Guidance Offered on Children With Wolff-Parkinson-White Syndrome

BOSTON – Although it ranks behind hypertrophic cardiomyopathy as a cause of sudden cardiac death in children and young adults, the Wolff-Parkinson-White electrocardiogram pattern warrants monitoring and, in some cases, intervention, according to authors of a consensus statement announced at the annual meeting of the Heart Rhythm Society.

The Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS) issued an expert consensus statement on the care of young, asymptomatic patients with the Wolff-Parkinson-White (WPW) electrocardiographic patterns, caused by an accessory cardiac electrical pathway.

The statement is intended as a guideline for clinicians who treat patients aged 8-21 years who have the WPW pattern but are otherwise asymptomatic, said lead author Dr. Mitchell I. Cohen, chief of pediatric cardiology and director of pediatric electrophysiology at Phoenix Children’s Hospital.

An estimated 65% of young patients with WPW are asymptomatic, Dr. Cohen said in a briefing. In those patients, "essentially one of three things can happen: They may remain asymptomatic; they may develop an arrhythmia that can be managed with medication or ablation; or, more concerning, they may have a life-threatening event and die suddenly. The incidence of sudden death is quite rare, but it’s not zero," he said.

The consensus panel, comprising both pediatric and adult electrophysiologists, estimates the prevalence of the WPW to range from 1 to 3 per 1,000. The incidence of sudden death from WPW, including resuscitated sudden cardiac death (SCD), is about 4.5 per 1,000 patient-years, on the basis of a study of asymptomatic adults with the pattern who were followed for a mean of 38 months (J. Am. Coll. Cardiol. 2003;41:239-44).

In contrast, the incidence of sudden death attributable to hypertrophic cardiomyopathy was about 7.4 per 1,000 person-years in one study. (N. Engl. J. Med. 2000;342:1778-85).

Symptoms of WPW may include palpitations, dizziness, syncope, and supraventricular tachycardia. Many young patients are diagnosed only after they undergo electrocardiograms required by many school districts prior to participation in organized sports.

Dr. Cohen says that although the condition can be effectively treated with catheter-based radiofrequency ablation, invasive techniques may not always be necessary or appropriate for younger patients.

Specifically, the statement recommends the following for patients aged 8-21 years who have the WPW ECG pattern:

• Patients should take an exercise stress test if the ambulatory ECG exhibits persistent pre-excitation.

• Invasive risk stratification (transesophageal or intracardiac) should be performed to assess the shortest pre-excited RR interval in atrial fibrillation in patients in whom noninvasive testing fails to demonstrate clear and abrupt loss of pre-excitation.

• Catheter ablation may be considered in young patients with a measurement of the SPERRI (Shortest Pre-Excited RR Interval) of 250 ms or less in atrial fibrillation, as they are at increased risk for SCD.

• Ablation may be safely deferred in lower-risk young patients with a SPERRI longer than 250 ms in atrial fibrillation.

• Catheter ablation may be considered in previously asymptomatic patients who subsequently develop cardiovascular symptoms such as syncope or palpitations.

• Ablation may be considered regardless of the anterograde characteristics of the accessory pathway in asymptomatic patients with a WPW ECG pattern and structural heart disease.

• Asymptomatic patients with a WPW ECG pattern and ventricular dysfunction secondary to dyssynchronous contractions, regardless of anterograde characteristics of the bypass tract, may benefit from ablation.

• It is safe to prescribe medications for attention-deficit/hyperactivity disorder (ADHD) for asymptomatic patients with a WPW ECG in accordance with American Heart Association guidelines, which state that ADHD medications may be used in this setting after cardiac evaluation and with intermittent monitoring and supervision by a pediatric cardiologist.

The consensus statement has been endorsed by the governing bodies of the PACES, the HRS, the American College of Cardiology Foundation, the American Heart Association, the American Academy of Pediatrics, and the Canadian Heart Rhythm Society.

