Early- and Middle-Term Surgical Outcomes in Patients with Heterotaxy Syndrome

Abstract

Objectives: Heterotaxy syndrome is a recognized risk factor for surgical cardiac interventions. We evaluated the early- and middle-term results of a surgical intervention for patients with heterotaxy syndrome. Methods: A total of 42 patients with heterotaxy syndrome were enrolled (September 2008 to March 2015). Left and right atrial isomerism were identified in 26% (11 out of 42) and 74% of patients (31 out of 42), respectively. The median age of the patients at the time of surgery was 6.8 months (range: 5 days to 22.3 years). Biventricular repair was completed in 3 patients with left atrial isomerism. Seventeen out of 39 patients who were scheduled for single ventricular repair completed a modified Fontan procedure. Results: The hospital mortality rate was 4.7% (2 out of 42). Another 5 deaths occurred in the remaining survivors following hospital discharge with a follow-up duration of 45.8 ± 23.6 months (range: 13-111 months). The 1-year and 5-year survival rates were 88.1% (37/42) and 83.3% (35/42), respectively. Univariate analysis and multivariate analysis identified pulmonary venous obstruction and atrioventricular valve replacement as additional risk factors for mortality. Conclusions: Right ventricular bypass surgery remains the preferred palliative procedure for patients with heterotaxy syndrome. Based on the current results, the early- and middle-term outcomes are satisfactory.

Cardiac surgery in patients with atrial isomerism: Long‐term results and outcomes

“…We found a predominance of RAI opposed to LAI, as reported in the literature, 13,14,16,[20][21][22][23][24][25] with a prevalence at our institution of 58.5% versus 41.5%, respectively. We agree with be appropriately diagnosed and classified in the preoperative setting with help from the auxiliary diagnostic team at our institution.…”

Changes in Prognosis of Heterotaxy Syndrome Over Time

“…HS has long been known to be a challenging disease with suboptimal short-and midterm outcomes. [4][5][6]12 Although some recent reports have suggested improved outcomes over time, [7][8][9]13,14 other studies have not found improved survival in the current era. 15,16 These discrepancies among reported outcomes in HS may, at least in part, be due to small sample sizes and short follow-up times in previous series.…”

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

“…Surgical outcomes for the Blalock-Taussig-Thomas shunt and the Glenn, Kawashima, and Fontan operations have improved over time, but overall, the prognosis for patients with heterotaxy syndrome is significantly worse than for patients with other congenital heart diseases, 5,21 possibly because the factors conferring increased mortality risk following these procedures-such as neonatal presentation requiring surgery, 22 pulmonary venous obstruction or stenosis, 10,22,23 atrioventricular valve dysfunction, [21][22][23] unplanned reoperation, 21 total anomalous pulmonary venous return, 21 extracorporeal membrane oxygenation use, 21 arrhythmia, 10 and longer duration of intensive care unit stay 10 -are more likely to be seen in patients with heterotaxy syndrome. In addition, functional asplenia in these patients carries a higher risk of sepsis, 10,17 and gastrointestinal issues can lead to poor feeding and poor weight gain.…”