Validation of scoring system predicting permanent pacemaker implantation after transcatheter aortic valve replacement.

BACKGROUND: Transcatheter aortic valve replacement (TAVR) is being increasingly performed in patients with severe aortic stenosis. Despite newer generation valves, atrioventricular (AV) conduction disturbance is a common complication, necessitating permanent pacemaker (PPM) implantation in about 10% of patients. Hence, it is imperative to improve periprocedural risk stratification to predict PPM implantation after TAVR. The objective of this study was to externally validate a novel risk-stratification model derived from the National Inpatient Sample (NIS) database that predicts risk of PPM from TAVR. METHODS: Components of the score included pre-TAVR left and right bundle branch block, sinus bradycardia, second-degree AV block, and transfemoral approach. The scoring system was applied to 917 patients undergoing TAVR at our institution from November 2011 to February 2017. We assessed its predictive accuracy by looking at two components: discrimination using the C-statistic and calibration using the Hosmer-Lemeshow goodness of fit test. RESULTS: Ninety patients (9.8%) required PPM. The scoring system showed good discrimination with C-statistic score of 0.6743 (95% CI: 0.618-0.729). Higher scores suggested increased PPM risk, that is, 7.3% with score ⩽3, 19.23% with score 4-6, and 37.50% with score ≥7. Patients requiring PPM were older (81.4 versus 78.7 years, P = .002). Length of stay and in-hospital mortality was significantly higher in PPM group. CONCLUSIONS: The NIS database derived PPM risk prediction model was successfully validated in our database with acceptable discriminative and gradation capacity. It is a simple but valuable tool for patient counseling pre-TAVR and in identifying high-risk patients.

Trends in complete heart block after transcatheter aortic valve replacement: A population based analysis.

OBJECTIVES: To define the national rate of complete heart block (CHB) after transcatheter aortic valve replacement (TAVR) and its impact on procedural mortality, overall cost, and length of hospital stay. BACKGROUND: CHB leading to permanent pacemaker (PPM) implantation is one of the most common complications post TAVR. National data on the temporal trend of CHB post TAVR are lacking. METHODS: We queried the 2012-2014 National Inpatient Sample databases to identify all patients who underwent TAVR. Patients with preoperative pacemakers or implantable cardioverter-defibrillators were excluded. Association between CHB and outcomes, and overall trends in rate of CHB, PPM implantation, and inpatient mortality were examined. RESULTS: Of 35,500 TAVR procedures, 3,675 (10.4%) had CHB. Overall, occurrence of CHB significantly increased from 8.4% in 2012 to 11.8% in 2014 (adjusted OR per year: 1.23; 95% confidence interval [CI]: 1.17-1.29, P trend <0.001). During the same period, PPM implantation increased from 9.5 to 13.7% (adjusted OR per year: 1.22; 95% CI: 1.16-1.28, P trend <0.001). Patients with CHB had higher odds of in-hospital mortality when compared to patients without CHB (5.9% vs. 4.2%, adjusted OR: 1.32; 95% CI: 1.12-1.56; p = 0.001). Moreover, CHB was also associated with longer length of stay (LOS) and higher hospitalization cost. CONCLUSIONS: There was a significant increase in rates of CHB and PPM implantation over the study period. Development of CHB was associated with increased in-hospital mortality, LOS, and hospitalization cost.

Dysrhythmias and heart failure complicating acute myocardial infarction: An emergency medicine review.

