gait and the fear of falling, such as on going up and down stairs, especially if there is no railing.
My hematologist, who had done the lab studies, called me to come in to discuss my situation. He had talked to my neurologists (the docs at OHSU are really good and caring). He recommended that I consider a new treatment that was just getting underway around the country; however, there were yet no completed studies but the results coming in were promising. Rituxan is a monoclonal antibody that is being used in the treatment of breast and colon cancer. It reduces the antibodies produced by the plasma cells in the bone marrow, including IgG and IgM which should then reduce their attack on the nerve cells. The effect is not permanent but so far it has been shown to last at least a year in most cases. If IgG and IgM go up in the future, I can have another series of treatments.
So, this Wednesday I go in for my first infusion of Rituxan which will take 5 – 6 hours I am told. I will then have 3 more infusions a week apart but these will be faster, taking only 2-3 hours. I told Patti to bring a book along to keep me company and I think I will bring a Sudoku with me
The Rituxan is not like the chemotherapies they use for cancer treatment. It has some side effects but not like the former. Incidentally, the cost of the treatment is $20,000. Thank goodness for Medicare.
Saturday, October 11, 2008
Lucentis: a New Miracle Drug
Those of us with Macular Degeneration (MD) have wondered when we would lose our useful vision; not if we would, but when, as this is the typical progression of the "wet type" of this disease.
In my particular case, I developed wet MD in 1999 at the age of 70. My father had it and went virtually blind in his mid 70s. Being genetic in origin, I knew I was a prime candidate to develop this disease. Back then, the only treatment was by Laser and after five treatments over a year period my wet MD was arrested but my vision was now 20/400 so I was statutorily blind in the left eye. Fortunately, I retained 20/20 vision in my right eye with no signs of MD on this side.
Fast forward seven years to the fall of 2006. While on a river cruise in Europe, I noticed one day a dimming of vision in my right, good eye on looking at a computer screen. On returning home I immediately contacted my retinologist who confirmed that I indeed had developed wet MD in my right eye. He recommended starting Laser tratment. Like many of us with MD I had kept abreast of research on MD and was familiar with the on-going studies involving both Avastin and Lucentis. On questioning my retinologist I was told that I was not a candidate for any of the new drugs but he could give me no good reason for this conclusion.
Knowing that I would likely lose all useful vision in my right eye, as had happened on the left with the Laser treatment, I opted for a second opinion. Searching Google, I discovered the Casey Eye Institute at the Oregon Health & Science University (OHSU) in Portland, one of the oldest and best eye clinics in the country. Their I met Dr. Andreas Lauer who would become my new retinologist. He explained that their center had been involved in the research studies on Lucentis. It had just been approved for commercial use by the FDA in June 2006 for the treatment of wet MD. They were presently using it almost to the exclusion of any other treatment including the old Laser treatment. He indicated that I would be an excellent candidate for use of Lucentis. I had my first injection of Lucentis into my eyeball that day. The procedure is a little daunting the first time but Dr. Lauer was clearly experienced and the whole procedure from prepping to the end took no more than five minutes. Within a week I noticed that my vision was improving. I returned for a second injection in three weeks and then a third injection four weeks later. By then my vision had returned to 20/20 and I noticed no visual defects on this side. I then skipped a month with no injection but then had two more injections a month apart. Thereafter, there was no evidence of any activity with no swelling of the macula from hemorrhage or edema evident on either the ocular CT scan or on visual examination.
Then another decision had to be made. I could continue to have monthly injections of Lucentis for the rest of my life which would most likely preclude the onset of any further wet MD or I could be followed at 4 - 6 week intervals and have no treatment until further wet MD developed. The Casey Eye Institute is participating in studies that include both protocols. I opted to have no further injections unless the wet MD reappeared. Fortunately, I have now had twelve months of quiescence and have a checkup every six weeks.
My only other treatment has been to take two Preservision capsules (Bausch & Lomb) a day as recommended by Dr. Lauer. This is an antioxidant vitamin (A, C & E) and mineral (Zinc & Copper) supplement that was shown to be clinically effective in the Age Related Eye Disease Study (AREDS) conducted by the National Eye Institute which is a part of the National Institutes of Health (NIH).
Now, a little background on the history of Lucentis. Genentech, a biopharmaceutical company, has been working for several decades on the biochemistry of Vascular Endothelial Growth Factor (VEGF), a normally occurring chemical in the body which initiates and stimulates the growth of blood vessels. It is present in tumors, in particular colon cancer, where it stimulates the growth of the tumor. Genentech developed an anti-vegf drug, Avastin, which has been effective in treating colon cancer by blocking the development of new blood vessels in the tumor.
We know that in wet MD the predominant lesion is the growth of new blood vessels which are fragile and burst causing hemorrhage and edema characteristic of the lesion. The process is caused by a VEGF. In wet MD the treatment is the same as for a tumor: initiate an antii-vegf regimen. Initially Avastin was used successfully in the treatment of wet MD. Genentech then reasoned that a smaller molecule than Avastin might better penetrate the retina and give better results; thus the creation of Lucentis. Avastin is still used by some physicians for the treatment of wet MD. Its big advantage is that it is much cheaper than Lucentis. To date, the results of treatment with either drug look very similar. However, the FDA has not approved the use of Avastin for the treatment of wet MD. Presently studies are underway to determine the efficacy of the two drugs. The Casey Eye Institute is participating in these studies.
The purpose of this blog is to inform readers of the status of research and treatment in the wet type of Macular Degeneration. My retinologist indicated that it may take as long as two years for all physicians in the country to begin using these two drugs. It would be a travesty today if someone became blind for having no knowledge of these new drugs, or worse, if their physician did not make them aware of the existence, avalability and treatment options of these drugs.
