Clinical Characteristics and Mid-term Follow-up in Children with Isolated Complete Atrioventricular Block (original) (raw)
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Outcomes of pacemaker implantation in isolated congenital atrioventricular block
Progress in Pediatric Cardiology, 2020
Background: Most patients with isolated congenital atrioventricular block will eventually require a pacemaker implantation at an early age. Aims: To document a single-center experience of all patients with pacemaker implantation secondary to isolated congenital atrioventricular block. Methods: A retrospective review of all patients with isolated congenital atrioventricular block and pacemaker implantation over a period of 11 years was conducted. All patients were identified by using a surgical database. Descriptive statistics were mainly used. Results: Eighteen patients met the inclusion criteria. The median follow-up time, gestational age, and birth weight were 3.8 years, 35 weeks, and 2.1 kg, respectively. Eleven patients were born to mothers with autoimmune disease (61%). The median age at the time of initial implantation, initial generator longevity, and hospital stay per intervention were 5 days, 5 years, and 7 days, respectively. All patients had epicardial pacing except for one patient, who had a transvenous system. Among the 18 patients, initial implants consisted of 13 single-chamber, three dual-chamber, and two with temporary pacing only. Two patients underwent an upgrade to a biventricular system due to associated dilated cardiomyopathy without response. To date, all initially implanted leads are functional except for four leads. Four patients died due to extreme prematurity and from complications of dilated cardiomyopathy. Conclusion: Isolated congenital atrioventricular block is associated with significant mortality, especially when associated with dilated cardiomyopathy. Baseline left bundle branch block escape rhythm was noted to be present in most patients with dilated cardiomyopathy. Overall, lead performance and battery longevity were excellent. However, transvenous-type temporary pacing was associated with significant complications.
The American Journal of Cardiology, 1981
Because an initial Adams-Stokes attack in the patient with congenital complete atrioventricuiar (A-V) block may sometimes prove fatal, there is a need to be able to identify the patient at great risk of having such attacks. Twenty-four children with congenital complete A-V block were followed up for 1 to 19 years to determine the efficacy of current methods of predicting risk for Adams-Stokes syncope and the usefulness of pacemaker therapy in relieving symptoms. The heart rate at rest, configuration of surface electrocardiographic complexes, data obtained during intracardiac electrophysiologic study and response to graded treadmill exercise testing were compared in children with and without syncope. One or more Adams-Stokes episodes were experienced by eight children, one of whom died. Only a persistent heart rate at rest of 50 beats/min or less demonstrated any significant (probability [p] <O.Ol) correlation with the incidence of syncope. Intracardiac electrophysiologic study was of little benefit because the site of block did not correlate with syncope. Although the increase in heart rate during treadmill exercise testing showed no correlation with prevalence of syncope or location of block, exercise-induced ventricular ectopic beats may have predictive value in older children and young adults.
First-degree atrioventricular block
Journal of Interventional Cardiac Electrophysiology, 2007
Marked first-degree AV block (PR ≥ 0.30 s) can produce a clinical condition similar to that of the pacemaker syndrome. Clinical evaluation often requires a treadmill stress test because patients are more likely to become symptomatic with mild or moderate exercise when the PR interval cannot adapt appropriately. Uncontrolled studies have shown that many such symptomatic patients with normal left ventricular (LV) function improve with conventional dual chamber pacing (Class IIa indication). In contrast, marked first-degree AV block with LV systolic dysfunction and heart failure is still a Class IIb indication, a recommendation that is now questionable because a conventional DDD (R) pacemaker would be committed to right ventricular pacing (and its attendant risks) virtually 100% of the time. It would seem prudent at this juncture to consider a biventricular DDD device in this situation. Patients with suboptimally programmed pacemakers may develop functional atrial undersensing because the P wave tends to migrate easily into the postventricular atrial refractory period (PVARP). Retrograde vetriculoatrial conduction block is uncommon in marked first-degree AV block so a relatively short PVARP can often be used at rest with little risk of endless loop tachycardia. The usefulness of a short PVARP may be negated by special PVARP functions in some pulse generators designed to time out a long PVARP at rest and a gradually shorter one with activity. First-degree AV block during cardiac resynchronization therapy (CRT) predisposes to loss of ventricular resynchronization during biventricular pacing because it favors the initiation of electrical "desyn-chronization" especially in association with a relatively fast atrial rate and a relatively slow programmed upper rate. Patients with first-degree AV block have a poorer outcome with CRT than patients with a normal PR interval, a response that may involve several mechanisms. (1) The long PR interval may be a marker of more advanced heart disease. (2) Patients with first-degree AV block may experience more episodes of undetected "electrical desynchronization". (3) "Concealed resynchronization" whereupon ventricular activation in patients with a normal PR interval may result from fusion of electrical wavefronts coming from the right bundle branch and the impulse from the LV electrode. The resultant hemodynamic response may be superior because the detrimental effects of right ventricular stimulation (required in the setting of a longer PR interval) are avoided.