INTRODUCTION: Patients with acute myocardial infarction (AMI) may suffer several complications after the acute event, including dysrhythmias and heart failure (HF). These complications place patients at risk for morbidity and mortality. OBJECTIVE: This narrative review evaluates literature and guideline recommendations relevant to the acute emergency department (ED) management of AMI complicated by dysrhythmia or HF, with a focus on evidence-based considerations for ED interventions. DISCUSSION: Limited evidence exists for ED management of dysrhythmias in AMI due to relatively low prevalence and frequent exclusion of patients with active cardiac ischemia from clinical studies. Management decisions for bradycardia in the setting of AMI are determined by location of infarction, timing of the dysrhythmia, rhythm assessment, and hemodynamic status of the patient. Atrial fibrillation is common in the setting of AMI, and caution is warranted in acute rate control for rapid ventricular rate given the possibility of compensation for decreased ventricular function. Regular wide complex tachycardia in the setting of AMI should be managed as ventricular tachycardia with electrocardioversion in the majority of cases. Management directed towards HF from left ventricular dysfunction in AMI consists of noninvasive positive pressure ventilation, nitroglycerin therapy, and early cardiac catheterization. Norepinephrine is the first line vasopressor for patients with cardiogenic shock and hypoperfusion on clinical examination. Early involvement of a multi-disciplinary team is recommended when caring for patients in cardiogenic shock. CONCLUSIONS: This review discusses considerations of ED management of dysrhythmias and HF associated with AMI.

Incidence and predictors of permanent pacemaker implantation following treatment with the repositionable Lotus™ transcatheter aortic valve.

OBJECTIVES: To determine the incidence and predictors of permanent pacemaker (PPM) requirement following transcatheter aortic valve replacement (TAVR) with the mechanically expanded LotusTM Valve System (Boston Scientific). BACKGROUND: Pacemaker implantation is the most common complication following TAVR. Predictors of pacing following TAVR with the Lotus valve have not been systematically assessed. METHODS: Consecutive patients with severe aortic stenosis who underwent Lotus valve implantation were prospectively recruited at a single-centre. Patients with a pre-existing PPM were excluded. Baseline ECG, echocardiographic and multiple detector computed tomography as well as procedural telemetry and depth of implantation were independently analyzed in a blinded manner. The primary endpoint was 30-day incidence of pacemaker requirement (PPM implantation or death while pacing-dependent). Multivariate analysis was performed to identify independent predictors of the primary endpoint. RESULTS: A total of 104 consecutive patients underwent TAVR with the Lotus valve with 9/104 (9%) with a pre-existing PPM excluded. New or worsened procedural LBBB occurred in 78%. Thirty-day incidence of the primary pacing endpoint was 28%. The most common indication for PPM implantation was complete heart block (CHB) (69%). Independent predictors of the primary endpoint included pre-existing RBBB (hazard ratio [HR] 2.8, 95% CI 1.1-7.0; P = 0.032) and depth of implantation below the noncoronary cusp (NCC) (HR 2.4, 95% CI 1.0-5.7; P = 0.045). CONCLUSIONS: Almost a third of Lotus valve recipients require pacemaker implantation within 30 days. The presence of pre-existing RBBB and the depth of prosthesis implantation below the NCC were significant pacing predictors. © 2016 Wiley Periodicals, Inc.

PR interval prolongation in coronary patients or risk equivalent: excess risk of ischemic stroke and vascular pathophysiological insights.

BACKGROUND: Whether PR prolongation independently predicts new-onset ischemic events of myocardial infarction and stroke was unclear. Underlying pathophysiological mechanisms of PR prolongation leading to adverse cardiovascular events were poorly understood. We investigated the role of PR prolongation in pathophysiologically-related adverse cardiovascular events and underlying mechanisms. METHODS: We prospectively investigated 597 high-risk cardiovascular outpatients (mean age 66 ± 11 yrs.; male 67%; coronary disease 55%, stroke 22%, diabetes 52%) for new-onset ischemic stroke, myocardial infarction (MI), congestive heart failure (CHF), and cardiovascular death. Vascular phenotype was determined by carotid intima-media thickness (IMT). RESULTS: PR prolongation >200 ms was present in 79 patients (13%) at baseline. PR prolongation >200 ms was associated with significantly higher mean carotid IMT (1.05 ± 0.37 mm vs 0.94 ± 0.28 mm, P = 0.010). After mean study period of 63 ± 11 months, increased PR interval significantly predicted new-onset ischemic stroke (P = 0.006), CHF (P = 0.040), cardiovascular death (P < 0.001), and combined cardiovascular endpoints (P < 0.001) at cut-off >200 ms. Using multivariable Cox regression, PR prolongation >200 ms independently predicted new-onset ischemic stroke (HR 8.6, 95% CI: 1.9-37.8, P = 0.005), cardiovascular death (HR 14.1, 95% CI: 3.8-51.4, P < 0.001) and combined cardiovascular endpoints (HR 2.4, 95% CI: 1.30-4.43, P = 0.005). PR interval predicts new-onset MI at the exploratory cut-off >162 ms (C-statistic 0.70, P = 0.001; HR: 8.0, 95% CI: 1.65-38.85, P = 0.010). CONCLUSIONS: PR prolongation strongly predicts new-onset ischemic stroke, MI, cardiovascular death, and combined cardiovascular endpoint including CHF in coronary patients or risk equivalent. Adverse vascular function may implicate an intermediate pathophysiological phenotype or mediating mechanism.

