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At least 217 records · Page 12Linked to original sources
PubMed1996

Outcome of isolated congenital complete heart block diagnosed in utero.

OBJECTIVE: To establish identifiable prenatal factors in fetal heart block which might predict death in utero, the need for intervention, or the probability of pacemaker requirement. SETTING: Tertiary referral unit for fetal echocardiography. SUBJECTS: 36 fetuses with congenital complete heart block and structurally normal hearts identified between 1980 and 1993. METHODS: Maternal anti-Ro antibody status was documented. Prenatal variables examined included absolute heart (ventricular) rate, change in rate, and development of hydrops fetalis. Postnatally, heart rate, need for pacing, and the indications for pacing were detailed. RESULTS: Of the total of 36 patients, there are 24 survivors; 11 are paced. Of those fetuses which died, two were electively aborted for severe hydrops, seven died in utero, two were immediate postnatal deaths, and one was an unrelated infant death. The trend was for the heart rate to decrease during fetal life and postnatally. Fetuses with deteriorating cardiac function did not always show the lowest heart rates. Bradycardia of less than 55 beats/min in early pregnancy or rapid decrease in heart rate prenatally were poor prognostic signs. Hydrops was also associated with bad outcome, 10 out of the 12 hydropic fetuses dying (83%). Of 10 fetuses presenting with a heart rate above 60/min, nine survived of whom three required pacing. Of seven presenting with heart rates of 50/min or less, only three survived and two of these required pacing. Of the two fetuses with negative maternal anti-Ro antibody status one died in utero and one required heart transplantation after pacemaker insertion. CONCLUSIONS: Isolated complete heart block identified in fetal life does not always have a good prognosis. An individual heart rate does not accurately predict the outcome in utero or the need for postnatal pacing. Regular, careful monitoring during pregnancy is required in order to optimise care and timing of any interventions.

Cardiac Pacing, Artificial↗
PubMed2003

Autoantibody-associated congenital heart block: the clinical perspective.

Congenital heart block (CHB) can occur in association with structural heart disease, such as atrioventricular septal defects, left atrial isomerism, and abnormalities of the great arteries, with tumors, such as mesotheliomas, or as an isolated defect. In 1928, Aylward reported the occurrence of CHB in two children whose mother "suffered from Mikulicz's disease." This curious clinical observation was further solidified by the 1970s, with reports of CHB in children whose mothers had autoimmune diseases and that the maternal sera contained antibodies to Ro ribonucleoproteins. It was subsequently reported that many mothers also had antibodies to La. Other abnormalities affecting the skin, liver, and blood elements were associated with anti-Ro/La antibodies in the maternal and fetal circulation, and are now grouped under the heading of neonatal lupus syndromes. Neonatal lupus was termed because the cutaneous lesions of the neonate resembled those seen in systemic lupus erythematosus.

Autoantibodies↗
PubMed1977

Familial congenital complete heart block and maternal systemic lupus erythematosis.

A family is reported in which two siblings had congenital complete heart block with resultant congestive heart failure, the father and paternal grandfather show adult-onset conduction defects, and the mother has systemic lupus erythematosis. The interaction of heredity and environment is discussed in this context. A review of the literature on familial complete heart block suggests that so-called pure congenital-onset familial heart block, originally felt to be genetic, may in fact have an important enivronmental component, specifically related to ongoing maternal factors such as systemic lupus erythematosis.

Adult↗
PubMedDate not supplied

Is there an increased risk of local recurrence under the heart block in patients with left-sided breast cancer?

UNLABELLED: Tangential radiotherapy for left-sided breast cancer may be cardiotoxic. Shaping the field with a heart block reduces cardiac exposure but may under-dose the breast and/or chest wall. We compared the incidence and location of local recurrences in patients irradiated with and without a heart block. METHODS AND MATERIALS: Between 1994 and 1998, 180 patients irradiated to the left breast and/or chest wall were retrospectively reviewed. The local recurrence rates in patients treated with and without a heart block were compared using a 2-tailed Fisher exact test. An in-depth dosimetric analysis was performed in 23 patients to assess the percentage of breast tissue under-dosed by inclusion of the heart block. RESULTS: Overall, the local recurrence rates in patients with or without a heart block were similar. In postlumpectomy patients with inferiorly located tumors, the rates of local recurrence with and without a heart block were 2 of 6 patients versus 0 of 19 patients, respectively. In the dosimetric analysis, the average percentage of breast tissue under-dosed by the inclusion of a heart block was 2.8% (range, 0%-11%). DISCUSSION: A heart block is a reasonable method to limit cardiac dose but should be used cautiously following a lumpectomy in patients with inferiorly located tumors. Additional study with larger numbers of patients is warranted.

Breast Neoplasms↗
PubMed2002

Favorable outcome in a pregnancy with complete fetal heart block and severe bradycardia.

