Valve Sparing Aortic Root Replacement in Children with Loeys-Dietz Syndrome

Loeys-Dietz syndrome (LDS) is an autosomal dominant connective tissue disorder that is characterized by aggressive arterial and aortic disease, often involving the formation of aortic aneurysms. We describe the cases of two children with LDS who were diagnosed with aortic root aneurysms and successfully treated by valve-sparing aortic root replacement (VSRR) with a Valsalva graft. VSRR is a safe and suitable operation for children that avoids prosthetic valve replacement.

Venovenous Extracorporeal Membrane Oxygenation for Postoperative Acute Respiratory Distress Syndrome

BACKGROUND: Extracorporeal membrane oxygenation (ECMO) has recently attracted interest as a treatment for severe acute respiratory distress syndrome (ARDS). However, the outcomes of this procedure in post-surgical settings have not yet been characterized. In this study, we evaluated the outcomes of ECMO in patients with severe postoperative ARDS. METHODS: From January 2007 to December 2012, a total of 69 patients (aged 58.3+/-11.5 years, 23 females) who underwent venovenous ECMO to treat severe postoperative ARDS were reviewed. Of these patients, 22 (31.9%) had undergone cardiothoracic surgery, 32 (46.4%) had undergone liver transplantation, and 15 (21.7%) had undergone other procedures. RESULTS: Thirty-four patients (49.3%) were successfully weaned from ECMO, while the other 35 patients (50.7%) died on ECMO support. Among the 34 patients who were successfully weaned from ECMO, 21 patients (30.4%) eventually died before discharge from the hospital, resulting in 13 hospital survivors (18.8%). Multivariable analysis showed that the duration of pre-ECMO ventilation was a significant independent predictor of death (odds ratio [OR], 2.25; 95% confidence interval [CI], 1.29 to 3.90; p=0.004), whereas the concomitant use of continuous venovenous hemodialysis (CVVHD) was associated with improved survival (OR, 0.55; 95% CI, 0.31 to 0.97; p=0.038). CONCLUSION: Although the overall survival rate of patients treated with ECMO for postoperative ARDS was unfavorable, ECMO offered an invaluable opportunity for survival to patients who would not have been expected to survive using conventional therapy. CVVHD may be beneficial in improving the outcomes of such patients, whereas a prolonged duration of pre-ECMO ventilator support was associated with poor survival.

Translocation of the Aortic Arch with Norwood Procedure for Hypoplastic Left Heart Syndrome Variant with Circumflex Retroesophageal Aortic Arch

Retroesophageal aortic arch, in which the aortic arch crosses the midline behind the esophagus to the contralateral side, is a rare form of vascular anomaly. The complete form may cause symptoms by compressing the esophagus or the trachea and need a surgical intervention. We report a rare case of a hypoplastic left heart syndrome variant with the left retroesophageal circumflex aortic arch in which the left aortic arch, retroesophageal circumflex aorta, and the right descending aorta with the aberrant right subclavian artery encircle the esophagus completely, thus causing central bronchial compression. Bilateral pulmonary artery banding and subsequent modified Norwood procedure with extensive mobilization and creation of the neo-aorta were performed. As a result of the successful translocation of the aorta, the airway compression was relieved. The patient underwent the second-stage operation and is doing well currently.

Tuberculous Aortitis Complicated with Pseudoaneurysm Formation in the Descending Thoracic Aorta: A Case Report

A 51-year-old male with sustained fever was diagnosed with military tuberculosis and tuberculous aortitis complicated with pseudoaneurysm formation at the proximal descending aorta. A follow-up computed tomography evaluation showed an increased size of the pseudoaneurysm in this area, suggestive of a contained rupture. Consequently, the patient underwent emergency excision and replacement of the aorta using a left heart bypass. The patient was discharged without postoperative complications on post-operative day 12. During the one-year follow-up period, the patient was free of any complications or recurrence of tuberculosis. We report a case of pseudoaneurysm of the descending aorta that was successfully surgically repaired.