Assessment of fluorinated steroids to avert progression and mortality in anti-SSA/Ro-associated cardiac injury limited to the fetal conduction system.

OBJECTIVES: Extension of disease beyond the atrioventricular (AV) node is associated with increased mortality in cardiac neonatal lupus (NL). Treatment of isolated heart block with fluorinated steroids to prevent disease progression has been considered but published data are limited and discordant regarding efficacy. This study evaluated whether fluorinated steroids given to manage isolated advanced block prevented development of disease beyond the AV node and conferred a survival benefit. METHODS: In this retrospective study of cases enrolled in the Research Registry for NL, inclusion was restricted to anti-SSA/Ro-exposed cases presenting with isolated advanced heart block in utero who either received fluorinated steroids within 1 week of detection (N=71) or no treatment (N=85). Outcomes evaluated were: development of endocardial fibroelastosis, dilated cardiomyopathy and/or hydrops fetalis; mortality and pacemaker implantation. RESULTS: In Cox proportional hazards regression analyses, fluorinated steroids did not significantly prevent development of disease beyond the AV node (adjusted HR=0.90; 95% CI 0.43 to 1.85; p=0.77), reduce mortality (HR=1.63; 95% CI 0.43 to 6.14; p=0.47) or forestall/prevent pacemaker implantation (HR=0.87; 95% CI 0.57 to 1.33; p=0.53). No risk factors for development of disease beyond the AV node were identified. CONCLUSIONS: These data do not provide evidence to support the use of fluorinated steroids to prevent disease progression or death in cases presenting with isolated heart block.

Effects of ventricular conduction block patterns on mortality in hospitalized patients with dilated cardiomyopathy: a single-center cohort study.

BACKGROUND: Ventricular conduction blocks (VCBs) are associated with poor outcomes in patients with known cardiac diseases. However, the prognostic implications of VCB patterns in dilated cardiomyopathy (DCM) patients need to be evaluated. The purpose of this study was to determine all-cause mortality in patients with DCM and VCB. METHODS: This cohort study included 1119 DCM patients with a median follow-up of 34.3 (19.5-60.8) months, patients were then divided into left bundle branch block (LBBB), right bundle branch block (RBBB), intraventricular conduction delays (IVCD) and narrow QRS groups. The all-cause mortality was assessed using Kaplan-Meier survival curves and Cox regression. RESULTS: Of those 1119 patients, the all-cause mortality rates were highest in patients with IVCD (47.8, n = 32), intermediate in those with RBBB (32.9, n = 27) and LBBB (27.1 %, n = 60), and lowest in those with narrow QRS (19.9 %, n = 149). The all-cause mortality risk was significantly different between the VCB and narrow QRS group (log-rank χ2 = 51.564, P < 0.001). The presence of RBBB, IVCD, PASP ≥ 40 mmHg, left atrium diameter and NYHA functional class were independent predictors of all-cause mortality in DCM patients. CONCLUSIONS: Our findings indicate that RBBB and IVCD at admission,but not LBBB, were independent predictors of all-cause mortality in patients with DCM.

Survival of 4 dogs with persistent atrial standstill treated by pacemaker implantation.

Pacemakers were implanted in 4 client-owned female dogs which had persistent atrial standstill. Three dogs were alive after 14 to 39 months and 1 dog was euthanized after 10.5 years. This report demonstrates that some dogs with persistent atrial standstill can survive for extended time periods.

