Background Deficient retro-aortic rim is of concern as a risk factor for aortic erosion following device closure of atrial septal defects (ASD). However, its prevalence and contribution to technical failure and adverse outcomes has not been delineated. Methods A single-center retrospective cohort study of children and adults undergoing cardiac catheterization for device occlusion of ASD from 1/1/1999 to 4/1/2012 was performed. Risk factors for technical failure and early adverse outcome were assessed using multivariate logistic regression. Results Four-hundred forty-five consecutive subjects of median age 5.9 years (range: 0.8–80 years) underwent catheterization during the study period. Of subjects with reviewable echocardiograms, 60% had deficient retro-aortic rim. No attempt was made at device closure in 3.6% of subjects. Of the remaining 429 subjects, 96% underwent successful device occlusion. Major early adverse events occurred in 1.2% (95% CI: 0.4–2.7%) of cases, all either device embolization or malposition. Deficient retro-aortic rim was not a risk factor for composite outcome of technical failure or early major adverse event. No deaths, late re-interventions, or erosion events occurred over 2,395 total person-years of follow-up (median 5.8 years). Deficient retro-aortic rim was associated with increased risk of device impingement on the aorta, but no association was seen between device impingement or deficient retro-aortic rim and the development of new/progressive aortic insufficiency. Conclusion Deficient retro-aortic rim is highly prevalent but did not increase risk of adverse outcomes. Its contribution to the risk of aortic erosion could not be addressed by this study.

Ectopia cordis (EC) is a rare congenital anomaly often associated with congenital heart disease (CHD). There is a lack of contemporary information on EC diagnosed prenatally. We sought to combine the experiences of two regional referral centers in order to evaluate current outcomes for EC. Clinical, echocardiographic features and perinatal outcomes of fetuses with EC managed at two large cardiac centers from 1995 to 2014 were retrospectively reviewed. Seventeen fetuses with EC were diagnosed at a median gestational age of 23 weeks (range 17-36). There were 6 thoracic EC and 11 thoracoabdominal. Fifteen had associated CHD: 10 conotruncal defects, 2 tricuspid atresia, 1 aortic stenosis, 1 atrial septal defect, and 1 atrioventricular septal defect. There were 2 terminations of pregnancy, 2 fetal deaths, 2 lost to follow-up, and 11 live born. Mean gestational age at birth was 36.4 weeks (range 26-39). Three patients died shortly after birth with comfort care, and 8 were actively managed. Six patients underwent postnatal cardiac intervention and are currently alive with a mean follow-up of 7.3 years (range 1.4-11.4), 2 of them with chronic dependency on ventilatory support. Two patients without CHD died after attempted chest closure. When diagnosed in utero, a high proportion of pregnancy termination or fetal demise is expected. In our cohort, conotruncal anomalies were the most common associated CHD. Though mortality in actively managed patients was not as high as previously reported, and cardiac surgical intervention may be achieved, EC is still associated with high mortality and significant long-term morbidity.

Current tetralogy of Fallot technical performance scores were not associated with selected outcomes in our postoperative population. Although returning to bypass and bypass length are not included as components in the current score, these are important factors influencing complications and resource use in our population. Revisions anticipated from a prospective trial should consider including these variables.