Dear waukon-ga,
Thanks for your question. Congrats on your upcoming study at MIT, and
condolances on your recent diagnosis with HCM. I lived in the Boston
area for over 15 years and worked at most of the Harvard hospitals
over the years, and have some familiarity with the medical environment
there. Many superb resources certainly exist there, with some great
centers (such as Tufts), sometimes living in the shadow of better
known academic centers in the area.
As you'll soon be in the Cambridge / Boston area, I will focus on the
many excellent resources in this area. You are right to wonder about
the distinction between good researchers and good physicians regarding
HCM. My experience in medicine has been that MDs or MD/PhDs who also
participate in research tend to be more in tune with recent clinical
developments and treatment techniques. They also often tend to be
more likely to practice evidence-based medicine (medicine based on
scientific analysis of interventions and outcomes). Also, as you
correctly surmise, the US News and similar rankings of medical centers
are too course a measure by which to judge what type of health care
one will receive for a given condition. At any medical center, there
are good and bad physicians. Those ranked highly by US News tend to
be strong on research, but they also tend not to suffer incompetent
physicians for very long either.
The complication rates for physicians are not usually documented or
publicly known. The only exceptions are usually published results of
clinical case series, which may not accurately reflect how good an
individual surgeon is at any given time, since they typically pool
together results from one physician over many years or multiple
physicians from a center or across the country. One measure of
physician competence is what number and type of actions have been
taken against them by their state Board of Medicine. It should be
noted, however, as stated at the Massachusetts Board site, that
"[s]ome studies have shown that there is no significant correlation
between malpractice history and a doctor's competence." This does not
seem to inhibit lawsuits, however, but that, perhaps, is the topic for
another day.
================================================
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Physicians
=========
First, the HCMA website you mention is a useful general resource.
Along with many physicians from Mayo, UCLA, and other institutions,
several physicians on the HCMA Board of Advisors practice in the
Boston area:
Dr. Christine Seidman - Brighams & Woman's Hospital / Harvard Medical
School, Boston, MA
Provider Name: Christine Edry Seidman, M.D.
Address: Harvard Medical School, Department of Genetics
77 Avenue Louis Pasteur NRB
Boston, MA 02115
Phone: (617) 732-4837
Department/Affiliations: Medicine, Cardiology
http://www.brighamandwomens.org/mdSearch/MedicalProfessionalDetail.asp?MPR=4432&N=Christine%20Edry%20Seidman,%20M.D.&D=Medicine&OP=2582&NP=Yes&L=No%20Data%20Available&PT=Specialist
cseidman@genetics.med.harvard.edu
Dr. Seidman's Medical Board profile can be found here:
http://profiles.massmedboard.org/Profiles/MA-Physician-Profile-View-Doctor.asp?Brn=56123
___________________________________________________
Dr. Martin Maron - New England Medical Center/TUFTS, Boston, MA
Dr. Maron works out of the NEMC HCM Center and was recently the lead
author of an article in the New England Journal of Medicine - one of
the top clinical journals in the world.
For some reason, Dr. Maron is not listed in the NEMC physician
directory. He is listed, however, in the Massachusetts Board of
Registration:
http://profiles.massmedboard.org/Profiles/MA-Physician-Profile-View-Doctor.asp?Brn=213784
Tufts New England Medica
750 Washington Street
BOSTON, MA 02111
(617) 636-8066
___________________________________________________
Dr. James Udelson - New England Medical Center/TUFTS, Boston, MA
Co-director of the NEMC HCM Center. Note that Dr. Udelson's name is
mispelled on the HCMA website as "Udleson."
