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Congenital heart block: successful prophylactic treatment with intravenous gamma globulin and corticosteroid therapy.

In mothers with anti-Ro-positive antibodies whose previous pregnancies have ended in deliveries of infants with congenital heart block, prophylactic therapeutic strategies are used to try to diminish the production and passage into fetal circulation of autoantibodies. Intravenous gamma globulin was given at 14 and 18 weeks' gestation and prednisone was given from 14 weeks' gestation to a woman with Sjögren's syndrome. The pregnancy ended with delivery of an infant without congenital heart block.

Adult↗

Double diastolic murmur in mitral stenosis with atrial fibrillation and complete heart block.

A double diastolic murmur was heard in a patient with mitral stenosis, atrial fibrillation, and complete heart block. Echo-phonocardiographic examination showed two separate opening and closing movements of the mitral valve in the same long diastole. A clear time relation was observed between the valve closing movements and the separate diastolic murmurs, giving support to the theory that the backward motion of the mitral valve against the forward blood flow through the valve is responsible for the production of these murmurs.

Atrial Fibrillation↗

The genesis of the diastolic murmur of complete heart block. Phono-echocardiographic observations.

A 72-year-old man with calcific aortic stenosis and complete heart block was noted to have an intermittent diastolic murmur. Using combined echocardiography and phonocardiography, it was demonstrated that the murmur occurred while the mitral valve was closing. This suggested that antegrade flow across a closing mitral orifice is of etiologic importance in the production of the murmur. Atrial sounds were also recorded and probably have a similar etiology. It is postulated that reduced ventricular compliance resulting in a more rapid rate of mitral valve closure was of etiologic importance in the genesis of the murmur.

Aged↗

Anatomy of congenital complete heart block and relation to maternal anti-Ro antibodies.

The anatomy of the conduction system was studied in 8 patients who had isolated congenital complete heart block. Atrial-axis discontinuity was seen in 7 hearts from children born to mothers with anti-Ro antibodies. The anticipated area of the atrioventricular node was occupied by fibrous and adipose tissue. The heart from the only child whose maternal serum was anti-Ro negative had nodoventricular discontinuity of the conduction system. This difference in anatomy may have etiologic significance.

Antibodies↗

Induction of antibodies reactive with SSA/Ro-SSB/La and development of congenital heart block in a murine model.

To correlate the arrhythmogenic effects of maternal autoantibodies with the genesis of congenital heart block, female BALB/c mice were immunized with human recombinant 48-kDa SSB/La, 60-kDa SSA/Ro, 52-kDa SSA/Ro (52alpha), and 52beta (amino acids 169-245 deleted) as well as with murine recombinant 52-kDa SSA/Ro. Control animals received beta-galactosidase or a polypeptide encoded by pET-28 alone. Following primary immunization and two boosters, high titer responses to the respective Ags were established by ELISA, immunoblotting, and immunoprecipitation. Sera from mice immunized with either human 52alpha or 52beta immunoprecipitated murine 52Ro. mRNA and protein expression of 52Ro was demonstrated in the newborn murine heart. A spectrum of atrioventricular nodal conduction abnormalities was identified by electrocardiogram. First-degree block was detected in 7% of 27 pups born to mothers immunized with 48La, 20% of 54 pups born to 60Ro-immunized mothers, 6% of 56 pups born to 52alpha-immunized mothers, 7% of 86 pups born to 52beta-immunized mothers, and 9% of 22 pups born to mothers immunized with murine 52Ro. Advanced conduction abnormalities were only identified in offspring of 52alpha- or 52beta-immunized mice. In the 52alpha group, one pup had complete block and another had second-degree block (Wenckebach type); in the 52beta group, five pups had complete block. Maternal Abs to the primary immunogens were detected in the pups. No control had any conduction abnormalities. This Ab-specific animal model provides strong evidence for a pathogenic role of anti-SSA/Ro-SSB/La Abs, particularly 52Ro, in the development of congenital heart block. The range and frequency of conduction defects suggest that additional factors promote disease expression.

Animals↗

Surgical heart block.

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Cardiac Surgical Procedures↗

Complete heart block in fetal lambs. I. Technique and acute physiological response.

Prenatally diagnosed complete heart block (CHB), without associated cardiac anomalies, is often refractory to attempts to increase heart rate and cardiac output by medical therapy, and results in fetal death. We developed a model of CHB in fetal lambs in order to evaluate the acute physiological consequences of CHB and to assess the feasibility of fetal cardiac ventricular (V) and atrioventricular (AV) pacing. After hysterotomy and fetal sternotomy AV-sequential pacing leads were placed on the right atrium and ventricle (RV). Electromagnetic flow transducers were placed around the pulmonary artery and the ascending aorta to measure combined ventricular output (CVO) in normal sinus rhythm (NSR), CHB, AV, and V pacing. CHB block was successfully induced in 19 of 21 (90%) fetal lambs at 100 to 110 days' gestation by injection of formalin into the interventricular septum. Two fetuses were refractory to CHB despite multiple injections. The mean ventricular escape rate was 52 +/- 3 (+/- SEM) beats/min (range, 45 to 60 beats/min). CHB lasted for the period of study (1 to 8 hours), and spontaneously reverted to NSR in one fetus after 3 hours. Combined ventricular output in NSR was 470 +/- 31 mL/min (+/- SEM), which fell to 213 +/- 13 mL/min in CHB, 45% of normal CVO (P less than .0001). CVO during AV-sequential and V pacing was 77% (P = .02) and 62% (P less than .001), respectively, of combined ventricular output in NSR. Both RV (27%) and left ventricles (LV) (37%) showed compensatory increase in stroke volume during CHB.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

Prevalence of retrograde conduction in heart block after DDD pacemaker implantation.