Dr. Cohen reported having no relevant disclosures.

BOSTON – Although it ranks behind hypertrophic cardiomyopathy as a cause of sudden cardiac death in children and young adults, the Wolff-Parkinson-White electrocardiogram pattern warrants monitoring and, in some cases, intervention, according to authors of a consensus statement announced at the annual meeting of the Heart Rhythm Society.

The Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS) issued an expert consensus statement on the care of young, asymptomatic patients with the Wolff-Parkinson-White (WPW) electrocardiographic patterns, caused by an accessory cardiac electrical pathway.

The statement is intended as a guideline for clinicians who treat patients aged 8-21 years who have the WPW pattern but are otherwise asymptomatic, said lead author Dr. Mitchell I. Cohen, chief of pediatric cardiology and director of pediatric electrophysiology at Phoenix Children’s Hospital.

An estimated 65% of young patients with WPW are asymptomatic, Dr. Cohen said in a briefing. In those patients, "essentially one of three things can happen: They may remain asymptomatic; they may develop an arrhythmia that can be managed with medication or ablation; or, more concerning, they may have a life-threatening event and die suddenly. The incidence of sudden death is quite rare, but it’s not zero," he said.

The consensus panel, comprising both pediatric and adult electrophysiologists, estimates the prevalence of the WPW to range from 1 to 3 per 1,000. The incidence of sudden death from WPW, including resuscitated sudden cardiac death (SCD), is about 4.5 per 1,000 patient-years, on the basis of a study of asymptomatic adults with the pattern who were followed for a mean of 38 months (J. Am. Coll. Cardiol. 2003;41:239-44).

In contrast, the incidence of sudden death attributable to hypertrophic cardiomyopathy was about 7.4 per 1,000 person-years in one study. (N. Engl. J. Med. 2000;342:1778-85).

Symptoms of WPW may include palpitations, dizziness, syncope, and supraventricular tachycardia. Many young patients are diagnosed only after they undergo electrocardiograms required by many school districts prior to participation in organized sports.

Dr. Cohen says that although the condition can be effectively treated with catheter-based radiofrequency ablation, invasive techniques may not always be necessary or appropriate for younger patients.

Specifically, the statement recommends the following for patients aged 8-21 years who have the WPW ECG pattern:

• Patients should take an exercise stress test if the ambulatory ECG exhibits persistent pre-excitation.

• Invasive risk stratification (transesophageal or intracardiac) should be performed to assess the shortest pre-excited RR interval in atrial fibrillation in patients in whom noninvasive testing fails to demonstrate clear and abrupt loss of pre-excitation.

• Catheter ablation may be considered in young patients with a measurement of the SPERRI (Shortest Pre-Excited RR Interval) of 250 ms or less in atrial fibrillation, as they are at increased risk for SCD.

• Ablation may be safely deferred in lower-risk young patients with a SPERRI longer than 250 ms in atrial fibrillation.

• Catheter ablation may be considered in previously asymptomatic patients who subsequently develop cardiovascular symptoms such as syncope or palpitations.

• Ablation may be considered regardless of the anterograde characteristics of the accessory pathway in asymptomatic patients with a WPW ECG pattern and structural heart disease.

• Asymptomatic patients with a WPW ECG pattern and ventricular dysfunction secondary to dyssynchronous contractions, regardless of anterograde characteristics of the bypass tract, may benefit from ablation.

• It is safe to prescribe medications for attention-deficit/hyperactivity disorder (ADHD) for asymptomatic patients with a WPW ECG in accordance with American Heart Association guidelines, which state that ADHD medications may be used in this setting after cardiac evaluation and with intermittent monitoring and supervision by a pediatric cardiologist.

The consensus statement has been endorsed by the governing bodies of the PACES, the HRS, the American College of Cardiology Foundation, the American Heart Association, the American Academy of Pediatrics, and the Canadian Heart Rhythm Society.

Dr. Cohen reported having no relevant disclosures.

