complete heart block – isolated

complete heart block – isolated

description

complete heart block(chb) occurs when atrial and ventricular contraction is completely dissociated or independent. this can be due to damage to the conducting tissue (usually caused by maternal auto-antibodies) or to abnormalities of the atrial nodes and conducting tissue, which can be a familial defect, or to congenital heart disease, particularly left atrial isomerism. in isolated chb, the fetal heart is structurally normal but the ventricular rate is sustained at less than 100 beats per minute. this is usually due to damage to the conducting tissue by circulating anti-ro antibodies (anti-ssa in some laboratories). the mother is usually asymptomatic but may have clinical sjogren’s syndrome or systemic lupus erythematosis. complete heart block may become evident at some time between 18-24 weeks gestation. it has been reported to develop later but this is rare. usually the affected fetus remains well in utero but in some cases, evidence of fetal hydrops develops. in this latter setting, the outcome tends to be poor. if the fetus survives to delivery close to term, pacing can be successfully carried out if it proves necessary. premature delivery with immediate pacemaker placement has salvaged some hydropic infants. many children do not need pacing for some years. rarely, isolated chb is due to an abnormality of the conduction tissues which is familial in aetiology. maternal antibodies are negative. this appears to have a less benign course in fetal life than those who are antibody positive.

diagnosis

the heart is dilated on measurement of the cardiothoracic ratio and is hypertrophied. the ventricular rate is visibly slow on cross-sectional scanning. on m-mode measurement, the atrial rate will usually be normal around 140 beats per minute with the ventricular rate anywhere between 45-80 beats per minute. there is no relationship between atrial and ventricular contraction on a simultaneous m-mode recording displaying the atrial and ventricular walls or the atrial wall and aortic valve. the dissociation of atrial and ventricular contraction can also be shown on a doppler recording of the mitral valve and aortic outflow tract. the heart structure is normal. the arterial doppler velocities are increased above the normal range. there may be evidence of atrioventricular valve insufficiency and hydrops fetalis if decompensation occurs. there may also be evidence of myocardial damage by antibodies, in terms of decreased left ventricular shortening fraction and increased ventricular wall echogenicity.

differential diagnosis

short episodes of sinus bradycardia are common in the midtrimester fetus. these are not sustained, which distinguish them from complete heart block. frequent blocked ectopic beats can also produce a bradycardia. these can usually be distinguished on the m-mode tracing demonstrating atrial and ventricular contraction. in blocked ectopics, the atrial wall will show two beats close together followed by a pause, whereas in chb the atrial wall movement is regular.

sonographic features

slow heart rate, less than 100bpm.

dissociation of atrial and ventricular contraction.

increase in c/t ratio, myocardial hypertrophy.

increased arterial dopplers.

may be increased myocardial echogenicity, diminished function.

may be av valve regurgitation.

may be fetal hydrops.

associated syndromes

  • charge
  • chromosomal
  • digeorge
  • partial situs inversus
  • thalidomide

references

  1. gillette pc, garson a, porter cj, mcnamara dg in: paediatric cardiology anderson rh, mccartney fj, shinebourne ea, tynan m (eds). churchill livingstone: edinburgh, p1278-9
  2. bharati s, lev m in: heart disease in infants, children and adolescents adams fh, emmanouilides gc, riemenschneider ta (eds). williams and wilkins: baltimore, p1010-1
  3. machado mvl, tynan mj, curry pvl, allan ld fetal complete heart block br heart j 60:512-515
  4. ho sy, fagg n, anderson r, cook a, allan ld disposition of the atrioventricular conduction tissues in the heart with isomerism of the atrial appendages: its relation to congenital complete heart j am coll cardiol 20:904-10