Long QT syndrome
Editor-In-Chief: Prab R Tumpati, MD
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| Long QT syndrome | |
|---|---|
| |
| Synonyms | LQTS |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Syncope, seizures, sudden cardiac death |
| Complications | Torsades de pointes, ventricular fibrillation |
| Onset | Congenital or acquired |
| Duration | Lifelong |
| Types | N/A |
| Causes | Genetic mutations, certain medications, electrolyte imbalances |
| Risks | Family history, certain medications, electrolyte disturbances |
| Diagnosis | Electrocardiogram, genetic testing |
| Differential diagnosis | Ventricular tachycardia, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia |
| Prevention | Avoiding QT-prolonging drugs, managing electrolyte levels |
| Treatment | Beta blockers, implantable cardioverter-defibrillator, left cardiac sympathetic denervation |
| Medication | N/A |
| Prognosis | Variable, risk of sudden death |
| Frequency | 1 in 2,000 people |
| Deaths | N/A |
Long QT syndrome (LQTS) is a genetic disorder that affects the heart's electrical activity. It is characterized by a prolonged QT interval on an electrocardiogram (ECG), which can lead to arrhythmias, syncope, and sudden cardiac arrest.
Pathophysiology
Long QT syndrome is caused by mutations in genes that encode for ion channels in the heart. These ion channels are responsible for the flow of potassium, sodium, and calcium ions, which are crucial for the heart's electrical activity. The most commonly affected genes include KCNQ1, KCNH2, and SCN5A. Mutations in these genes can disrupt the normal flow of ions, leading to a prolonged QT interval.
Types
There are several types of Long QT syndrome, classified based on the specific gene mutation:
- LQT1: Caused by mutations in the KCNQ1 gene.
- LQT2: Caused by mutations in the KCNH2 gene.
- LQT3: Caused by mutations in the SCN5A gene.
Symptoms
Individuals with Long QT syndrome may experience:
Diagnosis
Diagnosis of Long QT syndrome typically involves:
- Electrocardiogram (ECG) to measure the QT interval.
- Genetic testing to identify specific mutations.
- Holter monitor to record heart activity over 24-48 hours.
- Exercise stress test to observe heart activity during physical exertion.
Treatment
Treatment options for Long QT syndrome include:
- Beta blockers to reduce the risk of arrhythmias.
- Implantable cardioverter-defibrillator (ICD) for individuals at high risk of sudden cardiac arrest.
- Lifestyle modifications such as avoiding strenuous exercise and certain medications that can prolong the QT interval.
Prognosis
With appropriate treatment and lifestyle modifications, individuals with Long QT syndrome can lead relatively normal lives. However, the risk of sudden cardiac arrest remains, particularly in untreated individuals.
See also
Cardiovascular disease A-Z
Most common cardiac diseases
- Cardiac arrhythmia
- Cardiogenetic disorders
- Cardiomegaly
- Cardiomyopathy
- Cardiopulmonary resuscitation
- Chronic rheumatic heart diseases
- Congenital heart defects
- Heart neoplasia
- Ischemic heart diseases
- Pericardial disorders
- Syndromes affecting the heart
- Valvular heart disease
A | B | C | D | E | F | G | H | I | J | K | L | M | N | O | P | Q | R | S | T | U | V | W | X | Y | Z
A
- Accelerated idioventricular rhythm
- Acute decompensated heart failure
- Arteriosclerotic heart disease
- Athletic heart syndrome
- Atrial flutter
- Atrioventricular fistula
- Cardiovascular disease in Australia
- Autoimmune heart disease
B
C
- Ebb Cade
- Cardiac allograft vasculopathy
- Cardiac amyloidosis
- Cardiac asthma
- Cardiac tamponade
- Cardiogenic shock
- Cardiogeriatrics
- Cardiorenal syndrome
- Cardiotoxicity
- Carditis
- Coronary artery aneurysm
- Coronary artery anomaly
- Coronary artery disease
- Spontaneous coronary artery dissection
- Coronary artery ectasia
- Coronary occlusion
- Coronary steal
- Coronary thrombosis
- Coronary vasospasm
- Cœur en sabot
- Coxsackievirus-induced cardiomyopathy
D
E
H
- Heart attack
- Heart failure
- Heart failure with preserved ejection fraction
- Heart to Heart (1949 film)
- High-output heart failure
- Hyperdynamic precordium
A | B | C | D | E | F | G | H | I | J | K | L | M | N | O | P | Q | R | S | T | U | V | W | X | Y | Z
I
- Idiopathic giant-cell myocarditis
- Interventricular dyssynchrony
- Intraventricular dyssynchrony
- Isolated atrial amyloidosis
K
L
M
- Mydicar
- Myocardial bridge
- Myocardial disarray
- Myocardial rupture
- Myocardial scarring
- Myocardial stunning
- Myocarditis
N
O
P
- Papillary fibroelastoma
- Pathophysiology of heart failure
- Postpericardiotomy syndrome
- Pulmonary vein stenosis
R
S
- Saturated fat and cardiovascular disease
- SCAR-Fc
- Shone's syndrome
- Strain pattern
- Subacute bacterial endocarditis
- Sudden cardiac death of athletes
A | B | C | D | E | F | G | H | I | J | K | L | M | N | O | P | Q | R | S | T | U | V | W | X | Y | Z
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Contributors: Prab R. Tumpati, MD
