2. Hypertrophic Cardiomyopathy: A Detailed Look at the Thickening Heart
The next type of cardiomyopathy we’re exploring is Hypertrophic Cardiomyopathy (HCM). This condition is characterized by an abnormal thickening of the heart muscle, primarily impacting the interventricular septum, the wall that separates the left and right ventricles.
This irregular hypertrophy can cause the heart to work harder to pump blood, potentially leading to heart failure. More worrisomely, the thickened heart muscle can disrupt the heart’s electrical system, leading to life-threatening arrhythmias and sudden cardiac death.
Like DCM, the causes of HCM are quite varied. Many instances of HCM are inherited, caused by mutations in the genes responsible for the proteins that make up the heart muscle. This is often referred to as familial HCM and is typically an autosomal dominant trait, meaning that a child of a parent with HCM has a 50% chance of inheriting the condition.
Beyond genetics, conditions that cause the heart to work harder, like high blood pressure or aging, can sometimes cause the heart muscle to thicken, resulting in a form of HCM. Other conditions like Friedreich’s ataxia, Noonan syndrome, or Fabry disease may also cause HCM. However, there are cases where the cause is unknown, known as idiopathic HCM.
HCM symptoms can range from being completely asymptomatic to sudden cardiac death. The symptoms depend on the degree of hypertrophy and obstruction of blood flow.
They can include shortness of breath, chest pain (especially during physical activity), fainting, heart palpitations, and fatigue. There can also be more severe complications like mitral valve regurgitation, atrial fibrillation, or even stroke.
Management of HCM aims at alleviating symptoms, preventing sudden cardiac death, and slowing the progression of the disease. Medications are usually the first line of treatment and can include beta-blockers, calcium channel blockers, and antiarrhythmic agents.
For patients with severe symptoms or those at high risk of sudden cardiac death, more invasive treatment options may be recommended, such as septal myectomy or septal ablation, which aim to reduce the thickened muscle, or the implantation of an ICD.
A diagnosis of HCM also brings with it the need for lifestyle changes. Patients are usually advised to avoid strenuous or competitive activities. A balanced diet, limiting alcohol intake, and cessation of smoking are other recommendations. Additionally, regular follow-up with healthcare providers is necessary for close monitoring and management of the condition. (2)
