Ventricular Functional Analysis in Congenital Complete Heart Block Using Speckle Tracking: Left Ventricular Epicardial Compared to Right Ventricular Septal Pacing.

BACKGROUND: Chronic right ventricular (RV) apical pacing in patients with congenital complete atrioventricular block (CCAVB) is associated with left ventricle (LV) dyssynchrony and dysfunction. Hence, alternative pacing sites are advocated. The aim of this study was to compare LV function using STE in selected patients with LV epicardial pacing (LVEp) vs. RV transvenous pacing (RVSp). METHODS: This was a single-center, retrospective study in patients with CCAVB who underwent permanent pacemaker implant at age ≤ 18 years. Age- and gender-matched patients with a normal heart anatomy and function served as the control group. LV function was comprehensively assessed by conventional 2D Echocardiography and speckle-tracking echocardiography (STE). RESULTS: We included 24 patients in the pacemaker group [27.6% male, mean age of 17.1 at last follow-up, follow-up duration of 8.7 years, RVSp (n = 9; 62.5%)] compared to 48 matched healthy controls. Shortening fraction (SF) and ejection fraction (EF) were normal and similar between cases and controls. However, STE detected abnormal LV function in the pacemaker group compared to controls. The former demonstrated lower/abnormal, Peak Longitudinal Strain myocardial (PLS Myo) [- 12.0 ± 3.3 vs. - 18.1 ± 1.9, p < 0.001] and Peak Longitudinal Strain endocardial (PLS endo) [- 16.1 ± 4.1 vs. 1.7 ± 1.7, p < 0.001]. STE parameters of LV function were significantly more abnormal in LVEp vs. RVSp subgroup as demonstrated by lower values for PLS Myo (- 10.1 ± 3.2 vs. - 13.1 ± 2.9, p = 0.03) and PLS Endo (- 13.8 ± 4.4 vs. - 17.5 ± 3.3, p = 0.03). CONCLUSION: STE was more sensitive in detecting subtle differences in LV function relative to standard conventional 2D echocardiography (SF and EF) in selected patients with CCAVB and a permanent pacemaker. Furthermore, STE demonstrated that transvenous RV septal pacing was associated with better LV systolic function preservation than LV epicardial pacing for comparable post-implant intervals.

Leadless pacemaker use in a patient with a durable left ventricular assist device.

There is limited known safety and efficacy of leadless pacemaker device use in patients with durable left ventricular assist devices (LVADs). We present a case of a pacemaker-dependent LVAD patient with infection of permanent transvenous pacemaker who underwent successful implantation of Micra transcatheter pacing system (Medtronic).

Occurrence of complete heart block associated with acute renal infarction.

Complete heart block (CHB) and acute renal infarction (ARI) are both uncommon diseases and seldom encountered in the clinical practice. We describe a rare case of pre-existing left bundle branch block, presenting simultaneously with CHB and ARI. The possible mechanism depends on prior presence of either CHB or ARI. If ARI occurs first, severe pain and embolism may enhance the vagal tone resulting in decrease in the heart rate and transient intraventricular conduction interruption, which subsequently causes CHB. The opposite scenario, CHB preceding ARI, is also possible. CHB can be physiologic and transient, with higher risk of development in the circumstance of pre-existing conduction system disturbances. Patients with CHB are predisposed to formation of thrombi and thromboemboli, giving rise to ARI. In conclusion, awareness and timely identification of the clinical manifestations of these two diseases may facilitate early diagnosis and prompt management.

The prevention, screening and treatment of congenital heart block from neonatal lupus: a survey of provider practices.

Objective: To survey an international sample of providers to determine their current practices for the prevention, screening, and treatment of congenital heart block (CHB) due to maternal Ro/SSA antibodies. Methods: A survey was designed by the organizing committee of the 9th International Conference of Reproduction, Pregnancy and Rheumatic Diseases. It was sent to attendants of the conference and authors of recent publications or abstracts at ACR 2012, 2013 or 2014 on rheumatic diseases and pregnancy. Results: In anti-Ro/SSA positive women, 80% of 49 respondents recommended screening by serial fetal echocardiogram (ECHO), with most starting at week 16 (59%) and stopping at week 28 (25%), although the time to stop varied widely. For women without a prior infant with neonatal lupus, respondents recommend every other week (44%) or weekly (28%) fetal ECHOs. For women with a prior infant with neonatal lupus, 80% recommend weekly fetal ECHOs. To prevent CHB, HCQ was recommended by 67% of respondents and most would start pre-pregnancy (62%). Respondents were asked about medications to treat varying degrees of CHB in a 20-week pregnant, anti-Ro and La positive SLE patient. For first degree, respondents recommended starting dexamethasone (53%) or HCQ (43%). For second degree, respondents recommended starting dexamethasone (88%). For third degree, respondents recommended starting dexamethasone (55%) or IVIg (33%), although 27% would not start treatment. Conclusion: Despite the absence of official guidelines, many physicians with a focus on pregnancy and rheumatic disease have developed similar patterns in the screening, prevention and treatment of CHB.

