Atrial appendages and venoatrial connections in hearts from patients with visceral heterotaxy

Abstract

Background: Venoatrial connections are important when choosing surgical options for patients with visceral heterotaxy. The precise morphology of the atriums, however, is often obfuscated by the term "visceral heterotaxy." This morphologic study aims to clarify the features of significance to the cardiac surgeon.

Methods: We investigated 183 hearts from patients known from postmortem inspection to have so-called visceral heterotaxy. The connections of the systemic and pulmonary veins to the atriums, and the detailed morphology of the atriums, were examined in each case.

Results: Pectinate muscles extended bilaterally to the crux in 125 hearts determined to have isomeric morphologically right appendages. The other 58 hearts all exhibited bilaterally smooth-walled vestibules, and were diagnosed as having isomeric left appendages. Bilateral superior caval veins were frequent in both groups. The inferior caval vein was right- or left-sided with equal frequency in both groups, but was interrupted only in hearts with isomeric left appendages. The pulmonary veins connected in extraatrial fashion in 48% of cases with isomeric right appendages, whereas, most commonly, pulmonary veins were connected bilaterally to the atriums in those with isomeric left appendages (60%).

Conclusions: Both the morphology of the atrial appendages and the venoatrial connections need to be distinguished to establish precise diagnoses in patients with so-called visceral heterotaxy ("splenic syndromes").