Hereditary atrial tachyarrhythmia-infra-Hisian cardiac conduction disease

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ORPHA:436242OMIM:616117I45.8
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Overview

Hereditary atrial tachyarrhythmia with infra-Hisian cardiac conduction disease is a very rare inherited heart rhythm disorder. In this condition, the heart's electrical system does not work properly in two ways. First, the upper chambers of the heart (the atria) beat too fast, which is called atrial tachyarrhythmia. Second, there is a problem with how electrical signals travel through the lower part of the heart's conduction system, specifically below a structure called the bundle of His. This area is responsible for sending electrical signals from the upper chambers to the lower chambers (ventricles), so when it doesn't work correctly, the heartbeat can become dangerously slow or irregular. People with this condition may experience episodes of rapid heartbeat (palpitations), dizziness, fainting, fatigue, and shortness of breath. Because the disease affects both the fast and slow parts of the heart's rhythm, patients can swing between having a heart rate that is too fast and one that is too slow. This combination makes treatment particularly challenging. Treatment typically focuses on managing symptoms and preventing dangerous heart rhythms. This may include medications to control heart rate, blood thinners to reduce stroke risk from atrial arrhythmias, and in many cases, implantation of a pacemaker to address the slow conduction problem. Catheter ablation procedures may also be considered for the fast rhythm component. Because this is a hereditary condition, family screening with electrocardiograms (ECGs) and genetic testing is recommended for close relatives.

Key symptoms:

Rapid or racing heartbeat (palpitations)Fainting or near-fainting episodesDizziness or lightheadednessUnexplained fatigue or tirednessShortness of breathChest discomfort or tightnessExercise intoleranceIrregular heartbeatSlow heart rate (bradycardia)Episodes of fast heart rate followed by pauses

Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Hereditary atrial tachyarrhythmia-infra-Hisian cardiac conduction disease.

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No specialists are currently listed for Hereditary atrial tachyarrhythmia-infra-Hisian cardiac conduction disease.

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Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Hereditary atrial tachyarrhythmia-infra-Hisian cardiac conduction disease.

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Community

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.How severe is the conduction problem in my heart, and do I need a pacemaker now or in the future?,What medications are safe for me, and which ones should I avoid?,Should my family members be tested for this condition?,What physical activities or exercises are safe for me?,How often will I need follow-up appointments and heart monitoring?,What are the warning signs that I should go to the emergency room?,Are there any clinical trials or new treatments being studied for this condition?

Common questions about Hereditary atrial tachyarrhythmia-infra-Hisian cardiac conduction disease

What is Hereditary atrial tachyarrhythmia-infra-Hisian cardiac conduction disease?

Hereditary atrial tachyarrhythmia with infra-Hisian cardiac conduction disease is a very rare inherited heart rhythm disorder. In this condition, the heart's electrical system does not work properly in two ways. First, the upper chambers of the heart (the atria) beat too fast, which is called atrial tachyarrhythmia. Second, there is a problem with how electrical signals travel through the lower part of the heart's conduction system, specifically below a structure called the bundle of His. This area is responsible for sending electrical signals from the upper chambers to the lower chambers (ven

How is Hereditary atrial tachyarrhythmia-infra-Hisian cardiac conduction disease inherited?

Hereditary atrial tachyarrhythmia-infra-Hisian cardiac conduction disease follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.