NEW ENGLAND MEDICAL CTR
750 WASHINGTON ST BOX 70
BOSTON, MA 02111
Phone:
(617) 636-8066
Board of Medicine profile:
http://profiles.massmedboard.org/Profiles/MA-Physician-Profile-View-Doctor.asp?Brn=51223
___________________________________________________
Dr. Mark Link- New England Medical Center/TUFTS, Boston, MA
Co-director of the NEMC HCM Center
http://profiles.massmedboard.org/Profiles/MA-Physician-Profile-View-Doctor.asp?Brn=78045
Dr. N.A. Mark Estes - New England Medical Center/TUFTS, Boston, MA
http://profiles.massmedboard.org/Profiles/MA-Physician-Profile-View-Doctor.asp?Brn=78045
The focus of Drs. Link and Estes' work is in the area of
electrophysiology and pacing. While they may accept patients, they
are more likely to function as subspecialists, handling referrals
from other physicians primarily managing a patient's HCM. Here is his
Board of Medicine profile:
http://profiles.massmedboard.org/Profiles/MA-Physician-Profile-View-Doctor.asp?Brn=78045
___________________________________________________
Dr. Carolyn Ho - Brigham & Woman's Hospital / Harvard Medical School,
Boston, MA
Brigham and Women's Hosp
75 Francis Street, CV Di
BOSTON, MA 02115
(617) 732-5685
http://profiles.massmedboard.org/Profiles/MA-Physician-Profile-View-Doctor.asp?Brn=205472
___________________________________________________
Given that the Tufts NEMC HCM center is the first of its kind in the
northeast and the strength of the members of this group, I would
recommend contacting them first. Here is a little more information
about them:
A press release from 1/22/03 discussing the Tufts NEMC HCM Center,
where several of the above physicians are located, can be found here:
http://www.nemc.org/home/news/pressrel/2003/03012201.htm
This was the first such treatment facility in the northeast. They
stress a multidisciplinary approach to evaluation and treatment.
The main website for the HCM Center can be found here:
http://www.tufts-nemc.org/medicine/card/HypertrophicCardiomyopathyCenter.htm
Appointments as well as any questions concerning a clinic visit can be
made by calling Celeste Webster at 617-636-8066 or email:
cwebster@tufts-nemc.org.
========
Treatment
========
A good, updated overview discussing hypertrophic cardiomyopathy can be
found at eMedicine, written by Michael E Zevitz, MD, Clinical
Assistant Professor, Department of Medicine, Finch University of
Health Sciences, The Chicago Medical School.
http://www.emedicine.com/med/topic290.htm
The page above briefly discusses ablation for HCM, first described in
1995 by Ulrich Sigwart (Sigwart U. Non-surgical myocardial reduction
for hypertrophic obstructive cardiomyopathy. Lancet 1995;346:211-4.):
"Transvenous catheter ablation of the septal region has been performed
using selective arterial ethanol infusion to destroy myocardial
tissue.
The procedure involves infusing 96% ethanol down the first septal
branch of the left anterior descending artery and inducing a
therapeutic infarction of the proximal interventricular septal
myocardium. This leads to a remodeling of the septum, which decreases
the marked septal thickening characteristic of HCM and results in a
decrease of the gradient across the LV outflow tract. In this manner,
the procedure is analogous to a surgical myomectomy, in attempting to
decrease the amount of septal ventricular myocardium and thereby
reducing the LV outflow tract gradient.
The procedure has been used in clinical practice since the early
1990s, and the reported results have been excellent, with significant
reduction symptoms, particularly in the incidence of heart failure.
In many centers, it is the surgical procedure of choice for HCM."
___________________________________________________
A recent New England Journal of Medicine journal article discusses HCM
diagnosis, etiology, and treatment.
Nishimura RA. Holmes DR Jr. Clinical practice. Hypertrophic
obstructive cardiomyopathy.[erratum appears in N Engl J Med. 2004 Sep
2;351(10):1038]. New England Journal of Medicine. 350(13):1320-7, 2004
Mar 25.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=15044643&query_hl=3
With regard to ablation therapy, the article has the following to say:
"Alcohol-induced septal ablation is a newer method of treating
hypertrophic cardiomyopathy. This procedure is performed in the
catheterization laboratory, where 100 percent alcohol is infused
selectively into a septal perforator artery (or branch) that perfuses
the proximal septum, [24,25] producing a controlled myocardial
infarction. The subsequent thinning and remodeling of the basal septal
region decrease obstruction over a period of months. The initial
results from several centers have shown improvements in hemodynamic
variables and symptoms, with a decrease in the outflow gradient from
60 to 70 mm Hg to less than 20 mm Hg. Improved exercise performance
has been documented, but not to the extent that has been shown after
surgery. Initially, complete heart block requiring permanent pacing
occurred in 30 to 40 percent of cases, but in experienced centers
where smaller doses of alcohol were used in combination with
myocardial contrast echocardiography (to localize the area of
myocardium perfused by a septal artery), heart block occurred in fewer
than 15 to 20 percent. Other complications, such as a large myocardial
infarction, ventricular septal defect, intractable ventricular
fibrillation, and myocardial perforation, have been described,
although their incidence is uncertain, in part because these events
are probably underreported.
