Pediatric Sinus Node Dysfunction Follow-up

  • Author: M Silvana Horenstein, MD; Chief Editor: Stuart Berger, MD   more...
 
Updated: Aug 13, 2010
 

Complications

  • Symptomatic patients with sinus node dysfunction (SND) may develop syncope, exercise intolerance, and cardiac dysfunction due to bradycardia and loss of AV synchrony.
  • These patients can also develop atrial tachyarrhythmias, such as atrial flutter or fibrillation.
  • Treatment of symptoms is achieved with the implant of an atrial pacemaker to provide atrial rate support. This prevents symptoms related to bradycardia from occurring. In patients with atrial tachyarrhythmias, it is a useful adjunct to antiarrhythmic therapy.
  • After undergoing a Mustard procedure for transposition of the great arteries or a Fontan procedure for single ventricle, many pediatric patients who have SND and frequent episodes of atrial flutter or fibrillation currently receive an intracardiac defibrillator (ICD) device. The ICD lead is placed in the RA. When the atrial rate exceeds the programmed value, the ICD assumes that the patient has an atrial tachyarrhythmia and provides a shock of 10-30 J.
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Prognosis

  • Symptomatic patients with normal systemic ventricular function and SND have an overall good prognosis with atrial (rate-responsive) pacing.
  • The overall prognosis in patients with SND and additional systemic ventricular dysfunction (eg, numerous postoperative Mustard and Fontan patients) depends on their underlying ventricular dysfunction or degree of CHF.
  • A study has shown that in those patients who have undergone a Fontan surgery and developed SND, endocardial atrial leads can be implanted relatively safely and permit low-energy thresholds as long as 5 years after implant.[6]
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Patient Education

  • Because most of the pediatric patients with SND have already received surgery for CHD (eg, Mustard procedure, Fontan procedure), their education is focused on recognizing symptoms of CHF and tachyarrhythmias, such as atrial flutter/fibrillation, which are usually poorly tolerated.
  • Patients who are on antiarrhythmic medication for atrial flutter or fibrillation should be instructed to take their medications regularly and to visit the cardiologist as scheduled. They should also be cognizant of adverse effects and toxicity of the medication.
  • In patents who have already received a Mustard or Fontan procedure, undergoing yearly echocardiography to monitor cardiac function is advisable. If cardiac function is decreased, anti-CHF management should be started and close follow-ups with the cardiologist are advisable.
  • Patients who have a pacemaker should be instructed about how to have regular checks. This is usually achieved from home with a transtelephonic monitor that transmits to a central monitoring station, which in turn, contacts the cardiologist in case a problem is detected (eg, device malfunction, arrhythmia).
  • Patients who have an ICD device should receive the same instructions that are given to patients who have pacemakers. Because these patients often are placed on antiarrhythmic medication, they also should receive instruction regarding medication schedules and information about adverse effects and toxicity. In addition, in cases of frequent atrial flutter or fibrillation episodes, which are followed by a shock from the ICD, patients are instructed to avoid activities that may pose a risk to themselves and/or other people (eg, driving) and are instructed when to go to the cardiologist or the emergency department.
  • For excellent patient education resources, please refer to eMedicine's Heart Center and, for information specific to arrhythmias, see the eMedicine article Heart Rhythm Disorders.
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Contributor Information and Disclosures
Author

M Silvana Horenstein, MD  Assistant Professor, Department of Pediatrics, University of Texas Medical School at Houston; Medical Doctor Consultant, Legacy Department, Best Doctors, Inc

M Silvana Horenstein, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Peter P Karpawich, MD  Professor of Pediatric Medicine, Department of Pediatrics (Cardiology), Wayne State University School of Medicine; Director, Cardiac Electrophysiology and Pacemaker Services, Children's Hospital of Michigan

Peter P Karpawich, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Heart Rhythm Society, Michigan State Medical Society, and Pediatric Electrophysiology Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Paul M Seib, MD  Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas Children's Hospital

Paul M Seib, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Arkansas Medical Society, International Society for Heart and Lung Transplantation, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

John W Moore, MD, MPH  Professor of Clinical Pediatrics, Section of Pediatric Cardiology, Department of Pediatrics, University of California San Diego School of Medicine; Director of Cardiology, Rady Children's Hospital

John W Moore, MD, MPH is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Gilbert Z Herzberg, MD  Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center

Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD  Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

References

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This 12-lead ECG is from an asymptomatic 10 year-old girl, which was brought to our attention because of the irregularity of the P-P intervals. This ECG shows sinus arrhythmia at a rate of 65-75 beats per minute. The P waves all originate from the sinus node because they have a positive axis (upright) in leads I, II, and aVF. The PR interval is 104 milliseconds, and the QRS is narrow at 86 milliseconds, with a normal axis of 64°. The corrected QT (QTc) interval measures 402 milliseconds. Therefore, this is a normal ECG.
Below is an ECG of a 2-year-old girl who was referred to the clinic by the pediatrician for evaluation of a heart murmur. This ECG shows atrial rhythm originating most likely from the lower left atrium (P waves are inverted in lead I and are positive in II and aVF, with a frontal axis of 124°). The PR interval measures 113 milliseconds, and the QRS is narrow at 90 milliseconds. Right ventricular conduction delay is shown, which is best seen in the precordial leads V1 and V2. The QRS frontal axis shows right axis deviation (reference range for a 2-year-old child is 0-110°). The patient does not have right ventricular hypertrophy by voltage criteria. The inverted T waves in V1 are a normal finding at this age. An echocardiogram showed a moderately sized atrial septal defect. Nonsinus atrial rhythm is not a synonym of sinus node dysfunction.
This is a 12-lead ECG from a 12-year-old boy with history of syncope. This patient was healthy until 1 month earlier, when he started to experience episodes of lightheadedness. The ECG shows sinus arrhythmia (bradycardia) at a rate of 50-79 beats per minute with a PR interval of 136 milliseconds. Two junctional escape beats are present after a prolonged pause. The QRS is narrow at 85 milliseconds with a normal frontal axis of 70°. The corrected QT interval (QTc) is 411 milliseconds. A later electrophysiologic (EP) study showed prolonged sinus node recovery time (SNRT) and sinoatrial conduction time (SACT). Because of the patient's symptoms and his sinus node dysfunction, he received an atrial pacemaker. If this 12-lead ECG had been recorded from an asymptomatic patient, the findings would be considered within normal limits and no further workup would be indicated. In this case, the lightheadedness and, ultimately, the syncope define sick sinus syndrome, with the patient requiring pacemaker therapy.
 
 
 
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