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PubMed1985

Prenatal diagnosis of complete heart block.

Two cases of complete heart block diagnosed prenatally are presented. Proper evaluation of such cases by fetal echocardiography is necessary. In the absence of obstetric complication, vaginal delivery of such cases can be expected with biophysical and biochemical monitoring of the fetus in a tertiary care center.

Echocardiography↗
PubMed1969

Conservative treatment of chronic heart block.

A study of 203 patients with chronic heart block treated with oral long-acting isoprenaline showed that 85 (42%) were maintained satisfactorily on the drug for a mean period of 18.2 months. The survival rates at one, two, and three years were 76%, 64%, and 57% respectively. In 115 patients treatment by pacing became necessary to control symptoms, and in these patients the survival rates at one, two and three years were 83%, 72%, and 60%.The two most valuable guides to patients' response to oral isoprenaline are the response to a trial dose of intravenous isoprenaline and the type of dysrhythmia associated with their Adams-Stokes attacks. Patients with heart failure with slow ventricular rates and those with angina of effort do not respond to treatment with sympathomimetic drugs.The majority of patients with chronic heart block are elderly, and in view of the complexity of pacing systems, and the need for skilled supervision of paced patients, oral long-acting isoprenaline remains of value in the longterm management of chronic heart block, provided patients are carefully selected for this form of therapy.

Adams-Stokes Syndrome↗
PubMed1994

Complete congenital heart block in autoimmune hepatitis (SLA-positive).

Complete congenital heart block is a serious complication of neonatal lupus erythematosus which most often occurs in children of mothers suffering from connective tissue disease. We report the occurrence of complete congenital heart block associated with autoimmune hepatitis (SLA-positive). A 32-year-old woman was treated for more than 10 years for autoimmune hepatitis (SLA-/ANA-positive) and remained in clinical remission under immunosuppressive therapy. She showed an MHC-haplotype typical for autoimmune hepatitis (A1, B8, DR3). After a normal first pregnancy, an emergency caesarean section was performed in the 32nd week of her second pregnancy because of fetal bradycardia. The child died a few hours after delivery of complete congenital AV-block. Retrospective analysis of the maternal serum showed the emergence of SS-A/Ro-antibodies prior to the second pregnancy. The maternal serum antibodies were reactive with the 52 kD SS-A/Ro-antigen, as demonstrated by immunoblot employing recombinant SSA/Ro-antigen. The occurrence of complete congenital heart block has been shown to be associated with the presence of SS-A/Ro antibodies as well as the MHC-haplotype DR3. With respect to this genetic linkage, pregnant patients with autoimmune hepatitis and the MHC-haplotype DR3 should be examined for the presence of SS-A/Ro-antibodies. They should be closely followed during pregnancy to enable early detection of the development of congenital heart block, as prevention by plasmapheresis plus dexamethasone may be possible at an early stage.

Adult↗
PubMed2001

Long-term outcome of mothers of children with isolated heart block in Finland.

OBJECTIVE: To study the long-term outcome of mothers of children with isolated heart block in a defined population. METHODS: We reviewed the Finnish hospital registries for patients born between 1950 and 1999 who had been diagnosed as having isolated heart block before the age of 15 years. We identified 101 children with isolated congenital heart block (CHB) and 55 with isolated heart block detected after the newborn period. Eighty-three (91%) of the 91 mothers of children with CHB and 48 (87%) of the 55 mothers of children with heart block detected after the newborn period were studied according to a protocol defining clinical characteristics (mean 9.9 years, range 0-49 years, and mean 22.9 years, range 4-47 years after the index delivery, respectively). Maternal survival was compared with survival in an age-matched population of normal Finnish women. RESULTS: Before the index delivery, 29 (37%) of the 78 surviving mothers of children with CHB had a self-reported clinical diagnosis of a chronic autoimmune disease, and 55 (71%) had had symptoms, signs, or abnormal laboratory findings suggesting an underlying subclinical disease. Of the 23 mothers who were completely asymptomatic before the index delivery, 10 (13% of the surviving mothers) remained so after a mean followup of 9.6 years (range 0-21 years). In mothers of children with CHB, clinical characteristics different from those of healthy mothers were photosensitivity, fatigue, dry eyes, and dry mouth. Forty-eight (58%) of these 83 mothers developed an autoimmune disease during followup. The most common diagnosis was primary Sjögren's syndrome (22 definite, 11 probable), followed by systemic lupus erythematosus (SLE). The standardized mortality ratio of mothers of children with CHB was 5.1, and 3 of the 5 deaths were associated with SLE. Mothers of children with heart block detected after the newborn period had similar symptoms and signs of autoimmune diseases as the healthy controls, and their standardized mortality ratio was 1.9. CONCLUSION: Primary Sjögren's syndrome, either definite or subclinical, is the predominant autoimmune disorder in mothers of children with CHB. Mothers of children with isolated heart block detected after the newborn period do not, as a group, have clinical features suggestive of autoimmune diseases.

