CLINICAL EXPERIENCES WITH A NEW DRUG IN THE TREATMENT OF HEART BLOCK.
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Lyme carditis is becoming a more frequent complication of Lyme disease, primarily due to the increasing incidence of this disease in the United States. Cardiovascular manifestations of Lyme disease often occur within 21 days of exposure and include fluctuating degrees of atrioventricular (AV) block, acute myopericarditis or mild left ventricular dysfunction and rarely cardiomegaly or fatal pericarditis. AV block can vary from first-, second-, third-degree heart block, to junctional rhythm and asystolic pauses. Patients with suspected or known Lyme disease presenting with cardiac symptoms, or patients in an endemic area presenting with cardiac symptoms with no other cardiac risk factors should have a screening electrocardiogram along with Lyme titers. We present a case of third-degree AV block due to Lyme carditis, illustrating one of the cardiac complications of Lyme disease. This disease is usually self-limiting when treated appropriately with antibiotics, and does not require permanent cardiac pacing.
We report a permanent complete heart block, in a 27 year-old female patient, developed during mitral percutaneous valvotomy using a single inflation of the Inoue balloon catheter. After one month, even using corticoids, a Mobitz II second-degree atrioventricular block persisted, the patient became symptomatic to moderate efforts and a cardiac pacemaker was implanted.
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An experimental therapeutic regimen for congenital lupus erythematosus, which consists of treating the mother with high doses of dexamethasone and performing plasmapheresis (3 times per week), was developed. This regimen was administered to a woman whose pregnancy was complicated by the abrupt onset of fetal pericarditis, myocarditis, and complete heart block in the twenty-fourth week of gestation. The effectiveness of plasmapheresis was evidenced by a decrease in the very high titer of SS-B (La) antibodies in the mother. After 5 weeks of therapy, the cardiac disease was ameliorated. The infant tolerated delivery at week 31, and the heart block has persisted. The rationale for this therapy for fetal disease is discussed, with emphasis on 2 goals: to diminish the quantity of maternal antibody that is transmitted to the fetus, and to suppress the manifestations of fetal carditis with the use of glucocorticoids that are not inactivated by the placenta. Tentative recommendations for more intensive prenatal monitoring of maternal and fetal status for those at risk are outlined.
OBJECTIVE: To extend the information base on cutaneous manifestations of neonatal lupus erythematosus (NLE) with regard to maternal disease, sex of child, onset, localization, influence of UV light, prognosis, and recurrence rates in subsequent pregnancies. METHODS: Review of records from the Research Registry for Neonatal Lupus. RESULTS: The cohort includes 47 mothers (83% white) whose sera contain anti-SSA/Ro, anti-SSB/La, and/or anti-U1-ribonucleoprotein antibodies and their 57 infants (20 boys and 37 girls) diagnosed with cutaneous NLE (absent heart disease) between 1981 and 1997. At detection of the child's rash, 13 mothers were asymptomatic, 11 had an undifferentiated autoimmune syndrome (UAS), 9 had systemic lupus erythematosus (SLE), 7 Sjögren's syndrome (SS), 6 SLE/SS, and 1 rheumatoid arthritis/SS; 20 reported photosensitivity. Within 5 years, 7 asymptomatic mothers experienced disease progression: 1 developed photosensitivity, 2 SLE, 3 SS, 1 SLE/SS; in 2 mothers UAS progressed to SLE; and 2 mothers with SS developed SLE. The infant's rash often followed UV light exposure; mean age at detection was 6 weeks, and mean duration was 17 weeks. All had facial involvement (periorbital region most common) followed by the scalp, trunk, extremities, neck, and intertriginous areas. In 37, the rash resolved without sequelae, 43% of which were untreated. A quarter had residual sequelae that included telangiectasia and dyspigmentation. One child developed Hashimoto's thyroiditis, and 2 developed systemic-onset juvenile rheumatoid arthritis. Of 20 subsequent births, 7 children were healthy, 2 had congenital heart block (CHB) only, 4 CHB and skin rash, and 7 skin rash only. CONCLUSIONS: Future pregnancies should be monitored by serial echocardiograms, given the substantial risk for heart block. Affected children should be observed for later development of a rheumatic disease.
A case is described of complete heart block during spinal anaesthesia for Caesarean section in a fit 23 yr-old-woman. This developed shortly after the institution of the block, with the height of the block below T5 and in the absence of hypotension. The patient was resuscitated successfully with vagolytic and alpha-agonist drugs. A Wenckebach block persisted for a short period postoperatively. The importance of instituting monitoring before the beginning of anaesthesia and the immediate availability of atropine and alpha-agonists before the initiation of spinal anaesthesia is stressed.
A 34-year-old man with ankylosing spondylitis was admitted to the hospital because of syncopal attacks and heart block. Standard ECG, telemetry and an invasive electrophysiologic examination demonstrated right bundle branch block with left anterior fascicular block, sinus node dysfunction with spontaneous slowing of the sinus rate and, as a result, complete infrahisian atrioventricular block in the remaining fascicle at sinus rates below 57 beats per minute. Infrequent supraventricular extrasystoles and abruptly terminated atrial pacing led to the same result. Atrioventricular conduction resumed after three-to-four blocked atrial impulses with successively increasing rate. Pacemaker treatment provided complete symptomatic relief.
