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Preexcitation Syndrome

Lown-Ganong-Levine Syndrome

The preexcitation syndrome is a congenital disease caused by the existence of an accessory conduction pathway between the atria and the ventricles. This abnormality causes the disappearance of the normal delay of conduction in the atrioventricular node because this structure is bypassed as the impulse is conducted through the high speed abnormal cells. Patients are prone to developing ventricular arrhythmia. Several types of pathway with similar electrocardiographic expression leading to a short PR interval with or without a delta wave have been described.


Presentation

Preexcitation syndrome patients have complaints of varying severity, depending on the underlying arrhythmia. Furthermore, signs and symptoms vary with the age of the affected individual. Newborns and toddlers may exhibit irritability, modified behavior, and indications of heart failure. Feeding is difficult for them, which may lead to failure to thrive if episodes are frequent and prolonged. Symptoms most often occur during a febrile illness.

An older individual will describe rapid palpitations, possibly accompanied by breathing difficulties and chest pain, even if coronary atherosclerosis is not clinically relevant at rest. Palpitations are usually regular (when they are due to a supraventricular orthodromic tachyarrhythmia conducted 1:1 to the ventricles via the accessory pathway), but may also be irregular (when the substrate is atrial fibrillation) and cause diminished physical effort tolerance. Atrial fibrillation is potentially life-threatening in these patients, leading to ventricular fibrillation [1]. Antidromic tachycardia is another possibility that clinically manifests as dizziness or syncope in the context of rapid, regular palpitations. Paroxysmal episodes can be followed by polyuria.

During the arrhythmia, the patient may be pale, exhibit diaphoresis, develop cool extremities, and display symptoms of low blood pressure. The venous pressure may be increased, leading to jugular turgescence. Hepatomegaly caused by stasis and pulmonary congestion manifesting as tachypnea are sometimes observed. The heart rate varies between 200 and 250 beats/minute. The person is hemodynamically stable unless associated abnormalities, such as Ebstein anomaly or hypertrophic cardiomyopathy exist [2]. The first condition is usually accompanied by cyanosis during the arrhythmic event.

Pallor
  • Chittagong Keywords: Wolff-Parkingson-White syndrome, tachyzrrhythmia, preexcitation syndrome Abstract An adult unconscious patient was brought to intensive care unit (ICU) with impalpable peripheral pulse, non-recordable blood pressure (BP), gross pallor[banglajol.info]
Failure to Thrive
  • Feeding is difficult for them, which may lead to failure to thrive if episodes are frequent and prolonged. Symptoms most often occur during a febrile illness.[symptoma.com]
  • Fred Ferri’s popular "5 books in 1" format provides quick guidance on menorrhagia, Failure to Thrive (FTT), Cogan’s syndrome, and much more.[books.google.com]
Macroglossia
  • The clinical evaluation may describe muscle weakness, suggesting a glycogen-storage illness, mental retardation in Danon disease, or macroglossia and hepatomegaly, indicators of Pompe disease.[symptoma.com]
Hepatomegaly
  • Hepatomegaly caused by stasis and pulmonary congestion manifesting as tachypnea are sometimes observed. The heart rate varies between 200 and 250 beats/minute.[symptoma.com]
Heart Disease
  • Nineteen patients were free of associated heart disease. Average age for the group was 45 years (11 men and 11 women). Sustained atrial arrhythmias developed in four of 22 patients during MTT. All four were free of arrhythmia on subsequent MON.[ncbi.nlm.nih.gov]
  • RESULTS: Recurrences were more frequent in patients with spontaneous malignant form (34 vs. 21%), in congenital heart disease (4.2 vs. 0%) (P CONCLUSIONS: Reappearance of symptoms or a PS on ECG after AP ablation was not rare (18%) and was inconsistently[ncbi.nlm.nih.gov]
  • The CHD NSF UK Support Network Aims to support health care professionals in providing expert care to patients suffering form heart disease. A Yahoo group, with joining instructions, post and read mail.[curlie.org]
  • Join Us For A Heart Walk In Your Area Heart Walk is the American Heart Association's premiere event for raising funds to help save lives from heart disease and stroke.[americanheart.org]
Exertional Syncope
  • He did not give any history of palpitation, dyspnoea on exertion, syncope, dizziness or chest pain.[ijaweb.org]

Workup

The clinical evaluation may describe muscle weakness, suggesting a glycogen-storage illness, mental retardation in Danon disease, or macroglossia and hepatomegaly, indicators of Pompe disease. The blood workup should include the evaluation of electrolytes, such as potassium, calcium and magnesium. Screening for drugs, especially digoxin, is also important in cases where clinical judgement dictates, as they may act as precipitants for the arrhythmia. A thyroid panel can diagnose hyperthyroidism, another tachycardia trigger.

