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Familial Congenital Sinus Rhythm Anomalies: Clinical and Pathological Correlations
Author(s) -
BHARATI SAROJA,
SURAWICZ BORYS,
VIDAILLET HUMBERTO J.,
LEV MAURICE
Publication year - 1992
Publication title -
pacing and clinical electrophysiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.686
H-Index - 101
eISSN - 1540-8159
pISSN - 0147-8389
DOI - 10.1111/j.1540-8159.1992.tb02960.x
Subject(s) - medicine , pathological , cardiology , atrophy , sinus (botany) , atrial fibrillation , sinoatrial node , sick sinus syndrome , electrical conduction system of the heart , anatomy , electrocardiography , heart rate , botany , blood pressure , biology , genus
We describe pathological abnormalities in a 72‐year‐old male member of a family with a congenital absence of sinus rhythm and a tendency to develop atrial fibrillation at an early age, and in a 54‐year‐old female member of a family with cardiomyopathy and progressive conduction system disease manifested by first‐degree atrioventricular (AV) block, left bundle branch block, and atrial arrhythmias. Both patients died suddenly. The absence of sinus rhythm in case 1 could be explained by marked atrophy, degeneration, and isolation of the sinoatrial (SA) node. The SA node was also diseased in the member of the other family with atrial arrhythmias. Additional common features in both cases included: fatty metamorphosis and degenerative changes of the approaches to the SA node, the atrial preferential fibers, and the approaches to the AV node, a small AV node, degenerative changes of the bundle branches, and floppy AV valves. These findings show that the pathological substrate of familial supraventricular arrhythmias consists of a diffuse involvement of the entire conduction system, bearing resemblance to pathological findings in elderly subjects with acquired sick sinus syndrome.