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Prevalence of maternal Ro (SS-A) and La (SS-B) autoantibodies in relation to congenital heart block.

The prevalence of autoantibodies to ribonucleoprotein antigens in cases of congenital heart block was established using immunofluorescence, counterimmunoelectrophoresis, double immunodiffusion and Western blots. All of 35 mothers of babies with congenital heart block, none of five mothers of babies with other types of heart block, 10 of 29 women with connective tissue disease but no babies with heart block, four of 445 normal pregnant women and two of 109 healthy nonpregnant women had either Ro (SS-A) or La (SS-B) antibodies. Of 15 babies with congenital heart block, 10 of 10 who were less than 3 months old possessed antibody. Antibody titres in affected but not in normal infants were lower compared with their mothers' titres, suggesting deposition of antibodies in the baby's tissues. The findings indicate that placental transfer of anti-Ro (SS-A) or anti-La (SS-B) is essential for development of congenital complete heart block.

Antibodies, Antinuclear↗

Successful prenatal treatment of congenital heart block with ritodrine administered transplacentally.

Congenital heart block (CHB) is rather rare, and a poorer prognosis has been documented in fetuses with a ventricular rate <55 beats per minutes (bpm), in which therapeutic interventions during pregnancy have been warranted. We present a case of CHB associated with maternal anti-SSA/Ro antibody, diagnosed at 28 weeks' gestation. Fetal echocardiography revealed atrioventricular dissociation, with an atrial rate of 170 bpm and a ventricular rate of 54 bpm. To increase the fetal heart rate, maternal intravenous ritodrine infusion was undertaken, fetal ventricular rate was rapidly increased to 65 bpm. The pregnancy successfully continued until term, and a female infant weighing 2919 g was delivered by cesarean section with Apgar scores of 8 and 8 and 1 and 5 min. The infant is now 12 months of age and growing normally on oral terbutaline without pacing. In a case of fetal heart block, maternal administration of ritodrine may be a therapeutic intervention to improve the fetal and neonatal prognosis.

Adult↗

Complete heart block and physical performance.

Complete heart block (CCHB) is a very rare finding. The problems in such patients are: additional heart malformations, the appearance of Stokes-Adams or syncopal attacks, and the occurrence of different grades of ectopy. Most patients with CCHB without structural heart disease develop normally and can lead a normal life. If there are no Stokes-Adams attacks or high grades of ectopy at rest and exercise, they have a normal physical capacity and can perform strenuous work and sports. Increased stroke volume, cardiac enlargement, and extraction of very large amounts of oxygen from the blood were found to be compensatory mechanisms of adaptation. Some authors have discussed the possible role of training in this adaptation.

Adams-Stokes Syndrome↗

Pre-operative prophylactic transvenous cardiac pacing for bifascicular heart block.

Some authorities consider that the combination of right bundle-branch block with left axis deviation (bifascicular block) is not an indication for prophylactic insertion of a pacing generator in patients undergoing non-cardiac surgery. Five patients who developed peri-operative progression of bifascicular block to complete heart block are described. Bifascicular block, together with any other cardiovascular condition, advanced age or surgery in regions that promote vagal stimulation, merit consideration for prophylactic pacing. A cardiological opinion is an essential aspect of the pre-operative preparation of patients with this abnormality.

Aged↗

Complete heart block and cardiac tamponade secondary to Merkel cell carcinoma cardiac metastases.

This is a unique case of Merkel cell carcinoma, a rare neuroendocrine tumor, metastasizing to the heart and inducing a progression of cardiac complications such as new-onset atrial fibrillation, malignant pericardial effusion with tamponade physiology, first-degree heart block, and complete heart block. Metastases to the heart are relatively rare but should be suspected if a patient with a known neoplasm presents with new cardiac manifestations. This is the first case report of cardiac metastases from Merkel cell carcinoma causing cardiac tamponade or complete heart block. This case highlights the clinical decision-making involved in managing cardiac tamponade and complete heart block in the setting of metastatic disease to the heart.

Aged↗

Successful outcome in a fetus with an extremely low heart rate due to isolated complete congenital heart block.

Isolated complete congenital heart block (CCHB) in a fetus is usually associated with the presence of autoantibodies to SSA (Ro) and SSB (La) antigens in the maternal circulation. Although the prognosis for the majority of fetuses is good, it is less favorable in fetuses with a ventricular rate < 55 bpm in early pregnancy or with a decrease in the ventricular rate by >/= 5 bpm during pregnancy. It is not known if the same prognostic criteria apply for the occasional fetus with isolated non-autoimmune CCHB. We report a case of a single fetus with an isolated non-autoimmune CCHB with an extremely low ventricular rate (37 bpm) in which the outcome was favorable. Dilated cardiomyopathy is a rare complication in patients with isolated CCHB, despite early institution of cardiac pacing, and is usually recognized after several months of relative well-being. It is assumed that in the majority of patients it represents a sequel to in utero autoimmune or postnatal reactivation myocarditis. However, the possibility of a tachycardia-induced cardiomyopathy caused by an excessively high pacing rate should also be taken into consideration, as was clearly demonstrated in our patient.

Adult↗

Sarcoidosis associated with complete heart block.

