Kathemolaminergic Polymorphic Ventricular Tachycardia (KPVT) is a rare, inherited heart rhythm disorder that affects the cardiac muscle’s ability to function properly.

This condition causes sudden and irregular heartbeats that can be life-threatening. In this article, we will explore everything you need to know about KPVT, including its symptoms, causes, diagnosis, and treatment.

Symptoms

The symptoms of KPVT often appear during exercise or in stressful situations, such as during a fight or flight response. They can include fainting, dizziness, and palpitations (irregular heartbeats).

In some cases, individuals with KPVT may not experience any symptoms.

Causes

KPVT is caused by a mutation in one of two genes that control the cardiac muscle’s ability to contract. These genes are RYR2 and CASQ2.

A mutation in either of these genes can lead to abnormal calcium release in the cells, disrupting the heart’s electrical system and causing arrhythmias.

Diagnosis

Diagnosing KPVT can be challenging because of its similarity to other heart-related conditions. However, a thorough evaluation of an individual’s symptoms, medical history, and family history can help physicians determine the likelihood of KPVT.

A 12-lead electrocardiogram (ECG) can also be used to diagnose KPVT. In some cases, genetic testing may be needed to confirm the presence of the mutation.

Treatment

The goal of treatment for KPVT is to prevent arrhythmias from occurring and reduce the risk of sudden cardiac death. Treatment options include medication, lifestyle changes, and implantable devices.

Medications such as beta-blockers and calcium channel blockers can help regulate the heart’s electrical system and prevent arrhythmias.

Individuals with KPVT should also avoid high-intensity exercise and stressful situations that can trigger arrhythmias.

In some cases, an implantable cardioverter-defibrillator (ICD) may be recommended. An ICD is a small device that is implanted under the skin near the collarbone.

It monitors heart rhythms and delivers an electric shock if it detects an irregular heartbeat that could lead to sudden cardiac death.

Conclusion

KPVT is a rare, inherited heart rhythm disorder that can be life-threatening. It is caused by a mutation in one of two genes that control the cardiac muscle’s ability to contract.

Symptoms can include fainting, dizziness, and palpitations, and can appear during exercise or in stressful situations. Diagnosing KPVT can be challenging, but a thorough evaluation of an individual’s symptoms and medical history can help physicians determine the likelihood of KPVT. Treatment options include medication, lifestyle changes, and implantable devices.