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Torsades de pointes

MedGen UID:
21214
Concept ID:
C0040479
Disease or Syndrome
Synonym: Torsade de pointes
SNOMED CT: Ventricular tachycardia, polymorphic with Q-T prolongation (31722008); Torsade de pointes (31722008); Torsades de pointes (31722008); Twisting spikes (31722008)
 
HPO: HP:0001664
Monarch Initiative: MONDO:0005478

Definition

A type of ventricular tachycardia characterized by polymorphioc QRS complexes that change in amplitue and cycle length, and thus have the appearance of oscillating around the baseline in the EKG. [from HPO]

Conditions with this feature

Sick sinus syndrome 2, autosomal dominant
MedGen UID:
320273
Concept ID:
C1834144
Disease or Syndrome
Sick sinus syndrome (also known as sinus node dysfunction) is a group of related heart conditions that can affect how the heart beats. "Sick sinus" refers to the sino-atrial (SA) node, which is an area of specialized cells in the heart that functions as a natural pacemaker. The SA node generates electrical impulses that start each heartbeat. These signals travel from the SA node to the rest of the heart, signaling the heart (cardiac) muscle to contract and pump blood. In people with sick sinus syndrome, the SA node does not function normally. In some cases, it does not produce the right signals to trigger a regular heartbeat. In others, abnormalities disrupt the electrical impulses and prevent them from reaching the rest of the heart.\n\nSick sinus syndrome occurs most commonly in older adults, although it can be diagnosed in people of any age. The condition increases the risk of several life-threatening problems involving the heart and blood vessels. These include a heart rhythm abnormality called atrial fibrillation, heart failure, cardiac arrest, and stroke.\n\nSick sinus syndrome tends to cause the heartbeat to be too slow (bradycardia), although occasionally the heartbeat is too fast (tachycardia). In some cases, the heartbeat rapidly switches from being too fast to being too slow, a condition known as tachycardia-bradycardia syndrome. Symptoms related to abnormal heartbeats can include dizziness, light-headedness, fainting (syncope), a sensation of fluttering or pounding in the chest (palpitations), and confusion or memory problems. During exercise, many affected individuals experience chest pain, difficulty breathing, or excessive tiredness (fatigue). Once symptoms of sick sinus syndrome appear, they usually worsen with time. However, some people with the condition never experience any related health problems.
Long QT syndrome 3
MedGen UID:
349087
Concept ID:
C1859062
Disease or Syndrome
Long QT syndrome (LQTS) is a cardiac electrophysiologic disorder, characterized by QT prolongation and T-wave abnormalities on the EKG that are associated with tachyarrhythmias, typically the ventricular tachycardia torsade de pointes (TdP). TdP is usually self-terminating, thus causing a syncopal event, the most common symptom in individuals with LQTS. Such cardiac events typically occur during exercise and emotional stress, less frequently during sleep, and usually without warning. In some instances, TdP degenerates to ventricular fibrillation and causes aborted cardiac arrest (if the individual is defibrillated) or sudden death. Approximately 50% of untreated individuals with a pathogenic variant in one of the genes associated with LQTS have symptoms, usually one to a few syncopal events. While cardiac events may occur from infancy through middle age, they are most common from the preteen years through the 20s. Some types of LQTS are associated with a phenotype extending beyond cardiac arrhythmia. In addition to the prolonged QT interval, associations include muscle weakness and facial dysmorphism in Andersen-Tawil syndrome (LQTS type 7); hand/foot, facial, and neurodevelopmental features in Timothy syndrome (LQTS type 8); and profound sensorineural hearing loss in Jervell and Lange-Nielson syndrome.
Long QT syndrome 5
MedGen UID:
358092
Concept ID:
C1867904
Disease or Syndrome