Pediatric Cardiology, 2006
Low heart rate is the predominantly used indication for pacemaker intervention in patients with isolated congenital atrioventricular block (CAVB). The aim of this study was to compare the difference in heart rates recorded with ECG and Holter monitoring between paced (PM) and nonpaced (NPM) patients with isolated CAVB before pacemaker implantation to identify additional predictors for future PM need. Retrospective evaluation of atrial and ventricular rates (electrocardiography) and minimal and maximal (Holter) heart rates in 129 CAVB patients prior to PM implantation (n = 93) was performed, and results are expressed in V adjusted for age and sex. The average V score for the atrial rate was 0.51 (n = 50) in the PM group and 0.60 (n = 22) in the NPM group (not-significant). The average z score for the ventricular (average) rate was À0.91 (n = 83) in the PM group and À0.93 (n = 33) in the NPM group (not-significant). Minimal heart rate was À0.94 (n = 61) in the PM group and À0.86 (n = 25) in the NPM group (not significant). Maximal heart rate was À0.96 (n = 61) in the PM group and À0.95 (n = 26) in the NPM group (not significant). Initial recordings of the average heart rate and the minimal and maximal heart rate recorded during Holter monitoring do not seem to predict future pacemaker need in patients with CAVB. Studies with exercise stress tests are needed to confirm these findings.
Europace, 2002
Aims This study assessed survival, morbidity and impact of pacemaker (PM) therapy in children with Congenital Complete Atrioventricular Block (CCAVB). Methods and Results Data of 32 children, diagnosed as showing CCAVB at a median age of 0•4 years (range foetal-10 years), were retrospectively analysed. For comparison of clinical data patients were separated into two groups: CCAVB without structural heart disease (group 1; n=23) and with structural heart disease (group 2; n=9). Median follow-up time was 10•2 years. Pacemakers (PM) were implanted in 17 group 1 and all group 2 children. Frequency of PM therapy, age and symptoms before PM implantation did not differ significantly between the groups. Indications for PM implantation were bradycardia in 15, decreased exercise tolerance in 6, syncope in 3 and heart failure in 2 children. PM system related complications occurred in 11/26 (42%) children. Although 1 child died due to PM exit block no further CCAVB related symptoms were recorded in children with PM. Conclusion PM therapy reduces mortality and morbidity in children with CCAVB when compared with natural history data. Although children with PM are free from CCAVB related symptoms limited morbidity remains due to PM system related complications.
Atrioventricular block: Natural history after permanent ventricular pacing
The American Journal of Cardiology, 1978
The preimplantation status, postimplantation morbidlty and causes of late mortality were summarized for 246 patients who underwent pacing for atrioventricular (A-V) block at the University of Michigan for the 14 years from 1961 to 1974. The survival rate at 1,s and 10 years was 88, 61 and 49 percent, respectively. Risk of death was greatest among patients with antecedent ischemic or hypertensive heart disease or congestive heart failure In the period before pacemaker implantation, patients older than 74 years at inttlal implantation and those receiving a pacemaker before 1965. Forty-two percent of the 109 deaths were related to apparent progression of underlying cardiac disease. Pacing system malfunction was a contributing documented cause of only 3 deaths. Even with permanent pacemaker implantation, patients with A-V block have a higher age-specific mortality rate than the general U.S. population. Survival improved steadily over the period of study. This change is attributed to apparent improvements in treatment of cardiovascular disease including more effective treatment of congestive heart failure and valve replacement for selected patients as well as elimination of immediate postoperative mortality. Permanent ventricular pacing has been the recognized treatment for atrioventricular (A-V) block for more than 15 years. Previous reports14 have documented the continued morbidity and mortality in patients with A-V block who have received pacemakers. This report reviews the status of such patients to determine the natural history of this disorder after permanent pacemaker implantation, the causes of late morbidity and mortality and the changes in mortality that occurred during the follow-up period. Survival of this population group was also compared with that of the U.S. population matched for age, race and sex. Methods The records of all adult patients who received permanent pacemakers at the University of Michigan Medical Center from 1961 through 1974 were reviewed. Many of the patients have been the subject of previous