Recurrent symptomatic aortic aneurysm in a young girl with tuberous sclerosis complex and review of the literature.

An abdominal aortic aneurysm is a rare occurrence in pediatric populations. When present, it is usually associated with an underlying etiology such as a connective tissue disorder, inflammatory process, or noninflammatory medial degeneration. In the present report, we describe the case of a girl with tuberous sclerosis complex who underwent successful emergency open repair of a symptomatic infrarenal abdominal aortic aneurysm and recurrent type IV thoracoabdominal aortic aneurysm.

The Congenital Heart Surgeons' Society Kirklin/Ashburn Fellowship: The Fellows' Perspective.

Since its establishment in 2001, the Congenital Heart Surgeons' Society John W. Kirklin/David Ashburn Fellowship has contributed substantially to the field of congenital heart surgery research while simultaneously training the next generation of surgeon- scientists. To date, ten fellows (and counting) have successfully completed this rigorous training, producing over 40 published articles focused on longitudinal outcomes from the various Congenital Heart Surgeons' Society cohorts. As the Kirklin/Ashburn Fellowship expands and additional fellows matriculate, its legacy, the network of support, and the contribution to congenital heart surgery research will undoubtedly hold strong.

Transcatheter Approaches to Palliation for Tetralogy of Fallot.

To this day, controversy still exists regarding the optimal method to treat symptomatic neonates and infants with Tetralogy of Fallot (TOF). Symptomatic (severely cyanotic or ductal dependent) infants with TOF can undergo either a staged repair approach (consisting of initial palliation followed by complete repair) or primary repair. Traditionally, initial palliative procedures have been surgical, for example placement of a Blalock-Taussig-Thomas (BTT) shunt. Recent advances in technology have facilitated the introduction of catheter-based procedures as palliative techniques, for example, patent ductus arteriosus (PDA) stenting and right ventricular outflow tract (RVOT) stenting as more durable solutions than balloon pulmonary valvuloplasty (BPV). In this article, we discuss the rationale for these procedures, technical aspects of these procedures and outcomes data compared to traditional surgical procedures. Recent data have suggested that RVOT and PDA stenting procedures offer many advantages over traditional surgical palliative procedures as palliative methods in this patient population. This comes at a cost of increased reintervention burden, which may be considered part of the overall treatment strategy in smaller neonates and can be minimized with a focus on technical aspects and overall treatment strategies. Advanced surgical techniques are required at the eventual complete repair to negotiate removal of stent material and pulmonary artery reconstruction in some instances. Further adoption of catheter based palliative procedures for infants with symptomatic TOF has the potential to tip the outcomes towards favoring a staged approach, particularly in high-risk infants.

Heart Transplantation in a Case of Scimitar Syndrome.

We present a rare clinical scenario of a patient with tetralogy of Fallot, hypertrophic cardiomyopathy, and concomitant scimitar syndrome. We created a scimitar vein cuff from a recipient heart, performed its translocation, and subsequently performed heart transplantation. This ingenuity reduces the likelihood of scimitar vein obstruction.

Perioperative evaluation of neonatal aortic arch thrombosis.

Herein, we present a neonatal case of coarctation of the aorta, with aortic arch thrombus confirmed by echocardiography. We performed thrombus removal and aortic arch repair emergently. This critical condition necessitates quick preoperative evaluation with echocardiography. Moreover, postoperative evaluation using computed tomography is reasonable to assess an aortic arch configuration, and exclude the remnant thrombus.

Rapid-deployment aortic valve replacement after aortic root replacement: A safe alternative to redo root replacement.

Reoperative aortic root replacement, following prior biologic or mechanical valved conduit aortic root prosthesis, presents a technical challenge. The rapid-deployment aortic valve prosthesis is an approved alternative to traditional bioprosthetic aortic valve replacement. We present three clinical cases in which rapid-deployment aortic valve prostheses were utilized in lieu of reoperative full aortic root replacement. All three patients recovered uneventfully. The rapid-deployment valve insertion in a prior surgical aortic root prosthesis is a safe option to avoid reoperative full aortic root replacement.

Annulus-Sparing Tetralogy of Fallot Repair: Low Risk and Benefits to Right Ventricular Geometry.

BACKGROUND: Annulus-sparing repair of tetralogy of Fallot (TOF) carries a potential increased risk of reoperation for restenosis and unproven benefits on right ventricular (RV) geometry. METHODS: Primary TOF repairs (n = 434) between 2000 and 2012 were studied using risk-adjusted parametric techniques. Progression of cardiac dimensions was analyzed using repeated measures regression using reports of all 2,103 echocardiograms undertaken throughout the study period, to a maximum follow-up of 13.7 years. RESULTS: Repair was at a mean age of 180 days: AS approach in 296 (68%) patients; and transannular patch in 138 (32%). Intraoperative revisions (for residual stenosis) were required in 135 patients (29%). There have been 4 deaths (survival 99%). Surgical reoperation for recurrent right ventricular outflow tract stenosis was occasionally required in both groups at comparable rates (transannular patch, 5 of 136 [4%]; annulus-sparing repair, 14 of 296 [5%]; p = 0.83). Larger increases in RV end-diastolic dimensions were evident in transannular patch patients versus annulus-sparing repair patients (p < 0.0001). Other risks for RV dilation included worse grade of postoperative pulmonary regurgitation, larger right ventricular end-diastolic dimension at the time of diagnosis, and higher operative weight (all p < 0.0001). Factors associated with successful annulus-sparing repair included (1) pulmonary annulus greater than 7 mm, right ventricular end-diastolic dimension greater than 1.2 cm, and tricuspid annulus greater than 1.4 cm (all preoperatively); and (2) right ventricular outflow tract diameter greater than 10 mm and right ventricular systolic pressure less than 50 mm Hg (both intraoperatively after repair). CONCLUSIONS: Pursuit of annulus-sparing repair strategies can lower the use of transannular patch to approximately 30% with low risk of reoperation for the patient. Annulus-sparing repair is associated with significantly reduced long-term RV dilation. Pulmonary valve enlargement to approximately 10 mm and right ventricular systolic pressure less than 50 mm Hg during annulus-sparing repair are associated with low risk of recurrent stenosis.