Surgical treatment for patients aged above 5-month with heterotaxy with functional single ventricle and total anomalous pulmonary venous connection

Weidan CHEN; Xinxin CHEN; Shaoyi ZHENG

ABSTRACT

Objective:

To review the surgical outcomes for patients above 5-month old with heterotaxy syndrome with heterotaxy with functional single ventricle and total anomalous pulmonary venous connection(TAPVC) in a single center.

Methods:

From September 2008 to December 2018, there were 34 patients with heterotaxy syndrome with heterotaxy with functional single ventricle and TAPVC underwent cardiac operations at Guangzhou Women and Children Medical Center. 29 patients are aged above 5-month. The median age and body weight at surgery were 440 days(159 to 3 718 years) and 8.5 kg(4.9 to 19.2 kg). The type of TAPVC was supracardiac in 29 patients. All patients were diagnosed with complex cardiac anomaly, including complete atrioventricular canal defect 22 cases, tricuspid atresia 1 cases, mitral atresia 1 cases, atrioventricular valve regurgitation ≥moderate 6 cases, pulmonary venous stenosis 5 cases.

Results:

There were 4 died after initial operation. The causes of dead were pulmonary hypertension and low cardiac output syndrome. The follow-up duration was 11 to 130 months. 3 patients died of low cardiac output syndrome, protein-losing enteropathy and pulmonary venous stenosis respectively. Kaplan- Meier estimated survival at 1 and 5 years were 79.3% and 75.7%, respectively. 13 patients proceeded to a modified Fontan operation. Fontan completion was 44.8%(13/29). The mortality for patients with pulmonary venous stenosis(PVO) was 60%(3/5). Cox multivariate regression analysis indicated that preoperative PVO was the only risk factor for mortality.

Conclusion:

Delayed surgical treatment outcomes for patients with heterotaxy syndrome and TAPVC have improved significantly. Fontan completion was higher. However, preoperative PVO was the only risk factor for mortality, and outcomes for patients with PVO were still not promising. The outcomes could be better if these patients underwent initial operation at an early time. Early diagnosis and early intervention are still the principle strategy of surgical treatment of congenital heart disease.