Arrhythmogenic Right Ventricular Dysplasia

Arrhythmogenic right ventricular dysplasia (ARVD) is a rare type of cardiomyopathy. It occurs when the muscle tissue in the right ventricle dies and is replaced with scar tissue. This disrupts the heart's electrical signals and causes 心律失常. Symptoms include palpitations, fatigue, 晕倒 甚至 心脏骤停 体力活动后. Palpitations are feelings that your heart is skipping a beat, fluttering or beating too hard or too fast.

Teens and young adults are more likely than older people to have ARVD, although it’s rare in both groups. It can cause sudden 心脏骤停 (SCA) in young athletes.

ARVD的其他名称

  • Arrhythmogenic right ventricular cardiomyopathy
  • Right ventricular dysplasia

什么导致ARVD

  • Researchers believe that ARVD is an inherited disease due to gene mutations. Researchers have found at least 13 genes that may cause this condition when mutated. The mutated genes affect proteins that are responsible for heart muscle development. In 30%-50% of cases there is a family history of ARVD.
  • There are two patterns of inheritance:
    • Autosomal dominant: Where one parent has the mutation. Family members have a 50% chance of inheriting the abnormal gene.
    • Autosomal recessive: Where both parents have the mutation but no symptoms.

ARVD may also be related to other causes including congenital abnormalities, viral or inflammatory infections of the heart muscle or other unknown causes.

诊断

诊断 of ARVD is challenging since symptoms are common for other diseases. Your health care professional will use several tests to make the diagnosis including an 心电图, 超声心动图, 心脏核磁共振 and possibly a heart biopsy.

Treatment and management

The goal of treatment is to prevent or reduce the risk of sudden cardiac death.

  • 药物 will be used to manage 心律失常 and prevent clot formation.
  • 导管消融 may be used to stop the electrical impulses that cause irregular heart rhythms.
  • 手术放置 implantable cardiac defibrillator in the heart is the most effective way to prevent sudden death.
  • A 心脏移植手术 may be done if 心律失常 do not respond to interventions or the heart failure isn’t being controlled by the medications.

预后

With modern medicine and advance imaging protocols, 包括心脏MRI, the prognosis is favorable especially in families who undergo genetic testing.

Written by American Heart Association editorial staff and reviewed by science and medicine advisors.

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Last Reviewed: May 31, 2024