Atrioventricular Septal Defects: Lessons Learned About Patterns of Practice and Outcomes From the Congenital Heart Surgery Database of the Society of Thoracic Surgeons

Abstract

During the 4-year time interval of 2005 through 2008, the Society of Thoracic Surgeons Congenital Heart Surgery Database documented data about 2882 operations to repair atrioventricular (AV) canal defects: partial, 623 (21.5%); intermediate, 342 (11.8%);. complete, 1917 (66.3%). Mean age at complete repair (years) was as follows: partial, 6.1; intermediate, 2.9; complete, 0.6. Median age at complete repair (years) was as follows: partial, 2.6; intermediate, 0.9; complete, 0.4. Down syndrome was present in 1767 patients (61.1%). Debanding of the pulmonary artery was rarely performed: partial, 1 (0.2%); intermediate, 0 (0.0%); complete, 66 (3.4%). Deep hypothermic circulatory arrest was rarely used: partial, 6 (1.0%); intermediate, 5 (1.5%); complete, 52 (2.7%). Discharge mortality was low: partial, 2 (0.3%); intermediate, 3 (0.9%); complete, 38 (2.0%). Atrioventricular block requiring permanent pacemaker occurred but was uncommon: partial, 6 (1.0%); intermediate, 2 (0.6%); complete, 29 (1.5%). Unplanned reoperation prior to hospital discharge occurred in 3.9% of complete AV canal repairs. The sternum was left open in 3.0% of complete AV canal repairs. Postoperative cardiac arrest occurred in 1.9% of complete AV canal repairs. Mean postoperative length of stay (days) was as follows: partial, 5.2; intermediate, 7; complete, 13.1. Median postoperative length of stay (days) was as follows: partial, 4; intermediate, 4; complete, 7. This review of data from the Society of Thoracic Surgeons Congenital Heart Surgery Database allows for unique documentation of patterns of practice and outcomes. From this review, we learned that 98% to 99% of patients survive complete repair of AV canal and 96% to 97% survive complete repair of AV canal with no major complications.

Results of Palliation With an Initial Modified Blalock-Taussig Shunt in Neonates With Single Ventricle Anomalies Associated With Restrictive Pulmonary Blood Flow

“…The presence of obstructed TAPVC repair results in varying degrees of postoperative lung pathology and elevated pulmonary vascular resistance that complicate the postoperative management of those patients in the intensive care unit. The high incidence of associated intracardiac anomalies, particularly atrioventricular valve regurgitation and conduction abnormalities, in addition to the prevalence of extracardiac malformations in patients with heterotaxy, add to the challenge [12][13][14][15]. Similarly, outcomes of SV patients with PAIVS continue to be unfavorable and multiple studies have identified that PAIVS was an independent risk factor for mortality after mBTS [2,8,16].…”

“…A recent STS study examining hospital survival of 1,505 patients with heterotaxy who underwent surgery demonstrated that discharge mortality was higher in patients with heterotaxy compared with patients without heterotaxy for every procedure mortality risk category. Specifically, operative mortality after mBTS was 6.8% for SV patients other than heterotaxy compared with 10.8% in those with heterotaxy [12]. The association of TAPVC in heterotaxy patients is especially complicated and reported outcomes from various institutions continue to show high mortality rates [13][14][15].…”

“…We identified that survival was significantly worse in neonates born with PAIVS or atrial isomerism. Atrial isomerism, associated with the heterotaxy syndrome, continues to be a challenge [7,[12][13][14][15]. A recent STS study examining hospital survival of 1,505 patients with heterotaxy who underwent surgery demonstrated that discharge mortality was higher in patients with heterotaxy compared with patients without heterotaxy for every procedure mortality risk category.…”

Single Ventricle Palliation in Low Weight Patients Is Associated With Worse Early And Midterm Outcomes

“…The higher risk of early and late mortality in heterotaxy patients is due to the high incidence of associated intracardiac defects, particularly total anomalous pulmonary venous connection, atrioventricular valve regurgitation, and conduction abnormalities in addition to extracardiac anomalies affecting outcomes beyond hospital discharge [23][24][25]. In patients with PA/ IVS, the increased mortality is attributed to the higher prevalence of ventricular to coronary arteries sinusoids and right ventricle dependant coronary circulation with subsequent tenuous physiologic state and higher risk of ischemia and cardiac arrest prior to or after palliation [16,26,27].…”

Contemporary outcomes of complete atrioventricular septal defect repair: Analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database

“…Outcomes following surgical repair have improved over several decades due to refinements of technique and postoperative management. 1–7 The age for elective repair has steadily declined, from as late as 1 year a few decades ago to 3 to 6 months at most centers today. 6,8–12 Early repair is intended to minimize the risk of premature death or pulmonary vascular obstructive disease.…”