Survie de 4 chiennes atteintes de paralysie auriculaire persistante traitées à l'aide de l'implantation d'un cardiostimulateur. Des cardiostimulateurs ont été implantés chez 4 chiennes, appartenant à des propriétaires, atteintes de paralysie auriculaire persistante. Trois chiennes étaient vivantes après 14 à 39 mois et 1 chienne a été euthanasiée après 10,5 ans. Ce rapport démontre que certains chiens atteints de paralysie auriculaire persistante peuvent survivre pendant des périodes de temps prolongées.(Traduit par Isabelle Vallières).

Current status and future perspectives on alcohol septal ablation for hypertrophic obstructive cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiac disease. Patients may present with a wide variety of symptoms, ranging from relatively asymptomatic to heart failure, recurrent syncope, angina, or sudden death. Once diagnosed, a thorough clinical, anatomic and physiologic assessment should be undertaken. Treatment options include both pharmacologic and invasive therapies, with a goal to reduce symptoms and possibly extend longevity. Traditionally, the "gold standard" for treating severe obstructive HCM has been ventricular septal myotomy-myomectomy. Since its introduction in 1994, alcohol septal ablation (ASA) has emerged as an acceptable alternative in patients who meet strict anatomic criteria, and has been supported in recent guidelines. We review the indications, technique, competency requirements, alternatives, outcomes, complications, and future directions of ASA.

Prognostic values of clockwise and counterclockwise rotation for cardiovascular mortality in Japanese subjects: a 24-year follow-up of the National Integrated Project for Prospective Observation of Noncommunicable Disease and Its Trends in the Aged, 1980-2004 (NIPPON DATA80).

BACKGROUND: Although clockwise rotation and counterclockwise rotation are distinct findings of the ECG, their prognostic significance is rarely studied. METHODS AND RESULTS: We studied prognostic values of clockwise and counterclockwise rotation on total, cardiovascular disease (CVD), and subtype mortality using the National Integrated Project for Prospective Observation of Noncommunicable Disease and Its Trends in the Aged, 1980-2004 (NIPPON DATA80) database with a 24-year follow-up. At baseline in 1980, data were collected on study participants aged ≥30 years from randomly selected areas in Japan. We followed 9067 participants (44% men; mean age, 51 years). During the 24-year follow-up, mortality was as follows: 2581 total, 887 CVD, 179 coronary heart disease, 173 heart failure, and 411 stroke. The multivariate-adjusted hazard ratio (HR) with the use of the Cox model including biochemical and other ECG variables revealed that clockwise rotation was significantly positively associated with heart failure in men and women combined (HR=1.79; 95% confidence interval [CI], 1.13-2.83; P=0.013), CVD in men and in men and women combined (HR=1.49; 95% CI, 1.12-1.98; P=0.007 in men; HR=1.28; 95% CI, 1.02-1.59; P=0.030 in combined), and total mortality in men and in men and women combined (HR=1.19; 95% CI, 1.00-1.49; P=0.0496 in men; HR=1.15; 95% CI, 1.00-1.32; P=0.045 in combined). Counterclockwise rotation was significantly inversely associated stroke in men and women combined (HR=0.77; 95% CI, 0.62-0.96; P=0.017), CVD in men and in men and women combined (HR=0.74; 95% CI, 0.59-0.94; P=0.011 in men; HR=0.81; 95% CI, 0.70-0.94; P=0.006 in combined), and total mortality in women (HR=0.87; 95% CI, 0.77-0.98; P=0.023). CONCLUSIONS: We found a significant positive association of clockwise rotation and a significant inverse association of counterclockwise rotation with CVD mortality in men and in men and women combined, independent of confounding factors including other ECG changes.

Prognostic implications of progressive cardiac conduction disease.

