Concomitant valve-sparing aortic root replacement with tetralogy of Fallot repair in a 30-year-old patient: A case report.

INTRODUCTION AND IMPORTANCE: Aortic regurgitation and aortic root dilatation are common features in adults with both repaired and unrepaired tetralogy of Fallot (TOF). Valve-sparing aortic root replacement (VSARR) is an effective repair for aortic regurgitation due to progressive aortic root dilatation with TOF after repair. However, the effectiveness of this technique for unrepaired patients has rarely been reported. CASE PRESENTATION: We reported a case of a 30-year-old man with cyanosis and exertional dyspnea. Echocardiography and computed tomography showed unrepaired TOF with significant aortic regurgitation and massively dilated aortic root. Aortic root replacement was mandatory. He underwent successful concomitant VSARR with TOF repair. At 6-month follow-up, he remains stable with trivial aortic regurgitation on echocardiogram. CLINICAL DISCUSSION: In unrepaired TOF, the absence of sub-annular muscular rim and the unbalance of aortic sinuses make VSARR utmost challenging. If can be done successfully, VSARR preserves the native aortic valve and avoids lifelong anticoagulation therapy. CONCLUSION: Concomitant VSARR with TOF repair can be safely and effectively applied for unrepaired patients presenting with progressive aortic root dilatation and significant aortic regurgitation.

Interleukin-23 receptor signaling by interleukin-39 potentiates T cell pathogenicity in acute graft-versus-host disease.

IL-12 (p35/p40) and IL-23 (p19/p40) signal through IL-12R (IL-12Rß2/ß1) and IL-23R (IL-23Rα/IL-12Rß1), respectively, which can promote pathogenic T lymphocyte activation, differentiation, and function in graft-versus-host disease (GVHD). With the use of murine models of allogeneic hematopoietic cell transplantation (HCT), we found that IL-12Rß1 on donor T cells was dispensable to induce acute GVHD development in certain circumstances, while IL-23Rα was commonly required. This observation challenges the current paradigm regarding IL-12Rß1 as a prerequisite to transmit IL-23 signaling. We hypothesized that p19/EBI3 (IL-39) may have an important role during acute GVHD. With the use of gene transfection and immunoprecipitation approaches, we verified that p19 and EBI3 can form biological heterodimers. We found that IL-39 levels in recipient serum positively correlated with development of acute GVHD in experimental models and in clinical settings, thereby implicating IL-39 in the pathogenesis of acute GVHD. Furthermore, we observed that human T cells can signal in response to IL-39. In chronic GVHD, IL-23Rα and IL-12Rß1 were similarly required for donor T cell pathogenicity, and IL-39 levels were not significantly different from controls without GVHD. Collectively, we identify a novel cytokine, IL-39, as a pathogenic factor in acute GVHD, which represents a novel potential therapeutic target to control GVHD and other inflammatory disorders.

Successful repair of critical bronchial stenosis with coexisting bronchomalacia and congenital heart defects in infants by slide bronchoplasty.

Isolated bronchial stenosis in infancy is rare, difficult to manage and becomes more complex when associated with congenital heart defects. Given the small luminal diameter and the proximity of the lung parenchyma to the bronchial lesion in infants, reconstruction of the bronchial stenosis is surgically challenging. We present 2 infant cases having isolated critical bronchial obstruction with congenital heart defects, which were successfully managed by primary one-stage slide bronchoplasty with concomitant heart repair. Both cases had excellent results after the surgery and did well at home without requiring respiratory support.