Adult↗
PubMed1988

Fetal complete heart block.

In a series of over 6000 patients referred for fetal echocardiography during an eight year period, 37 fetuses were found to have complete heart block. There were 16 cases of isolated heart block and 21 cases associated with structural heart disease. All mothers of fetuses with isolated complete heart block had evidence of circulating syndrome Sjögren A antibody (Ro). Only one mother had clinical evidence of connective tissue disease. In the 21 cases associated with structural heart disease there were 17 cases of atrioventricular septal defect, one case of secundum atrial and perimembranous ventricular septal defects, two cases of tetralogy of Fallot, and one case of pulmonary stenosis. All fetuses with atrioventricular septal defects and complete heart block had left atrial isomerism. Additional abnormalities of the great arteries were often found in this group; these were double outlet right ventricle, transposition of the great arteries, pulmonary atresia, coarctation of aorta, and stenosis of the pulmonary or aortic valves. Intrauterine congestive heart failure was a feature of four cases in the group with isolated complete heart block and 11 cases of the group with associated structural heart disease. The outcome in the fetuses with isolated complete heart block was better than in those with heart disease: 12 of the 16 fetuses are alive, two of them have a pacemaker. But only three of the group of 21 fetuses with cardiac malformation are alive, and two of them have a pacemaker.

Echocardiography↗
PubMed1992

Prospective evaluation of heart block complicating early Lyme disease.

Lyme disease is a recognized cause of heart block/carditis. The incidence of heart block complicating early Lyme disease has not been prospectively evaluated. In this study, 61 patients with early Lyme disease documented by the rash of erythema migrans were prospectively evaluated for carditis. Fifty five of 61 patients had a repeat examination 3 to 4 weeks after initiation of antibiotic therapy. Only one of 61 patients (1.6%) presented with heart block, which resolved with antibiotics. None of the 54 patients without heart block on initial presentation had a change in any measured electrocardiographic parameter or progressed to heart block after antibiotics. Therefore, early Lyme disease appears to be infrequently complicated by heart block. Early administration of antibiotics may prevent the development of heart block/carditis.

Adult↗
PubMedDate not supplied

Adult heart block is associated with disease activity in primary Sjögren's syndrome.

OBJECTIVES: Congenital heart block occurring in the foetus and neonate may be associated with maternal anti-SS-A/anti-SS-B autoantibodies (anti-SSA/anti-SSB). The adult atrioventricular node is generally thought to be resistant to the damaging effects of anti-SSA/anti-SSB. However, case reports suggest that heart block developing in adult Sjögren's syndrome (SS) patients may be associated with these autoantibodies. Therefore, we investigated the relationship between serum antibodies and heart block in adult SS patients. METHODS: We abstracted data from clinic patient records. Diagnosis of primary SS was based on American-European classification criteria. Electrocardiograms (EKGs), laboratory immunology parameters, lipid profiles, and focus scores from labial salivary gland biopsies were available for 51 SS patients. Fifteen patients had follow-up EKGs. PR interval200 ms was considered to be first-degree heart block. RESULTS: Five patients showed prolonged PR intervals; the presence of heart block was not related to the presence of anti-SSA antibodies. However, significant differences between patients with prolonged and normal PR intervals were seen for mean focus scores (p<0.0001), anti-cardiolipin immunoglobulin IgG (p = 0.0009), age (p = 0.01), IgG (p = 0.02), anti-SSB antibodies (p = 0.02), and high density lipoprotein (HDL) cholesterol levels (p = 0.03). These parameters correlated with prolonged PR intervals. CONCLUSIONS: These results suggest an association between disease activity, the presence of anti-SSB antibodies, and the occurrence of first-degree heart block in adults with primary SS.

