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Cardiac complications are common in tricyclic antidepressant poisoning, but are rare with overdose of mianserin, which is a tetracyclic antidepressant. We report a case in which repeated episodes of complete heart block occurred following overdose with mianserin.
A young man with IHSS who developed complete heart block was successfully treated with a permanent pacemaker. Echocardiography and other noninvasive techniques showed marked cycle-to-cycle variation in the evidence of subvalvular obstruction which decreased markedly when atrial systole preceded the ensuing paced complex by an appropriate interval. Because cycle length and therefore afterload were constant, it is concluded that diminished obstruction resulted from augmented ventricular end-diastolic volume produced by atrial contraction. The mitral valve echocardiogram showed unusual movements in diastole dependent upon the timing of atrial systole. Early reopening of the leaflets was a direct result of atrial contraction when the P waves were appropriately timed in presystole, whereas late reopening was passive and a result of ventricular filling in mid-diastole. Variations in intensity of the first heart sound correlated with the position of the mitral valve leaflets at the onset of ventricular systole.
OBJECTIVE: Since there is suggestive but, to date, indirect evidence that maternal anti-SSA(Ro) and SSB(La) antibodies are pathogenic in congenital heart block (CHB), we explored the hypothesis that binding to fetal tissues would result in selective depletion of autoantibodies from the neonatal circulation. METHODS: Maternal and umbilical cord levels of anti-48 kDa SSB(La), anti-52 kDa SSA(Ro) and anti-60 kDa SSA(Ro) antibodies were measured by ELISA and immunoprecipitation at parturition in 15 pregnancies complicated by CHB. A control group consisted of 13 pregnancies in which the mother was known to have antibodies to either SSA(Ro) and/or SSB(La) and the children did not have CHB. RESULTS: The ratios of maternal to cord serum levels of anti-48 SSB(La), anti-52 SSA(Ro) and anti-60 kDa SSA(Ro) antibodies ranged from 0.71 to 2.38 in both affected and unaffected pregnancies. The mean ratio obtained for each of the 3 autoantibodies was not significantly different between the 2 groups. Moreover these ratios did not significantly differ from the mean ratios obtained for total IgG levels in either group. CONCLUSION: These data demonstrate that maternal antibodies to all components of the SSA(Ro) SSB(La) system are efficiently transported across the placenta and are not selectively depleted in the circulation of neonates with CHB.
A 65-year-old female was hospitalized for syncope due to new onset complete heart block. An apical holosystolic murmur was detected on physical examination. Echocardiography revealed corrected transposition of great arteries, and Ebstein-like anomaly with regurgitation at the left-sided tricuspid valve. A single chamber (VVI) pacemaker was implanted and patient has been asymptomatic thereafter.
OBJECTIVE: To assess the longterm outcome of mothers of children with isolated congenital complete heart block (CCHB), and the maternal specific immunoblot pattern and HLA antigens. METHODS: Fifteen mothers of 16 children with isolated CCHB were investigated; their followup extended up to 15.8 years on average after the index delivery. Anti-Ro and La antibodies were detected by counterimmunoelectrophoresis and ELISA; anti-Ro antibodies were studied by immunoblot. HLA typing was done using a microcytotoxicity test. RESULTS: One mother has systemic lupus erythematosus (SLE) before the index delivery. The other mothers developed only minor symptoms (arthralgia, dry eyes and photosensitivity) resembling primary Sjögren's syndrome more than classic lupus. All 15 mothers were anti-Ro and 9 were also anti-La positive, a mean of 12.5 years after the index delivery. Eight mothers reacted with the 52 kDa SSA(Ro) component, and 2 also with the 60 kDa SSA(Ro) component. The prevalence of the DR3 antigen and of the B44/DR5, DR3/DQ2 and A1/Cw7/B8/DR3/DQ2 haplotypes was significantly increased. CONCLUSION: The longterm outcome for the mothers of children with CCHB is more reassuring than generally assumed. All the mothers were anti-Ro positive by sensitive ELISA: Reactivity to the denaturated 52 kDa SSA(Ro) component seems characteristic of these mothers, who presented a particular immunogenetic background.
An 18-year-old girl with systemic lupus erythematosus developed progressive electrocardiographic abnormalities over a period of 16 years, culminating in complete heart block with Adams-Stokes attacks. A permanent ventricular pacing system was implanted successfully.