The association between sarcoidosis and complete heart block was explored by a register study, a description of 9 own cases and a survey of 48 cases collected from the literature. Incidences were calculated from statistics of hospital in-patient diagnoses in a Swedish county over a 13-year period. Strong association was found between the two conditions, with significantly higher risk (RR = 16.7, P less than 0.001) of heart block in sarcoidosis patients than in the general population. Pacemaker management is often complicated in patients with sarcoidosis. The association should be kept in mind when dealing with patients presenting with sarcoidosis or complete heart block.

Adult↗

Complete heart block complicating hyperthyroidism: a case report.

Complete heart block developed in a 29-year-old man with hyperthyroidism and acute febrile illness. The definite cause of acute febrile illness was unknown. The results of bacteriological and viral studies were negative. Endomyocardial biopsy revealed no evidence of carditis which is the common cause of heart block. All the abnormalities resolved completely after the fever subsided and the patient was treated with an antithyroid drug. Available information indicates that a high degree of heart block can sometimes occur in hyperthyroidism in either the presence or absence of additional factors which can independently impair atrioventricular conduction. This course of events which occurred in this patient suggest that complete heartblock may have been the direct manifestation of the hyperthyroid state, however, the acute febrile illness may have been an aggravating factor in the development of abnormal atrioventricular conduction.

Adult↗

A new therapeutic approach to the fetus with congenital complete heart block: preemptive, targeted therapy with dexamethasone.

BACKGROUND: Therapy of established congenital complete heart block in the fetus has resulted in improved survival but persistence of heart block. This exposes the infant to the morbidity of heart block, including the risk of sudden death and pacemaker implantation. CASE: A 35-year-old gravida 2, para 1, with Sjogren syndrome and a previous pregnancy complicated by congenital complete heart block presented during her second pregnancy. Intensive fetal monitoring with echocardiography was employed. Early evidence of myocardial dysfunction and dysrhythmia was found, dexamethasone therapy was initiated, and the dysfunction and dysrhythmia resolved. The pregnancy went to term without further complication. CONCLUSION: This represents a new and successful strategy to identify very early signs of myocardial disease in a fetus at high risk of congenital complete heart block, enabling targeted, preemptive therapy.

Adult↗

Pacemaker therapy in children with complete heart block.

Permanent pacemaker therapy in children with complete heart block is necessary occasionally. Ten patients ranging in age from 8 months to 15 years were treated with an implanted P-wave, synchronous epicardial pacemaker. Indications for implantation were persisting postsurgical heart block, congestive failure, syncopal attacks, and arrhythmias. There were two deaths not attributed to pacemaker malfunction. The remaining eight children have been followed up for 38 to 108 months. There have been 27 pulse generator replacements. Twenty-three were for battery exhaustion, three for electromechanical failure, and one was due to arrhythmia. Rhythm disturbances have occurred on eight occasions. There have been no infections. It is concluded that the implanted P-wave synchronous pacemaker is an effective method of therapy when indicated for children with complete heart block.

Adolescent↗

Nocturnal enuresis secondary to heart block: report of cure by cardiac pacemaker implantation.

This report describes an unusual case of secondary nocturnal enuresis presumptively secondary to progressive bradycardia from complete heart block. Congenital complete heart block occurs in approximately 1 of 22,000 livebirths and is typically associated with structural congenital heart disease or maternal collagen vascular diseases. It can be entirely asymptomatic during infancy and childhood, depending in part on the escape rate and rhythm and other hemodynamic variables. The case described above was not diagnosed until the patient coincidentally underwent cardiac monitoring. The picture was confusing initially, as a tricyclic antidepressant medication had been ingested. Heart block is one of the known cardiovascular effects of tricyclic antidepressant overdose. However, the conduction disturbance should have resolved as the drug was excreted from the body. As children with congenital complete heart block get older, the ventricular escape rate typically decreases. In addition, as activity increases with age, more demand is placed for cardiac output. The resting end-diastolic volume is increased to elevate stroke volume in compensation for lower heart rate. As the escape rate decreases and the metabolic demand increases, patients with congenital complete heart block then may begin to develop symptoms. Typical symptoms in children include dizziness, Stokes-Adams syncopal attacks, fatigue, daytime somnolence, and other somatic complaints. Bedwetting has not been reported as an initial symptom, but in this case is likely secondary to the excessive somnolence and difficulty with arousal.(ABSTRACT TRUNCATED AT 250 WORDS)

Child, Preschool↗

Exercise-induced ventricular ectopy in children and young adults with complete heart block.

Twenty-five children and young adults with complete heart block and 50 normal control subjects were maximally exercised on a treadmill and their electrocardiograms were analyzed for the presence and degree of ventricular ectopy activity. Sixty-eight % (17 of 25) of the heart block patients and significant ventricular ectopy (frequent unifocal ectopy or worse) on exercise compared to 2% (one of 50) of the controls (p = less than 0.01). In the patients with block there was a significant trend toward more frequent and more severe ectopy with increasing age (p = less than 0.05) independent of heart rate. More severe ventricular ectopy was also seen in those patients with QRS prolongation (p = less than 0.05). No significant differences were found between the 14 patients with isolated "congenital" heart block, the seven with associated defects, and the four with surgically acquired block. We believe that severe ventricular ectopy is common at exercise in patients with complete heart block and they may have an increased risk for sudden death.

Adolescent↗