BACKGROUND: Pregnancies complicated by congenital heart block usually have a poor prognosis when there is severe fetal bradycardia. CASE: We present a pregnancy with fetal heart rate of 40 beats per minute. She previously delivered a child with third-degree heart block by cesarean at 28 weeks. This pregnancy was complicated by a high ribonucleoprotein antibody anti-Ro/SSA titer and fetal bradycardia. The patient was treated with steroids and beta-mimetics. The fetus continued to grow normally with reassuring biophysical profiles. After fetal lung maturity documentation at 34 weeks, she delivered by repeat cesarean a healthy 2349-g infant who required a permanent pacemaker. CONCLUSION: Reassuring antepartum testing and normal growth in pregnant women with anti-Ro/SSA antibodies and congenital heart block may allow expectant management until fetal maturity.

Adult↗
PubMed1995

Production of complete heart block and utero cardiac pacing in fetal lambs.

We produced a complete heart block in 5 fetal lambs at 120 to 135 days of gestation by injecting 10% formaldehyde into the His bundle. We obtained acute experimental data in 5 fetuses and chronic experimental data in 2 fetuses. Immediately after production of the complete heart block, cardiac output was acutely reduced to 43 +/- 9% when compared with the cardiac output at sinus rhythm. It was possible to restore the cardiac output to 80 +/- 7% of the preoperative levels by atrioventricular pacing at the same rate as had been present before the heart block was produced. When the heart rate decreased below 100 beats per minute, further increase of stroke volume did not occur, and output was markedly diminished. This model allows us to study the pathophysiological response of a complete heart block, and offers basic information useful for understanding fetal cardiac functioning.

Animals↗
PubMed2004

First-degree heart block in the fetus of an anti-SSA/Ro-positive mother: reversal after a short course of dexamethasone treatment.

Isolated congenital heart block is almost invariably associated with the presence of antibodies to SSA/Ro and SSB/La antigens in the maternal circulation. Once established, third-degree congenital heart block is permanent. However, a lesser degree of autoantibody-associated heart block in a fetus can be reversed if it is recognized and treated early enough with fluorinated glucocorticosteroids. The only method available clinically for the recognition of first-degree heart block in a fetus is measurement of the mechanical PR interval by pulsed Doppler echocardiography. This is the first report of a fetus in whom a diagnosis of first-degree heart block and the consequent decision to intervene were based solely on this technique. In addition, the first-degree heart block resolved completely after only 2 weeks of dexamethasone treatment, and the heart rhythm remained stable throughout the remainder of the pregnancy despite early discontinuation of therapy due to oligohydramnios.

Adult↗
PubMed1977

Postoperative complete heart block in 64 children treated with and without cardiac pacing.

Between 1957 and 1973, a total of 64 children had complete heart block during intracardiac surgery were treated by one of three methods: (1) No pacemaker: Six of the 13 patients in this group had reversion to sinus rhythm. Seven patients continued to have complete heart block, and five of these died of Stokes-Adams episodes. Two patients continue to have complete heart block without pacemaker insertion. (2) Temporary pacemaker: Seventeen of 25 patients had reversion to sinus rhythm and the temporary pacemaker was removed; the other 8 continued to have complete heart block. Five of the eight died--three of Stokes-Adams episodes and two at the time of cardiac reoperation. Three remain asymptomatic without placement of a permanent pacemaker. Six died of postoperative complications unrelated to heart block or pacemaker implantation. None of the other 20 experienced Stokes-Adams attacks. There was one late death in this group. Although there are problems in using permanent pacemakers and electrode systems, in these patients with operatively induced complete heart block their use was associated with a low mortality rate.

Adolescent↗
PubMed1984

Congenital complete heart block.

Isolated reports of patients, including aviators, with congenital complete heart block suggest a benign prognosis. However, prospective studies of adolescents and adults with congenital complete heart block reveal an unpredictable incidence of impairment of consciousness and sudden death. Congenital complete heart block is, therefore, a contraindication to professional pilot status.

Adolescent↗
PubMed1990

Ascites not due to congestive heart failure in a fetus with lupus-induced heart block.

Hydrops developing in fetuses with lupus-associated heart block has usually been assumed to result from congestive heart failure. We present a case in which fetal ascites associated with complete heart block resolved promptly after administration of betamethasone and prednisone to the mother, who had anti-SSA and who developed clinical lupus erythematosus. This resolution occurred with no demonstrable change in fetal cardiac function. We conclude that an immune mechanism, rather than congestive heart failure, was responsible for the fetal ascites.

Adult↗
PubMed1976

Ankylosing spondylitis with complete heart block.

