Overview
Congenital short QT syndrome (SQTS) is a rare inherited heart rhythm disorder. It gets its name from the 'QT interval' — a measurement on a heart tracing (ECG) that reflects how long it takes the heart's electrical system to reset between beats. In people with SQTS, this interval is unusually short, meaning the heart resets too quickly. This disrupts the normal electrical signals that keep the heart beating in a steady, organized rhythm. Because the heart's electrical system is unstable, people with SQTS are at risk for dangerous irregular heartbeats called arrhythmias. The most serious of these — ventricular fibrillation — can cause the heart to stop pumping blood effectively, leading to sudden cardiac arrest. This can happen even in young, otherwise healthy people, including infants and children. Some people with SQTS also experience atrial fibrillation, which causes a fast, fluttery heartbeat and can lead to dizziness or fainting. Treatment focuses on preventing sudden cardiac death. The most reliable option is an implantable cardioverter-defibrillator (ICD), a small device placed under the skin that monitors the heart and delivers a shock if a life-threatening rhythm is detected. Certain medications, particularly quinidine, may also help by lengthening the QT interval back toward normal. Early diagnosis is critical, as the first sign of the condition can sometimes be a life-threatening event.
Also known as:
Key symptoms:
Fainting or blacking out, especially during physical activity or stressHeart palpitations — a feeling that the heart is racing, fluttering, or poundingDizziness or lightheadednessShortness of breathSudden cardiac arrest (the heart stops pumping effectively)Atrial fibrillation — an irregular, often fast heartbeatNo symptoms at all in some people (discovered by chance on an ECG)
Clinical phenotype terms (9)— hover any for plain English
Autosomal dominant
Passed on from just one parent; each child has about a 50% chance of inheriting it
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for Congenital short QT syndrome.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Congenital short QT syndrome at this time.
New trials open frequently. Follow this disease to get notified.
Specialists
View all specialists →No specialists are currently listed for Congenital short QT syndrome.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Congenital short QT syndrome.
Community
No community posts yet. Be the first to share your experience with Congenital short QT syndrome.
Start the conversation →Latest news about Congenital short QT syndrome
No recent news articles for Congenital short QT syndrome.
Follow this condition to be notified when news becomes available.
Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.Based on my ECG and genetic results, how high is my personal risk of sudden cardiac arrest?,Do I need an ICD, and what are the risks and benefits for someone my age?,Is quinidine or another medication appropriate for me, and what are the side effects?,Which physical activities or sports are safe for me to do?,Should my family members be tested, and if so, who should be tested first?,What should I do — and what should people around me do — if I collapse or lose consciousness?,How often do I need follow-up appointments and heart monitoring?
Common questions about Congenital short QT syndrome
What is Congenital short QT syndrome?
Congenital short QT syndrome (SQTS) is a rare inherited heart rhythm disorder. It gets its name from the 'QT interval' — a measurement on a heart tracing (ECG) that reflects how long it takes the heart's electrical system to reset between beats. In people with SQTS, this interval is unusually short, meaning the heart resets too quickly. This disrupts the normal electrical signals that keep the heart beating in a steady, organized rhythm. Because the heart's electrical system is unstable, people with SQTS are at risk for dangerous irregular heartbeats called arrhythmias. The most serious of th
How is Congenital short QT syndrome inherited?
Congenital short QT syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.