Measurement of fetal atrioventricular time intervals: A comparison of 3 spectral Doppler techniques.

OBJECTIVE: To establish nomograms for fetal atrioventricular (AV) time intervals assessed by 3 different pulsed-wave Doppler techniques: left ventricular inflow and outflow tracts (LV in/out), superior vena cava and ascending aorta (SVC/AA), and pulmonary artery and pulmonary vein (PA/PV). METHODS: A cross-sectional study was performed in 311 normal fetuses divided into 5 groups between 16 and 38 weeks. Pulsed-wave Doppler-derived AV intervals were measured by interrogation of flow in LV in/out, SVC/AA, and PA/PV. Linear regression analyses were performed to examine correlations with gestational age (GA) and fetal heart rate (FHR). Intraclass correlation coefficients for reproducibility of each method were compared. RESULTS: Pulmonary artery and pulmonary vein revealed the longest mean AV time intervals (P < .001). The AV intervals in all methods were positively correlated with GA (R2  = 0.20-0.36; P < .001) and negatively correlated with FHR (R2  = 0.09-0.19; P < .001). The SCV/AA time intervals demonstrated the weakest influence of FHR. For LV in/out, SVC/AA, and PA/PV, intraobserver and interobserver reliability coefficients showed excellent agreements (all intraclass correlation coefficients ≥ 0.80). CONCLUSION: All pulsed-wave Doppler-derived AV time intervals increased with advancing GA and decreased with increasing FHR. Fetal AV interval measurements can be obtained in a clinically viable fashion with excellent reproducibility.

An unusual course of anti-Ro antibody-mediated fetal complete heart block.

Fetal hydrops is a serious complication of immune-mediated congenital complete atrioventricular block. We present the case of a fetus with severe hydrops and profound bradycardia and an unusual favourable outcome. This case enhances the importance of considering the contribution of ventricular ectopic beats to the cardiac output when counselling and predicting outcome of complete heart block.

Prenatal sonographic monitoring of progressive cardiac damages caused by anti-Ro antibodies: A case report.

Maternal autoantibodies to Ro/SSA are often linked to congenital heart block and rarely associated with structural defects. We describe the case of a fetus with anti-Ro-mediated second-degree block at 19 weeks, which progressed to a complete block, fibroelastosis, atrioventricular valve insufficiency, and semilunar valve stenosis/insufficiency at 20, 22, 24, and 26 weeks, respectively, although the fetus received transplacental anti-arrhythmic drugs. The 2150-g fetus was vaginally delivered at 35 weeks. An external pacemaker was inserted immediately after birth and replaced with permanent pacemaker at the age of 3 months. The newborn has had a good outcome with well-controlled heart rate.

Complete heart block in young adult with acute rheumatic fever.

Acute Rheumatic fever (ARF) is commonly associated with ECG abnormalities particularly atrioventricular block. However, third degree atrioventricular block or complete heart block is a rare manifestation. Most cases occurred in children. We reported a 25 year old man who developed complete heart block during an acute episode of ARF. He presented to hospital with five days history of fever, malaise and migrating arthralgia, followed by pleuritic chest pain. One day after admission his electrocardiogram (ECG) revealed complete heart block. Transthoracic echocardiography showed good left ventricular function with thickened, mild mitral regurgitation with minimal pericardial effusion. ASOT titer was positive with elevated white blood count and acute phase reactant. A temporary pacemaker was inserted in view of symptomatic bradycardia. The complete heart block resolved after medical therapy. He was successfully treated with penicillin, steroid and aspirin. He was discharged well with oral penicillin. The rarity of this presentation is highlighted.

Factors influencing fetal cardiac conduction in anti-Ro/SSA-positive pregnancies.

Objectives: Congenital heart block (CHB) develops in 1-2% of anti-Ro/SSA-positive pregnancies and has a recurrence rate of 12-20%, which indicates that factors other than maternal autoantibodies are crucial for CHB to occur. Here, we aimed to evaluate the influence of factors previously associated with CHB on the occurrence of milder forms of fetal cardiac conduction disturbances, shown to occur in up to 30% of anti-Ro/SSA-positive pregnancies, and on neonatal outcome in a large cohort of prospectively followed pregnancies. Methods: The association of maternal age, season of the year and history of atrioventricular block (AVB) with the development of fetal Doppler and neonatal ECG conduction disturbances was evaluated in 212 anti-Ro52/SSA-positive singleton pregnancies. Results: Maternal age was significantly higher in AVB II-III pregnancies but was not correlated with fetal AV time intervals in fetuses without signs of AVB II-III. AV time intervals of fetuses surveilled during the winter were significantly longer than those of fetuses surveilled during the summer. Fetal AV time intervals in consecutive pregnancies from the same women were significantly correlated. A history of AVB II-III was associated with significantly longer AV time intervals, and AVB I-III was observed at birth in 38% of babies born after a sibling with abnormal fetal AV conduction. Conclusion: Our study shows that AV time intervals in anti-Ro/SSA antibody-exposed fetuses during the CHB risk period are influenced by the season of the year, and reveals that the recurrence of conduction disturbances in antibody-exposed fetuses is higher than previously reported when milder forms are taken into account.

