Surgical management of hearts with isomeric atrial appendages

Abstract

Background and Aim: On the basis of previously published accounts, coupled with our own experience, we have assessed the surgical approaches to patients with isomeric atrial appendages. Methods:We reviewed pertinent published studies on surgical treatment of individuals with isomeric atrial appendages, with the pertinent surgical details provided by most of the manuscripts.Results: Half of patients with right isomerism, and two-thirds of those with left isomerism have bilateral superior caval veins. Azygos extension of the inferior caval vein is reported in three-quarters of those with left isomerism. The coronary sinus is universally absent in right isomerism, along with totally anomalous pulmonary venous connection, and is absent in two-fifths of those with left isomerism. Univentricular atrioventricular connections are expected in up to three-quarters of those with right isomerism. Atrioventricular septal defect is reported in up to fourfifths, more frequently in right isomerism, with such patients typically having discordant ventriculoatrial connections or double outlet right ventricle. Reported mortalities extend to 85% for those with right, and 50% for those with left isomerism. In right isomerism, mortality is up to 54% for systemic-to-pulmonary arterial shunting, up to 75% for univentricular repair, and up to 95% for repair of totally anomalous pulmonary venous connection itself. No more than one-quarter had undergone Fontan completion, with reported mortalities of 21%. Conclusion:Early surgical results are satisfactory in patients with left isomerism, but disappointing for those with right. Recent advances in cardiac and liver transplantation may offer improved survival.

Long-Term Surgical Outcomes of Patients With Isomeric Right and Left Atrial Appendages

“…It is now beginning to be accepted that individuals with the so-called “heterotaxy” are best segregated according to the morphology of the atrial appendages. 4–9 ,E3-E5,E8,E9,E38,E39 Our study now shows that computed tomographic imaging is capable of determining appendage morphology, thus facilitating surgical planning (Figures 1-4). 3,5 ,E7,E9 It also proved possible to confirm the distinguishing features during the surgical procedures.…”

“…4–9 ,E3-E5,E8,E9,E38,E39 Our study now shows that computed tomographic imaging is capable of determining appendage morphology, thus facilitating surgical planning (Figures 1-4). 3,5 ,E7,E9 It also proved possible to confirm the distinguishing features during the surgical procedures. Cyanosis as a presenting symptom was present in both groups, and about half of the patients in each group also had congestive cardiac failure (Table E3).…”

“…Studies assessing the surgical outcomes for individuals with isomeric atrial appendages are limited. 4–9 ,E3-E5,E9,E10 To our knowledge, our cohort of 431 patients undergoing surgery consecutively by a single surgeon is one of the largest studies currently available in the English literature. It is now beginning to be accepted that individuals with the so-called “heterotaxy” are best segregated according to the morphology of the atrial appendages.…”

“…It is now accepted that the extent of the pectinate muscles relative to the vestibules of the atrioventricular junctions permits the distinction of right versus left isomerism. 1–5 Individuals with these morphologies are characterized by complex intracardiac anatomies, particularly regarding the systemic and pulmonary venous connections, for which surgical corrections are commonly performed. 1–9 ,E1-E5 To date, however, little emphasis has been placed on whether surgical approaches should be tailored specifically to one or other of the subsets.…”

“…1–5 Individuals with these morphologies are characterized by complex intracardiac anatomies, particularly regarding the systemic and pulmonary venous connections, for which surgical corrections are commonly performed. 1–9 ,E1-E5 To date, however, little emphasis has been placed on whether surgical approaches should be tailored specifically to one or other of the subsets. Studies on long-term outcomes, furthermore, are limited.…”