A case of complete fetal heart block associated with intra-uterine growth retardation, SSA- and SSB-antibodies is presented. The fetus died in utero. Autopsy revealed damage to the fetal cardiac conducting system together with severe placental fibrosis and recent infarctions. These findings and a slightly prolonged activated partial thromboplastin time indicated also the presence of the lupus anticoagulant.
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The aims of this study were to assess morbidity, mortality, and long-term cardiac outcome of children with congenital complete heart block (CHB) diagnosed between the ages of 3 months and 15 years. The study population consists of 61 children with CHB diagnosed in five tertiary referral centers in Finland. There were two study groups: CHB diagnosed 3 months to 2 years of age (group 1; n = 29) and CHB diagnosed between the age of 2 and 15 years (group 2; n = 32). Neonatal morbidity was significantly higher in group 1 than in group 2 (p = 0.047). In the whole study population, permanent pacemaker implantation with the main indications of low ventricular rate and syncope (52%) was performed in 80% of cases, with a median age of 11.2 years. Structural heart defect not causally associated with CHB was evident in 9 of 61 patients (15%). Of 8 patients with atrial septal defect (ASD) secundum, 6 (75%) were operated on and 7 (88%) were paced. One patient had small ventricular septal defect. The incidence of dilated cardiomyopathy was 7%. The mortality with cardiomyopathy was very high (75%). Of the 61 children, 3 (5%) died due to cardiomyopathy at the ages of 2, 26, and 31 years. In the long-term follow-up of 17 years (median, range 2.9-46 years) 17% of patients who survived had cardiac problems: arrhythmias in 5, mitral valve insufficiency in 3, ASD secundum in 1, and cardiomyopathy in 1. The long-term outcome did not depend on the age at which CHB was diagnosed. In conclusion, CHB diagnosed after the newborn period carries relatively low mortality and morbidity. Pacemaker application was indicated in most children. The possibility of an associated heart defect or dilated cardiomyopathy indicates regular echocardiographic monitoring in all children and young adults with CHB.
Two different effects of maternal autoantibodies presented in a third-trimester pregnancy. The first was complete fetal heart block, demonstrated ultrasonographically, which correlated with the presence of anti-Ro and anti-La antibodies in the maternal serum. The second effect was decidual vasculopathy and thrombosis, a morphologic finding in the placenta that caused massive placental infarction and intrauterine death. The placental pathology correlated with the presence of anticardiolipin antibodies in the maternal serum at the time of stillbirth.
A 70-year-old man who had been drinking a bottle of whisky each day was scheduled for laser resection of a tongue tumor. His electrocardiogram showed sinus bradycardia (heart rate was 35-40 bpm), and transient complete heart block was observed. Echocardiography showed hypertrophic cardiomyopathy. After a temporary transvenous pacemaker had been inserted, anesthesia was induced with thiopental and vecuronium bromide, and maintained with sevoflurane and fentanyl. Heart rate was 45.min-1 before the induction of anesthesia, and after the induction increased to 70-80.min-1. Analysis of heart rate variability suggested that the increase in heart rate was due to augmentation of sympathetic nervous activity after intubation and operation stress. After the operation his bradycardia improved gradually, and after 3 months heart rate settled at about 55.min-1. Cadiomyopathy is known to be one of the complications of alcoholism. It was reported that alcoholic heart disease was improved promptly by abstinence from alcohol. During his long hospitalization, abstinence might have improved his severe bradycardia. Thiopental is useful for induction of anesthesia in a patient with severe bradycardia from alcoholic cadiomyopathy.
Data were analyzed from 698 patients with proved acute myocardial infarction (AMI) to develop a method to predict the occurrence of complete heart block (CHB). The presence of electrocardiographic abnormalities of atrioventricular or intraventricular conduction during hospitalization was determined for each patient. The electrocardiographic risk factors considered were: first-degree atrioventricular block, Mobitz type I atrioventricular block, Mobitz type II atrioventricular block, left anterior hemiblock, left posterior hemiblock, right bundle branch block and left bundle branch block. A CHB risk score was developed that consisted of the sum of each patient's individual risk factors. CHB risk scores of 0, 1, 2 or 3 or more were associated with incidences of CHB of 1.2, 7.8, 25.0 and 36.4%, respectively. When applied to an independent AMI data base, as well as to the summed results of 6 previously reported series that identified predictors of CHB during AMI, a similar incremental risk of CHB as predicted by the risk score method was demonstrated.
