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. 2021 Sep 22:8:664752.
doi: 10.3389/fcvm.2021.664752. eCollection 2021.

Outcomes of the Surgical Management of Atrial Isomerism and Functional Single Ventricle: A Single-Centered Cohort From China

Ming-Hui Zou  1   2 , Fan Cao  1   2 , Li Ma  1   2 , Wei-Dan Chen  1   2 , Wen-Lei Li  1   2 , Jia Li  1   2 , Xin-Xin Chen  1   2
Affiliations

Outcomes of the Surgical Management of Atrial Isomerism and Functional Single Ventricle: A Single-Centered Cohort From China

Ming-Hui Zou et al. Front Cardiovasc Med. .

Abstract

Objectives: The management of atrial isomerism with complex congenital heart disease remains challenging. Experience has been largely obtained in advanced countries. The clinical diversity is greater in China. We evaluated the early- and medium-term outcomes of surgical treatment of these patients. Methods: We reviewed 86 patients of atrial isomerism with complex congenital heart disease undergoing varied surgeries in our center in 2008-2020. Cox regression models were used to analyze the risk factors for mortality. Results: There were 75 cases of right and 11 of left atrial isomerism. Eighty-three (96.5%) patients underwent single-ventricle staged palliation approach, with 10 early and 7 late deaths. The overall 1-, 5-, and 10-year survival rates were 84.7, 79.3, and 79.3%, respectively. Thirty-six (43.4%) patients completed the Fontan procedure with median age of 48 months and freedom from death or Fontan failure at 1-, 5-, and 8-years were 94.4, 87.4, and 80.7%, respectively. Concomitant total anomalous pulmonary venous connection [hazard ratio (HR): 5.15 (1.95-12.94), p = 0.008], more than moderate atrioventricular valve regurgitation [HR: 4.82 (2.42-6.79), p = 0.003], and the need for first-stage palliative surgery [HR: 4.58 (1.64-10.76), p = 0.015] were independent risk factors for mortality. Conclusions: Despite even greater clinical diversity, the surgical outcomes of atrial isomerism with complex congenital heart disease are improving in China. The early and intermediate outcomes are comparable to many previous reports. Concomitant total anomalous pulmonary venous connection, moderate or severe atrioventricular valve regurgitation, and the need for a first-stage palliative surgery are still independent risk factors for mortality.

Keywords: Fontan; Heterotaxy syndrome; left atrial isomerism; right atrial isomerism; single ventricle.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Flow chart of interventions in 86 patients with atrial isomerism.
Figure 2
Figure 2
Survival for patients after first-stage palliative surgery.
Figure 3
Figure 3
Survival for patients after Glenn shunt.
Figure 4
Figure 4
Survival for patients after Fontan completion.
Figure 5
Figure 5
The Kaplan–Meier curves depict estimated survival. (A) Survival for the entire cohort of 86 patients. (B) Comparison of right and left atrial isomerism survival probability.
See this image and copyright information in PMC

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