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.
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 . 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 . The first condition is usually accompanied by cyanosis during the arrhythmic event.
Entire Body System
- Surgical Procedure
However, treatment options do exist including medications and surgical procedures. Medications include antiarrhythmics to control tachycardia, but they are not always effective. [web.archive.org]
A surgical procedure known as catheter ablation may be used in such cases. This procedure may also be used in individuals who are at high risk of developing cardiac arrest and sudden death including some asymptomatic individuals. [rarediseases.org]
Lithotomy position was made for the surgical procedure. After 30 minutes of surgery, ECG rhythm showed few slurred QRS rhythms with delta wave for 2 minutes with intermittent NSR followed by continuous rhythm suggestive of WPW syndrome [Figure 2]a. [ijaweb.org]
Complications As with any surgical procedure, cardiac catheterizations come with a generic list of possible complications. One of the complications that are sometimes reported involves some temporary nerve involvement. [en.wikipedia.org]
In other cases, a doctor may recommend a surgical procedure to determine the location of the extra electrical pathway, followed by a catheter ablation. [rarediseases.info.nih.gov]
Abstract Lown Ganong Levine (LGL) which is a preexcitation syndrome is defined with paroxysmal tachycardia, dyspnea, anxiety, angina, and the complaint of fatigue accompanied by characteristic changes on ECG (short PR). [bibliomed.org]
She denied chest pain, shortness of breath, dyspnea, and walks 15 minutes per day without difficulty. Figure 1 Show Answer Please share your thoughts on this Patient Case Quiz in the comments section below. [acc.org]
Symptoms of WPW may include one or more of the following: Heart palpitations – a sudden pounding, fluttering or Racing feeling in your chest Dizziness – feeling lightheaded or faint Shortness of breath (dyspnea) Anxiety Rarely, cardiac arrest (sudden [my.clevelandclinic.org]
Lipomatous hypertrophy of the interatrial septum presenting as an obstructive right atrial mass in a patient with exertional dyspnea. J Am Soc Echocardiogr 2007;20:1319.e3-5. 11. Burke AP, Litovsky S, Virmani R. [ijcva.com]
She also gave history of chest deformity since birth, but denied history of any syncopal attacks, dyspnea, and cyanosis. There was no history of deafness, abnormalities of genitalia, or secondary sexual characteristics. [ijdvl.com]
Paroxysmal tachycardia. Arch. Int. Med. 27: 571, 1921. [johnsonfrancis.org]
Following tiapamil it was not possible to initiate the tachycardia in 4 cases and atrial echoes in 1 case; in 2 patients the tachycardia zone widened and in 3 it was not altered. In the latter, the cycle length of the tachycardia increased. [ncbi.nlm.nih.gov]
- Heart Disease
You will find sites that serve the needs of people who need support for Congenital Heart Defects, along with Coronary Heart Disease. The sites provide information and resources. Many sites provide up to the minuet news on issues related to health. [curlie.org]
Signs of organic heart disease were not present. Electrocardiogram showed, in addition to typical features of ventricular pre-excitation, a QS pattern in leads V4-V6. [moh-it.pure.elsevier.com]
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]
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]
We conclude that the abnormalities of signal conduction described in association with LGL syndrome is probably liable to be affected by some cardiac structural disease and interestingly we are reporting right side heart disease in such problem. [omicsonline.org]
- Heart Block
Ablation of the accessory pathway is more difficult in LGL syndrome compared to WPW syndrome since it is located very close to the AV node and there is risk of causing complete heart block necessitating permanent pacemaker implantation. [healio.com]
[…] disease I45 - Other conduction disorders I45.0 - Right fascicular block I45.1 - Other and unspecified right bundle-branch block I45.2 - Bifascicular block I45.3 - Trifascicular block I45.4 - Nonspecific intraventricular block I45.5 - Other specified [unboundmedicine.com]
Extreme care if administering a beta blocker to a patient already taking verapamil (may precipitate extreme bradycardia or heart block); in case of need, esmolol probably is the perioperative beta blocker of choice. [accessanesthesiology.mhmedical.com]