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Six hermetically sealed single cell rechargeable mercury-zinc pacemakers (B.T.) that will run continuously for over 4 years between rechargings have paced dogs with complete heart block for from 2 to 3 years. To maintain full cell capacity (over 1,000 mA hours) requires recharging for from 2-3 min/day to 60 to 80 hr once every 4 years, with any variation between these extremes being acceptable. Six realtime bench tests continue after over 7 years and accelerated tests have simulated a minimum of 50 years continuous pacing. Battery voltage is assessed by direct telemetry, eliminating the risk of patient intrinsic rhythm-pacemaker competition which is present with all current indirect (stimulation rate change) battery assessment techniques. The B.T. is an excellent 4-5 year primary pacemaker, fully rechargeable after several 4 year periods of battery rundown. A clinical test series has been initiated.
OBJECTIVE: To investigate which maternal HLA allele or haplotype is primarily associated with isolated congenital complete heart block (CCHB) in offspring. METHODS: HLA class II typings were assessed by line probe assay and polymerase chain reaction-sequence-specific oligonucleotide probe methods, and HLA class I by the microlymphocytotoxicity test, in 13 Italian anti-Ro-positive mothers of children with CCHB and 41 anti-Ro-positive mothers with healthy children (20 mothers with systemic lupus erythematosus [SLE] and 21 with Sjögren's syndrome [SS]). Anti-Ro antibodies were studied by immunoblot. RESULTS: HLA-DRB1*03011 and DRB1*03011; DQA1*0501;DQB1*0201 were more frequent in mothers of infants with CCHB than in mothers who had SLE, but not in mothers who had SS and whose children were healthy. Mothers of infants with CCHB were either HLA-B5/35, B17, or B44 positive and had a higher prevalence of B44;DRB11;DQA1*0501;DQB1*0301 and isolated anti-52-kd antibodies, which were absent in SS and SLE controls. CONCLUSION: Mothers of infants with CCHB presented a strong genetic similarity to mothers who had SS, except for HLA class I phenotype. HLA-DRB1*03011;DQA1*0501;DQB1*0201 seemed not to be primary CCHB-associated genes, but were involved in an SS-like anti-Ro/La response. The combined presence of HLA-DRB1*03011 and anti-52-kd SSA/Ro antibodies conveyed the highest risk of giving birth to an affected child.
High-grade atrioventricular heart block (HGAVB) occurring as a manifestation of systemic lupus erythematosus is an exceedingly rare event. We describe a patient with HGAVB who had myositis and antibodies to nuclear RNP (previously associated with myocarditis). A review of the literature on HGAVB associated with systemic lupus erythematosus is also presented.
A case of varicella myocarditis in a previously healthy 6-year-old child was reviewed. The patient presented with third-degree heart block and shock as the sole manifestation of her cardiac involvement. Bradyarrhythmias required temporary transvenous pacing. Intravenous acyclovir was used. The patient recovered without permanent sequelae. The natural history, clinical presentation, and treatment of varicella myocarditis are reviewed.
OBJECTIVE: To analyze the humoral immune response to Ro/SSA and La/SSB antigens in detail, in order to identify markers in mothers at high risk of having children with congenital heart block (CHB). METHODS: Serum samples were obtained from 9 Ro/La-positive mothers who gave birth to affected children, from their 8 newborns with CHB, and from 26 Ro/La-positive mothers whose children were healthy. Antibodies against Ro 52-kd, Ro 60-kd, and La were analyzed by enzyme-linked immunosorbent assay and immunoblotting, using recombinant proteins and synthetic peptides. RESULTS: IgG anti-Ro 52-kd antibodies were detected in all mothers who gave birth to children with CHB, as well as in their affected children, but were less frequent and at lower levels in control mothers. Fine mapping revealed a striking difference in which the response in mothers with affected children was dominated by antibodies to amino acids 200-239 of the Ro 52-kd protein (P = 0.0002), whereas the primary activity in control mothers was against amino acids 176-196 (P = 0.001). Furthermore, 8 of 9 mothers of children with CHB had antibody reactivity against amino acids 1-135 of the Ro 52-kd protein, containing 2 putative zinc fingers reconstituted under reducing conditions. CONCLUSION: The results suggest that development of CHB is strongly dependent on a specific antibody profile to Ro 52-kd, which may be a useful tool to identify pregnant Ro/La-positive women at risk of delivering a baby with CHB. Close monitoring of mothers at high risk would enable early detection of a block that is still developing and allow early treatment to combat more serious complications.