One last thought, I have read several blogs indicating that not all insurers will cover the expense of Lucentis. Medicare covers my expense which is $2500 just for the medicine alone. I believe that Avastin is about one-tenth that cost; however, it does not have FDA approval which may influence some insurers. It is my hope that no one will be denied anti-vegf treatment because of cost.
Note: The opinions in this blog are solely those of the author and are not meant as recommendations for treatment. Patients should consult their physician for diagnosis and treatment.
A series of blogs are planned on Atherosclerosis and Coronary Artery Disease.
Macular Degeneration will be updated as new developments occur.
Richard W. Blide, M.D.
Those of us with Macular Degeneration (MD) have wondered when we would lose our useful vision; not if we would, but when, as this is the typical progression of the "wet type" of this disease.
In my particular case, I developed wet MD in 1999 at the age of 70. My father had it and went virtually blind in his mid 70s. Being genetic in origin, I knew I was a prime candidate to develop this disease. Back then, the only treatment was by Laser and after five treatments over a year period my wet MD was arrested but my vision was now 20/400 so I was statutorily blind in the left eye. Fortunately, I retained 20/20 vision in my right eye with no signs of MD on this side.
Fast forward seven years to the fall of 2006. While on a river cruise in Europe, I noticed one day a dimming of vision in my right, good eye on looking at a computer screen. On returning home I immediately contacted my retinologist who confirmed that I indeed had developed wet MD in my right eye. He recommended starting Laser tratment. Like many of us with MD I had kept abreast of research on MD and was familiar with the on-going studies involving both Avastin and Lucentis. On questioning my retinologist I was told that I was not a candidate for any of the new drugs but he could give me no good reason for this conclusion.
Knowing that I would likely lose all useful vision in my right eye, as had happened on the left with the Laser treatment, I opted for a second opinion. Searching Google, I discovered the Casey Eye Institute at the Oregon Health & Science University (OHSU) in Portland, one of the oldest and best eye clinics in the country. Their I met Dr. Andreas Lauer who would become my new retinologist. He explained that their center had been involved in the research studies on Lucentis. It had just been approved for commercial use by the FDA in June 2006 for the treatment of wet MD. They were presently using it almost to the exclusion of any other treatment including the old Laser treatment. He indicated that I would be an excellent candidate for use of Lucentis. I had my first injection of Lucentis into my eyeball that day. The procedure is a little daunting the first time but Dr. Lauer was clearly experienced and the whole procedure from prepping to the end took no more than five minutes. Within a week I noticed that my vision was improving. I returned for a second injection in three weeks and then a third injection four weeks later. By then my vision had returned to 20/20 and I noticed no visual defects on this side. I then skipped a month with no injection but then had two more injections a month apart. Thereafter, there was no evidence of any activity with no swelling of the macula from hemorrhage or edema evident on either the ocular CT scan or on visual examination.
Then another decision had to be made. I could continue to have monthly injections of Lucentis for the rest of my life which would most likely preclude the onset of any further wet MD or I could be followed at 4 - 6 week intervals and have no treatment until further wet MD developed. The Casey Eye Institute is participating in studies that include both protocols. I opted to have no further injections unless the wet MD reappeared. Fortunately, I have now had twelve months of quiescence and have a checkup every six weeks.
My only other treatment has been to take two Preservision capsules (Bausch & Lomb) a day as recommended by Dr. Lauer. This is an antioxidant vitamin (A, C & E) and mineral (Zinc & Copper) supplement that was shown to be clinically effective in the Age Related Eye Disease Study (AREDS) conducted by the National Eye Institute which is a part of the National Institutes of Health (NIH).
Now, a little background on the history of Lucentis. Genentech, a biopharmaceutical company, has been working for several decades on the biochemistry of Vascular Endothelial Growth Factor (VEGF), a normally occurring chemical in the body which initiates and stimulates the growth of blood vessels. It is present in tumors, in particular colon cancer, where it stimulates the growth of the tumor. Genentech developed an anti-vegf drug, Avastin, which has been effective in treating colon cancer by blocking the development of new blood vessels in the tumor.
We know that in wet MD the predominant lesion is the growth of new blood vessels which are fragile and burst causing hemorrhage and edema characteristic of the lesion. The process is caused by a VEGF. In wet MD the treatment is the same as for a tumor: initiate an antii-vegf regimen. Initially Avastin was used successfully in the treatment of wet MD. Genentech then reasoned that a smaller molecule than Avastin might better penetrate the retina and give better results; thus the creation of Lucentis. Avastin is still used by some physicians for the treatment of wet MD. Its big advantage is that it is much cheaper than Lucentis. To date, the results of treatment with either drug look very similar. However, the FDA has not approved the use of Avastin for the treatment of wet MD. Presently studies are underway to determine the efficacy of the two drugs. The Casey Eye Institute is participating in these studies.
The purpose of this blog is to inform readers of the status of research and treatment in the wet type of Macular Degeneration. My retinologist indicated that it may take as long as two years for all physicians in the country to begin using these two drugs. It would be a travesty today if someone became blind for having no knowledge of these new drugs, or worse, if their physician did not make them aware of the existence, avalability and treatment options of these drugs.
One last thought, I have read several blogs indicating that not all insurers will cover the expense of Lucentis. Medicare covers my expense which is $2500 just for the medicine alone. I believe that Avastin is about one-tenth that cost; however, it does not have FDA approval which may influence some insurers. It is my hope that no one will be denied anti-vegf treatment because of cost.
Note: The opinions in this blog are solely those of the author and are not meant as recommendations for treatment. Patients should consult their physician for diagnosis and treatment.
A series of blogs are planned on Atherosclerosis and Coronary Artery Disease.
Macular Degeneration will be updated as new developments occur.
Richard W. Blide, M.D.
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