[III-S10-05] 内臓錯位症候群を伴う機能的単心室症の外科治療成績
Keywords:heterotaxy, single ventricle, Fontan
Background: Heterotaxy syndrome with single ventricle (SV) is frequently associated with variety of cardiac malformations, including anomalous pulmonary/systemic venous connections, atrio-ventricular (AV) valve incompetency, and rhythm disturbances, which are obstacles for Fontan completion.
Methods: Among 607 patients with SV (excluding HLHS) who underwent surgical treatment in our institution from 1995 to 2015, heterotaxy was diagnosed in 177 patients (right isomerism in 142, left isomerism in 35). Sixty-four patients (36.2%) had extra-cardiac total anomalous pulmonary venous connection, 66 patients (37.3%) demonstrated pulmonary atresia and 149 patients (84.2%) had a common AV valve. The course of surgical treatment and the results, predictors of mortality and hemodynamics after Fontan completion, were assessed. Follow-up time was 6.5±6.0 years.
Results: During the entire course of surgical treatment, 86 AV valve surgeries, 69 pulmonary artery plasties, 74 surgeries for pulmonary vein, and 5 pacemaker implantations were performed as additional procedures. There were 50 mortalities before and 5 mortalities after the Fontan operation, and 98 patients completed and 13 post-Glenn patients await Fontan operation. Actuarial survival at 5 and 10 years was 66.8% and 63.5%, which were significantly lower than that in non-heterotaxy patients (84.7% and 83.6%, p<0.0001). Multivariate analysis revealed that a predictor for mortality was AV valve surgery at the first palliative operation (p=0.007). Post-Fontan catheterization (n=93) showed a central venous pressure of 10.4±2.5 mmHg, ventricular end-diastolic pressure of 6.1±3.0 mmHg, and arterial oxygen saturation of 94.6±1.7%. Seventy-three patients (74.5%) demonstrated mild or less AV valve regurgitation at the last follow-up.
Conclusions: Heterotaxy syndrome with SV requires frequent additional procedures for associated cardiac malformations and demonstrates a higher mortality than non-heterotaxy patients. However, if patients undergo Fontan operation, satisfactory Fontan circulation can be achieved.
Methods: Among 607 patients with SV (excluding HLHS) who underwent surgical treatment in our institution from 1995 to 2015, heterotaxy was diagnosed in 177 patients (right isomerism in 142, left isomerism in 35). Sixty-four patients (36.2%) had extra-cardiac total anomalous pulmonary venous connection, 66 patients (37.3%) demonstrated pulmonary atresia and 149 patients (84.2%) had a common AV valve. The course of surgical treatment and the results, predictors of mortality and hemodynamics after Fontan completion, were assessed. Follow-up time was 6.5±6.0 years.
Results: During the entire course of surgical treatment, 86 AV valve surgeries, 69 pulmonary artery plasties, 74 surgeries for pulmonary vein, and 5 pacemaker implantations were performed as additional procedures. There were 50 mortalities before and 5 mortalities after the Fontan operation, and 98 patients completed and 13 post-Glenn patients await Fontan operation. Actuarial survival at 5 and 10 years was 66.8% and 63.5%, which were significantly lower than that in non-heterotaxy patients (84.7% and 83.6%, p<0.0001). Multivariate analysis revealed that a predictor for mortality was AV valve surgery at the first palliative operation (p=0.007). Post-Fontan catheterization (n=93) showed a central venous pressure of 10.4±2.5 mmHg, ventricular end-diastolic pressure of 6.1±3.0 mmHg, and arterial oxygen saturation of 94.6±1.7%. Seventy-three patients (74.5%) demonstrated mild or less AV valve regurgitation at the last follow-up.
Conclusions: Heterotaxy syndrome with SV requires frequent additional procedures for associated cardiac malformations and demonstrates a higher mortality than non-heterotaxy patients. However, if patients undergo Fontan operation, satisfactory Fontan circulation can be achieved.