BOSTON – Although it ranks behind hypertrophic cardiomyopathy as a cause of sudden cardiac death in children and young adults, the Wolff-Parkinson-White electrocardiogram pattern warrants monitoring and, in some cases, intervention, according to authors of a consensus statement announced at the annual meeting of the Heart Rhythm Society.

The Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS) issued an expert consensus statement on the care of young, asymptomatic patients with the Wolff-Parkinson-White (WPW) electrocardiographic patterns, caused by an accessory cardiac electrical pathway.

The statement is intended as a guideline for clinicians who treat patients aged 8-21 years who have the WPW pattern but are otherwise asymptomatic, said lead author Dr. Mitchell I. Cohen, chief of pediatric cardiology and director of pediatric electrophysiology at Phoenix Children’s Hospital.

An estimated 65% of young patients with WPW are asymptomatic, Dr. Cohen said in a briefing. In those patients, "essentially one of three things can happen: They may remain asymptomatic; they may develop an arrhythmia that can be managed with medication or ablation; or, more concerning, they may have a life-threatening event and die suddenly. The incidence of sudden death is quite rare, but it’s not zero," he said.

The consensus panel, comprising both pediatric and adult electrophysiologists, estimates the prevalence of the WPW to range from 1 to 3 per 1,000. The incidence of sudden death from WPW, including resuscitated sudden cardiac death (SCD), is about 4.5 per 1,000 patient-years, on the basis of a study of asymptomatic adults with the pattern who were followed for a mean of 38 months (J. Am. Coll. Cardiol. 2003;41:239-44).

In contrast, the incidence of sudden death attributable to hypertrophic cardiomyopathy was about 7.4 per 1,000 person-years in one study. (N. Engl. J. Med. 2000;342:1778-85).

Symptoms of WPW may include palpitations, dizziness, syncope, and supraventricular tachycardia. Many young patients are diagnosed only after they undergo electrocardiograms required by many school districts prior to participation in organized sports.

Dr. Cohen says that although the condition can be effectively treated with catheter-based radiofrequency ablation, invasive techniques may not always be necessary or appropriate for younger patients.

Specifically, the statement recommends the following for patients aged 8-21 years who have the WPW ECG pattern:

• Patients should take an exercise stress test if the ambulatory ECG exhibits persistent pre-excitation.

• Invasive risk stratification (transesophageal or intracardiac) should be performed to assess the shortest pre-excited RR interval in atrial fibrillation in patients in whom noninvasive testing fails to demonstrate clear and abrupt loss of pre-excitation.

• Catheter ablation may be considered in young patients with a measurement of the SPERRI (Shortest Pre-Excited RR Interval) of 250 ms or less in atrial fibrillation, as they are at increased risk for SCD.

• Ablation may be safely deferred in lower-risk young patients with a SPERRI longer than 250 ms in atrial fibrillation.

• Catheter ablation may be considered in previously asymptomatic patients who subsequently develop cardiovascular symptoms such as syncope or palpitations.

• Ablation may be considered regardless of the anterograde characteristics of the accessory pathway in asymptomatic patients with a WPW ECG pattern and structural heart disease.

• Asymptomatic patients with a WPW ECG pattern and ventricular dysfunction secondary to dyssynchronous contractions, regardless of anterograde characteristics of the bypass tract, may benefit from ablation.

• It is safe to prescribe medications for attention-deficit/hyperactivity disorder (ADHD) for asymptomatic patients with a WPW ECG in accordance with American Heart Association guidelines, which state that ADHD medications may be used in this setting after cardiac evaluation and with intermittent monitoring and supervision by a pediatric cardiologist.

The consensus statement has been endorsed by the governing bodies of the PACES, the HRS, the American College of Cardiology Foundation, the American Heart Association, the American Academy of Pediatrics, and the Canadian Heart Rhythm Society.

Dr. Cohen reported having no relevant disclosures.