Preliminary findings on the safety of 1.5 and 3 Tesla magnetic resonance imaging in cardiac pacemaker patients.

INTRODUCTION: Modern pacemakers are designed to allow patients to undergo magnetic resonance imaging (MRI) under a set of specific conditions. Aim of this study is to provide confirmatory evidence of safety and performance of a new pacing system (ImageReady™, Boston Scientific) in patients undergoing 1.5 and 3T MRI. METHODS AND RESULTS: Two prospective, nonrandomized, single-arm studies were designed to provide confirmatory data of no impact of MRI on device function, lead parameters, and patient conditions in subjects implanted with the system undergoing a clinically non-indicated 1.5T and 3T MRI, respectively. Device measurements were done within 1 hour before and after the scan and at 1 month follow-up. Thirty-two subjects underwent MRI visit (17 subjects with 1.5T MRI and 15 subjects with 3T MRI). There were no unanticipated adverse effects related to the pacemaker. Device measurements taken pre- and post-MRI scan did not show any clinical relevant change that could indicate an effect of the MRI on the device or at the lead-tissue interface (RV threshold change: 0.01 ± 0.13 V, P = 0.60; RA threshold change: 0.01 ± 0.11 V, P = 0.53; R wave change: -0.44 ± 1.73 mV, P = 0.36; R wave change: 0.12 ± 1.67 mV, P = 0.73), with data confirmed at 1-month follow-up visit. CONCLUSION: The study documented safety of the pacing system in the 1.5T and 3T MRI environment by showing no adverse events related to device or MRI scan. Additional data are required to cover the more complex scenarios involving different diagnostic needs, conditions of use, clinical conditions, and new emerging technologies.

Use of adenosine to shorten the post ablation waiting period for cavotricuspid isthmus-dependent atrial flutter.

BACKGROUND: Dormant conduction unmasked by adenosine predicts clinical recurrences of cavotricuspid isthmus (CTI) dependent atrial flutter following catheter ablation. Conventional practice involves a waiting period of 20 to 30 minutes after achievement of a bidirectional line of block (BDB) to monitor for recovery of conduction. OBJECTIVE: Assess whether abolition of dormant conduction with adenosine immediately after CTI ablation and BDB can predict the lack of CTI conduction recovery during the following 30 minutes. METHODS: Consecutive patients undergoing catheter ablation for CTI-dependent atrial flutter were studied. Following the completion of CTI ablation and documentation of BDB, adenosine (≥12 mg IV) was administered immediately. In cases of dormant conduction, the CTI was ablated again until its abolition. After the achievement of BDB without dormant conduction, spontaneous CTI reconnection during the following 30 minutes and dormant conduction with adenosine at 30 minutes were evaluated. RESULTS: A CTI block was achieved in 171 patients. Nine patients (5.3%) had dormant conduction across the CTI immediately after ablation and BDB, and required further ablation. Two patients (1.2%) had subsequent spontaneous time-dependent reconnection within 30 minutes. Two other patients (1.2%) developed late dormant conduction with adenosine at 30 minutes. All 4 patients underwent further ablation. CONCLUSION: A negative adenosine challenge immediately after CTI ablation with bidirectional block, or after abolition of dormant conduction with further ablation, strongly predicted the absence of subsequent spontaneous reconnection within 30 minutes. Based on these results, the conventional waiting period is unnecessary in 97.6% patients without dormant conduction after CTI-dependent flutter ablation.

Persistent atrial standstill following the Cox-maze III procedure: reversal with sustained atrial pacing.

Atrial standstill is a rare disorder of cardiac rhythm that is characterized by total absence of electrical activity in one or both atria. We report herein the case of a patient with atrial fibrillation and symptomatic 4.0 s pauses who received a ventricular demand pacemaker. The patient later underwent mitral valve replacement with a pericardial tissue valve and the Cox-maze III procedure for symptomatic mitral stenosis and atrial fibrillation. Following surgery, he developed atrial standstill and became pacemaker dependent. The pacemaker was later revised to an atrioventricular sequential pacemaker. Twelve hours after revision, atrioventricular sequential pacing was noted and mechanical function of the atria was confirmed by Doppler echocardiography.