Although no randomized trials comparing septal ablation with septal
myectomy have been conducted, the rate of complete abolition of
obstruction and relief of symptoms appears to be lower with septal
ablation than with septal myectomy. This difference may be explained
by the highly variable anatomical course of the septal perforator
arteries [26]; up to 20 percent of patients may not have a perforator
artery that supplies the critical area of septal hypertrophy.
Moreover, benefit may not be obtained because coexisting conditions,
such as intrinsic mitral-valve disease, midventricular obstruction, or
fixed subaortic obstruction, may be present; these conditions are
amenable only to operative intervention. [27]"
Numbers in brackets, [ ], refer to references in the article cited above.
___________________________________________________
Another potentially interesting review article was published by the
British journal Heart:
Frenneaux MP. Assessing the risk of sudden cardiac death in a patient
with hypertrophic cardiomyopathy. Heart (British Cardiac Society).
90(5):570-5, 2004 May.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=15084566&query_hl=1
The full text of this article is available free from the link above.
___________________________________________________
The Journal of the American Medical Association also recently
published a review article on HCM:
Maron BJ. Hypertrophic cardiomyopathy: a systematic review. JAMA.
287(10):1308-20, 2002 Mar 13.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=11886323&query_hl=6
I don't know if Dr. Maron from the Minneapolis Heart Institute
Foundation (of the above article) is any relation to the Dr. Maron at
Tufts.
A search of the NIH clinical trials database did not turn up any
clinical trials for HCM that are currently accruing patients outside
of Maryland (only 1 there). If you'd like to search in the future,
you can use this link:
http://clinicaltrials.gov/
___________________________________________________
The American Heart Association discusses alcohol ablation, describing
it as experimental:
http://www.americanheart.org/presenter.jhtml?identifier=4468
"Alcohol ablation is another nonsurgical treatment being developed for
hypertrophic obstructive cardiomyopathy. It involves injecting alcohol
down a small branch of one of the heart arteries to the extra heart
muscle. This destroys the extra heart muscle without having to cut it
out surgically.
People undergoing this procedure usually suffer chest pain during the
alcohol injection. The alcohol can also disrupt normal heart rhythms
and require the insertion of a pacemaker. Alcohol ablation is a
relatively new procedure being performed at only a few specialized
centers in the United States. It's too soon to know whether this
treatment will result in long-term benefit. It's still considered
experimental."
The procedure is generally reserved for those who are poor candidates
for or who wish to avoid open heart surgery.
___________________________________________________
Some additional information can be found at the Cleveland Clinic site for HCM:
http://www.clevelandclinicmeded.com/diseasemanagement/cardiology/hypertrophic/hypertrophic.htm
This site is a little more detailed and quotes a surgical mortality of ~1%.
"At the Cleveland Clinic, most alcohol ablations have been performed
on elderly, suboptimal surgical candidates. We generally prefer that
the septum be between 1.8 cm and 3.0 cm to provide a safety margin; if
the septum is too thick, favorable ablation results may be difficult
to attain. Complications of alcohol ablation include complete heart
block (requiring a permanent pacemaker), a large anterior wall
myocardial infarction, ventricular tachycardia or fibrillation, and
pericarditis. The risk of alcohol ablation include a 2%-4% procedural
mortality rate and a 9%-27% incidence of patients requiring permanent
pacemakers.[11-14] Like septal myectomy, alcohol ablation has not been
shown to improve survival due to the lack of randomized controlled
trials and a suitable control population. However, septal myectomy
does result in both short-term and long-term significant decreases in
the LVOT gradient as well as a significant improvement in New York
Heart Association classification. [9,12] In 3-month follow-up data,
Qin, et al., reported a decrease in LVOT gradient from 64 mm Hg to 28
mm Hg and an improvement in NYHA class from 3.5 to 1.9 after alcohol
ablation. [9]"
They go on to give a summary of the current state of treatment of HCM:
"Surgical outcomes for HCM are excellent; operative mortality is
generally less than 2% for septal myectomy. As described previously,
most patients report improvement in their symptoms. Outcomes for
alcohol ablation are more limited, with follow-up averaging three to
five years, as compared with decades for myectomy. At three-month
follow-up, both myectomy and alcohol ablation are effective in
improving symptoms and reducing LVOT gradients, but myectomy results
in larger improvements in LVOT gradients. [9] Alcohol ablation is a
promising therapeutic option for HCM, with a major advantage being its
less invasive nature. However, presently, septal myectomy remains the
preferred treatment of choice for most HCM patients."