"Experimental production of complete heart block by electrocoagulation in the closed chest dog". Am. Heart J. 104 (6): 1328–34. doi:10.1016/0002-8703(82)90163-6. PMID 7148651. ^ Keane, D; Ruskin, J (Fall 2002). [en.wikipedia.org]
block: AV block (First degree, Second degree, Third degree) - Bundle branch block (Left, Right) - Bifascicular block - Trifascicular block Pre-excitation syndrome (Wolff-Parkinson-White, Lown-Ganong-Levine) - Long QT syndrome - Adams-Stokes syndrome [bionity.com]
Accelerated atrioventricular conduction Accessory atrioventricular conduction Anomalous atrioventricular excitation Lown-Ganong-Levine syndrome Pre-excitation atrioventricular conduction Wolff-Parkinson-White syndrome MS-DRG Mapping DRG Group #308-310 [icd.codes]
Code I45.6 - Pre-excitation syndrome ⑩ [Billable] Includes Accelerated atrioventricular conduction Accessory atrioventricular conduction Anomalous atrioventricular excitation Lown-Ganong-Levine syndrome Pre-excitation atrioventricular conduction Wolff-Parkinson-White [unboundmedicine.com]
Electrocardiographic Signs of Pre-excitation As the stimulus originates from the sinus node, the P wave will be normal. In pre-excitation, ventricles depolarize from two different points: bundle of His and the accessory pathway. [en.my-ekg.com]
Update: Repeat ECG after three days showing disappearance of pre-excitation Repeat ECG after three days showing disappearance of pre-excitation No pre-excitation is seen in this repeat ECG, meaning that there is no conduction through the accessory pathway [cardiophile.org]
Olympic sprinter shares his stroke story Learn the warning signs of stroke No bones about it: It’s time to fight heart disease with Best Friend Fridays The American Heart Association’s new effort looks to put pets in the workplace, while helping raise [americanheart.org]
True/False: Upon conversion of atrial fibrillation to sinus rhythm, the patient should be observed for signs of possible stroke. [quizlet.com]
Earlier this year, she went onstage at the Delete Bone Cancer Gala in New York and shared that the condition caused her to suffer a stroke when she was just 18. She thought she would never get better. [web.archive.org]
Escalated stroke output. Reduced cardiac output. How Can Lown Ganong Levine Syndrome Be Diagnosed? There are several diagnostic tools for the diagnosis and confirmation of Lown Ganong Levine syndrome. Mentioned below are a few of them. [icliniq.com]
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   or electrophysiological studies . Atrial fibrillation in preexcitation syndrome patients may cause hemodynamic instability  and should be suspected if the electrocardiogram shows a rapid succession of bizarre, shape changing complexes in a young individual . The location of the accessory pathway is suggested by the lead that contains the maximally preexcited complexes .
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.
- Wide QRS Complex
In 1913 Cohn and Fraser published the first patient with a short P-R interval, wide QRS complexes, and paroxysmal tachycardia. [ncbi.nlm.nih.gov]
Short PR interval and normal width of QRS complex in a young woman. The absence of delta wave and wide QRS complex excludes the diagnosis of WPW syndrome. Click here for a more detailed ECG ECG 2. [metealpaslan.com]
This results in a short PR interval and a wide QRS complex with a delta wave (an initial slurring of the QRS complex, which represents early, abnormal ventricular depolarization). [journals.lww.com]
- Short PR Interval
In the seminal paper Lown B et al observed that out of the 200 patients with short PR interval, 23 had paroxysmal tachycardia (11%), compared with 0.5 to 1% in a control group of 200 patients with normal PR interval. [johnsonfrancis.org]
Short PR interval PR interval < 120 msec (< 3 small squares). P wave is normal: upright in leads II, III and avF. QRS width is normal. [metealpaslan.com]
Historically, some authors have referred to patients with a short PR interval and normal QRS duration as having LGL. [emedicine.medscape.com]
Patients who display reentrant tachycardia with short PR intervals may benefit from an accessory pathway or partial AV nodal ablation. [ncbi.nlm.nih.gov]
Other ECG Findings
- Abnormal ECG
It's the perfect book to turn to for clear and clinically relevant guidance on all of today’s ECG applications. Comprehensively and expertly describes how to capture and interpret all normal and abnormal ECG findings in adults and children. [books.google.com]