BACKGROUND: Progressive cardiac conduction disease (PCCD), characterized by temporal increase in PR interval and QRS duration, may be attributed to diverse pathophysiological mechanisms. This study aimed to investigate whether PCCD is associated with increased risk of cardiovascular morbidity and mortality. METHODS AND RESULTS: Digital analysis of 12-lead ECG was performed to select patients with PCCD from among a database containing 359,737 ECGs. Long-term prognosis of PCCD was assessed in a large hospital-based population: 458 patients (341 males; mean age, 57.9 ± 14.7 years) with PCCD were enrolled. During a mean follow-up of 13.3 ± 6.4 years, 109 patients were hospitalized for heart failure (HF), and there were 16 and 59 deaths from cardiovascular diseases and all causes, respectively. Multivariate Cox proportional hazards analysis confirmed (1) a significant association of temporal incremental rate of PR interval (≥ 2 ms/year) and QRS duration (≥ 3 ms/year) with HF hospitalization (hazard ratio [HR], 2.34; 95% confidence interval [CI], 1.36-4.05; P=0.002 and HR, 2.08; 95% CI, 1.25-3.53; P=0.01, respectively) and (2) a significant association of temporal incremental rate of PR interval (≥ 4 ms/year) and QRS duration (≥ 5 ms/year) with cardiovascular mortality (HR, 6.9; 95% CI, 1.47-36.96; P=0.02 and HR, 4.31; 95% CI, 1.19-16.5; P=0.03, respectively). CONCLUSIONS: The severity of PCCD was independently and significantly associated with HF hospitalization and cardiovascular mortality.

The ventriculophasic response: relationship to sinus arrhythmia and the duration of interposed QRS complexes.

BACKGROUND: The ventriculophasic response (VR) refers to shortening of sinus cycle length during heart block when a QRS complex is interposed between 2 P waves. Our purpose was to analyze its relationship to respiratory sinus arrhythmia (SA) and to compare VR in relation to paced versus intrinsic QRS complexes. METHODS: Patients with advanced heart block had their pacer devices temporarily programmed to ventricular inhibited mode at 30 ppm. In 35 subjects, we analyzed VR and SA before, during and after 3 cycles of deep breathing. In 16 other patients we compared VR in the presence of paced versus narrower intrinsic QRS complexes. RESULTS: The magnitude of P-P interval shortening surrounding QRS complexes during inspiration correlated with SA (r = 0.36, P = 0.03). The prevalence of VR increased from 37% at baseline to 77% of subjects during deep breathing (P = 0.02). The mean P-P interval shortening was greater surrounding intrinsic QRS complexes than paced QRS complexes (3.6 ± 3.6% vs. 1.4 ± 1.1%, P = 0.02). The prevalence of VR increased from 25% during paced rhythm to 56% when intrinsic complexes were present. CONCLUSION: VR, like SA, increases with deep breathing and likely reflects intact parasympathetic nervous system function. Its increase in the presence of narrower beats suggests it may reflect ventricular synchrony.

Heart rate variability risk score for prediction of acute cardiac complications in ED patients with chest pain.

BACKGROUND: We aimed to develop a risk score incorporating heart rate variability (HRV) and traditional vital signs for the prediction of early mortality and complications in patients during the initial presentation to the emergency department (ED) with chest pain. METHODS: We conducted a prospective observational study of patients with a primary complaint of chest pain at the ED of a tertiary hospital. The primary outcome was a composite of mortality, cardiac arrest, ventricular tachycardia, hypotension requiring inotropes or intraaortic balloon pump insertion, intubation or mechanical ventilation, complete heart block, bradycardia requiring pacing, and recurrent ischemia requiring revascularization, all within 72 hours of arrival at ED. RESULTS: Three hundred nine patients were recruited, and 25 patients met the primary outcome. Backwards stepwise logistic regression was used to derive a scoring model that included heart rate, systolic blood pressure, respiratory rate, and low frequency to high frequency ratio. For predicting complications within 72 hours, the risk score performed with an area under the curve of 0.835 (95% confidence interval [CI], 0.749-0.920); and a cutoff of 4 and higher in the risk score gave a sensitivity of 0.880 (95% CI, 0.677-0.968), specificity of 0.680 (95% CI, 0.621-0.733), positive predictive value of 0.195, and negative predictive value of 0.985. The risk score performed better than ST elevation/depression and troponin T in predicting complications within 72 hours. CONCLUSION: A risk score incorporating heart rate variability and vital signs performed well in predicting mortality and other complications within 72 hours after arrival at ED in patients with chest pain.