The numbers in brackets, [ ], refer to references at the end of the
above article. The article includes a total of 18 references.
___________________________________________________
The following article, published in the North Carolina Medical
Journal, also discusses the current state of alcohol ablation for HCM:
Charles E. Mayes, Jr, MD, Andrew Wang, MD, John J. Warner, MD, Richard
A. Krasuski, MD, Katherine B Kisslo, RDCS, Thomas M. Bashore, MD, J.
Kevin Harrison, MD. Alcohol Ablation of the Interventricular Septum
in Symptomatic Patients with Hypertrophic Obstructive Cardiomyopathy.
NCMJ, Volume 63 Number 3. May/June 2002.
://www.google.com/url?sa=U&start=12&q=http://www.ncmedicaljournal.com/may-june-02/ar050203.pdf&e=9707
===============
Pressure gradients
===============
With regard to pressure gradients, the eMedicine article cited above
discusses the doppler ultrasound findings typically identified in HCM:
"The hallmark of HCM associated with a pressure gradient is the
abnormal systolic motion of the anterior leaflet of the mitral valve
(ie, systolic anterior motion) and, in rare cases, the systolic motion
of the posterior leaflet. Three explanations for the systolic anterior
motion of the mitral valve have been offered, as follows:
The mitral valve is pulled against the septum by contraction of the
papillary muscles, which occurs because of the valve's abnormal
location and septal hypertrophy altering the orientation of the
papillary muscles.
The mitral valve is pushed against the septum because of its abnormal
position in the outflow tract.
The mitral valve is drawn toward the septum because of the lower
pressure that occurs as blood is ejected at high velocity through a
narrowed outflow tract (Venturi effect)."
The last paragraph touches on why there is a pressure gradient - it
takes a lot of force to overcome the outlet obstruction present in
HCM, but once this is overcome, there is relatively little resistance,
resulting in a high fluid velocity across the aortic valve. The
pressure gradient occurs between the left ventricle and the aorta.
More details on the 2D ultrasound findings typically seen in HCM are
discussed in the remainder of the eMedicine article above.
Although the article is primarily discussing valvular aortic stenosis,
you can find a good discussion of trans-aortic gradients here:
://www.google.com/url?sa=U&start=3&q=http://www.utmem.edu/cardiology/articles/quantification%2520of%2520valvular%2520AS%2520ACCJR03.pdf&e=9707
___________________________________________________
===
Etc.
===
Another potentially useful, interesting, and free resource is the
National Guideline Clearinghouse archive. The Archive includes
current management recommendations for a large number of diseases,
including HCM. Their 27 page HCM guideline document can be found
here:
http://www.guideline.gov/summary/summary.aspx?doc_id=4767&nbr=3447&string=hypertrophic+AND+cardiomyopathy
The PDF version is easier to read and is here:
http://www.escardio.org/NR/rdonlyres/D2085338-DDC0-4A7E-B4B5-F1F520DB5140/0/HCM_281003.pdf
This is a detailed overview of HCM, including genetic etiology,
diagnosis, and treatment.
___________________________________________________
As a student at MIT, I believe you will have access to the Harvard
medical library (Countway), which will have about any journal article
you wish to access, if these are not yet freely available online. The
library, like the medical school is located in Boston, in the Longwood
medical area, behind the main medical school building (to the left as
your facing the common). You should also have access to the Ovid
search tool to dig through medical references in the PubMed NIH
medical database. For details of how to go about this, you should
visit your libraries website:
http://libraries.mit.edu/
I hope this information was useful. Feel free to request clarification.
Best of luck in your studies,
-welte-ga |
,
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