A septal myomectomy is a type of open-heart surgery used to treat severe hypertrophic obstructive cardiomyopathy (HOCM). HOCM may be inherited and result in sudden death in young adults. People with HOCM have an abnormal growth of muscle fibers in their heart that results in a thickened and stiffened septum (the muscular wall separating the right and left ventricles). This enlarged area interferes with blood that is being pumped by the left ventricle and may reduce the heart’s ability to fill with blood. HOCM may produce symptoms such as shortness of breath, dizziness, fainting and/or chest pain. In very severe cases, patients are also at increased risk of cardiac arrest.

A septal myomectomy is designed to remove part of the enlarged septum, thus relieving the obstruction and improving blood flow and reducing the risk of potentially fatal consequences from HOCM. Because this is an open-heart surgery, patients will need to remain in the hospital for one to two weeks while recovering, and will need additional recovery time after returning home. In some cases, the physician may also replace a faulty mitral valve at the same time the surgery is conducted.

HOCM can also be treated by other techniques, depending on its level of severity. Physicians may choose to treat HOCM with medications only, with surgery or with an implantable defibrillator. HOCM also may be treated with a newer procedure called septal alcohol ablation, which involves destroying excess septal muscle with ethanol alcohol. Surgery and septal alcohol ablation are usually recommended for patients who have severe symptoms despite drug treatment.

A septal myomectomy is a type of open-heart surgery used to treat severe hypertrophic obstructive cardiomyopathy (HOCM), an inherited heart condition that can cause sudden death in young adults. This condition is characterized by excess septal muscle. The septum is the wall of muscle separating the left and right sides of the heart. When there is excess muscle, it may interfere with the flow of blood through the ventricle.

This condition is called an outflow tract obstruction and may result in a reduced blood flow to the body. In some rare cases, the outflow tract obstruction may also interfere with the functioning of the mitral valve. This valve sits between the left atrium and left ventricle, and the outflow tract obstruction can cause blood to flow backward from the left ventricle into the left atrium (mitral valve regurgitation). Other complications of this condition include atrial fibrillation, syncope, congestive heart failure and cardiac arrest.

There are four options for treating HOCM:

• Septal myomectomy surgery, an open–heart surgery in which a surgeon removes some of the excess muscle. In some cases, the physician may choose to also replace a faulty mitral valve in conjunction with the surgery, depending if the HOCM is causing mitral valve regurgitation.
  
  
• Septal alcohol ablation, a less-invasive catheter-based procedure in which a physician uses ethanol alcohol to create an infarction, or mini heart attack, in the septum and thins the excess muscle. Studies have shown this relatively new procedure has roughly the same long-term outcome as surgery, with the advantage of less surgical trauma for the patient.

The HOCM’s severity and the patient profile dictates which approach the physician will usually choose as treatment. About 95 percent of cases can be handled with either medication or an implanted pacemaker with ICD. Of the remaining 5 percent, septal alcohol ablation is usually recommended for elderly patients and others who cannot withstand the rigors of open-heart surgery. Surgery is often recommended for younger patients who have severe symptoms that are not responding to medication.

Several weeks before surgery, the patient may be required to undergo a test called a transesophageal echocardiogram (TEE) to find the diseased area in the septum. To perform this test, the physician inserts a thin tube (endoscope) into the patient’s esophagus (the long tube that connects the throat with the stomach), which ends just behind the patient’s heart. At the far end of the endoscope is a small device called a transducer, which sends and receives high-frequency sound waves (ultrasound signals). A computer near the operating table then calculates the travel time of these sound waves to and from the heart, thereby constructing a very clear and virtually unobstructed image of the heart’s size and function. This may be recorded on video monitors, paper (still pictures) and/or videotape.

A few days before surgery, the patient will undergo a number of tests. Even if these tests were performed days or weeks earlier, they will be done again just before surgery to be sure that the patient’s medical condition has not changed. They include:

Eight to twelve hours before surgery, all patients are placed on NPO (non per os; nothing by mouth) status. That means that they are not permitted to eat, drink or take anything by mouth until after their surgery. Smokers will have been advised to completely avoid smoking for at least two weeks before their surgery to prevent problems in blood flow, clotting or breathing. Certain medications may need to be reduced or stopped temporarily, so patients should discuss their medication schedules with their cardiologist before surgery.

The patient is usually admitted the morning of surgery. He or she will be given specific pre-operative medications and then “prepped” for surgery. The chest is shaved, and a bacteria-killing (bactericidal) solution is applied to the operative site and surrounding area. The patient is then put to sleep with a medication that is given through an intravenous (IV) line in the arm or hand. The patient will continue to breathe a mixture of oxygen and anesthetic gas (general anesthesia) to make sure that he or she remains asleep throughout the entire surgery.