Long QT syndrome (LQTS) is a cardiac electrophysiologic disorder, characterized by QT prolongation and T-wave abnormalities on the EKG that are associated with tachyarrhythmias, typically the ventricular tachycardia torsade de pointes (TdP). TdP is usually self-terminating, thus causing a syncopal event, the most common symptom in individuals with LQTS. Such cardiac events typically occur during exercise and emotional stress, less frequently during sleep, and usually without warning. In some instances, TdP degenerates to ventricular fibrillation and causes aborted cardiac arrest (if the individual is defibrillated) or sudden death. Approximately 50% of untreated individuals with a pathogenic variant in one of the genes associated with LQTS have symptoms, usually one to a few syncopal events. While cardiac events may occur from infancy through middle age, they are most common from the preteen years through the 20s. Some types of LQTS are associated with a phenotype extending beyond cardiac arrhythmia. In addition to the prolonged QT interval, associations include muscle weakness and facial dysmorphism in Andersen-Tawil syndrome (LQTS type 7); hand/foot, facial, and neurodevelopmental features in Timothy syndrome (LQTS type 8); and profound sensorineural hearing loss in Jervell and Lange-Nielson syndrome.
Jervell and Lange-Nielsen syndrome 2
MedGen UID:
394108
Concept ID:
C2676723
Disease or Syndrome
Jervell and Lange-Nielsen syndrome (JLNS) is characterized by congenital profound bilateral sensorineural hearing loss and long QTc, usually >500 msec. Prolongation of the QTc interval is associated with tachyarrhythmias, including ventricular tachycardia, episodes of torsade de pointes ventricular tachycardia, and ventricular fibrillation, which may culminate in syncope or sudden death. Iron-deficient anemia and elevated levels of gastrin are also frequent features of JLNS. The classic presentation of JLNS is a deaf child who experiences syncopal episodes during periods of stress, exercise, or fright. Fifty percent of individuals with JLNS had cardiac events before age three years. More than half of untreated children with JLNS die before age 15 years.
Long QT syndrome 12
MedGen UID:
442824
Concept ID:
C2751830
Disease or Syndrome
Long QT syndrome (LQTS) is a cardiac electrophysiologic disorder, characterized by QT prolongation and T-wave abnormalities on the EKG that are associated with tachyarrhythmias, typically the ventricular tachycardia torsade de pointes (TdP). TdP is usually self-terminating, thus causing a syncopal event, the most common symptom in individuals with LQTS. Such cardiac events typically occur during exercise and emotional stress, less frequently during sleep, and usually without warning. In some instances, TdP degenerates to ventricular fibrillation and causes aborted cardiac arrest (if the individual is defibrillated) or sudden death. Approximately 50% of untreated individuals with a pathogenic variant in one of the genes associated with LQTS have symptoms, usually one to a few syncopal events. While cardiac events may occur from infancy through middle age, they are most common from the preteen years through the 20s. Some types of LQTS are associated with a phenotype extending beyond cardiac arrhythmia. In addition to the prolonged QT interval, associations include muscle weakness and facial dysmorphism in Andersen-Tawil syndrome (LQTS type 7); hand/foot, facial, and neurodevelopmental features in Timothy syndrome (LQTS type 8); and profound sensorineural hearing loss in Jervell and Lange-Nielson syndrome.
Long QT syndrome 13
MedGen UID:
462083
Concept ID:
C3150733
Disease or Syndrome
