Progressive cardiac conduction defect
(Redirected from Heart block progressive, familial)
| Progressive cardiac conduction defect | |
|---|---|
| Other names | Familial Lenègre disease, Familial Lev disease, Familial Lev-Lenègre disease, Familial PCCD, Familial progressive heart block, Progressive familial heart block, Hereditary bundle branch defect |
| Specialty | Cardiology |
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.[1]
Diagnosis
[edit]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.[2]
Treatment
[edit]At the moment, implanting an implantable pacemaker is the only proven treatment for PCCD, regardless of the underlying cause.[3]
References
[edit]- ^ 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.
- ^ 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.
- ^ 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.
Further reading
[edit]- Asatryan, Babken; Medeiros-Domingo, Argelia (April 26, 2019). "Molecular and genetic insights into progressive cardiac conduction disease". EP Europace. 21 (8). Oxford University Press (OUP): 1145–1158. doi:10.1093/europace/euz109. ISSN 1099-5129.