Pacemaker Syndrome


Article Author:
Arshad Muhammad Iqbal


Article Editor:
Syed Jamal


Editors In Chief:
Stacy Mandras


Managing Editors:
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Frank Smeeks
Kristina Soman-Faulkner
Benjamin Eovaldi
Radia Jamil
Sobhan Daneshfar
Saad Nazir
William Gossman
Pritesh Sheth
Hassam Zulfiqar
Navid Mahabadi
Steve Bhimji
John Shell
Matthew Varacallo
Ahmad Malik
Mark Pellegrini
James Hughes
Beata Beatty
Hajira Basit
Phillip Hynes


Updated:
5/5/2019 11:37:46 PM

Introduction

Pacemakers have become an established modality for long-term management of life-threatening arrhythmias and improve the quality of life of affected patients significantly. Pacemakers have also become a cornerstone in the management of cardiomyopathies.

Conventionally, these devices pace the ventricle in isolation, often leading to improper or mistimed atrial and ventricular contraction causing a reduction in cardiac output. This may be due to mistimed atrial contraction occurring against closed atrioventricular (AV) valve or atrial contraction occurring too close to ventricular contraction, producing back pressure in venous circulation systems and loss of atrial contribution to the ventricular output.

Since the invention of this therapy in 1958, physicians have reported reductions in cardiac output as a response to ventricular pacing. Although baroreceptor reflexes do compensate for this by increasing peripheral vascular resistance, this is not always the case. In patients who fail to compensate for the loss of cardiac output with these mechanisms, a wide variety of symptoms are noted and mostly attributable to a loss in cardiac output and decreased peripheral resistance. This phenomenon and the resulting constellation of signs and symptoms is referred to as pacemaker syndrome.

Etiology

Pacemaker syndrome is most commonly seen in the setting of a single chamber device with ventricular sensing and pacing lead. Since there is no atrial sensing lead to guide the ventricle, the ventricle contracts at the programmed rate regardless of the timing of atrial contraction. This leads to loss of AV synchrony which leads to a loss in stroke volume and cardiac output. VA conduction, which is usually a ventricular beat finding its way upwards to the atria also leads to a mistimed atrial contraction and produces similar effects.

Epidemiology

The incidence of pacemaker syndrome varies widely, but has been reported as high as 80%[1] and as low as 5%[2], and is seen fairly constantly in both genders.[3]

With single-chamber and ventricular-paced devices in VVI mode, the incidence approaches 20% as has been reported in the MOST trial.[4]

In large trials as MOST, PASE and CTTOP, it was observed the pacing mode change or device revision to dual chamber pacing (DDD) helped the most and was often what was required to alleviate the symptoms in the majority of patients.[5][6][7]

Pathophysiology

The physiologic electrical coupling and synchronization of atria and ventricles are altered whenever a pacing device is implanted. This is less of a concern with temporary pacemakers which are implanted for a shorter duration, but with permanent pacemakers, this physiologic coupling gets impaired and leads to electrical dyssynchrony which effects both AV coordination and VV coordination commonly referred to as AV dyssynchrony and VV dyssynchrony.

AV dyssynchrony results in mistimed atrial contractions which cause back pressure in venous circulation systems that causes congestion (peripheral and pulmonary) as well as loss of atrial contribution leading to decreased cardiac output. Frequently a ventricular beat that is conducted upward to the atrium also gives rise to a similar phenomenon.[8]

VV dyssynchrony leads to mistiming of contraction of opposing ventricular walls, as RV pacing causing left bundle branch block (LBBB) pattern which is associated with asynchronous contraction of the inter-ventricular septum and opposing LV walls. This event often leads to a loss in output of the left ventricle and increases back pressure in the pulmonary circulation.[9][10]

Hence, AV dyssynchrony and VV dyssynchrony and retrograde conduction of ventricular beats form the pathophysiological basis of this entity.[11]

History and Physical

Diagnosis is often straightforward due to the correlation of timing of device implantation/onset of pacing and the timing of symptoms, as well as more frequent and persistent symptoms in patients who are pacing dependant in the setting of single chamber devices.

In the aging population, however, there might be some confusion in reporting and remembering symptoms, and most of these could be non-specific which can often be attributed to aging.

Symptoms often include the following;

  • Neurologic: Fatigability, memory loss, confusion, and often transient blackouts
  • Cardiovascular: Low blood pressures, exertional dyspnea, orthopnea, palpitations, a decrease in exercise capacity, syncope
  • Hemodynamic: Prominent neck pulsations, abdominal pulsations, right upper quadrant (RUQ) pain

Examination often yields the following signs:

  • Hypotension, tachypnea, low oxygen saturation, lung rales
  • Narrow pulse pressure and low pulse volume
  • Neck vein distension and cannon wave in JVP tracing
  • Precordial examination may reveal regurgitant murmurs and variability in the intensity of first heart sound
  • Pulsatile liver and ascites may be present in severe cases
  • Lower extremity edema
  • Neurologic examination may reveal confusion, dizziness, or altered mental status

Evaluation

Chest x-ray usually reveals a single pacemaker lead stationed in RV.