Long QT syndrome (LQTS) is a cardiac electrophysiologic disorder, characterized by QT prolongation and T-wave abnormalities on the EKG that are associated with tachyarrhythmias, typically the ventricular tachycardia torsade de pointes (TdP). TdP is usually self-terminating, thus causing a syncopal event, the most common symptom in individuals with LQTS. Such cardiac events typically occur during exercise and emotional stress, less frequently during sleep, and usually without warning. In some instances, TdP degenerates to ventricular fibrillation and causes aborted cardiac arrest (if the individual is defibrillated) or sudden death. Approximately 50% of untreated individuals with a pathogenic variant in one of the genes associated with LQTS have symptoms, usually one to a few syncopal events. While cardiac events may occur from infancy through middle age, they are most common from the preteen years through the 20s. Some types of LQTS are associated with a phenotype extending beyond cardiac arrhythmia. In addition to the prolonged QT interval, associations include muscle weakness and facial dysmorphism in Andersen-Tawil syndrome (LQTS type 7); hand/foot, facial, and neurodevelopmental features in Timothy syndrome (LQTS type 8); and profound sensorineural hearing loss in Jervell and Lange-Nielson syndrome.
Long QT syndrome 2
MedGen UID:
462293
Concept ID:
C3150943
Disease or Syndrome
Long QT syndrome (LQTS) is a cardiac electrophysiologic disorder, characterized by QT prolongation and T-wave abnormalities on the EKG that are associated with tachyarrhythmias, typically the ventricular tachycardia torsade de pointes (TdP). TdP is usually self-terminating, thus causing a syncopal event, the most common symptom in individuals with LQTS. Such cardiac events typically occur during exercise and emotional stress, less frequently during sleep, and usually without warning. In some instances, TdP degenerates to ventricular fibrillation and causes aborted cardiac arrest (if the individual is defibrillated) or sudden death. Approximately 50% of untreated individuals with a pathogenic variant in one of the genes associated with LQTS have symptoms, usually one to a few syncopal events. While cardiac events may occur from infancy through middle age, they are most common from the preteen years through the 20s. Some types of LQTS are associated with a phenotype extending beyond cardiac arrhythmia. In addition to the prolonged QT interval, associations include muscle weakness and facial dysmorphism in Andersen-Tawil syndrome (LQTS type 7); hand/foot, facial, and neurodevelopmental features in Timothy syndrome (LQTS type 8); and profound sensorineural hearing loss in Jervell and Lange-Nielson syndrome.
Long QT syndrome 6
MedGen UID:
462303
Concept ID:
C3150953
Disease or Syndrome
Long QT syndrome (LQTS) is a cardiac electrophysiologic disorder, characterized by QT prolongation and T-wave abnormalities on the EKG that are associated with tachyarrhythmias, typically the ventricular tachycardia torsade de pointes (TdP). TdP is usually self-terminating, thus causing a syncopal event, the most common symptom in individuals with LQTS. Such cardiac events typically occur during exercise and emotional stress, less frequently during sleep, and usually without warning. In some instances, TdP degenerates to ventricular fibrillation and causes aborted cardiac arrest (if the individual is defibrillated) or sudden death. Approximately 50% of untreated individuals with a pathogenic variant in one of the genes associated with LQTS have symptoms, usually one to a few syncopal events. While cardiac events may occur from infancy through middle age, they are most common from the preteen years through the 20s. Some types of LQTS are associated with a phenotype extending beyond cardiac arrhythmia. In addition to the prolonged QT interval, associations include muscle weakness and facial dysmorphism in Andersen-Tawil syndrome (LQTS type 7); hand/foot, facial, and neurodevelopmental features in Timothy syndrome (LQTS type 8); and profound sensorineural hearing loss in Jervell and Lange-Nielson syndrome.
Ogden syndrome
MedGen UID:
477078
Concept ID:
C3275447
Disease or Syndrome
Ogden syndrome (OGDNS) is an X-linked neurodevelopmental disorder characterized by postnatal growth failure, severely delayed psychomotor development, variable dysmorphic features, and hypotonia. Many patients also have cardiac malformations or arrhythmias (summary by Popp et al., 2015).
Jervell and Lange-Nielsen syndrome 1
MedGen UID:
1646925
Concept ID:
C4551509
Disease or Syndrome
