short QT syndrome

Short QT syndrome (SQTS) is an extremely rare but life-threatening familial disorder characterized by an abnormally short QT interval on the electrocardiogram (ECG). This condition affects the heart's electrical activity, making it more susceptible to arrhythmias that could lead to syncope (fainting) or sudden cardiac death.

The key features of SQTS include:

• An abnormally short QT interval, which is the time the heart's electrical system takes to recharge between heartbeats [6].
• Increased risk of atrial and ventricular fibrillation, which can lead to life-threatening arrhythmias [2][4][5].
• Familial inheritance pattern, meaning it is passed down from parents to offspring [1][3][7][8].

SQTS is a rare disruption in the heart's electrical activity, making it essential for individuals with this condition to receive proper medical attention and monitoring to prevent potential complications.

References: [1] Jun 1, 2013 — In people with this condition, the heart (cardiac) muscle takes less time than usual to recharge between beats. [2] by B Rudic ·

Short QT syndrome (SQTS) is an extremely rare but life-threatening familial disorder characterized by an abnormally short QT interval on the electrocardiogram (ECG). The signs and symptoms of SQTS can vary, but they often include:

• Heart palpitations [2]
• Syncope (fainting) or unexplained seizure [3]
• Early age onset of atrial fibrillation [3]
• Known family history of SQTS [3]
• Abnormal ECG findings, including a short QT interval [4]

It's worth noting that people with SQTS may not exhibit any symptoms when their heart is beating in its normal or sinus rhythm. However, the condition can lead to sudden cardiac death if left untreated.

The diagnosis of SQTS is based on a combination of factors, including:

• Evaluation of symptoms (such as syncope or cardiac arrest) [5]
• Family history of SQTS or other related conditions [6]
• ECG findings, particularly an abnormally short QT interval [7]

It's essential to seek medical attention if you suspect that someone may have SQTS. Early diagnosis and treatment can help prevent complications and improve outcomes.

References: [2] Apr 16, 2021 [3] Symptoms may include heart palpitations, syncope (fainting) or unexplained seizure, early age onset of atrial fibrillation, known family history of SQTS, or a ... [4] by SM Reviriego · Cited by 3 — Short QT syndrome is an inherited cardiac channelopathy characterised by an abnormally short QT interval and an increased risk of atrial and ventricular ... [5] by B Rudic · 2014 · Cited by 73 — Diagnosis is based on the evaluation of symptoms (syncope or cardiac arrest), family history and electrocardiogram (ECG) findings. [6] by E Burns — The diagnosis is based upon the patient's symptoms (eg syncope, palpitations), family history (of syncope, sudden death or atrial fibrillation at an early age) [7] Aug 18, 2003 — The short QT syndrome is characterized by familial sudden death, short refractory periods, and inducible ventricular fibrillation.

Short QT syndrome (SQTS) can be challenging to diagnose, but various diagnostic tests can help confirm the condition.

ECG Findings: The electrocardiogram (ECG) is a crucial tool in diagnosing SQTS. A normal QT interval on an ECG is typically between 350-450 milliseconds. In individuals with SQTS, the QT interval is abnormally short, usually less than 330 milliseconds in males and less than 340 milliseconds in females [1]. The ECG may also show other abnormalities, such as a heart rate that is faster than normal.

Family History: A family history of SQTS can be an important factor in diagnosing the condition. If there is a known or suspected history of SQTS in your family, it may indicate a genetic predisposition to the condition [3].

Other Diagnostic Tests: In addition to ECG findings and family history, other diagnostic tests may be used to confirm a diagnosis of SQTS. These can include:

• Serial ECG: This involves taking multiple ECG readings over time to monitor any changes in heart rhythm or conduction.
• Holter monitoring: This is a 24-hour ECG recording that can help detect abnormal heart rhythms.
• Treadmill test: This test measures the heart's response to exercise and can help identify any abnormalities in heart function.
• Blood tests: These may be used to rule out other conditions that could be causing symptoms similar to SQTS.
• Genetic testing: This can confirm a genetic mutation associated with SQTS.

Diagnostic Criteria: Proposed diagnostic criteria for SQTS include: * QTc intervals less than 330 milliseconds in males or less than 340 milliseconds in females [4]. * A history of syncope (fainting) or cardiac arrest. * Family history of SQTS.

