Congenital heart block

See also: Neonatal lupus erythematosus
Congenital heart block
The conduction system of the heart (shown in yellow)
SpecialtyMedical genetics Edit this on Wikidata
Symptomsslow heart rate[1]
Usual onsetin utero.[1]
Diagnostic methodfetal echocardiogram and Doppler and ELISA for the mother[1]
Treatmentfluorinated steroids, beta agonists, IVIG, HCQ, pace maker implantation and maternal plasmapheresis.[1][2]
Frequency1 child in every 15000-20000[3]

The congenital heart block (CHB) is the heart block that is diagnosed in fetus (in utero) or within the first 28 days after birth[1][4] (neonatal period), some studies also include the diagnosis during early childhood to the definition of CHB.[5] It refers to the disorder in the electrical conduction system within the heart muscle,[4] which leads to the failure in pumping the blood efficiently into the aorta and the pulmonary trunk. The result of CHB can be first, second, or third-degree (complete) atrioventricular block (a block in the atrioventricular node) in which no electric signals move from the atrium to the ventricles[5]

The congenital heart block is a rare disease that affects around 1 child in every 15,000–20,000 births.[3] However, its high mortality (which can be as high as 85% in some severe cases) makes the early diagnosis and intervention very important.[1] CHB can be isolated, where the fetus does not suffer from any other problems, or it can be a result of other diseases either in the child or in the mother.[1]

In most cases, the congenital heart block is associated with other diseases,[5][4][1] and therefore, the symptoms vary a lot between patients. However, low heart rate is usually the main clinical presentation that leads to the diagnosis.[6][5][1] Also, the treatment varies as well due to the associated diseases and it can be non-invasive (medications given to the pregnant woman or to the child),[2][7][1] or a surgery in some cases when the CHB is resulted from anatomical disorders in the heart.

  1. ^ a b c d e f g h i j Friedman, DM; Duncanson, LJ; Glickstein, J; Buyon, JP (2003). "A review of congenital heart block". Images in Paediatric Cardiology. 5 (3): 36–48. ISSN 1729-441X. PMC 3232542. PMID 22368629.
  2. ^ a b Saxena, Amit; Izmirly, Peter M.; Mendez, Barbara; Buyon, Jill P.; Friedman, Deborah M. (November 2014). "Prevention and treatment in utero of autoimmune-associated congenital heart block". Cardiology in Review. 22 (6): 263–267. doi:10.1097/CRD.0000000000000026. ISSN 1538-4683. PMC 4539276. PMID 25050975.
  3. ^ a b Michaëlsson, M.; Engle, M. A. (1972). "Congenital complete heart block: an international study of the natural history". Cardiovascular Clinics. 4 (3): 85–101. ISSN 0069-0384. PMID 4273004.
  4. ^ a b c Ambrosi, Aurélie; Wahren-Herlenius, Marie (2012-04-26). "Congenital heart block: evidence for a pathogenic role of maternal autoantibodies". Arthritis Research & Therapy. 14 (2): 208. doi:10.1186/ar3787. ISSN 1478-6362. PMC 3446439. PMID 22546326.
  5. ^ a b c d Brito-Zerón, Pilar; Izmirly, Peter M.; Ramos-Casals, Manuel; Buyon, Jill P.; Khamashta, Munther A. (May 2015). "The clinical spectrum of autoimmune congenital heart block". Nature Reviews. Rheumatology. 11 (5): 301–312. doi:10.1038/nrrheum.2015.29. ISSN 1759-4804. PMC 5551504. PMID 25800217.
  6. ^ Cite error: The named reference :3 was invoked but never defined (see the help page).
  7. ^ Cite error: The named reference :4 was invoked but never defined (see the help page).