Jervell and Lange-Nielsen syndrome (JLNS) is characterized by congenital profound bilateral sensorineural hearing loss and long QTc, usually >500 msec. Prolongation of the QTc interval is associated with tachyarrhythmias, including ventricular tachycardia, episodes of torsade de pointes ventricular tachycardia, and ventricular fibrillation, which may culminate in syncope or sudden death. Iron-deficient anemia and elevated levels of gastrin are also frequent features of JLNS. The classic presentation of JLNS is a deaf child who experiences syncopal episodes during periods of stress, exercise, or fright. Fifty percent of individuals with JLNS had cardiac events before age three years. More than half of untreated children with JLNS die before age 15 years.
Long QT syndrome 1
MedGen UID:
1641146
Concept ID:
C4551647
Disease or Syndrome
Long QT syndrome (LQTS) is a cardiac electrophysiologic disorder, characterized by QT prolongation and T-wave abnormalities on the EKG that are associated with tachyarrhythmias, typically the ventricular tachycardia torsade de pointes (TdP). TdP is usually self-terminating, thus causing a syncopal event, the most common symptom in individuals with LQTS. Such cardiac events typically occur during exercise and emotional stress, less frequently during sleep, and usually without warning. In some instances, TdP degenerates to ventricular fibrillation and causes aborted cardiac arrest (if the individual is defibrillated) or sudden death. Approximately 50% of untreated individuals with a pathogenic variant in one of the genes associated with LQTS have symptoms, usually one to a few syncopal events. While cardiac events may occur from infancy through middle age, they are most common from the preteen years through the 20s. Some types of LQTS are associated with a phenotype extending beyond cardiac arrhythmia. In addition to the prolonged QT interval, associations include muscle weakness and facial dysmorphism in Andersen-Tawil syndrome (LQTS type 7); hand/foot, facial, and neurodevelopmental features in Timothy syndrome (LQTS type 8); and profound sensorineural hearing loss in Jervell and Lange-Nielson syndrome.
Ventricular arrhythmias due to cardiac ryanodine receptor calcium release deficiency syndrome
MedGen UID:
1781114
Concept ID:
C5542154
Disease or Syndrome
Ventricular arrhythmias due to cardiac ryanodine receptor calcium release deficiency syndrome (VACRDS) is characterized by syncope, cardiac arrest, and/or sudden unexpected death. Polymorphic ventricular tachycardia and ventricular fibrillation have been documented in these patients. Symptoms generally occur with physical activity or emotional stress, but unlike typical catecholaminergic ventricular tachycardia (CPVT), arrhythmias are not reproducible on exercise stress testing or adrenaline challenge (Sun et al., 2021). Mutation in the RYR2 gene also causes catecholaminergic polymorphic ventricular tachycardia-1 (CPVT1; 604772).
Recurrent metabolic encephalomyopathic crises-rhabdomyolysis-cardiac arrhythmia-intellectual disability syndrome
MedGen UID:
1798947
Concept ID:
C5567524
Disease or Syndrome
Individuals with TANGO2-related metabolic encephalopathy and arrhythmias can present in acute metabolic crisis (hypoglycemia, elevated lactate, mild hyperammonemia) or with developmental delay, regression, and/or seizures. The acute presentation varies from profound muscle weakness, ataxia, and/or disorientation to a comatose state. Individuals can present with intermittent acute episodes of rhabdomyolysis. The first episode of myoglobinuria has been known to occur as early as age five months. Acute renal tubular damage due to myoglobinuria can result in acute kidney injury and renal failure. During acute illness, transient electrocardiogram changes can be seen; the most common is QT prolongation. Life-threatening recurrent ventricular tachycardia or torsade de pointes occurs primarily during times of acute illness. Individuals who do not present in metabolic crises may present with gait incoordination, progressively unsteady gait, difficulty with speech, or clumsiness. Intellectual disability of variable severity is observed in almost all individuals. Seizures are observed outside the periods of crises in more than 75% of individuals. Hypothyroidism has been reported in more than one third of individuals.

