Outcomes of single ventricle palliation in infants with heterotaxy syndrome.

Outcomes of single ventricle palliation in infants with heterotaxy syndrome.

Vodiskar J, Kido T, Strbad M, Cleuziou J, Hager A, Ewert P, Hörer J, Ono M.

Eur J Cardiothorac Surg. 2021 Mar 30:ezab141. doi: 10.1093/ejcts/ezab141.


Take Home Messages:

  • Single ventricle physiology with heterotaxy pertains a high mortality risk.
  • Restricted pulmonary blood flow and atrioventricular valve regurgitation are The two most important risk factors for mortality.
  • Providing unrestricted pulmonary blood flow and repairing total anomalous pulmonary venous connection offers survival advantages.


Commentary by Dr. Frederic Jacques (Québec city, Canada), chief section editor of Congenital Heart Surgery Journal Watch:  Heterotaxy syndrome is a recognized risk factor for overall poor outcome with single ventricle patients. This syndrome that mainly defines abnormal abdominal visceral configuration is associated with atrial appendages isomerism. Vodiskar et al. (Munich, Germany), reported their experience with 53 patients operated from 2001 to 2019. Their cohort comprises 66% right dominant ventricle, 49% total anomalous pulmonary venous connection, and 80% pulmonary stenosis/atresia. Thirteen patients were born with moderate to severe atrioventricular regurgitation. Consequently, these patients were treated with a non-heterogenous surgical protocol. In fact, 87% required some sort of neonatal palliation of which roughly one quarter died. A group of 41 patients underwent a bilateral cavopulmonary connection (17% as an initial surgery). About one tenth died. Of the 58% that reached the total cavopulmonary connection, none died. Unplanned reintervention occurred in 60% after Stage-1, mainly to better adjust pulmonary inflow, but also to address atrioventricular valve regurgitation. Through multivariate analyses, the authors identified restrictive pulmonary blood flow (HR 3.23; 95% CI 1.02–10.2; P = 0.05) and moderate to severe atrioventricular regurgitation (HR 3.57; 95% CI 1.27–10.0; P = 0.02) as independent risk factors for mortality. Providing a sustainable pulmonary inflow and correcting the pulmonary venous connection were two ways identified to come up with better survival.




This paper reports on a large cohort of patients with heterotaxy in addition to single ventricle physiology. Not surprisingly, this is a motley group of patients comprising various combinations of systemic and pulmonary venous connections, various types and degrees of outflow obstruction, as well as various atrioventricular valve configurations and degree of regurgitation. Nevertheless, the authors did an honest effort in trying to pinpoint risk factors for mortality and potential factors to tackle down in order to devise better outcomes. The findings echo intuitions, as with one functioning ventricle, one could expect that any inflow or outflow anomaly would lead to worse outcomes, and that correcting those would potentially reverse this burden, at least partially. The paper, just like similar previous papers from other authors, also highlight how limited this kind of single centre analysis is for such a rare disease. Even large cohorts from large institutions have somewhat been limited in their capacity to derive meaningful messages that go further what can be expected form the insight of common sense. In order to change in a more radical fashion the outcomes with this very difficult group of patients to deal with, multicentre strategies are likely mandated. An international registry comprising all the anatomical variables faced with the spectrum within the heterotaxy syndrome is needed in order to get more granular in the analysis of these patients.