Maternal and fetal factors associated with mortality and morbidity in a multi-racial/ethnic registry of anti-SSA/Ro-associated cardiac neonatal lupus.

BACKGROUND: Cardiac manifestations of neonatal lupus include conduction disease and, rarely, an isolated cardiomyopathy. This study was initiated to determine the mortality and morbidity of cardiac neonatal lupus and associated risk factors in a multi-racial/ethnic US-based registry to provide insights into the pathogenesis of antibody-mediated injury and data for counseling. METHODS AND RESULTS: Three hundred twenty-five offspring exposed to maternal anti-SSA/Ro antibodies with cardiac neonatal lupus met entry criteria. Maternal, fetal echocardiographic, and neonatal risk factors were assessed for association with mortality. Fifty-seven (17.5%) died, 30% in utero. The probability of in utero death was 6%. The cumulative probability of survival at 10 years for a child born alive was 86%. Fetal echocardiographic risk factors associated with increased mortality in a multivariable analysis of all cases included hydrops and endocardial fibroelastosis. Significant predictors of in utero death were hydrops and earlier diagnosis, and of postnatal death were hydrops, endocardial fibroelastosis, and lower ventricular rate. Isolated heart block was associated with a 7.8% case fatality rate, whereas the concomitant presence of dilated cardiomyopathy or endocardial fibroelastosis quadrupled the case fatality rate. There was a significantly higher case fatality rate in minorities compared with whites, who were at a lower risk of hydrops and endocardial fibroelastosis. Pacing was required in 70%; cardiac transplantation was required in 4 children. CONCLUSION: Nearly one fifth of fetuses who develop cardiac neonatal lupus die of complications predicted by echocardiographic abnormalities consistent with antibody-associated disease beyond the atrioventricular node. The disparity in outcomes observed between minorities and whites warrants further investigation.

Anatomical and pathological findings in hearts from fetuses and infants with cardiac manifestations of neonatal lupus.

OBJECTIVE: The autopsy and clinical information on children dying with anti-SSA/Ro-associated cardiac manifestations of neonatal lupus (cardiac NL) were examined to identify patterns of disease, gain insight into pathogenesis and enhance the search for biomarkers and preventive therapies. METHODS: A retrospective analysis evaluating reports from 18 autopsies of cardiac NL cases and clinical data from the Research Registry for Neonatal Lupus was performed. RESULTS: Of the 18 cases with autopsies, 15 had advanced heart block, including 3 who died in the second trimester, 9 in the third trimester and 3 post-natally. Three others died of cardiomyopathy without advanced block, including two dying pre-natally and one after birth. Pathological findings included fibrosis/calcification of the atrioventricular (AV) node, sinoatrial (SA) node and bundle of His, endocardial fibroelastosis (EFE), papillary muscle fibrosis, valvular disease, calcification of the atrial septum and mononuclear pancarditis. There was no association of pathology with the timing of death except that in the third-trimester deaths more valvular disease and/or extensive conduction system abnormalities were observed. Clinical rhythm did not always correlate with pathology of the conduction system, and the pre-mortem echocardiograms did not consistently detect the extent of pathology. CONCLUSION: Fibrosis of the AV node/distal conduction system is the most characteristic histopathological finding. Fibrosis of the SA node and bundle of His, EFE and valve damage are also part of the anti-Ro spectrum of injury. Discordance between echocardiograms and pathology findings should prompt the search for more sensitive methods to accurately study the phenotype of antibody damage.

Postoperative heart block in children with common forms of congenital heart disease: results from the KID Database.