Hypoplastic Left Heart Syndrome with Congenital Complete Heart Block.

Congenital complete atrioventricular block (CCAVB) is a rare condition with an incidence of 1 of 20,000 live births. Hypoplastic left heart syndrome (HLHS) occurs more frequently than CCAVB and occurs in 1 of 5000 live births. HLHS in association with CCAVB is exceedingly rare. In this report, we describe a rare case of HLHS and CCAVB diagnosed in utero. Postnatal diagnosis, management and outcome are presented as well as review of the medical literature.

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.

Anti-Ro/SSA autoantibody-positive women's experience of information given on the risk of congenital heart block.

OBJECTIVE: Congenital heart block (CHB) may develop in fetuses of women with anti-Ro/SSA autoantibodies, and carries substantial morbidity and mortality. The aim was to evaluate how information on CHB is imparted and identify areas of improvement. METHODS: A questionnaire was distributed to anti-Ro/SSA antibody-positive women who had either participated in a surveillance programme but whose expected child did not develop CHB (n = 100, denoted Doppler-Assessed Pregnancies (DAP) group) or given birth to a child with CHB (n = 88, denoted CHB-Affected Pregnancies (CAP) group). RESULTS: The response rate was 83% (157/188). Most women received the information on CHB when they were already pregnant (DAP group 60%, CAP group 83%). However, a majority of them would have wanted the information before pregnancy (DAP group 52%, CAP group 56%), and most stated that it would not have influenced their decision to have a child (DAP group 77%, CAP group 58%). The ability to both understand the information and to perceive the information as sufficient were significantly higher when someone trained in paediatric cardiology gave the information. CONCLUSIONS: Our findings indicate that information on CHB should be given to women before pregnancy. The data further highlight the importance of having specific knowledge for giving relevant and understandable, yet sufficient information.

Imaging of cardiac sarcoidosis.

Sarcoidosis is a multisystem inflammatory disease. Cardiac involvement is described in up to 50% of the cases. The disease spectrum is wide and cardiac manifestations ranges from being asymptomatic to heart failure, arrhythmias and sudden cardiac death. The diagnosis of cardiac sarcoidosis can be challenging due to its non-specific nature and the focal involvement of the heart. In this review, we discuss the utility of a stepwise approach with multimodality cardiac imaging in the diagnosis and management of CS.

Ro/SSA autoantibody-positive pregnancy: reactions to serial fetal Doppler echocardiographic surveillance.

OBJECTIVE: The risk for congenital heart block (CHB) associated with maternal Ro/SSA autoantibodies is low, but the possibility of treating early stages of disease has seen the introduction of Doppler echocardiographic surveillance programs with serial examinations during the CHB susceptibility weeks of pregnancy. The aim of the present study was to understand how Ro/SSA autoantibody-positive women having undergone Doppler echocardiographic surveillance programs and giving birth to children without CHB experienced their pregnancy and frequent ultrasound examinations. METHODS: A validated questionnaire based on data from an interview-study was distributed to Ro/SSA-positive women supervised with Doppler examinations during their pregnancy (n = 100). RESULTS: The response rate was 79%. The majority of the women (61%) reported that the increased number of ultrasound examinations influenced their pregnancy, but in a positive way, with qualified information and additional support from health care personnel in conjunction with the examinations. Further, the visits to the clinic provided opportunities to see the ultrasound picture of the expected infant. However, one-third of the women also reported stress in relation to the examinations. CONCLUSIONS: Fetal echocardiographic surveillance holds many and predominantly positive effects for Ro/SSA-positive women during pregnancy in addition to the medical advantages.

Prevalence of Left Ventricular Dyssynchrony in Patients with Congenital Atrioventricular Block and Long-Term Pacing: A Three-Dimensional Echocardiographic Study.

BACKGROUND: 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. OBJECTIVE: 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. METHOD: 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. RESULTS: LV systolic dysfunction was present in 16 patients (32%) by two-dimensional and in 20 patients (40%) by RT3DE. There was a good correlation between LV ejection fraction by two-dimensional and RT3DE (r = 0.75; P < 0.001). Fourteen (28%) patients had intraventricular dyssynchrony by TDI, while 12 (24%) had intraventricular dyssynchrony by RT3DE. There was a good agreement between LV dyssynchrony by TDI and RT3DE (Kappa = 0.735; P < 0.001). There was a negative correlation between LV ejection fraction and SDI obtained by RT3DE (r = -0.58; P < 0.001) CONCLUSIONS: In patients with CCAVB and long-term pacing, LV dyssynchrony occurred in one-third of patients and was related to LV dysfunction. There was a good correlation between dyssynchrony obtained by RT3DE and TDI.