Progressive cardiac conduction defect explained
| Progressive cardiac conduction defect |
| Management: | --> |
| Risk: | --> |
| Cause: | --> |
| Synonyms: | 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
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
At the moment, implanting an implantable pacemaker is the only proven treatment for PCCD, regardless of the underlying cause.[3]
Further reading
- Asatryan . Babken . Medeiros-Domingo . Argelia . Molecular and genetic insights into progressive cardiac conduction disease . EP Europace . Oxford University Press (OUP) . 21 . 8 . April 26, 2019 . 1099-5129 . 10.1093/europace/euz109 . 1145–1158 . none.
Notes and References
- Lynch . H T . Mohiuddin . S . Sketch . M H . Krush . A J . Carter . S . Runco . V . Hereditary progressive atrioventricular conduction defect. A new syndrome? . JAMA . September 17, 1973 . 225 . 12 . 1465–1470 . 10.1001/jama.1973.03220400011003 . 4740717 . 22 January 2024.
- Baruteau . Alban-Elouen . Probst . Vincent . Abriel . Hugues . Inherited progressive cardiac conduction disorders . Current Opinion in Cardiology . Ovid Technologies (Wolters Kluwer Health) . 30 . 1 . 2015 . 0268-4705 . 10.1097/hco.0000000000000134 . 33–39. 25426816 . 13213870 .
- Balmer . C . Long-term follow up of children with congenital complete atrioventricular block and the impact of pacemaker therapy . Europace . Oxford University Press (OUP) . 4 . 4 . 2002 . 1099-5129 . 10.1053/eupc.2002.0266 . 345–349.