Aged↗
PubMed2004

Treatment of fetal heart block with maternal steroid therapy: case report and review of the literature.

The presence of maternal autoantibodies to SS-A/Ro and/or SS-B/La is associated with the development of fetal heart block. There are data suggesting that maternal treatment with steroids might reverse heart block. We report on a pregnancy in a mother with secondary Sjögren syndrome and systemic lupus erythematosus with presence of autoantibodies to SS-A/Ro and SS-B/La, which was complicated by the development of incomplete fetal heart block. Oral dexamethasone treatment could not prevent progression to complete heart block and was associated with a number of complications.A review of the literature revealed 19 studies (including ours) in which 93 cases of fetal heart block were treated with maternal steroid therapy. Complete heart block proved irreversible in all cases; and of 13 fetuses with incomplete heart block which received maternal steroid therapy, three had a reduction in their degree of block and one reverted to sinus rhythm. Maternal steroid therapy, initiated early in pregnancy and potentially preventing the onset of heart block, did not decrease the incidence of heart block in nine studies with 43 cases. Furthermore, the literature review revealed numerous serious side effects of maternal steroid administration during pregnancy. Data on these potential side effects are lacking in the 28 studies discussed in this review. Maternal dexamethasone therapy to prevent or treat fetal heart block remains, in our opinion, a questionable intervention and can as yet not be recommended in the clinical situation.

Adult↗
PubMedDate not supplied

Transient complete heart block complicating acute rheumatic fever.

First-degree heart block is a common electrocardiographic manifestation of acute rheumatic fever and is included in Jones' diagnostic criteria. Other electrocardiographic changes such as sinus tachycardia, bundle branch blocks. nonspecific ST-T wave changes, atrial and ventricular premature complexes have been reported with variable frequency. However, complete heart block is an exceptionally rare manifestation of acute rheumatic fever. We report the clinical course of a 16-year-old boy with acute rheumatic fever who had prolonged P-R interval in the electrocardiogram on admission which subsequently progressed to complete heart block. The patient regained normal sinus rhythm within a few minutes without any pharmacologic or electrical intervention.

Acute Disease↗
PubMed1998

Heart block in patients with obstructive sleep apnoea: pathogenetic factors and effects of treatment.

Heart block during sleep has been described in up to 10% of patients with obstructive sleep apnoea. The aim of this study was to determine the relationship between sleep stage, oxygen desaturation and apnoea-associated bradyarrhythmias as well as the effect of nasal continuous positive airway pressure (nCPAP)/nasal bi-level positive airway pressure (nBiPAP) therapy on these arrhythmias in patients without electrophysiological abnormalities. Sixteen patients (14 males and two females, mean age 49.6+/-10.4 yrs) with sleep apnoea and nocturnal heart block underwent polysomnography after exclusion of electrophysiological abnormalities of the sinus node function and atrioventricular (AV) conduction system by invasive electrophysiological evaluation. During sleep, 651 episodes of heart block were recorded, 572 (87.9%) occurred during rapid eye movement (REM) sleep and 79 (12.1%) during nonrapid eye movement (NREM) sleep stages 1 and 2. During REM sleep, the frequency of heart block was significantly higher than during NREM sleep: 0.69+/-0.99 versus 0.02+/-0.04 episodes of heart block x min(-1) of the respective sleep stage (p<0.001). During apnoeas or hypopnoeas, 609 bradyarrhythmias (93.5%) occurred with a desaturation of at least 4%. With nCPAP/ nBiPAP therapy, apnoea/hypopnoea index (AHI) decreased from 75.5+/-39.6 x h(-1) to 3.0+/-6.6 x h(-1) (p<0.01) and the number of arrhythmias from 651 to 72 (p<0.01). We conclude that: 1) 87.9% of apnoea-associated bradyarrhythmias occur during rapid eye movement sleep; 2) the vast majority of heart block episodes occur during a desaturation of at least 4% without a previously described threshold value of 72%; and 3) nasal continuous positive airway pressure or nasal bi-level positive airway pressure is the therapy of choice in patients with apnoea-associated bradyarrhythmias.

