n Cardiovascular Journal of South Africa - Progressive familial heart block type II (PFHBII) : a clinical profile from 1977 to 2003 : cardiovascular topics
|Article Title||Progressive familial heart block type II (PFHBII) : a clinical profile from 1977 to 2003 : cardiovascular topics|
|© Publisher:||Clinics Cardive Publishing|
|Journal||Cardiovascular Journal of South Africa|
|Author||P. Fernandez, V.A. Corfield and P.A. Brink|
|Publication Date||May 2004|
|Pages||129 - 132|
An evaluation of a 38-year-old Caucasian woman, who was referred to Tygerberg Hospital (Western Cape Province, RSA) with Wenckebach second-degree or possibly complete atrioventricular (AV) block that had progressed from first-degree AV block, identified a family history of the cardiac conduction system disorder progressive familial heart block type II (PFHBII). This prompted a retrospective clinical review of the subjects described in the original study, as well as additional family members who had not been examined in the original study. Progression of clinical features was observed, but more importantly, PFHBII was clinically redefined as an AV nodal disorder, which may progress to dilated cardiomyopathy (DCM).
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