hypertrophic cardiomyopathy

Hypertrophic Cardiomyopathy Association

January 14, 2010 By Larry Creswell, MD 2 Comments

I got a kind email yesterday from Lisa Salberg, the Executive Director of the Hypertrophic Cardiomyopathy Association (HCMA) and thought I’d share some information here at the blog.

I actually met Lisa a couple years ago at a medical conference here in Jackson that was devoted to the topic of sudden death in athletes. My interest in the various issues regarding athletes and heart disease was actually stimulated by that conference.

The HCMA is a non-profit organization that is devoted to providing information and advocacy for patients with hypertrophic cardiomyopathy (HCM), their families, and their physicians. Their website, www.4hcm.org, is a great source of easy-to-read, practical information about HCM and would be a valuable resource for both athletes and non-athletes. Please take a moment to visit their website.

I’ll add a link to HCMA in my list of resources.

Reggie Lewis, NBA player, 1965-1993

December 28, 2009 By Larry Creswell, MD Leave a Comment

Reggie Lewis, a superstar NBA player with the Boston Celtics, died at the age of 27 on July 27, 1993. He collapsed suddenly during an off-season practice at Brandeis University and received bystander CPR, but was pronounced dead at a local hospital a couple hours later. The cause of his death was attributed to hypertrophic cardiomyopathy (HCM), a condition characterized by hypertrophy (enlargement) of the heart walls and a susceptibility to potentially lethal arrhythmias (abnormal heartbeat).

There’s an interesting twist to Lewis’s story, though. It turns out that 3 months earlier, he passed out during an NBA playoff game against the Charlotte Hornets on April 29, 1993. He was evaluated by a prominent group of cardiologists at the New England Baptist Hospital and was diagnosed with HCM. Those doctors admitted that there was often some difficulty in distinguishing HCM from the “athlete’s heart syndrome,” the normal enlargement of the heart due to cardiovascular training, but they recommended that Lewis no longer play basketball.

Lewis sought additional opinions, first from physicians at the Brigham & Women’s Hospital in Boston and later at UCLA. The cardiologists at Brigham & Women’s Hospital felt that Lewis did not have HCM, but rather had collapsed from a relatively harmless condition known as neurogenic syncope. The doctors at UCLA weren’t able to reach a definitive diagnosis.

We can understand why any professional athlete would be conflicted with the recommendation that he no longer play, but as we know now, the result was tragic.

This story illustrates the common scenario of sudden death in athletes who, unknowingly, have HCM….and illustrates the common problem that HCM can be difficult to distinguish from “athlete’s heart syndrome.”

Hypertrophic Cardiomyopathy (HCM)

October 13, 2009 By Larry Creswell, MD 5 Comments

I received an email inquiry yesterday from a reader who was interested in hypertrophic cardiomyopathy (HCM), a newly-diagnosed condition which had sidelined him from the sport that he loves. I thought I’d share some information about HCM that I shared with him yesterday. This is an uncommon problem, but it is a potentially lethal problem for athletes in sports with high cardiovascular demands.

What is HCM?

We use the terms hypertrophic cardiomyopathy (HCM) when there is thickening (that we call hypertrophy) of the ventricular (lower heart chamber) walls, if there is no other explanation. This disorder has been known by several other names in recent years, including hypertrophic obstructive cardiomyopathy (HOCM) and idiopathic hypertrophic subaortic stenosis. There are several patterns, but in the most common situation the septum (that divides the left and right ventricles) enlarges to >1.3 cm, while the other ventricular walls remain

In approximately 35% of cases of HCM, there is also obstruction to the flow of blood as it leaves the heart. This obstruction can be due to the thickening of the ventricular septum or to a condition known as systolic anterior motion of the mitral valve (SAM). With SAM, the mitral valve moves forward and gets in the way of blood that is heading toward the aortic valve to leave the heart.

Symptoms

Many individuals with HCM will have no symptoms at all. Some patients report instances of chest pain, with or without exertion, shortness of breath, palpitations, or syncope (blacking out), either during exertion or afterwards.

Role in Sudden Death

In the United States, HCM is probably the leading cause of death among athletes with sudden cardiac death. In most reports, HCM is found at autopsy in as many as 40% of young athletes with sudden death. Unfortunately, sudden death in individuals with HCM tends to occur in young people with no previous warning signs, who are engaged in moderate to strenuous physical activity.

Diagnosis

The diagnosis of HCM is established with an echocardiogram (an evaluation of the heart’s structure using ultrasound). The echocardiogram makes detailed pictures of the various heart walls and heart valves and the thickness of the various heart walls can be measured. The echocardiogram can also be used to estimate the degree of obstruction to blood flow exiting the heart.

Treatment

The treatment for HCM will depend on many variables that are specific to the individual patient. In general, treatment is designed to: 1) treat (or prevent) heart failure that may arise and 2) prevent sudden death. Potential treatments include medications, surgery (to remove some of the thickened heart wall or to replace the mitral valve), injection of alcohol into the heart wall (alcohol ablation) to cause it to shrink, insertion of a pacemaker, or insertion of a defibrillator (that delivers a shock to restart the heart in the event of sudden death).

Distinguishing from “Athlete’s Heart”

For athletes, there can sometimes be confusion about the diagnosis of HCM. This stems from the fact that well-trained athletes often have thickening of the ventricular walls that is simply a physiologic consequence of training. The distinction between HCM and athlete’s heart can be particularly problematic for individuals with ventricular wall thickness between 1.0 and 1.5 cm. Athletes in this situation would be well-served by consultation with a cardiologist with particular expertise in this area. Features that would favor athlete’s heart over HCM include: left ventricular cavity size >55 mm in diameter, and decrease in wall thickness with deconditioning. Features that would favor HCM include: family history of HCM, abnormal ECG, left ventricular cavity

Recommendations for Athletes

Consensus panels have convened to make recommendations for athletes with HCM. Athletes with HCM who are younger than 30 years old should not participate in sports with high cardiovascular demands. Athletes with HCM who are older than 30 years old should not participate in such sports if they have any other worrisome features of the disease: ventricular arrhythmias, syncope (blacking out spells), moderate or worse obstruction to ventricular outflow (such as with systolic anterior motion of the mitral valve), intermittent atrial arrhythmias, or enlarged left atrium. Older athletes HCM who do not have these features should consult with their cardiologist about continued participation.