reports from this institution.P1l Since 1965, all patients have been followed up in a pacemaker clinic. Data on the history, clinical course, specific arrhythmias seen and follow-up findings were transferred to computer tape. Survival curves were determined with the life table method12J3 utilizing the Michigan Interactive Data Analysis System (MIDAS)14 and the Interactive Graphic Survival Analysis System (IGSAS).15 For each patient survival time was calculated from the date of the first pacemaker implantation until death or the end of the observation period, March 1,1976. For the one patient who was lost to follow-up, survival was calculated from the date of implantation to the date of last follow-up. Patient selection: The study population consisted of 246 adult patients who received their initial pacemaker at the University of Michigan Medical Center for an A-V conduction disturbance during the years specified. Patients with bifascicular block and syncope, but without documentation of complete A-V block, and two patients with atrial fibrillation and advanced second degree block
Indicators of a Poor Outcome after Temporary Pacing in Patients with Complete Atrioventricular Block
Annals of King Edward Medical University, 2013
Abstract: Background: Temporary pace maker (tpm) implantation is an emergency procedure, performed in symptomatic patients with AV block We report our experience of temporary pacing at a tertiary care center. It is the first study of its type from a local center. Objectives: To study the clinical course of patients after tpm implantation and to determine indicators of a poor outcome after tpm implantation. Methods: This was descriptive retrospective study. Data of all tpm procedures performed at Children Hospital from 2006 to 2012 was retrieved. We excluded patients with surgically placed tpm leads. All patients recieving transvenous tpm were included in our study. Results: Total of 12 patients received tpm, 8 were male (66.6%) and 4 were female(33.3%). Median age was 6 years ( range 1.4 -13 year ). Mean weight was 30kg, (range 8.7 - 50kg). All of them presented with complete heart block.One patient each had post diphtheria cardiomyopathy, unknown poisoning. The rest had congenital...
BioMed research international, 2018
The aim of the study was to compare the two approaches to chronic right ventricular pacing currently adopted in clinical practice: right ventricular apical (RVA) and non-RVA pacing. Chronic RVA pacing is associated with an increased risk of atrial fibrillation, morbidity, and even mortality. Non-RVA pacing may yield more physiologic ventricular activation and provide potential long-term benefits and has recently been adopted as standard procedure at many implanting centers. The Right Pace study was a multicenter, prospective, single-blind, nonrandomized trial involving 437 patients indicated for dual-chamber pacemaker implantation with a high percentage of RV pacing. RV lead-tip target location was the apex or the interventricular septum. RVA (274) and non-RVA patients (163) did not differ in baseline characteristics. During a median follow-up of 19 months (25th-75th percentiles, 13-25), 17 patients died. The rates of the primary outcome of death due to any cause or hospitalization ...
Echocardiography (Mount Kisco, N.Y.), 2015
Left ventricular (LV) dysfunction is the major reason for poor outcomes in patients with congenital complete atrioventricular block (CCAVB) and pacemaker. Long-term pacing has been associated with LV mechanical dyssynchrony. However, the relationship of dyssynchrony and LV dysfunction is not clear. We sought to evaluate the prevalence of LV dyssynchrony by real time three-dimensional echocardiography (RT3DE) in patients with CCAVB and its association with LV dysfunction. In addition, we evaluated the agreement between RT3DE and tissue Doppler imaging (TDI) for detecting LV dyssynchrony. We studied 50 patients [median age 20 years old (5 months to 62 years), 68% women] with CCAVB and pacemaker who underwent complete two-dimensional echocardiography and RT3DE. LV dyssynchrony was considered if the systolic dyssynchrony index (SDI) was ≥5%. Intraventricular mechanical delay was defined by TDI when differences in electromechanical activation between LV walls were >65 msec. LV systoli...
Congenital Complete Atrioventricular Block and Dilated Cardiomyopathy: New Light for an Old Disease
Case reports in cardiology, 2012
We present a case of a patient with known complete congenital atrioventricular block (CAVB) since the age of 7 years old that developed dilated cardiomyopathy ten years after VVI-R pacemaker implantation. He presented severe biventricular dysfunction and was symptomatic despite optimal medical therapy. Cardiac resynchronization therapy was used, and he showed clinical and electrocardiographic improvement a month later.