The main tool used to establish the preexcitation syndrome diagnosis is the 12-lead electrocardiogram. The QRS complex may be narrow, wide, or can change morphology in cases where there are multiple accessory pathways. Its aspect can also change if intermittent preexcitation exists. Preexcitation alternans is characterized by the alternation of a delta wave QRS complex with a normal one, whereas in concertina preexcitation the degree of preexcitation varies from one complex to the other. Typically, preexcitation syndromes exhibit a diminished PR interval (below 0.12 sec) and the presence of a delta wave (broad upstroke of the R wave). The QRS duration is increased above 0.12 sec and repolarization abnormalities are present. In Lown-Ganong-Levine syndrome, the QRS complex has no abnormalities, but the PR interval is short. More detailed information is offered by a 252-lead electrocardiogram combined with computed tomography imaging [3] [4] or electrophysiological studies [5]. Atrial fibrillation in preexcitation syndrome patients may cause hemodynamic instability [6] and should be suspected if the electrocardiogram shows a rapid succession of bizarre, shape changing complexes in a young individual [7]. The location of the accessory pathway is suggested by the lead that contains the maximally preexcited complexes [8].

An echocardiogram is needed in order to evaluate the presence of an Ebstein abnormality, hypertrophic cardiomyopathy, coronary sinus diverticula, L-transposition of the great vessels or atrial and ventricular septal defects. Parietal motion may be depressed during the acute arrhythmic episode, but should normalize when the rhythm returns to normal.

In cases where the arrhythmia has a short duration, a variety of recording devices, such as Holter monitors or implantable loop recorders can be used in order to diagnose it.

Short PR Interval
  • The classic electrocardiogram in Wolff-Parkinson-White (WPW) syndrome is characterized by a short PR interval and prolonged QRS duration in the presence of sinus rhythm with initial slurring.[ncbi.nlm.nih.gov]
  • Wolff-Par·kin·son-White syn·drome ( wūlf park'in-son wīt ), [MIM*194200] an electrocardiographic pattern sometimes associated with paroxysmal tachycardia; it consists of a short PR interval (usually 0.1 second or less; occasionally normal) together with[medical-dictionary.thefreedictionary.com]
  • However the main basis for this diagnosis is the ECG which associates short PR interval and widening of QRS complex with a delta wave.[ncbi.nlm.nih.gov]
  • CONCLUSIONS: The vast majority of young patients with a short PR interval do not have evidence of an accessory pathway and have a favorable prognosis. Thus, the yield of adenosine test in young combat recruits is questionable.[ncbi.nlm.nih.gov]
  • Short PR interval. Normal QRS complex. Norma T wave.[en.my-ekg.com]
HLA-A3
  • The participation of HLA-A3 antigen was significantly less among patients (P 0.03), while HLA-B14 antigen was not found in patients with preexcitation.[ncbi.nlm.nih.gov]
HLA-B5
  • There was an increased frequency of HLA-A9 and HLA-B5 (P 0.026 and 0.0092) in the investigated population as a whole.[ncbi.nlm.nih.gov]
HLA-DR7
  • Within 10 HLA-DR locuses, HLA-DR7 antigen was rather more frequently present, although this was not statistically significant (P 0.173).[ncbi.nlm.nih.gov]
HLA-A9
  • There was an increased frequency of HLA-A9 and HLA-B5 (P 0.026 and 0.0092) in the investigated population as a whole.[ncbi.nlm.nih.gov]

Treatment

  • Oral treatment was discontinued early because of proarrhythmic effects in 2 patients, and after 2 1/2 months because of headaches in 1 patient.[ncbi.nlm.nih.gov]
  • WPW Syndrome ECG Treatment of Wolff-Parkinson-White syndrome : Asymptomatic patients, showing only ECG changes do not require treatment.[doctortipster.com]
  • AP ablation actually is the first treatment of PS. METHODS: Successful AP ablation was performed in 261 patients; reappearance of symptoms or PS on ECG occurred in 47 patients (18%) from 20minutes to several years.[ncbi.nlm.nih.gov]
  • At no time during any of the 15 HBO2 treatments could typical EKG changes be demonstrated, nor did the patient experience any PSVT symptoms. Despite an intercurrent acute rhinitis, the patient completed the treatments uneventfully.[archive.rubicon-foundation.org]
  • & symptoms, diagnosis, complications, treatment, prevention, prognosis, and additional useful information HERE.[dovemed.com]