INTRODUCTION: Cardiac conduction system injury is a cause of postoperative cardiac morbidity following repair of congenital heart disease (CHD). The national occurrence of postoperative complete heart block (CHB) following surgical repair of CHD is unknown. We sought to describe the occurrence of and costs related to postoperative CHB following surgical repair of common forms of CHD using a large national database. METHODS AND RESULTS: Retrospective, observational analysis performed over a 10-year period (2000-2009) using the Kids' Inpatient Database (KID). Visits for patients ≤24 months of age were identified who underwent surgical repair of ventricular septal defects (VSD), atrioventricular canal defects (AVC), and tetralogy of Fallot (TOF). Patients were identified who were diagnosed with postoperative CHB, further identifying those requiring a new pacemaker placement during the same hospitalization. Costs associated with visits were calculated. There were 16,105 surgical visits: 7,146 VSD, 3,480 AVC, and 5,480 TOF. There was a decrease in postoperative mortality (P = 0.0001) with no significant change in postoperative CHB. Hospital stay and cost were higher with CHB and placement of a permanent pacemaker. Repair of AVC (OR 1.77; [1.32-2.38]) was associated with a higher rate of postoperative CHB. Length of hospital stay and total cost were significantly increased with the development of postoperative CHB and increased further with placement of a permanent pacemaker. CONCLUSION: There has been little change over time in the frequency of postoperative CHB in patients undergoing repair of VSD, AVC, and TOF. Postoperative CHB results in major added cost to the healthcare system.

Extracorporeal membrane oxygenation support for intractable primary arrhythmias and complete congenital heart block in newborns and infants: short-term and medium-term outcomes.

OBJECTIVES: To describe the experience with extracorporeal membrane oxygenation support for intractable primary arrhythmias in newborns and infants. DESIGN: Retrospective study. SETTING: A tertiary care pediatric hospital. PATIENTS: Patients younger than 1 yr supported with extracorporeal membrane oxygenation for primary cardiac arrhythmias were identified from the institutional extracorporeal membrane oxygenation registry. INTERVENTIONS: Extracorporeal membrane oxygenation support. MEASUREMENTS AND MAIN RESULTS: Clinical characteristics and outcomes were investigated for patients with primary cardiac arrhythmia supported with extracorporeal membrane oxygenation. Outcomes investigated were time from initiation of extracorporeal membrane oxygenation support to arrhythmia control, duration of extracorporeal membrane oxygenation support, and results of interventions performed while supported with extracorporeal membrane oxygenation. We summarized the independent categorical and continuous variables using frequencies, percentages, and medians and ranges, respectively. Extracorporeal membrane oxygenation support was used in nine patients for rescue therapy for primary tachyarrhythmia and bradycardia. The primary arrhythmias were: focal atrial tachycardia (n = 2); reentrant supraventricular tachycardia (n = 3); junctional ectopic tachycardia (n = 2); and congenital complete atrioventricular block (n = 2) patients. Seven patients presented with severe hemodynamic compromise, with six patients requiring extracorporeal cardiopulmonary resuscitation. All patients required extracorporeal membrane oxygenation within 24 hrs of initial presentation. Balloon atrial septostomy was performed in three patients and ablation was performed in two patients. Sinus rhythm was achieved in all reentrant supraventricular tachycardia and rate control was established in both patients with focal atrial tachycardia and in one patient with junctional ectopic tachycardia while using extracorporeal membrane oxygenation support. All patients survived to hospital discharge, and median follow-up for the cohort was 5 yrs. There was one late death; all survivors had good overall and neurologic outcomes. CONCLUSIONS: The requirement of extracorporeal membrane oxygenation support in newborns and infants with intractable arrhythmia is rare. Extracorporeal membrane oxygenation support does potentially carry morbidity; however, to prevent arrhythmia-related mortality, extracorporeal membrane oxygenation support and/or extracorporeal cardiopulmonary resuscitation should be considered in the management of hemodynamically unstable primary arrhythmias as an emergent lifesaving procedure.

Congenital complete heart block: single tertiary centre experience.