After the patient is asleep, a device called the Swan-Ganz catheter is often inserted through a needle stick into the jugular vein (in the neck) and threaded to the pulmonary artery, which goes from the heart to the lungs. The catheter can be used to measure heart function, measure the pressures in both the heart and lungs, and give any necessary medications. A urinary catheter is also inserted and connected to a collection bag to measure the patient’s urine output.

An 11- to 12-centimeter incision (about 4-1/2 inches) is then made in the chest, through the breastbone (sternum), and the two halves of the breastbone are divided (a full median sternotomy). A retractor is used to gently pull back the two halves of the breastbone to give the surgeon plenty of room to work.

The functions of the heart, including blood flow and oxygenation, are rerouted through a heart-lung machine via tubes (cannulas) that are placed in the body. While this machine takes care of the heart’s functions, the heart can be carefully stopped with a cardioplegic solution so the surgeon can perform his or her very delicate work. Some surgeons may further protect the heart by decreasing its temperature. The heart will remain stopped for about 30 to 90 minutes during the two to four hours (on average) of surgery.

Based on the results of the transesophageal echocardiogram, the surgeon knows where and how much of the overgrown muscle cells need to be removed. The surgeon makes an incision in the aorta through which the the surgeon can access the heart and remove excess septal muscle. Once the procedure is completed, the incision in the aorta is closed and the heart is restarted. When the surgical team is satisfied that the heart is beating strongly again, the heart-lung machine will be removed. The chest incisions are then closed (sutured).

Blood transfusion may be needed during any type of open-heart surgery, including a septal myomectomy. The patient or family members may donate blood prior to the surgery. If blood is used from a  blood bank, it has been tested to make sure it is free from any diseases, such as hepatitis or human immunodeficiency virus (HIV).

After surgery, the patient will be transferred to a cardiac intensive care unit, where he or she will be monitored continually. The breathing tube and respirator will only be removed when it is decided that the patient can breathe independently. Painkillers may be administered, blood samples will be taken and an electrocardiogram (EKG) monitor will continuously record the electrical activity of the heart.

Barring any unforeseen complications, an individual will typically spend about four to eight days in the hospital before being discharged. The patient and family members will be given specific instructions as to care of the surgical site, necessary follow-up appointments and any activity restrictions. For many people, they will be able to participate in normal activities. Some individuals will experience difficulties with strenuous activities or physically demanding tasks. Some physicians advise patients to avoid participating in competitive sports or activities that require strong physical effort. Patients will need to be monitored by a cardiologist throughout their life.

The following results have been reported from a successful septal myomectomy:

Potential complications of septal myomectomy include:

• Excessive removal of septal muscle may create a ventricular septal defect, or hole in the wall between the right ventricle and left ventricle.
  
  
• Possible surgical damage to the aortic valve that necessitates aortic valve replacement at a later date.
  
  
• Possible damage to the heart’s electrical conduction system, which may require permanent pacing. This occurs in about 5 to 10 percent of patients.

There is also some risk associated with the open-heart surgery itself, especially among older people and/or those with more severe conditions and surgeries that involve use of the heart lung machine. About 5 to 10 percent of patients experience heart attacks, strokes or “mini-strokes” (transient ischemic attacks) either during or shortly after open-heart surgery. Other complications include bleeding and infection. 

 Conventional open-heart surgery, which has been around for almost 50 years, requires the use of the heart-lung machine to take over the heart’s functions during surgery so the heart can be carefully stopped for treatment. Since the advent of open-heart surgery, the techniques have been refined to make it safer and less-invasive. Today, open-heart surgery has an excellent track record, with the incidence of side effects approaching 1 percent in some surgeries.

Preparing questions in advance can help patients to have more meaningful discussions with their physicians regarding their conditions. Patients may wish to ask their doctor the following questions related to septal myomectomy:

1. What heart defect(s) do I have that require septal myomectomy?
   
   
2. What tests will be used to determine the extent of my defect?
   
   
3. How soon do I need to have this surgery?
   
   
4. Will any other procedures be performed at the time of my septal myomectomy?
   
   
5. Do I have any alternatives to this surgery for treatment?
   
   
6. How effective is the surgery in correcting my defect?
   
   
7. What are the risks associated with septal myomectomy?
   
   
8. How long will it take me to recover from the surgery?
   
   
9. What are my restrictions following this surgery?
   
   
10. Will I need to take any medications after the procedure?
    
    
11. Will I have any heart problems or conditions later in life as a result of this procedure?
    
    
12. How will my heart health be monitored through life?
    
    
13. Do my children have a higher risk of having a congenital heart defect?
    
    
14. How will pregnancy affect my ability to undergo this surgery?
    
    
15. What is my long-term prognosis?