Congenital complete heart block (CCHB) occurs in 2-5% of pregnancies with positive anti-Ro/SSA and/or anti-La/SSB antibodies, and has a recurrence rate of 12-25% in a subsequent pregnancy. After trans-placental passage, these autoantibodies attack and destroy the atrioventricular (AV) node in susceptible fetuses with the highest-risk period observed between 16 and 28 weeks' gestational age. Many mothers are asymptomatic carriers, while <1/3 have a preexisting diagnosis of a rheumatic disease. The mortality of CCHB is predominant in utero and in the first months of life, reaching 15-30%. The diagnosis of CCHB can be confirmed by fetal echocardiography before birth and by electrocardiography after birth. Whether early in-utero detection and treatment might prevent or reverse this condition remains controversial. In addition to autoantibody-associated CCHB, there is also an isolated (absent structural heart disease) nonimmune early- or late-onset heart block detected later in childhood that may be associated with specific genetic markers or other pathogenic mechanisms. In isolated immune or non-immune CCHB, cardiac pacemakers are implanted in symptomatic patients, however, data on the natural history of CCHB in the adult life indicate that all patients, even if asymptomatic, should receive a pacemaker when first diagnosed. However, important issues have emerged in these patients wherein life-long conventional right ventricular apical pacing may produce left ventricular dysfunction (pacing-induced cardiomyopathy) necessitating a priori alternate site pacing or subsequent upgrading to biventricular pacing. All these issues are herein reviewed and two algorithms are proposed for diagnosis and management of CCHB in the fetus and in the older individual.
Keywords: AV node; Anti-La/SSB antibodies; Anti‑Ro/SSA antibodies; Atrioventricular block; Autoantibodies; Cardiac pacing; Cardiac resynchronization therapy; Congenital heart block; Connective tissue diseases; Heart block; Heart failure; His bundle pacing; Neonatal lupus; Pacing-induced cardiomyopathy; Sjogren's syndrome; Systemic lupus erythematosus.
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