A patient with complete heart block due to ankylosing spondylitis exhibited an unusually brisk increase in ventricular rate with exercise. Despite broad ventricular (QRS) complexes, His bundle electrograms demonstrated a His deflection before each QRS complex and thus established the level of atrioventricular (A-V) block to be proximal to or localized to the His bundle. A junctional pacemaker accounted for the brisk response to exercise, atropine, or isoproteronol. Left bundle branch block pattern and prolongation of infranodal conduction time (H-Q) suggested additional disease involving the His bundle and/or right fascicle. Wide-spread disease affecting A-V nodal and infranodal conduction was compatible with pathologic changes seen in this disease. Because the pacemaker was able to respond to stress promptly and with appropriate increase in heart rate, the patient appeared not to warrant pacemaker implantation in his present state.

Atropine↗
PubMed1990

Management of a hydropic infant with congenital heart block.

A baby with congenital heart block and hydrops fetalis diagnosed prenatally was delivered at 36 weeks of gestation. Heart failure was controlled with temporary cardiac pacing which was complicated by perforation of the myocardium. She made an uneventful recovery after implantation of a permanent pacemaker. Sjögren's syndrome A antibodies were detected in her serum. Coincidentally, she also had pulmonary valvular stenosis as a structural abnormality.

Female↗
PubMed1996

Successful surgical repair of aortic dissection presenting with complete heart block.

Acute dissection of the ascending aorta can present with complete heart block if the dissecting hematoma involves the interatrial septum near the atrioventricular node. We report a case of acute type A dissection presenting with complete heart block treated with emergency grafting of the ascending aorta, aortic valve replacement, and coronary artery bypass grafting. The patient survived, although complete heart block persisted requiring permanent pacemaker implantation.

Aortic Dissection↗
PubMed1992

Transient high grade heart block following autologous bone marrow infusion.

We performed a retrospective analysis of 42 consecutive patients undergoing autologous BMT to determine the incidence of second and third degree heart block following the infusion of cryopreserved autologous bone marrow and to identify any predisposing characteristics. A decrease in heart rate > or = 10 beats/min was observed in 80.5% of patients, with a mean decrement of 27 +/- 7 beats/min. 48.8% of patients developed absolute bradycardia (< or = 60 beats/min). Four of 41 patients (9.7%) experienced high-grade heart block: 9.7% second degree and 4.8% third degree. Heart block patients did not differ from the non-heart block group with respect to age, interval from diagnosis or bone marrow harvest to transplant, cardiac risk factors, pretransplant electrocardiograms or radionuclide angiograms, transplant chemotherapy regimens or serum chemistry values. There was an increased incidence of heart block in patients with prior exposure to cyclophosphamide (p < 0.05) and vinca alkaloids (p < 0.05). There appears to be a high incidence of transient second and third degree heart block following autologous marrow infusion. This may be related to prior chemotherapy, but more likely is an effect of the infusate itself. Predisposing factors were not identified.

Adolescent↗
PubMed2002

Subcellular redistribution of la/SSB autoantigen during physiologic apoptosis in the fetal mouse heart and conduction system: a clue to the pathogenesis of congenital heart block.

OBJECTIVE: In isolated congenital heart block, the mechanism by which maternal autoantibodies target the intracellular components of the Ro/La RNP complex is unclear. Previous studies have demonstrated that cultured fetal cardiac myocytes rendered apoptotic bind antibodies to 48-kd La/SSB. This study further investigated the subcellular distribution of the La antigen during apoptosis in the fetal mouse heart and conduction system. METHODS: The atrioventricular (AV) node, AV bundle, and sinoatrial (SA) node were identified in serial sections prepared from paraffin blocks of normal mouse fetuses on days 15, 17, and 19 of gestation. Apoptosis was detected by TUNEL assay. Under confocal microscopy, fluorescent labeling of fragmented DNA in apoptotic cells was assessed by TUNEL, and La protein localization was visualized simultaneously using a murine monoclonal antibody or affinity-purified human polyclonal anti-La antibodies. RESULTS: Apoptotic cells were detected in and at the periphery of the AV and SA nodes as well as in the fetal heart valve insertions and working myocardium. In contrast, no apoptosis was detected in the adult heart AV node or surrounding myocardium. As expected, the La antigen was predominantly immunolocalized to the nucleus in nonapoptotic cells. However, apoptotic cells showed a marked reduction of nuclear La and redistribution of La to the cytoplasm. High-resolution confocal microscopy revealed that in cells that had undergone apoptosis, La antigen asymmetrically clustered near the surface of TUNEL-positive nuclei and apoptotic bodies. CONCLUSION: These data provide the first in vivo demonstration of the subcellular translocation of La autoantigen during apoptosis in the fetal heart and the conduction system under physiologic conditions. This observation supports the hypothesis that subcellular redistribution of La in the normally developing heart facilitates the binding of cognate maternal antibodies and subsequent tissue damage.

Animals↗