Outcomes of Multistage Palliation of Infants With Single Ventricle and Atrioventricular Septal Defect

Abstract

Background: Published palliation outcomes of infants with functional single ventricle (SV) and common atrioventricular septal defect (AVSD) are poor due to associated cardiac and extracardiac anomalies and development of atrioventricular valve (AVV) regurgitation. We report current palliation results. Methods: From 2002 to 2012, 80 infants with functional SV with AVSD underwent multistage palliation. Competing-risks analyses modeled events after first-stage surgery and Glenn (death/transplantation vs next palliation surgery) and examined factors associated with survival and AVV intervention. Results: Sixty-eight (80%) patients received neonatal palliation: modified Blalock-Taussig shunt (n = 33, 41%), Norwood (n = 20, 25%), and pulmonary artery band (n = 15, 19%), whereas 12 (15%) received primary Glenn. On competing-risks analysis, one-year following first-stage surgery, 29% of patients had died or received transplantation and 62% had undergone Glenn. Five years following Glenn, 9% of patients had died or received transplantation and 68% had undergone Fontan. Overall eight-year survival was 64% and was lower in patients with genetic syndromes (53% vs 82%), patients requiring concomitant total anomalous pulmonary venous connection repair (53% vs 69%), and those requiring neonatal palliation (48% vs 100%). Factors associated with mortality were unplanned reoperation (hazard ratio [HR]: 3.7 [1.7-8.0], P = .001) and extracorporeal membrane oxygenation use (HR: 7.1 [3.0-16.6], P < .001). Initial AVV regurgitation ≥ moderate was associated with AVV intervention (HR: 6.2 [2.4-16.1], P = .002) with eight-year freedom from death or AVV intervention of 25% in those patients. Conclusions: Patients with SV with AVSD are a distinct group and commonly have associated cardiac and extracardiac malformations that complicate care and affect survival. The development of AVV regurgitation requiring intervention is common but does not affect survival.

Commentary: Pulmonary artery banding in infants with atrioventricular septal defect, valid strategy or backward move?

“…Patients with unbalanced AVSD are at greater risk for mortality compared with other single-ventricle anomalies; this is due to many factors, including genetic conditions (association with Down and heterotaxy syndromes), extracardiac malformations, and associated cardiac problems (atrioventricular valve regurgitation, heart block, pulmonary venous anomalies, double outlet right ventricle, etc). 9 At the same time, those studies, along with the current series from Devlin and colleagues, highlighted specific problems related to PAB in patients with unbalanced ASVD, especially those with dominant right ventricle and associated arch obstruction. [8][9][10][11] In those patients, initial palliation with PAB might produce double-outlet obstruction by intensifying the degree of left ventricular outflow tract obstruction (a well-recognized problem).…”

“…9 At the same time, those studies, along with the current series from Devlin and colleagues, highlighted specific problems related to PAB in patients with unbalanced ASVD, especially those with dominant right ventricle and associated arch obstruction. [8][9][10][11] In those patients, initial palliation with PAB might produce double-outlet obstruction by intensifying the degree of left ventricular outflow tract obstruction (a well-recognized problem). A different strategy such as aortopulmonary amalgamation and shunt might be a better approach in those patients.…”

“…In that study, 8-year survival was 64% and was 100%, 55%, 43%, and 43% in those who had initial Glenn, Norwood, PAB, and aortopulmonary shunt, respectively. 9 Similarly, a study from Melbourne showed that 15-year survival was 66% following single-ventricle palliation of 139 patients with unbalanced AVSD. 10 An additional study from Atlanta examined outcomes of single-ventricle patients who were initially palliated with PAB.…”

“…7 In those conditions, PAB might seem necessary, despite the high-risk nature of this procedure. 2,8,9 In the current issue of the Journal, Devlin and colleagues 8 reviewed the Congenital Heart Surgeons Society complete AVSD cohort of patients (n ¼ 474) and examined 50 patients (11%) who received initial PAB with focus on atrioventricular valve function and survival based on the intended management pathway: clear eventual biventricular repair (n ¼ 24), definite single-ventricle repair (n ¼ 3), and deferred decision for biventricular repair candidacy (n ¼ 23). 8 Their findings were interesting (although not overly surprising) and basically emphasized the important correlation between outcomes following PAB and the intended surgical strategy that is fundamentally linked to underlying morphology.…”

Atrioventricular valve surgery: Restoration of the fibrous skeleton of the heart

“…16,17 Recently, we demonstrated that the outcomes of these patients were better than previously thought, with 60% of patients reaching Fontan completion and a 25-year survival of 80% in those patients who reached Fontan completion. 1,18 We have learned from this experience that it is crucial to have AVV competence to achieve a long-term survival in these challenging patients 2 as transplantation-free survival of those left with moderate or great AVV insufficiency was approximately 30% at 2 years after attempted AVV repair. 2 In sharp contrast, those who had common AVV replacement had 75% survival at 10 years.…”