Progressive cardiac conduction defect

Progressive cardiac conduction defect
Other namesFamilial Lenègre disease, Familial Lev disease, Familial Lev-Lenègre disease, Familial PCCD, Familial progressive heart block, Progressive familial heart block, Hereditary bundle branch defect
SpecialtyCardiology

Progressive cardiac conduction defect (PCCD) is a hereditary cardiac condition marked by a progressive delay in impulse conduction via the His-Purkinje system, resulting in right or left bundle branch block (RBBB or LBBB), syncope, and occasionally sudden cardiac death.

Cause

Diagnosis

When progressive conduction abnormalities in people under 50 with structurally normal hearts are present but skeletal myopathies are absent, progressive cardiac conduction defect is primarily diagnosed, particularly if there is a family history of PCCD.

Treatment

At the moment, implanting an implantable pacemaker is the only proven treatment for PCCD, regardless of the underlying cause.

References

  1. ↑ Lynch, H T; Mohiuddin, S; Sketch, M H; Krush, A J; Carter, S; Runco, V (September 17, 1973). "Hereditary progressive atrioventricular conduction defect. A new syndrome?". JAMA. 225 (12): 1465–1470. doi:10.1001/jama.1973.03220400011003. PMID 4740717. Retrieved 22 January 2024.
  2. ↑ Baruteau, Alban-Elouen; Probst, Vincent; Abriel, Hugues (2015). "Inherited progressive cardiac conduction disorders". Current Opinion in Cardiology. 30 (1). Ovid Technologies (Wolters Kluwer Health): 33–39. doi:10.1097/hco.0000000000000134. ISSN 0268-4705. PMID 25426816. S2CID 13213870.
  3. ↑ Balmer, C (2002). "Long-term follow up of children with congenital complete atrioventricular block and the impact of pacemaker therapy". Europace. 4 (4). Oxford University Press (OUP): 345–349. doi:10.1053/eupc.2002.0266. ISSN 1099-5129. PMID 12408251.

Further reading