Electrophysiologic studies were performed before DDD pacemaker implantation in 50 patients with symptomatic heart block. The patients were separated into 2 groups. Group I consisted of patients with intact retrograde conduction and group II consisted of patients with blocked retrograde conduction. After pacemaker implantation, postventricular atrial refractory periods in patients in group I were programmed at 50 to 100 ms, in excess of the retrograde conduction times measured during electrophysiologic studies. In group II patients, postventricular atrial refractory periods were routinely programmed at 300 ms. During follow-up, patients visited the outpatient clinic at 3-month intervals for noninvasive assessment of the prevalence of retrograde conduction, and to test the inducibility of pacemaker-mediated tachycardias. The mean follow-up of group I (15 patients) was 27 +/- 10 months, whereas the mean follow-up of group II (35 patients) was 19 +/- 9 months. The mean number of noninvasive tests performed during follow-up was 8 +/- 3 per patient for group I and 5 +/- 3 per patient for group II. In group I, retrograde conduction remained intact in 12 patients (p less than 0.01). In 29 of 31 patients in group II, retrograde conduction remained absent (p less than 0.01). In 4 patients in group II, chronic atrial fibrillation occurred during follow-up. Chronic atrial fibrillation did not occur in any patient in group I. During serial electrophysiologic testing, no pacemaker-mediated tachycardias could be induced in any patient in group I or II.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Complete congenital heart block followed by anti-Ro/SSA in adult life. Studies of an informative family.

A family is described in which an asymptomatic woman gave birth to a male child with complete congenital heart block. Precipitating antibodies to the Ro/SSA antigen have developed in this child, now aged 33, who is clinically well. In the mother, features of both systemic lupus erythematosus and Sjögren's syndrome developed 26 years after the birth of her son. Her serum currently has precipitating antibodies to both the Ro/SSA and La/SSB antigens. The presence of anti-Ro/SSA in the child with complete congenital heart block and the extremely long delay of 26 years between the delivery of the child and the development of clinical disease in the mother are discussed in light of investigators' developing knowledge of both neonatal and adult systemic lupus erythematosus.

Adult↗

Progressive familial heart block type II (PFHBII): a clinical profile from 1977 to 2003.

An evaluation of a 38-year-old Caucasian woman, who was referred to Tygerberg Hospital (Western Cape Province, RSA) with Wenckebach second-degree or possibly complete atrioventricular (AV) block that had progressed from first-degree AV block, identified a family history of the cardiac conduction system disorder progressive familial heart block type II (PFHBII). This prompted a retrospective clinical review of the subjects described in the original study, as well as additional family members who had not been examined in the original study. Progression of clinical features was observed, but more importantly, PFHBII was clinically redefined as an AV nodal disorder, which may progress to dilated cardiomyopathy (DCM).

Adult↗

Complete heart block associated with lupus in a dog.

A 5-year-old Poodle-cross was initially presented for exercise intolerance and difficulty in chewing and yawning. Some months later it acutely developed lethargy referable to complete heart block. Further investigations before and after permanent pacemaker implantation demonstrated Coombs-positive immune-mediated haemolytic anaemia, presumptive masticatory myositis and hypoadrenocorticism, suggesting the possibility of multisystem auto-immune disease. A diagnosis of systemic lupus erythematosus (SLE) was made based on these findings and a positive anti-nuclear antibody titre. It was thought that immune-mediated destruction of cardiac conduction tissues was responsible for the development of atrioventricular conduction block. Glucocorticoid deficiency was corrected using cortisone replacement therapy. SLE was controlled successfully for 10 months using azathioprine monotherapy until signs, subsequently shown to be due to subacute bacterial endocarditis, resulted in the death of the patient. Lupus should be considered as a potential underlying aetiology in dogs that develop heart block.

Animals↗

Common form of Lyme borreliosis carditis--complete heart block with syncope: report on 3 cases.

Third degree atrioventricular (AV) heart block with severe Adams-Stokes attacks in 3 patients with Lyme borreliosis is described. All patients had similar clinical manifestations: previously healthy, they experience syncope as an abrupt onset of the disease. Data on skin changes--erythema migrans--was subsequently obtained, although the patients did not recall being bitten by a tick. Diagnoses were based on clinical manifestations and on positive serologic test results to borrelia. Following AV block returned to sinus rhythm with normal AV conduction in all patients.

Adams-Stokes Syndrome↗

Congenital heart block in one of the HLA identical twins.

A case of HLA identical twins with one affected by congenital heart block is reported. Both twins, as their mother, had more than 12-fold higher anti-Ro antibody titers compared to healthy controls, but no differences were observed between the affected and the healthy baby. It is possible that there is a third factor causing the manifestation of this disease.

Adult↗