Professional guidelines

PubMed

Strasburger JF, Eckstein G, Butler M, Noffke P, Wacker-Gussmann A
J Clin Pharmacol 2022 Sep;62 Suppl 1(Suppl 1):S53-S66. doi: 10.1002/jcph.2129. PMID: 36106782Free PMC Article
Al-Khatib SM, Stevenson WG, Ackerman MJ, Bryant WJ, Callans DJ, Curtis AB, Deal BJ, Dickfeld T, Field ME, Fonarow GC, Gillis AM, Granger CB, Hammill SC, Hlatky MA, Joglar JA, Kay GN, Matlock DD, Myerburg RJ, Page RL
J Am Coll Cardiol 2018 Oct 2;72(14):e91-e220. Epub 2018 Aug 16 doi: 10.1016/j.jacc.2017.10.054. PMID: 29097296
Thomas SH, Behr ER
Br J Clin Pharmacol 2016 Mar;81(3):420-7. Epub 2015 Oct 26 doi: 10.1111/bcp.12726. PMID: 26183037Free PMC Article

Recent clinical studies

Etiology

Beach SR, Gross AF, Hartney KE, Taylor JB, Rundell JR
Gen Hosp Psychiatry 2020 Nov-Dec;67:42-50. Epub 2020 Aug 22 doi: 10.1016/j.genhosppsych.2020.08.008. PMID: 32979582
Beach SR, Celano CM, Sugrue AM, Adams C, Ackerman MJ, Noseworthy PA, Huffman JC
Psychosomatics 2018 Mar-Apr;59(2):105-122. Epub 2017 Nov 3 doi: 10.1016/j.psym.2017.10.009. PMID: 29275963
Thomas SH, Behr ER
Br J Clin Pharmacol 2016 Mar;81(3):420-7. Epub 2015 Oct 26 doi: 10.1111/bcp.12726. PMID: 26183037Free PMC Article
Trinkley KE, Page RL 2nd, Lien H, Yamanouye K, Tisdale JE
Curr Med Res Opin 2013 Dec;29(12):1719-26. Epub 2013 Sep 23 doi: 10.1185/03007995.2013.840568. PMID: 24020938
Beach SR, Celano CM, Noseworthy PA, Januzzi JL, Huffman JC
Psychosomatics 2013 Jan-Feb;54(1):1-13. doi: 10.1016/j.psym.2012.11.001. PMID: 23295003

Diagnosis

Viskin S, Chorin E, Schwartz AL, Kukla P, Rosso R
Circulation 2022 Oct 11;146(15):1170-1181. Epub 2022 Oct 10 doi: 10.1161/CIRCULATIONAHA.122.061259. PMID: 36214133
Strasburger JF, Eckstein G, Butler M, Noffke P, Wacker-Gussmann A
J Clin Pharmacol 2022 Sep;62 Suppl 1(Suppl 1):S53-S66. doi: 10.1002/jcph.2129. PMID: 36106782Free PMC Article
Woosley RL, Black K, Heise CW, Romero K
Trends Cardiovasc Med 2018 Feb;28(2):94-99. Epub 2017 Aug 1 doi: 10.1016/j.tcm.2017.07.010. PMID: 28801207
Tisdale JE, Jaynes HA, Kingery JR, Mourad NA, Trujillo TN, Overholser BR, Kovacs RJ
Circ Cardiovasc Qual Outcomes 2013 Jul;6(4):479-87. Epub 2013 May 28 doi: 10.1161/CIRCOUTCOMES.113.000152. PMID: 23716032Free PMC Article
Bishop D
Confed Aust Crit Care Nurses J 1990 Dec;3(4):32-5. doi: 10.1016/s1033-3355(11)80053-6. PMID: 2129926

Therapy

Beach SR, Gross AF, Hartney KE, Taylor JB, Rundell JR
Gen Hosp Psychiatry 2020 Nov-Dec;67:42-50. Epub 2020 Aug 22 doi: 10.1016/j.genhosppsych.2020.08.008. PMID: 32979582
Gorelik E, Masarwa R, Perlman A, Rotshild V, Abbasi M, Muszkat M, Matok I
Drug Saf 2019 Apr;42(4):529-538. doi: 10.1007/s40264-018-0751-2. PMID: 30368737
Thomas SH, Behr ER
Br J Clin Pharmacol 2016 Mar;81(3):420-7. Epub 2015 Oct 26 doi: 10.1111/bcp.12726. PMID: 26183037Free PMC Article
Jasiak NM, Bostwick JR
Ann Pharmacother 2014 Dec;48(12):1620-8. Epub 2014 Sep 9 doi: 10.1177/1060028014550645. PMID: 25204465
Briasoulis A, Agarwal V, Pierce WJ
Cardiology 2011;120(2):103-10. Epub 2011 Dec 13 doi: 10.1159/000334441. PMID: 22156660

