Left ventricular (LV) pacing in newborns and infants: Echo assessment of LV systolic function and synchrony at 5-year follow-up.

BACKGROUND: Small retrospective studies reported that left ventricular (LV) pacing is likely to preserve LV function in children with isolated congenital complete atrioventricular block (CCAVB). The aim of this study was to prospectively evaluate LV contractility and synchrony in a cohort of neonates/infants at pacemaker implantation and follow-up. METHODS: Patients with CCAVB who underwent LV pacing were evaluated with electrocardiogram and echocardiogram in a single-center, prospective study. Data were collected at implantation, at 1-month and every year of follow-up, up to 5 years. LV ventricular dimensions (diameters and volumes), systolic function (ejection fraction [EF] and global longitudinal strain [GLS]), and synchrony were evaluated. Data are reported as median (25th-75th centiles). RESULTS: Twenty consecutive patients with CCAVB underwent pacemaker implantation (12 single-chamber pacemaker [VVIR] and eight dual-chamber pacemaker [DDD]) with epicardial leads: 17 on the LV apex and three on the free wall. Age at implantation was 0.3 months (1 day-4.5 months). Patients showed good clinical status, normal LV dimensions, preserved systolic function, and synchrony at 60 (30-60) months follow-up. EF increased to normal values in patients with preimplantation EF <50%. Presence of antibodies and pacing mode (DDD vs VVIR) had no impact on the outcome. CONCLUSIONS: LV pacing preserved LV systolic function and synchrony in neonates and infants with CCAVB at 5-year follow-up. LV EF improved in patients with low preimplantation EF. Pacing mode or the presence of autoantibodies did not demonstrated an impact on LV contractility and synchrony.

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.

Ro52 autoantibody-positive women's experience of being pregnant and giving birth to a child with congenital heart block.

OBJECTIVE: congenital heart block may develop in the fetus of women with Ro/SSA autoantibodies. The aim of this study was to investigate how women expecting a child with congenital heart block (CHB) experienced their pregnancy and post-partum period. DESIGN, SETTING AND PARTICIPANTS: women giving birth to a child with CHB in Sweden during 2000-2009 were identified in a population-based manner and individually interviewed post-pregnancy using a semi-structured interview guide. The interviews (n=21) were audiotaped, transcribed verbatim and analysed by qualitative content analysis. FINDINGS: three categories emerged from the responses: learning, suspense and facing. Learning contained both learning about the child's heart block, but frequently also about autoantibody-positivity and a potential rheumatic diagnosis in the mother (16/21). The medical procedures and information differed considerably depending on the area of residence and who was encountered in the health-care system. In many cases, ignorance about this rare condition caused a delay in treatment and surveillance. Suspense described the women's struggle to cope with the feeling of guilt and that the child had a serious heart condition and might not survive the pregnancy. Facing included the post-partum period, leaving the hospital and adjusting to everyday life. The women had tended to put their pregnancies 'on hold', and some described that they needed prolonged time to bond with their newborn child. CONCLUSION: increased awareness and knowledge of CHB are needed to provide adequate care. Offering psychological support by a health-care professional to facilitate early bonding with the child should be considered. IMPLICATIONS FOR PRACTICE: there is a need for structured programs for surveillance of the pregnancies. Such programme should implement guidelines for the involved personnel in the chain of care and make relevant information accessible for the women and families.

Fetal echocardiographic assessment of endocardial fibroelastosis in maternal anti-SSA antibody-associated complete heart block.

BACKGROUND: There are few reports describing the features of maternal anti-SSA antibody-associated congenital complete heart block (CCHB) patients developing endocardial fibroelastosis (EFE). The aim of this study was to describe the clinical features and the outcome of patients with CCHB, with or without EFE. METHODS AND RESULTS: Over a 20-year period, 12 consecutive patients diagnosed with maternal anti-SSA antibody-associated CCHB were identified. The maternal anti-SSA antibody levels were measured and fetal echocardiographic findings were reviewed. The ratios of the thickness of the endocardium to that of the whole wall of the left ventricle (LE/W) and right ventricle (RE/W) were measured to investigate the degree of endocardial thickening. A total of 7 patients survived (living group) and were not diagnosed as having EFE. The remaining 5 patients died and were diagnosed with EFE during autopsy (dead group). Fetal echocardiography of the patients showed differences in the thickening and hyperintensity of the endocardium. The RE/W value was significantly higher in the dead group than in the living group. The titers of both maternal anti-52-kDa and anti-60-kDa SSA antibodies were high, but showed no significant differences between the 2 patient groups. CONCLUSIONS: EFE was the major negative prognostic factor for CCHB. Myocardial damage, predominantly in the right ventricle, was related to the outcome of CCHB associated with EFE.

