Giant right atrial aneurysm is a rare form of congenital heart disease with a wide spectrum of clinical presentation varying from asymptomatic patients to those with refractory atrial arrhythmias or severe airway obstruction. Diagnosis is often confused with other causes of right atrial dilation such as Ebstein disease. Because of its rare occurrence and variable clinical presentation, inconsistencies in medical and surgical management strategies exist between centers. We present five cases of giant right atrial aneurysm managed at our institution and discuss the clinical presentation, diagnostic challenges, and medical and surgical management.

These data indicate that for up to 120 months of patient follow-up, the ASO continues to be a safe device. Residual shunts and arrhythmias have low incidence post-ASO placement. Given the mortality in one high-risk patient, further investigation into anti-platelet therapy after device placement is warranted.

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Cryopreserved pulmonary allograft is frequently used to reconstruct the hypoplastic aortic arch. A decellularized graft preparation is available as an alternative reconstructive material. This case series profiles our experience using this material for neonatal aortic arch reconstruction. Data from 14 patients who received decellularized pulmonary allograft (DCPA) from 2001 to 2003 included the following: diagnosis; age at implantation; perioperative complications (graft bleeding or dehiscence); intermediate-term results, including infection, recurrent arch gradient (>10 mmHg), graft calcification, or aneurysm; need for graft removal; and cause of death. Eleven (79%) patients survived their initial procedure. Median follow-up was 26 (range: 1-110) months. No early graft dehiscence was reported. No child developed aortic arch obstruction or graft calcification. Twelve patients (86%) underwent postoperative cardiac catheterizations with no measurable arch gradient. No intermediate-term aneurysm, dehiscence, or graft removal occurred. Three early deaths were due to arrhythmia, non-shunt-related hypoxia, and renal failure, and one, intermediate-term death was respiratory syncytial virus related. This series found that DCPA reconstructed arches have a low adverse event profile. No complications occurred. Because this graft material, which may be less immunogenic, has important implications for this subgroup, who are at increased future transplant risk, larger trials evaluating longer-term safety and immunogenicity are warranted.