Prognosis

Strasburger JF, Eckstein G, Butler M, Noffke P, Wacker-Gussmann A
J Clin Pharmacol 2022 Sep;62 Suppl 1(Suppl 1):S53-S66. doi: 10.1002/jcph.2129. PMID: 36106782Free PMC Article
Neira V, Enriquez A, Simpson C, Baranchuk A
J Cardiovasc Electrophysiol 2019 Dec;30(12):3068-3078. Epub 2019 Oct 14 doi: 10.1111/jce.14227. PMID: 31596038
Roden DM
J Physiol 2016 May 1;594(9):2459-68. Epub 2016 Jan 18 doi: 10.1113/JP270526. PMID: 26660066Free PMC Article
Tisdale JE, Jaynes HA, Kingery JR, Mourad NA, Trujillo TN, Overholser BR, Kovacs RJ
Circ Cardiovasc Qual Outcomes 2013 Jul;6(4):479-87. Epub 2013 May 28 doi: 10.1161/CIRCOUTCOMES.113.000152. PMID: 23716032Free PMC Article
Curtis AB, Narasimha D
Clin Cardiol 2012 Mar;35(3):166-71. doi: 10.1002/clc.21975. PMID: 22389121Free PMC Article

Clinical prediction guides

Strasburger JF, Eckstein G, Butler M, Noffke P, Wacker-Gussmann A
J Clin Pharmacol 2022 Sep;62 Suppl 1(Suppl 1):S53-S66. doi: 10.1002/jcph.2129. PMID: 36106782Free PMC Article
Gorelik E, Masarwa R, Perlman A, Rotshild V, Abbasi M, Muszkat M, Matok I
Drug Saf 2019 Apr;42(4):529-538. doi: 10.1007/s40264-018-0751-2. PMID: 30368737
Van Laecke S
Acta Clin Belg 2019 Feb;74(1):41-47. Epub 2018 Sep 17 doi: 10.1080/17843286.2018.1516173. PMID: 30220246
Roden DM
J Physiol 2016 May 1;594(9):2459-68. Epub 2016 Jan 18 doi: 10.1113/JP270526. PMID: 26660066Free PMC Article
Tisdale JE, Jaynes HA, Kingery JR, Mourad NA, Trujillo TN, Overholser BR, Kovacs RJ
Circ Cardiovasc Qual Outcomes 2013 Jul;6(4):479-87. Epub 2013 May 28 doi: 10.1161/CIRCOUTCOMES.113.000152. PMID: 23716032Free PMC Article

Recent systematic reviews

Plasencia-García BO, Rodríguez-Menéndez G, Rico-Rangel MI, Rubio-García A, Torelló-Iserte J, Crespo-Facorro B
Psychopharmacology (Berl) 2021 Feb;238(2):329-340. Epub 2021 Jan 7 doi: 10.1007/s00213-020-05716-4. PMID: 33410987Free PMC Article
Beach SR, Gross AF, Hartney KE, Taylor JB, Rundell JR
Gen Hosp Psychiatry 2020 Nov-Dec;67:42-50. Epub 2020 Aug 22 doi: 10.1016/j.genhosppsych.2020.08.008. PMID: 32979582
Gorelik E, Masarwa R, Perlman A, Rotshild V, Abbasi M, Muszkat M, Matok I
Drug Saf 2019 Apr;42(4):529-538. doi: 10.1007/s40264-018-0751-2. PMID: 30368737
Tampi RR, Balderas M, Carter KV, Tampi DJ, Moca M, Knudsen A, May J
Psychosomatics 2015 Jan-Feb;56(1):36-43. Epub 2014 Sep 6 doi: 10.1016/j.psym.2014.09.002. PMID: 25619672
Johnston J, Pal S, Nagele P
Anesth Analg 2013 Sep;117(3):559-564. Epub 2013 Jun 6 doi: 10.1213/ANE.0b013e318290c380. PMID: 23744954Free PMC Article

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