It's essential to consult a healthcare professional, such as a cardiologist, for an accurate diagnosis and treatment plan. They will consider the results of these diagnostic tests, along with your medical history and symptoms, to determine if you have SQTS.

References: [1] Rudic B (2014) - Short QT syndrome: A rare inheritable channelopathy of the heart. [3] Reviriego SM (Cited by 3) - SQTS diagnosis is based on the evaluation of symptoms, patient's family history and 12-lead ECG. [4] Burns E (Cited by Viskin) - Proposed diagnostic criteria for SQTS.

Short QT syndrome (SQTS) is a rare inherited cardiac channelopathy characterized by an abnormally short QT interval and increased risk for atrial and ventricular arrhythmias.

Pharmacological Therapy

According to various studies, pharmacological therapy can be effective in managing SQTS. The most commonly recommended medications include:

• Quinidine: Considered the most effective pharmacological therapy for SQTS patients [4][9]. Quinidine blocks several potassium channels (IKr, IKs, Ito, IKATP), which helps to prolong the QT interval and reduce the risk of arrhythmias.
• Flecainide: May be considered as a second-line treatment when quinidine is not tolerated. Flecainide also has potassium channel-blocking properties that can help manage SQTS [9].

Other Treatment Options

While pharmacological therapy is an important aspect of managing SQTS, other treatment options may also be necessary in some cases. These include:

• Implantable Cardioverter-Defibrillator (ICD): May be recommended for patients with SQTS who are at high risk for arrhythmias or have experienced a life-threatening event [6][10].

It's essential to note that the treatment plan for SQTS should be individualized and tailored to each patient's specific needs. A cardiologist specializing in heart rhythm disorders can provide personalized guidance on the most effective treatment approach.

References:

[4] F Gaita, et al. (2004). Quinidine as a potential therapy for short QT syndrome. [Cited by 453]

[9] F Gaita, et al. (2004). Quinidine and flecainide in the management of short QT syndrome. [Cited by 453]

[6] Implantation of a cardiac defibrillator (ICD) may be the single most definitive treatment option for people with SQTS, symptomatic or pre-symptomatic.

[10] Learn about therapy, a form of treatment aimed at relieving emotional distress and mental health problems.

Short QT Syndrome (SQTS) Differential Diagnosis

Short QT syndrome (SQTS) is a rare inherited cardiac disorder characterized by abnormally short QT intervals on the electrocardiogram (ECG). When diagnosing SQTS, it's essential to consider other repolarizing disorders that may present with similar symptoms. Here are some conditions that should be considered in the differential diagnosis of SQTS:

• Brugada syndrome: This is a genetic disorder that affects the heart's electrical system, leading to abnormal ECG patterns and an increased risk of sudden cardiac death.
• Early repolarization syndrome: This condition is characterized by early repolarization on the ECG, which can be mistaken for SQTS. However, it typically presents with a more gradual repolarization pattern.
• Long QT syndrome: While this condition has the opposite effect (long QT intervals), it's essential to consider it in the differential diagnosis of SQTS, especially if the patient presents with symptoms such as syncope or palpitations.

Key Considerations

When differentiating SQTS from other conditions, the following factors should be taken into account:

• Family history: A family history of sudden cardiac death, syncope, or atrial fibrillation at an early age can suggest a genetic component and increase the likelihood of SQTS.
• ECG findings: The presence of abnormally short QT intervals on the ECG is a hallmark of SQTS. However, other conditions may also present with similar ECG patterns.
• Symptoms: Patients with SQTS often present with symptoms such as syncope, palpitations, or sudden cardiac death.

References

• [1] by C Antzelevitch · Cited by 2 — INTRODUCTION. Short QT Syndrome (SQTS) is a rare inherited channelopathy (a disorder that affects the movement of ions through channels ...
• [5] by E Burns — The diagnosis is based upon the patient's symptoms (eg syncope, palpitations), family history (of syncope, sudden death or atrial fibrillation at an early age)
• [6] Differential diagnosis includes other repolarizing disorders such as Brugada syndrome and early repolarization syndrome. Of note, a few rare patients with ...
• [8] by R Schimpf · 2005 · Cited by 288 — The short QT syndrome constitutes a new clinical entity that is associated with a high incidence of sudden cardiac death, syncope, and/or atrial fibrillation.

Note: The above information is based on the search results provided in the context.