The spectrum of atrioventricular discordance. A clinical study.

Abstract

SUMMARY The clinical, investigative, and surgical findings were reviewed in 47 patients with atrioventricular discordance who presented to the Brompton Hospital between January 1962 and June 1981. Although the unifying feature was the atria connecting to morphologically inappropriate ventricles, the hearts differed widely in other respects. In most cases there was the usual visceral and atrial arrangement, but six had a mirror image arrangement of the atria and viscera. Among those patients with usual atrial arrangement (solitus) the aorta was not always anterior and left sided, and ventricular "inversion" was not invariable. Only 35 of the 47 patients also had a discordant ventriculoarterial connexion, the majority of the remainder having a double outlet right ventricle.The specific diagnosis of atrioventricular discordance depended on echocardiographic and angiographic examination. The other investigations did not distinguish between different ventriculoarterial connexions and were not specific even for the presence of atrioventricular discordance. For those patients with the usual atrial arrangement the anticipated right heart border was present in only just over one third, and the reversal of Q wave progression in the precordial leads of the standard electrocardiogram was found in under a half.Many patients with atrioventricular discordance progressed normally to adult life. In 20 cases no surgery was performed. The results of total correction showed a mortality of 25% (three cases), including two deaths after a modified Fontan procedure for exceedingly complex associated lesions. The results of surgery in the survivors were excellent, and awareness of the disposition of the atrioventricular conduction tissue made it possible to avoid atrioventricular dissociation.

A Straight Left Heart Border: A New Radiological Sign of a Hemopericardium

“…The congenital pericardial absence is due to left common cardiac vein atrophy with ischemia of the pericardium, resulting in its agenesis [21]. A corrected transposition of the great vessels also creates a SLHB due to the contour of the transposed ascending aorta [22, 23]. A SLHB may also indicate a previous left lower lobectomy [24].…”

Transposition physiology in the setting of concordant ventriculo‐arterial connections

“…A number of cases have now been described in which, as predicted by Geipel, 1 despite the existence of concordant ventriculo‐arterial connections, the physiological picture produced is that of transposition 8–54 . Understanding of these accounts has been hindered by the varied terms used for description.…”

Ventricular inversion with normally connected great vessels in situs solitus (atrioventricular discordance with ventriculoarterial concordance)

“…With ventricular inversion the great vessels are generally either transposed, resulting in corrected transposition of the great vessels, or both great vessels arise from the mRV . When both great vessels originate from the inverted mRV, the aorta usually lies to the left of the pulmonary trunk [5] . The purpose of this report is to place on record a case of ventricular inversion with the great vessels normally connected (ventriculoarterial concordance) in situs solitus because of its rarity .…”