ECG reveals pacing spikes with adequate capture but no relation/synchrony between P waves and QRS complexes.

Laboratory studies are usually not required; however, cardiac troponins and an echocardiogram may reveal new wall motion abnormalities or a decline in EF suggesting cardiomyopathy. Clinicians best reach diagnosis when they correlate patients' symptoms with the cardiac rhythm. Historically, a drop in systolic blood pressures during episodes of ventricular pacing has been used as a perimeter to diagnose this entity, but this is not always the case and may not be practical.

Diagnosis is clinical and often one of exclusion. This may include the use of a Holter monitor or event recorder to look for arrhythmias. Often, device interrogation reveals pacemaker dependancy which also gives a clue to the presence of this entity.

Treatment / Management

Treatment includes switching to DDD or VDD modes in a dual chamber device. Up-gradation to a dual chamber device is necessary for single-chamber pacemakers. Concomitantly if the patient has LV dysfunction and low EF, then a CRT will be the preferred device for implantation/up-gradation.

Standard heart failure therapy is warranted for patients with LV dysfunction.

Differential Diagnosis

Tachyarrhythmias, most commonly atrial fibrillation or flutter, are the usual differential and can be easily excluded by an ECG and device interrogation. LV dysfunction and cardiomyopathies which usually present with similar symptoms can be excluded by echocardiography with tissue characterization by cardiac MRI (if the device is MRI compatible).

Prognosis

Prognosis is favorable because this entity is widely recognized and easily treated. However, if not recognized, and left untreated, it can result in the development of LV dysfunction, RV dysfunction, tachyarrhythmias and poor quality of life due to severe and debilitating symptoms as described above.

Complications

  • Poor quality of life
  • LV dysfunction
  • RV dysfunction
  • Heart failure
  • Tachyarrhythmias

Consultations

Such patients should be managed in consortium with a cardiologist and a cardiac electrophysiologist.

Deterrence and Patient Education

Providers should educate patients about the need for the device and based on the indications and pacing needs, what type of device will benefit most. This must be done before device implantation.

However, if single chamber ventricular lead devices are to be implanted then the patient must be counseled in detail about the possibility, prevalence and signs/symptoms of this condition. Every attempt must be made to maintain electrical synchrony in patients undergoing cardiac rhythm device implantation.

Pearls and Other Issues

Despite advances in cardiac device therapy, it is recognized that complete electrical synchrony between cardiac chambers is not attainable at present and there still might be some patients who will develop this constellation of signs and symptoms. However, it is the clinician and electrophysiologist's job to make sure that all attempts are made to maintain and restore AV and VV synchrony to avoid pacemaker syndrome.

Enhancing Healthcare Team Outcomes

The right device for the right patient should be the provider's rationale.

Discussion and effective communication between the primary or referring physician, the cardiologist, and the cardiac electrophysiologist should be ensured at all times. This will not only minimize the development of the syndrome by selecting the right device for the right patient but will also help in effective management that improves patient outcomes and ensures patient safety.


Interested in Participating?

We are looking for contributors to author, edit, and peer review our vast library of review articles and multiple choice questions. In as little as 2-3 hours you can make a significant contribution to your specialty. In return for a small amount of your time, you will receive free access to all content and you will be published as an author or editor in eBooks, apps, online CME/CE activities, and an online Learning Management System for students, teachers, and program directors that allows access to review materials in over 500 specialties.

Improve Content - Become an Author or Editor

This is an academic project designed to provide inexpensive peer-reviewed Apps, eBooks, and very soon an online CME/CE system to help students identify weaknesses and improve knowledge. We would like you to consider being an author or editor. Please click here to learn more. Thank you for you for your interest, the StatPearls Publishing Editorial Team.

Pacemaker Syndrome - Questions

Take a quiz of the questions on this article.

Take Quiz
A 30-year-old male with Lyme disease develops complete heart block and is treated with a single lead pacemaker and long-term antibiotics. At follow-up, he complains of confusion, cough, fatigue, and palpitations. Vital signs show a heart rate of 60 beats/min, blood pressure of 125/75 mmHg, and respirations of 20. There is jugular venous distention and an occasionally prominent pulsation over neck vein that are regular. Lung auscultation reveals rales. There is no pedal edema. ECG shows ventricular pacing with adequate spike and capture and ventricular rate of 60 beats/min. What is the most likely cause of this patient's symptoms?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
In which mode is pacemaker syndrome most commonly observed?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A patient with sick sinus syndrome and AV block has a DDD pacemaker. She presented with palpitations, was found to be in atrial fibrillation with a ventricular response rate of 120 bpm, and her pacemaker was reprogrammed to the VVI mode. One week later she complains of dyspnea on exertion, weakness, and lightheadedness. ECG shows sinus rhythm with pacing at 70 bpm. Which of the following is appropriate?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A 73-year-old male with a ventricular inhibited pacemaker presents with shortness of breath and fatigue. The pacemaker was placed seven years ago for sinus pauses leading to syncope. The patient has been on verapamil for hypertension for six months. Holter monitor shows mostly ventricular pacing at 70 bpm. Select the treatment option that should be avoided.