This abnormal ECG finding is called a delta wave, a finding not seen in the normal heart. A normal ECG, and an ECG with a delta wave, as seen in patients with Wolff-Parkinson-White Syndrome. [uwhealthkids.org]
Alternative names : Preexcitation syndrome; WPW Definition Wolff-Parkinson-White syndrome involves episodes of rapid heart rate (tachycardia) and baseline abnormal ECG caused by abnormal electrical pathways (circuits) in the heart. [forum.softpedia.com]
This is especially true in asymptomatic young patients who have been told of their abnormal ECG results. [emedicine.medscape.com]
WPW Syndrome ECG Treatment of Wolff-Parkinson-White syndrome : Asymptomatic patients, showing only ECG changes do not require treatment. [doctortipster.com]
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]
What Is the Treatment for Lown Ganong Levine Syndrome? Treatment of Lown-Ganong-Levine is presently focused on the utilization of antiarrhythmic drugs. [icliniq.com]
Dementia or Psychiatric Illness/Treatment. [cancertherapyadvisor.com]
& symptoms, diagnosis, complications, treatment, prevention, prognosis, and additional useful information HERE. [dovemed.com]
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]
Prognosis The overall prognosis of patients with LGL syndrome is good. [wikidoc.org]
Lown-Ganong-Levine Syndrome In this article Epidemiology Presentation Investigations Management Prognosis Historical The Lown-Ganong-Levine (LGL) syndrome is one of the pre-excitation syndromes of which Wolff-Parkinson-White (WPW) syndrome is the best [patient.info]
Etiology Some evidence suggests that LGL is a hereditary condition in some families. No definitive structural/anatomic anomaly has been identified as yet. [clinicalgate.com]
[…] of palpitations is long, and in some cases, the etiology is unable to be determined. In one study reporting the etiology of palpitations, 43% were found to be of cardiac etiology, 31% of psychiatric etiology and approximately 10% were classified as [en.wikipedia.org]
They are organised into groups, and further divided into clinical, etiological or histopathological sub-types. [orpha.net]
Tricuspid regurgitation is functional and is a satellite of left-sided heart disease and/or elevated pulmonary artery pressure most of the time; and when progressive, it worsens the patient prognosis whatever the underlying etiology . [omicsonline.org]
The Lown-Ganong-Levine (LGL) pattern and enhanced AV nodal conduction (EAVNC) share some common features and have often been considered to have a similar etiology. [medilib.ir]
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]
Lown-Ganong-Levine syndrome Microchapters Overview Historical Perspective Classification Pathophysiology Causes Differentiating Lown-Ganong-Levine Syndrome from other Diseases Epidemiology and Demographics Risk Factors Natural History, Complications and [wikidoc.org]
Epidemiology Lown suggested that 17% of people with a PR interval of less than 120 ms would have the condition. It is however very rare, with prevalence estimated to be less than one in a million. [patient.info]
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]
The pathophysiology of this syndrome includes an accessory pathway connecting the atria and the atrioventricular (AV) node (James fiber), or between the atria and the His bundle (Brechenmacher fiber). [amjcaserep.com]
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]
Probably the main drawback in our case is the absence of electrophysiological study which, if were done, it could explain a new idea about the pathophysiology of this syndrome. [omicsonline.org]
This page covers the pathophysiology and ECG features of pre-excitation syndromes in sinus rhythm. [litfl.com]
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]
Prevention There are no primary preventive measures available for LGL syndrome. References ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 1.8 1.9 LOWN, BERNARD; GANONG, WILLIAM F.; LEVINE, SAMUEL A. (1952). [wikidoc.org]
- Sethi KK, Dhall A, Chadha DS, Garg S, Malani SK, Mathew OP. WPW and preexcitation syndromes. J Assoc Physicians India. 2007;55:10-15.
- 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.
- Shah A, Hocini M, Haissaguerre M, Jais P. Non-invasive mapping of cardiac arrhythmias. Curr Cardiol Rep. 2015;17(8):60.
- 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.
- 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.
- Mark DG, Brady WJ, Pines JM. Preexcitation syndromes: diagnostic consideration in the ED. Am J Emerg Med. 2009;27(7):878-888.
- 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.
- 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.