Hypertrophic Cardiomyopathy Myectomy: A Cardiac Surgical Solution for Hypertrophic Cardiomyopathy

What Is Hypertrophic Cardiomyopathy?

Hypertrophic cardiomyopathy is an inherited disorder characterized by thickening of the heart muscles, especially those related to the right ventricle (the significant pumping chamber of the heart). Here, the walls or septum of the left ventricle become bulged and stiffened, affecting the pumping potential of the heart. Hence, the heart cannot function and pump adequate blood during every heartbeat, causing the associated effects.

There are two primary types of hypertrophic cardiomyopathy.

• Obstructive Hypertrophic Cardiomyopathy: This is the most common form of hypertrophic cardiomyopathy where the septum (wall) connecting the left ventricle and right ventricle tends to thicken, obstruct the blood flow within the left ventricle and the chief artery of the human system called the aorta.

• Nonobstructive Hypertrophic Cardiomyopathy: Here, the cardiac muscle surrounding the heart thickens, but this will not impede the blood flow.

What Is the Etiology of Hypertrophic Cardiomyopathy?

Hypertrophic cardiomyopathy primarily stems from genetic mutations that affect the proteins essential for cardiac muscle function. Such mutations often involve genes encoding sarcomere proteins, including MYH7, MYBPC3, and TNNT2. The sarcomere refers to the basic contractile unit of the heart muscle, and mutations in these genes could result in affiliated structural and functional abnormalities. Hypertrophic cardiomyopathy typically follows an autosomal dominant inheritance pattern, where there will be a chance of passing on the genetic mutation to fifty percent of the offspring from parents. While many cases are inherited, some individuals might develop this condition due to spontaneous de novo mutations.

At the cellular level, these genetic mutations disrupt the normal regulation of muscle contraction and relaxation, resulting in hypertrophy, specifically in the left ventricle. Dysfunctional signaling pathways and cellular remodeling are attributable to the hypertrophic response. Nevertheless, the clinical expression of this condition varies widely, and additional factors, such as environmental influences or lifestyle, might influence the clinical presentation of the disease.

What Are the Symptoms Associated With Hypertrophic Cardiomyopathy?

The signs and symptoms of hypertrophic cardiomyopathy vary from one individual to another. This discrepancy occurs more often within individuals of the same inheritance. Hypertrophic cardiomyopathy does not manifest any signs in some people. On the contrary, others might encounter the following signs and symptoms.

• Chest pain, specifically accompanied by physical activities.

• Fatigue.

• Arrhythmia (abnormal heart rhythm).

• Rapid and pounding heartbeat.

• Heart murmurs.

• Fainting.

• Dyspnea (shortness of breath).

• Lightheadedness.

• Dizziness.

• Swelling in the ankles, legs, and stomach.

What Is Hypertrophic Cardiomyopathy Myectomy?

Hypertrophic cardiomyopathy myectomy, also known as septal myectomy, is a kind of open-heart surgical intervention performed in the treatment of hypertrophic cardiomyopathy. This procedure helps relieve the symptoms associated with the thickened heart muscle and the condition. The excess part of the cardiac muscle forming the thickened septum is removed during the procedure. This is followed by allowing the blood to empty from the ventricle from the affected side with no impediment.

In many cases of hypertrophic cardiomyopathy, appropriate medications are effective in relieving the symptoms. Nevertheless, a septal myectomy for hypertrophic cardiomyopathy is recommended when the medications seem ineffective. The myomectomy procedure is a moderately safe and effective surgical procedure. Many women with hypertrophic cardiomyopathy might require surgery to be done before pregnancy, regardless of the worsening or severity of the symptoms.

How Is Myectomy for Hypertrophic Cardiomyopathy Done?

The healthcare providers might recommend the following diagnostic aids before the procedure.

• Certain blood tests.

• Cardiac-related MRIs (magnetic resonance imaging).

• Chest X-rays.

• Electrocardiography is for recording and determining the electrical activity of the heart.

• Echocardiography is a medical imaging procedure that utilizes ultrasound waves to present a comprehensive image of the heart.

Generally, the hypertrophic cardiomyopathy myectomy procedure is done by incorporating the following steps.

• The anesthesia will be given before the surgical intervention. The anesthesia makes one get into a deeper sleep state, witnessing painless surgery.

• Then, an incision is made in the middle aspect of the chest to separate the part of the breastbone, assessing the heart.

• When the incision is made, a cardiopulmonary machine is attached to the individual. This machine contributes to providing oxygen supply to the blood and pumping adequate blood to the vital systems during the surgical procedure.

• The thickened part of the muscle lining of the affected septum is then eliminated.

• The disconnection of the heart-lung machine follows this.

• The incised parts are then wired together, and the incision is closed using absorbable sutures.

How Is the Outcome of Hypertrophic Cardiomyopathy Myectomy?

The primary goal of the procedure is to eliminate excess heart muscle that obstructs blood flow from the heart. The outcomes of hypertrophic cardiomyopathy myectomy can be favorable, with many people reporting significant improvement in symptoms such as syncope, shortness of breath, and chest pain. The surgery can also reduce the gradient of obstruction in the left ventricle, making allowance for better blood flow.

However, as with any surgical intervention, there are risks and potential complications related to hypertrophic cardiomyopathy myectomy. These can include bleeding, infection, arrhythmias, and damage to adjacent structures in rare cases. The overall success and outcome of the procedure depend on various determinants, including the patient’s specific conditions and any associated health complications.

What Are the Risks Associated With Hypertrophic Cardiomyopathy Myectomy?

The hypertrophic cardiomyopathy myectomy procedure successfully treats the condition. It helps relieve symptoms like chest pain, unconsciousness, and shortness of breath. The risks are usually less with this kind of septal myectomy. The risks depend upon the severity of the condition, healing, age, and certain other attributes. Here are some potential complications associated with hypertrophic cardiomyopathy myectomy.

• Infection.

• Bleeding.

• Abnormal heart rhythms.

• Blood clots, potentially resulting in heart attack and stroke.

• Complications related to anesthesia.

• Aortic valve problems because of the valve movements during the surgery.

• Elimination of more cardiac muscle.

• Blood flow issues during surgery, potentially resulting in poor heart pumping function.

Several determinants and underlying medical conditions are known to increase the risk of hypertrophic cardiomyopathy myectomy.

• Lung problems.

• Increased age.

• Obesity.

• Infections.

• Chronic illnesses.

• Heart-related illnesses.

• Smoking habit.

Conclusion:

Hypertrophic cardiomyopathy myectomy stands as a significant and effective intervention for individuals suffering from such cardiac conditions. The surgical procedure aims to alleviate symptoms associated with hypertrophic cardiomyopathy by eliminating excess cardiac muscle that impedes healthy blood flow. By addressing the underlying anatomical abnormalities, particularly in the septum, myectomy can substantially improve the overall quality of life and reduce the burden of symptoms such as syncope, dyspnea, and chest pain. Moreover, the procedure often reduces left ventricular outflow tract obstruction, enhancing cardiac function.