Bloqueio átrio-ventricular total congênito: avaliação experimental de novo eletrodo para tratamento intra-uterino / Congenital Complete Heart Block: Experimental Assessment of a New Electrode for Intra-Uterine Treatment

Desenvolvemos um novo eletrodo para implante de marcapasso fetal, com o objetivo de se evitar a cirurgia intra-uterina a céu aberto. O eletrodo foi implantado por punção miocárdica a céu aberto nos corações de cinco fetos caprinos, para avaliação eletrofisiológica. A resistência foi de 1050,4e a onda R foi de 8,64mV. Os limiares de estimulação foram baixos, permanecendo relativamente constantes (>0,70 V) para larguras de pulso acima de 0,50 mseg, o que representa ótimo desempenho. O sistema de implante percutâneo de marcapasso fetal proposto poderá representar alternativa interessante para tratar o bloqueio átrio-ventricular total congênito, sem necessidade de exposição fetal / We have developed a new electrode for fetal pacing that avoids the need for intrauterine open surgical procedures. The electrode was implanted by myocardium puncture in five fetal goats for electrophysiological assessment. The stimulation resistance was 1050,4 and sensed fetal R wave of 8.64 mV. The acute stimulation thresholds were consistently low, remaining relatively constant (>0.70 V) at pulse widths greater than 0.5 msec, with excellent performance. The proposed percutaneous fetal pacing system may represent an interesting alternative to treat congenital complete heart block, with no need for fetal exposure...

Assessment of fetal rhythm in complete congenital heart block by magnetocardiography.

We report high precision assessment of fetal rhythm in utero in a case of isolated congenital complete heart block using fetal magnetocardiography. The recordings reveal a remarkably strong tendency for the atria and ventricles to synchronize, which is manifested by the continual presence of ventriculophasic sinus arrhythmia and frequent episodes of accrochage and isorhythmic AV dissociation.

Fetal echocardiography in the assessment of lupus pregnancies.

Fetal echocardiography is the most sensitive tool in detecting the earliest possible changes of the cardiovascular system related to maternal lupus. The institution of aggressive therapy at that time may still be able to reverse the process before permanent fetal cardiac injury occurs. Experience with this technique is described.

[Assessment of third-degree congenital atrioventricular block by ergometric tests]. / Avaliação do bloqueio atrioventricular do 3 degrees. grau congênito pelo teste ergométrico.

PURPOSE: To analyse the use of the exercise testing as the method of initial evaluation, following a prognostic indicative of patients with congenital complete heart block. METHODS: Five patients were analysed (3 men and 2 woman) with ages between 7 and 34 years (mean = 22.8). The patients were submitted to a treadmill exercise testing using the Bruce protocol 1 and symptom limited. RESULTS: In all patients the atrial frequency increased from a median of 74.40 bpm in the basal to 155.20 bpm in the maximum effort; the atrial chronotropism was a little below that calculated based on the age of the patients. The median of the ventricular frequency in the maximum effort was 94.80 bpm, very different from that foreseen and showing a deficit of ventricular chronotropism. The median consumption of oxygen was 35.68ml0(2)/Kg/min. In one patients (20%) there was not any change in the ventricular frequency with the effort, in 3 (60%) complex ventricular arrhythmia arise during the effort and in one (20%) a definitive ventricular pacemaker was implanted. CONCLUSIONS: The exercise testing is a simple method of initial evaluation, providing information as chronotropism, functional capacity and the presence of arrhythmias, that can be very useful in the evaluation of prognostic. The presence of complex ventricular arrhythmias during the exercise is indicative of a more regular follow-up.

Combined echocardiographic and Doppler assessment of fetal congenital atrioventricular block.

Fetal heart rate monitoring was combined with fetal echocardiography for examination of atrial reactivity during labour in five fetuses with second or third degree heart block. Alterations in vagal tone accompanying uterine contraction influence atrial rate, even when the ventricle is not under atrial 'control'. Fetal echocardiography enabled diagnosis of the underlying basis of the arrhythmia and located the optimal position for recording atrial activity with an external heart rate monitor. External and internal monitoring of atrial activity demonstrated reactivity during labour. Two patients were delivered vaginally after monitoring throughout labour. One mother preferred elective caesarean delivery. Caesarean section was required in another for cephalopelvic disproportion and in the remaining woman for late decelerations. These monitoring techniques provide an assessment of fetal well-being in the presence of fetal bradycardia due to variable degrees of heart block.