Adult↗
PubMed1987

Cardiac immunoglobulin deposition in congenital heart block associated with maternal anti-Ro autoantibodies.

Isolated congenital complete heart block is frequently found in offspring of mothers who have IgG anti-Ro/SSA autoantibodies. IgG deposition was reported in the heart of a baby who died from heart block and heart failure. The pattern of antibody deposition observed was the same as that reproduced in animal models for anti-Ro/SSA binding. The IgG deposition was present in, but not limited to, the conduction system. Scattered small infiltrates of mononuclear cells and a limited, patchy deposition of complement were also observed. These findings support the possibility that isolated congenital heart block may be causally related to autoantibodies.

Adult↗
PubMed1986

Complete heart block in Reiter's syndrome.

Complete heart block with Stokes-Adams attacks was diagnosed in a 30 year old male, who had suffered from a Reiter's syndrome for only 3 1/2 years. The heart block was unaffected by treatment with high dose corticosteroids, and a permanent pacemaker was implanted. There were no signs of concomitant aortic insufficiency or spondylitis/sacroiliitis.

Adams-Stokes Syndrome↗
PubMed2004

Octreotide-induced bradycardia and heart block during surgical resection of a carcinoid tumor.

UNLABELLED: Octreotide may be a life-saving treatment in the case of an acute carcinoid crisis, but when given as an i.v. bolus in larger doses, it may cause significant effects on the cardiac conduction system. We describe cardiac conduction impairment observed during octreotide administration in a patient undergoing carcinoid tumor surgery. In this patient, i.v. boluses of 100 microg of octreotide resulted in symptomatic bradycardia, Mobitz type II atrioventricular block, and complete heart block. Perioperative physicians especially need to be aware of these potential effects because they may be more likely to occur during surgery because of the larger doses and boluses that are used to treat acute symptoms secondary to tumor manipulation. IMPLICATIONS: In some susceptible patients, i.v. bolus administration of octreotide may cause significant bradycardia and cardiac conduction defects. Therefore, when octreotide is administered as a bolus, it may be advisable to give it slowly while monitoring the electrocardiogram.

Aged↗
PubMed2006

Is complete heart block after surgical closure of ventricular septum defects still an issue?

BACKGROUND: A serious complication after surgical closure of ventricular septal defect (VSD) is complete heart block. In this retrospective study, we reviewed the incidence of complete heart block after surgical closure of a VSD at Great Ormond Street Hospital from 1976 to 2001 to identify any particular anatomic features that still predisposed patients to surgically-induced complete heart block and to provide anatomic guidelines to avoid this in future. METHODS: Data were extracted from our local database for patients having (1) isolated VSD or VSD in the setting of (2) tetralogy of Fallot with pulmonary stenosis or (3) tetralogy of Fallot with pulmonary atresia; (4) absent pulmonary valve syndrome; (5 and 6) coarctation or interruption of the aortic arch; and (7) subaortic fibrous shelf. We carefully reviewed the operative notes from all patients with postoperative complete heart block to discover any predisposing anatomical reasons to explain the complication. RESULTS: Two thousand seventy-nine patients had a VSD closure. Permanent complete heart block developed in 7 of 996 patients (0.7%) with an isolated defect and in 1 of 847 patients (0.1%) with tetralogy of Fallot. Four more patients had postoperative complete heart block. CONCLUSIONS: Instances of iatrogenic complete heart block continue to occur after surgical VSD closure, either because of unexpected biological variations or because of unawareness of the disposition of the atrioventricular conduction axis in particular circumstances. This report emphasizes the latter aspect in details and suggests a risk of iatrogenic complete heart block of less than 1%.

Aortic Coarctation↗