Interstage mortality for functional single ventricle with heterotaxy syndrome: a retrospective study of the clinical experience of a single tertiary center

Abstract

BackgroundIn spite of improved survival after palliation for single ventricle, interstage mortality for a single ventricle with heterotaxy syndrome is unknown. The purpose of this study was to quantify interstage mortality and influence mortality risk factors.MethodsFrom November 1994 until February 2012, all patients that had a functional single ventricle and heterotaxy syndrome who underwent palliative operations at our center were included. Patients with hypoplastic left heart syndrome and operative mortality cases were excluded. The factors that influenced interstage mortality were determined by multivariate Cox analysis.ResultsThere were 16 patients with interstage mortality (41.0%), much higher than the non-heterotaxy group (vs. 11.3%, P = 0.001, OR = 5.478). The major presumptive causes of death were infection or sepsis (37.5%) and unknown sudden death (31.3%). When we compared the survival group and the mortality group with heterotaxy syndrome, Blalock-Taussig shunt as a 1st palliation is most common for both groups but there were more for the mortality group (81.2% vs. 52.2%), and there were more with bidirectional cavo-pulmonary shunt as a 1st palliation in the survival group (10 patients vs. 2 patients). The existence of pulmonary vein stenosis at initial diagnosis was more common for the mortality group. In multivariate Cox analysis, however, the duration of hospitalization at palliation, the duration of intensive care unit stay after palliation and the existence of pulmonary vein stenosis at diagnosis were significant risk factors.ConclusionInterstage mortality for a functional single ventricle with heterotaxy syndrome is significantly higher than for non-heterotaxy syndrome. Therefore more attention should be given to the prevention of interstage mortality in these patients with risk factors.

Survival in Children With Down Syndrome Undergoing Single-Ventricle Palliation

“…Patients with unbalanced AVSD were chosen for comparison, given that this lesion was present in most of the DS cohort. Patients with heterotaxy complex (isomerism) were excluded because of a known increased risk of death [10,11]. Clinical diagnosis, demographic characteristics, gestational age, dominant ventricle (morphologic left versus right), age at surgical procedure, and type of pre-SCPC surgical procedure (pulmonary artery band, systemic-to-pulmonary artery shunt, or other) were recorded.…”

Predictors of Mortality in Patients with Isomerism

“…These have included obstructed pulmonary venous connection, anomalous pulmonary venous connection, functionally univentricular circulation, congenital heart block, having noncardiac anomalies, mild or greater atrioventricular valvar regurgitation, having a common atrioventricular junction, aortic coarctation, need for neonatal surgery, and right ventricular outflow tract obstruction [19,[28][29][30][31][32][33][34][35][36][37][38][39]. None of these features, however, proved to be associated with mortality in our analysis.…”

Successful Palliation via Kawashima Procedure of an Infant With Heterotaxy Syndrome and Left-Atrial Isomerism

“…4 If circulation is relatively balanced prior to this step, as was the case with our patient, one of these procedures may be the first palliative step, meaning patients may be older at the time of their first intervention than those who require a Blalock-Taussig-Thomas shunt or pulmonary artery banding to balance their circulation. 10 Oxygen saturation is carefully monitored at follow-up, as formation of venous collaterals or development of arteriovenous malformations thought to be attributable to impaired delivery of vasoconstrictive cytokines from the liver are common at this stage. Patients who undergo a Kawashima procedure usually have a follow-up procedure in which the hepatocardiac venous blood is diverted to the pulmonary circulation to prevent pulmonary arteriovenous malformations.…”

“…11 Data show that mortality rates following completion of a Glenn/Kawashima procedure are lower compared to preprocedure mortality rates; therefore, performing the Glenn/Kawashima procedure as early as tolerated can be advantageous. 10,12 On the other hand, a concern is that redirection of the majority of the systemic circulation to the pulmonary vascular bed and a significant change in ventricular preload will be less easily tolerated in infants. 8,11 Another concern is that younger age at the time of the Glenn/Kawashima procedure appears to be related to greater risk of developing pulmonary arteriovenous malformations, although at least one study shows low rates of pulmonary arteriovenous malformations when Glenn/Kawashima procedures are performed in patients as young as 8 months.…”