Prognosis

  • CONCLUSIONS: The vast majority of young patients with a short PR interval do not have evidence of an accessory pathway and have a favorable prognosis. Thus, the yield of adenosine test in young combat recruits is questionable.[ncbi.nlm.nih.gov]
  • & symptoms, diagnosis, complications, treatment, prevention, prognosis, and additional useful information HERE.[dovemed.com]
  • Prognosis [ 1 ] Prognosis is excellent once treated and catheter ablation may be curative. Sudden death is rare ( In asymptomatic patients, the capacity for antegrade conduction across the accessory pathway often decreases with age.[web.archive.org]
  • Prognosis Prognosis is usually very good once treated. [ 16 ] Catheter ablation may be curative.[patient.info]

Etiology

  • They are organised into groups, and further divided into clinical, etiological or histopathological sub-types.[orpha.net]
  • However, the molecular etiology of Wolff-Parkinson-White syndrome not associated with this genetic syndrome is likely quite different.[doi.org]

Epidemiology

  • METHODS AND RESULTS We identified 113 residents of Olmsted County, Minnesota, during the period 1953-1989 using the centralized records-linkage system provided by the Mayo Clinic and the Rochester Epidemiology Program Project.[doi.org]
  • Skip to content Home Wellbeing Health Information Medicines Professional Reference Forums Directory Blogs Patient Access Welcome to Patient.co.uk 32 Users are discussing this topic On this page Causes Epidemiology Presentation Differential diagnosis Investigations[web.archive.org]
  • The symptoms were few and there was no significant morbidity. preexcitation Wolff-Parkinson-White syndrome children epidemiology Statistics from Altmetric.com Request Permissions If you wish to reuse any or all of this article please use the link below[heart.bmj.com]
  • He was the chief consultant to the National Heart Institute from 1948 to 1955 and was a major impetus in the Framingham Heart Study , which was the first major epidemiologic work to identify risk factors in coronary artery disease.[en.wikipedia.org]
  • Epidemiology WPW syndrome is relatively common and found in 2 to 4 people per 1,000 population. In adults, it is more common in males. [ 2 ] WPW syndrome is found in all ages, although it is most common in young, previously healthy people.[patient.info]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology [ edit ] Normally, the atria and the ventricles are electrically isolated, and electrical contact between them exists only at the " atrioventricular node ".[en.wikipedia.org]
  • Pathophysiology of WPW Firstly, it is important to know about the normal conducting pathway of the heart, and how the impulses spread uniformly in the whole heart.[lecturio.com]
  • Discussion The pathophysiology behind WPW syndrome lies in an aberrant re-entrant electrical pathway in the conducting system of the heart, which is responsible for all the rhythm changes.[jnaccjournal.org]
  • Pathophysiology In WPW, the heart’s cardiomyocytes develop fibrous tissue between the atria and ventricle during the gestational stages of the seventh and twelfth week.[eplabdigest.com]

Prevention

  • Intravenous flecainide prevented induction of atrial fibrillation in 4 of 9 patients and eliminated anterograde accessory pathway conduction in 9 of the 16 patients.[ncbi.nlm.nih.gov]
  • Tiapamil appears to be of therapeutic value for the termination of tachycardia and also for its prevention in some cases. In others, it may facilitate the initiation of tachycardia.[ncbi.nlm.nih.gov]
  • Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets[books.google.com]

References

Article

  1. Sethi KK, Dhall A, Chadha DS, Garg S, Malani SK, Mathew OP. WPW and preexcitation syndromes. J Assoc Physicians India. 2007;55:10-15.
  2. Gollob MH, Green MS, Tang AS, et al. Identification of a gene responsible for familial Wolff-Parkinson-White syndrome. N Engl J Med. 2001;344(24):1823-1831.
  3. Shah A, Hocini M, Haissaguerre M, Jais P. Non-invasive mapping of cardiac arrhythmias. Curr Cardiol Rep. 2015;17(8):60.
  4. Ramanathan C, Jia P, Ghanem RN, Ryu K, Rudy Y. Activation and repolarization of the normal human heart under complete physiological conditions. Proc Nat Acad Sci USA. 2006;103:6309–6314.
  5. Calkins H, Sousa J, el-Atassi R, et al. Diagnosis and cure of the Wolff-Parkinson-White syndrome or paroxysmal supraventricular tachycardias during a single electrophysiologic test. N Engl J Med. 1991;324(23):1612-1618.
  6. Mark DG, Brady WJ, Pines JM. Preexcitation syndromes: diagnostic consideration in the ED. Am J Emerg Med. 2009;27(7):878-888.
  7. Fengler BT, Brady WJ, Plautz CU. Atrial fibrillation in the Wolff-Parkinson-White syndrome: ECG recognition and treatment in the ED. Am J Emerg Med. 2007;25(5):576-583.
  8. Arruda MS, McClelland JH, Wang X, et al. Development and validation of an ECG algorithm for identifying accessory pathway ablation site in Wolff-Parkinson-White syndrome. J Cardiovasc Electrophysiol. 1998;9(1):2-12.

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Last updated: 2018-06-22 00:00