Congenital atrioventricular block in right atrial isomerism (asplenia). A case due to atrionodal discontinuity.

Abstract:

:The association of complex congenital heart disease with asplenia syndrome and congenital AV block is described in a newborn. Cardiovascular lesions were right atrial isomerism, d-loop, discordant ventriculoarterial connection, pulmonary stenosis, total anomalous pulmonary venous drainage, absent right pulmonary artery, and bilateral superior vena cava. The ECG recording disclosed complete AV block with narrow QRS (suprahisian block). The histology of the conduction system revealed two AV nodes, one anterior and one posterior, none of which connected with the atrial myocardium; only the posterior node was continuous with a penetrating and bifurcating bundle. The histologic findings were consistent with the suprahisian AV block due to lack of connection between the atria and the regular posterior AV specialized junction.

journal_name

Chest

journal_title

Chest

authors

Rossi L,Montella S,Frescura C,Thiene G

doi

10.1378/chest.85.4.578

subject

Has Abstract

pub_date

1984-04-01 00:00:00

pages

578-80

issue

4

eissn

0012-3692

issn

1931-3543

pii

S0012-3692(16)37866-7

journal_volume

85

pub_type

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