Hartzell Schaff, M.D., Cardiovascular Surgery, Mayo Clinic: I'm Hartzell Schaff. I'm a cardiac surgeon in Mayo Clinic, Rochester. Hypertrophic Cardiomyopathy is a congenital heart problem in which there's hypertrophy, or a thickening, of the left ventricle. Patients who have hypertrophic cardiomyopathy can present with shortness of breath, chest pain, or in some cases with syncope or dizzy spells. In general, patients who are symptomatic with hypertrophic cardiomyopathy are first managed by the cardiologist with medicines, primarily beta blockers. In some patients, disopyramide is used to try to improve their symptoms. However, in many patients, the medicines are not successful in relieving the symptoms, and then are referred for surgical myectomy. The patients who are candidates for myectomy are patients who have outflow tract obstruction and there have been many advances in the last 5 to 10 years in the management of patients both surgically and diagnostically. For example, 10 years ago, we didn't appreciate how many patients with hypertrophic cardiomyopathy had obstruction to the outflow of blood and it's the obstructive form of hypertrophic cardiomyopathy that can be helped with surgery. We now know that as many as 60 to 70% of patients with hypertrophic cardiomyopathy have outflow tract obstruction. In some instances, the outflow tract obstruction is not detected until the patients are exercised and have an echocardiogram done during exercise, and it's this activity that brings out the symptoms and brings out the latent obstruction that's present. And these patients are good candidates for surgery, because relief of the obstruction almost always relieves their symptoms.

The operation for a septal myectomy is done through a short skin incision in the upper part of the chest, what we call a median sternotomy or partial median sternotomy. The patients are on the heart-lung machine for a period of 30 minutes, or so, while we work through the aorta to remove muscle from beneath the aortic valve. The amount of muscle that we remove is a little different in each patient, but generally it's about the size of one's thumb and that's done with the heart arrested and on the heart-lung machine. The total length of surgery takes about three to four hours. In general, patients are in the hospital for five to seven days for recovery and then can return to full activities in four to six weeks. Patients who are very symptomatic, with shortness of breath and fatigability, often will notice improvement in their symptoms, even before they go home. And if recovered completely from surgery, they can have a sense of improved perfusion, a sense of improved well being. And we've seen a number of patients who have remarked, even the day or two after surgery, that they can tell a difference in their breathing and in their perfusion immediately after surgery.

There's a second group of patients who we can help with surgery, now, who have hypertrophic cardiomyopathy and this is a little different than the standard surgical myectomy. The second group of patients are those who have apical hypertrophic cardiomyopathy and this is a particular type of hypertrophic cardiomyopathy where the enlargement of the ventricles localized to the apex of heart. Many patients with apical hypertrophic cardiomyopathy have no symptoms and require no treatment, either medical or surgical. But patients who have apical hypertrophic cardiomyopathy and have symptoms due to diastolic dysfunction can be helped with an operation called an apical myectomy. Diastolic dysfunction results from having too small of ventricular cavity size because the hypertrophied muscle encroaches on the left ventricular cavity. These patients often will have shortness of breath. Again, some of them have chest pain as a symptom from diastolic dysfunction, and their symptoms can be improved by enlarging their ventricles. And we do that by performing a myectomy through the apex of the heart, and we've treated approximately 50 patients with apical hypertrophic cardiomyopathy with this apical myectomy.

The cardiologist and surgeons at the Mayo Clinic have a large experience with septal myectomy and apical myectomy. We've done well over 1,500 operations for this problem. The cardiologists here have seen thousands of patients with the disease and I think are in a very good position to be able to give advice on which patients need medical treatment, which patients need surgical treatment, and which patients need a defibrillator. The second point is that the patients require long-term follow-up and evaluation of other family members. And so I think that's something that the cardiologist have been very good at at Mayo, and that's investigating other family members to look for this genetic disease in patients who are family members, who might not be symptomatic. Patients who have hypertrophic cardiomyopathy should know that there's a surgical option. I think too often we see patients who have been followed, who continue on medical treatment, and are dissatisfied either with the improvement that they get with the medical treatment or they're dissatisfied with the side effects from the medicine and just aren't aware that there's a surgical option. So patients should ask their physicians, if they have hypertrophic cardiomyopathy, whether they might be a candidate for an operation. And if there's any question, of course, we'd be happy to look at their echocardiograms, or evaluate their clinical data, and see and give them an opinion about whether surgery might be helpful to them.

Oct. 28, 2023