BACKGROUND: Congenital complete heart block (CCHB) is an uncommon although important and potentially serious condition contributing to neonatal morbidity and mortality. AIMS: To study the characteristics and outcomes of infants born with CCHB at a single tertiary centre. METHODS: A retrospective review of all infants with CCHB over the last 20 years was carried out to determine the outcomes, and the indications and timing of pacemaker insertion. RESULTS: Fifteen live born infants (10 male, 5 female) with CCHB were identified. Their mean (and SD) gestation and birth weight were 37 (3.3) weeks and 3100 (448) grams respectively. Maternal systemic lupus erythematosus (SLE) antibodies were present in eight (53%) pregnancies and two infants had congenitally corrected transposition of the great arteries (cCTGA). The median heart rate/minute at birth was 60 (range 40-80). Thirteen (87%) patients to date required a pacemaker. The median age of insertion of a pacemaker device was six months (range 2 days-16 years). All patients were paced epicardially - six initially with a single chamber and five with a dual chamber pacemaker. At the time of generator change, dual chamber pacemakers were used. The median life of an implanted pacemaker was six years (3-10 years). Except for a patient with cCTGA who has undergone a double switch procedure, all the patients had good systemic ventricular function. There was one death in the group unrelated to CCHB. CONCLUSIONS: CCHB is a uncommon but potentially serious condition in infancy. While a significant number of infants need a pacemaker, the overall outcome of infants with CCHB in our experience is good.

Effect of long-term right ventricular pacing in young adults with structurally normal heart.

BACKGROUND: Right ventricular pacing increases the risk of heart failure in adults with structural heart disease. The impact of prolonged right ventricular pacing in adults without structural heart disease is not fully characterized and may depend on interactions of pacing with abnormal substrate predisposing to ventricular dysfunction. METHODS AND RESULTS: We assessed the effect of right ventricular pacing in patients who underwent pacemaker implantation for isolated congenital atrioventricular block between 1964 and 2005. To assess for immunologic contribution to cardiac dysfunction, outcomes were compared between patients with (Ab(+)) and without (Ab(-)) antinuclear antibody during adulthood and an age- and sex-matched Olmsted County, Minnesota, population. Of 103 patients (mean+/-SD age, 32+/-19 years), 18 were Ab(+). Long-term survival free of new heart failure after pacemaker implantation in isolated congenital atrioventricular block patients was worse than in the matched population (P<0.001). This difference was attributable to the development of heart failure in 12 Ab(+) patients (67%; P<0.001), without differences between Ab(-) patients (2%) and the matched population (2%; P=0.7). Compared with baseline, at last follow-up, left ventricular ejection fraction did not decline in Ab(-) (53+/-9% to 57+/-12%) but decreased in Ab(+)(52+/-10% to 38+/-12%; P=0.03) patients. Survival was similar in Ab(-) patients and the Minnesota population (98%; P=0.7) but worse in Ab(+) patients (79%; P<0.01). CONCLUSIONS: The natural history of patients with isolated congenital atrioventricular block who require pacing depends upon their antibody status. Antinuclear antibody status was a predictor for the development of heart failure and death. Long-term right ventricular pacing alone does not appear to be associated with development of heart failure, deterioration in ventricular function, or reduced survival in Ab(-) isolated congenital atrioventricular block patients.

Long-term follow-up after steroid-eluting epicardial pacemaker implantation in young children: a single centre experience.

AIMS: The purpose of this paper is the retrospective investigation of the clinical outcome and modes of failure leading to reoperation, as well as the report of the long-term results, in a group of young children who underwent epicardial pacemaker implantation. METHODS AND RESULTS: Between 2000 and 2008, 45 young children underwent epicardial pacemaker implantation at 3.2 +/- 2.5 years of age for congenital (n = 27) or post-operative (n = 18) atrioventricular block. The follow-up time was 5.7 years +/- 5 months (range: 6 months to 7.3 years). Five lead malfunction events (11%) were detected during the follow-up time, three of which were due to ventricular lead fracture. All revisions could be performed without complications, and all revised pacemakers showed stable pacing and sensing parameters during long-term follow-up. The actuarial freedom from reoperation at 6 years was 88.8 +/- 2%. Median epicardial ventricular and atrial pacing thresholds were stable and excellent at the latest follow-up, with means of 1.1 +/- 0.5 V and 0.7 +/- 0.8 V, respectively. CONCLUSION: In our patient cohort of 45 young children, epicardial pacing was associated with a satisfactory clinical outcome and acceptable long-term results. The major cause of reoperation in our series was lead fracture. Reoperations were performed at a low risk.