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Ann Thorac Surg 1998;66:681-682
© 1998 The Society of Thoracic Surgeons
a Division of Cardiology, Department of Paediatrics, The Hospital for Sick Children and University of Toronto Faculty of Medicine, Toronto, Ontario, Canada
Address reprint requests to Dr Freedom, The Hospital for Sick Children, Rm 1503C, 555 University Ave, Toronto, ON, Canada, M5G 1X8
Presented at the Workshop on "One and One-Half Ventricle Repairs," Gubbio, Italy, Dec 6–7, 1996.
Abstract
There is now a considerable literature that babies with right atrial isomerism have a poor outcome. The reasons for this are complex and multifactorial, but may be related at least in part to intrinsically small and abnormal pulmonary veins. We reviewed a series of consecutive patients seen at a single institution and found that babies with right atrial isomerism, severe pulmonary outflow tract obstruction or atresia, and total anomalous obstructed pulmonary veins had a grim outlook, especially those requiring operation in the neonatal period. Others have reported a similarly concerning outcome.
Among patients with major anomalies of pulmonary venous connections and complex intracardiac anatomy amenable either to a one ventricle or a one and a half ventricle repair, the majority will have the syndrome of right atrial isomerism, with or without asplenia [1–6]. There are of course exceptions to this, but in this article we will explore the relationship between abnormal pulmonary venous connections and outcome. Although the hypoplastic left heart syndrome has been identified as "the worst heart disease" [7], patients with right atrial isomerism, especially those with obstructed pulmonary venous connections, have a poor long-term outlook.
Prognosis for patients with right atrial isomerism
Data from the Toronto Hospital for Sick Children’s Cardiology, Cardiovascular Surgical, and Department of Pathology’s databases have identified 91 patients with right atrial isomerism from 1970 to 1996 [8]. The majority of patients presented at birth (62%), with 87% presenting in the first month of life. Cardiac anomalies included an abnormal ventriculoarterial connection in 97%, pulmonary outflow tract obstruction in 88%, anomalies of pulmonary venous connection in 86%, pulmonary venous obstruction in 28%, a common atrioventricular orifice in 78%, and ventricular hypoplasia or some form of univentricular atrioventricular connection in 74%. For this entire cohort of patients with right atrial isomerism identified at a single institution, overall mortality was 60%. The Kaplan-Meier survival was 88% at 7 days, 77% at 1 month, 57% at 1 year, 44% at 5 years, 42% at 10 years, and 33% at 20 years. Risk factors for death in this particular series included pulmonary vein obstruction, prostaglandin dependence at presentation, and lower birth weight. Other studies have also shown that severe pulmonary vein obstruction also correlates with poor outcome in these patients [9–13].
The pulmonary veins and visceroatrial heterotaxia
Jenkins and colleagues have investigated whether mortality in patients with bilaterally total anomalous pulmonary venous connections could be predicted from preoperative individual pulmonary vein size [11, 12]. Using echocardiographic measurements, their data framed in a Cox proportional hazards model revealed that the small sum of individual pulmonary vein diameters, small confluence size, and presence of heterotaxy syndrome were each significant univariate predictors of survival. Patients with the heterotaxy syndrome in their study had significantly smaller pulmonary veins than those without heterotaxy. However, both by stratified analysis and by multivariate modeling, the strong association between individual pulmonary vein size and survival was independent of the presence or absence of heterotaxy.
Surgical management
The management of the newborn with right atrial isomerism and severely obstructed pulmonary venous connections and either severe pulmonary outflow tract obstruction or pulmonary atresia or the less common situation of the patient with right atrial isomerism but no pulmonary outflow tract obstruction carries a very substantial mortality [2, 8, 9]. Although some authors have advocated the surgical anastomosis of the pulmonary vein confluence to the back wall of the atrium without cardiopulmonary bypass [14], this has not substantially improved surgical results, at least in our institution [8]. For those patients with total anomalous pulmonary venous connections that are not obstructed or only mildly so, we advocate repair or redirection of the pulmonary venous connections at the time of staging with the bidirectional cavopulmonary connection. Any impedance to pulmonary venous return can disadvantage the peculiar Fontan circulation. Finally, despite the increasing number of reports documenting remarkable surgical successes in patients with right atrial isomerism [15–20], the overall outlook for patients with right atrial isomerism, severely reduced pulmonary blood flow, and obstructed pulmonary venous connections must remain guarded [8]. One might speculate that cardiac transplantation might be a better option for at least some of these patients.
References
This article has been cited by other articles:
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  M. Koudieh, E D. McKenzie, and C. D Fraser Jr Outcome of Glenn Anastomosis for Heterotaxy Syndrome with Single Ventricle Asian Cardiovasc Thorac Ann, June 1, 2006; 14(3): 235 - 238. [Abstract] [Full Text] [PDF]
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 T.-J. Yun, O. O. Al-Radi, I. Adatia, C. A. Caldarone, J. G. Coles, W. G. Williams, J. Smallhorn, and G. S. Van Arsdell Contemporary management of right atrial isomerism: Effect of evolving therapeutic strategies J. Thorac. Cardiovasc. Surg., May 1, 2006; 131(5): 1108 - 1113. [Abstract] [Full Text] [PDF]
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T.-J. Yun, J. G. Coles, I. E. Konstantinov, O. O. Al-Radi, R. M. Wald, V. Guerra, N. C. de Oliveira, G. S. Van Arsdell, W. G. Williams, J. Smallhorn, et al. Conventional and sutureless techniques for management of the pulmonary veins: Evolution of indications from postrepair pulmonary vein stenosis to primary pulmonary vein anomalies J. Thorac. Cardiovasc. Surg., January 1, 2005; 129(1): 167 - 174. [Abstract] [Full Text] [PDF]
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