Two cases of thyrotoxicosis including a first-degree heart block are reported; a study of action potentials of the bundle of His was achieved in the second case. The association atrioventricular block-hyperthyroidism is unusual but does not seem to be a pure coincidence. The direct effect of thyroid hormon on the myocardium is supposed to be responsible for the conduction disturbance; its disappearance with the cure of the thyrotoxicosis is a favourable argument for a relation of causality. The therapeutic consequence of this association is to exclude completely the use of betablockers.
A simple technique for the diagnosis of fetal congenital heart block by simultaneous Doppler blood-flow velocimetry of the fetal abdominal aorta and inferior vena cava is described. Furthermore, a simple method of monitoring the fetal atrial reactivity during the antenatal period; and simultaneous recording of atrial and ventricular rates during labour are also described. These simple techniques can be applied for the diagnosis and monitoring of the fetus in other types of cardiac arrhythmias.
A 48-year-old man with symptoms of presyncope and congestive cardiac failure had hypertrophic cardiomyopathy (HCM) without obstruction. Complete heart block (CHB), a rare complication of this disease, was preceded by complete left bundle-branch block. Right ventricular (RV) heart failure was a dominant clinical feature but improved dramatically after temporary transvenous RV pacing prompting the insertion of a permanent RV inhibited pacemaker. Repeated ventricular fibrillation was successfully controlled by amiodarone. This is the seventh case of HCM complicated by CHB reported in the literature, and the first in which RV endomyocardial biopsies were undertaken. Two other patients reported in the literature had RV inhibited permanent pacemakers implanted, and a further 2 had atrioventricular sequential pacemakers.
Isolated congenital heart block (ICHB) is frequently associated with neonatal lupus syndrome (NLS). Therefore few data are available regarding the long-term cardiac outcome of newborns with ICHB and the pathogenic mechanisms are not yet defined. In order to compare demographic features and cardiological outcome of patients with ICHB submitted to pacemaker (PM) implantation with and without NLS, forty ICHB patients were evaluated pre- and post-PM implantation, by clinical, electrocardiogram, Holter Monitoring, treadmill test, and electrophysiological study. According to the presence of antibodies to 52 and 60 kDa Ro/SSA and La/SSB proteins in mother's sera, it was found that 60% (24/40) of patients had ICHB associated to NLS (ICHB/NL+). Twenty-three of 24 ICHB/NL+ patients were asymptomatic, and 16 (67%) were female (P = 0.013). The frequency of syncope, mitral insufficiency (MI), and congestive heart failure (CHF) was similar pre-PM implantation in both ICHB/NL+ and ICHB/NL- groups (P > 0.05). After PM implantation, MI and CHF were only observed in ICHB/NL+ patients, although not statistically significant. Interestingly, 67% of ICHB/NL+ were noticed before one year of age while only one fourth of ICHB/NL- was diagnosed in this period (P = 0.024). Almost half (46%) of ICHB/NL+ patients required PMs in the first 24 months of life, whereas only one in the ICHB/NL- received a PM at the same age (P = 0.02). In ICHB patients requiring PM implantation, the antibody-mediated lesion seems to be associated with an earlier onset and a more severe heart disease, in spite of the uniform criteria for PM indication.
OBJECTIVE: To determine whether anti-Ro/SS-A antibodies selectively bind to neonatal cardiac cells and alter membrane repolarization. METHODS: An in vitro electrophysiologic and immunocytochemical experimental model contrasting neonatal and rabbit cardiac tissue was employed. RESULTS: Sera and IgG-enriched fractions from anti-Ro/SS-A antibody-positive mothers of infants with neonatal lupus erythematosus and congenital heart block bind to neonatal, rather than adult, rabbit cardiac tissue and alter the transmembrane action potential (i.e., inhibit repolarization). The additional presence of anti-La/SS-B antibodies was not additive or synergistic for these immunocytochemical and electrophysiologic features. Sera containing other antibody specificities (i.e., anti-native DNA, cardiolipin, Sm, and nuclear RNP) failed to stain the neonatal cardiac tissue or produced alterations in membrane repolarization. CONCLUSION: Anti-Ro/SS-A antibodies may play a pathophysiologic role in the development of congenital heart block in neonatal lupus.
Acute and subacute complete heart block (CHB) are sequelae of alcohol septal ablation (ASA) for obstructive hypertrophic cardiomyopathy. Temporary pacemakers are routinely placed at the time of ASA, but there are no widely accepted guidelines for their management. This study examined acute predictors of subacute CHB in 52 consecutive ASA procedures in 48 patients without preexisting permanent pacemakers. Acute CHB occurred during 32 ASA procedures (62%), with the return of atrioventricular conduction on the day of ASA in all cases. New intraventricular conduction defects (IVCDs) were noted after 32 procedures (62%); in 9 of these, there was new first-degree atrioventricular block as well. CHB recurred subacutely 36 +/- 22 hours after 13 ASA procedures (25%). In 5 of these cases, there was absent or inconsistent ventricular escape rhythm. Subacute CHB did not occur in 9 cases without acute CHB during ASA or new IVCDs after ASA. Acute CHB during ASA, new IVCDs after ASA, and new first-degree atrioventricular block after ASA incrementally increased the risk for subacute CHB. In conclusion, patients with acute CHB during ASA or new IVCDs after ASA are at high risk for developing subacute CHB, sometimes without a reliable escape rhythm; these patients should therefore have temporary pacing support for > or = 48 hours after ASA or the last occurrence of CHB. Patients without acute CHB during ASA or new IVCDs after ASA are at low risk for subacute CHB.
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