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Which is not a symptom of pacemaker syndrome?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
What pacing mode is associated with pacemaker syndrome?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up

Pacemaker Syndrome - References

References

Heldman D,Mulvihill D,Nguyen H,Messenger JC,Rylaarsdam A,Evans K,Castellanet MJ, True incidence of pacemaker syndrome. Pacing and clinical electrophysiology : PACE. 1990 Dec     [PubMed]
Andersen HR,Thuesen L,Bagger JP,Vesterlund T,Thomsen PE, Prospective randomised trial of atrial versus ventricular pacing in sick-sinus syndrome. Lancet (London, England). 1994 Dec 3     [PubMed]
Lamas GA,Lee K,Sweeney M,Leon A,Yee R,Ellenbogen K,Greer S,Wilber D,Silverman R,Marinchak R,Bernstein R,Mittleman RS,Lieberman EH,Sullivan C,Zorn L,Flaker G,Schron E,Orav EJ,Goldman L, The mode selection trial (MOST) in sinus node dysfunction: design, rationale, and baseline characteristics of the first 1000 patients. American heart journal. 2000 Oct     [PubMed]
Link MS,Hellkamp AS,Estes NA 3rd,Orav EJ,Ellenbogen KA,Ibrahim B,Greenspon A,Rizo-Patron C,Goldman L,Lee KL,Lamas GA, High incidence of pacemaker syndrome in patients with sinus node dysfunction treated with ventricular-based pacing in the Mode Selection Trial (MOST). Journal of the American College of Cardiology. 2004 Jun 2     [PubMed]
Lamas GA,Orav EJ,Stambler BS,Ellenbogen KA,Sgarbossa EB,Huang SK,Marinchak RA,Estes NA 3rd,Mitchell GF,Lieberman EH,Mangione CM,Goldman L, Quality of life and clinical outcomes in elderly patients treated with ventricular pacing as compared with dual-chamber pacing. Pacemaker Selection in the Elderly Investigators. The New England journal of medicine. 1998 Apr 16     [PubMed]
Connolly SJ,Kerr CR,Gent M,Roberts RS,Yusuf S,Gillis AM,Sami MH,Talajic M,Tang AS,Klein GJ,Lau C,Newman DM, Effects of physiologic pacing versus ventricular pacing on the risk of stroke and death due to cardiovascular causes. Canadian Trial of Physiologic Pacing Investigators. The New England journal of medicine. 2000 May 11     [PubMed]
Lamas GA,Lee KL,Sweeney MO,Silverman R,Leon A,Yee R,Marinchak RA,Flaker G,Schron E,Orav EJ,Hellkamp AS,Greer S,McAnulty J,Ellenbogen K,Ehlert F,Freedman RA,Estes NA 3rd,Greenspon A,Goldman L, Ventricular pacing or dual-chamber pacing for sinus-node dysfunction. The New England journal of medicine. 2002 Jun 13     [PubMed]
Ausubel K,Furman S, The pacemaker syndrome. Annals of internal medicine. 1985 Sep     [PubMed]
Rosenqvist M,Isaaz K,Botvinick EH,Dae MW,Cockrell J,Abbott JA,Schiller NB,Griffin JC, Relative importance of activation sequence compared to atrioventricular synchrony in left ventricular function. The American journal of cardiology. 1991 Jan 15     [PubMed]
Grines CL,Bashore TM,Boudoulas H,Olson S,Shafer P,Wooley CF, Functional abnormalities in isolated left bundle branch block. The effect of interventricular asynchrony. Circulation. 1989 Apr     [PubMed]
Lamas GA,Ellenbogen KA, Evidence base for pacemaker mode selection: from physiology to randomized trials. Circulation. 2004 Feb 3     [PubMed]

Disclaimer

The intent of StatPearls is to provide practice questions and explanations to assist you in identifying and resolving knowledge deficits. These questions and explanations are not intended to be a source of the knowledge base of all of medicine, nor is it intended to be a board or certification review of Cardiology-Failure/Transplant. The authors or editors do not warrant the information is complete or accurate. The reader is encouraged to verify each answer and explanation in several references. All drug indications and dosages should be verified before administration.

StatPearls offers the most comprehensive database of free multiple-choice questions with explanations and short review chapters ever developed. This system helps physicians, medical students, dentists, nurses, pharmacists, and allied health professionals identify education deficits and learn new concepts. StatPearls is not a board or certification review system for Cardiology-Failure/Transplant, it is a learning system that you can use to help improve your knowledge base of medicine for life-long learning. StatPearls will help you identify your weaknesses so that when you are ready to study for a board or certification exam in Cardiology-Failure/Transplant, you will already be prepared.

Our content is updated continuously through a multi-step peer review process that will help you be prepared and review for a thorough knowledge of Cardiology-Failure/Transplant. When it is time for the Cardiology-Failure/Transplant board and certification exam, you will already be ready. Besides online study quizzes, we also publish our peer-reviewed content in eBooks and mobile Apps. We also offer inexpensive CME/CE, so our content can be used to attain education credits while you study Cardiology-Failure/Transplant.