Case Report: Nontuberculous mycobacterial infections in children with complete DiGeorge anomaly.

Children with complete DiGeorge anomaly (cDGA) have congenital athymia, resulting in severe T cell immunodeficiency and susceptibility to a broad range of infections. We report the clinical course, immunologic phenotypes, treatment, and outcomes of three cases of disseminated nontuberculous mycobacterial infections (NTM) in patients with cDGA who underwent cultured thymus tissue implantation (CTTI). Two patients were diagnosed with complex (MAC) and one patient with .

T Cell Repertoire Maturation Induced by Persistent and Latent Viral Infection Is Insufficient to Induce Costimulation Blockade Resistant Organ Allograft Rejection in Mice.

CD28:CD80/86 pathway costimulation blockade (CoB) with the CD80/86-specific fusion protein CTLA4-Ig prevents T cell-mediated allograft rejection in mice. However, in humans, transplantation with CoB has been hampered by CoB-resistant rejection (CoBRR). CoBRR has been attributed in part to pathogen-driven T cell repertoire maturation and resultant heterologous alloreactive memory.

Repair of Bicuspid Aortic Valve Syndrome with Anomalous Right Coronary Artery in Osteogenesis Imperfecta.

Osteogenesis imperfecta (OI) is an inherited connective tissue disorder caused by the defective synthesis of type I collagen. The clinical phenotype is dominated by bone fragility, but cardiovascular tissue involvement has also been reported. Here, the case is described of a 37-year-old man with OI who presented with aortic insufficiency, bicuspid aortic valve, dilated aortic root, and anomalous right coronary artery.

Elevated Cardiac Troponin I in Preservation Solution Is Associated With Primary Graft Dysfunction.

Background: Although primary graft dysfunction (PGD) is a leading cause of mortality and morbidity early post-heart transplant, relatively little is known regarding mechanisms involved in PGD development.

Methods And Results: We examined the relationship between cardiac troponin I (cTnI) concentrations in the preservation solution from 43 heart transplant procedures and the development of PGD. Donor hearts were flushed with cold preservation solution (University of Wisconsin [UW] or Custodiol) and stored in the same solution.

Surgical treatment of a cavernous haemangioma of the heart.

Cardiac haemangioma is an extremely rare, benign vascular tumour. A 42-year-old female patient who was previously very active presented with complaints of exertional chest pain as well as lower extremity oedema. Computed tomography and magnetic resonance imaging showed a large mass in the anterior mediastinum originating from the right atrioventricular groove and abutting the superior vena cava, right atrium, right ventricular outflow tract and aortic root.

Myocardial scar in sarcoidosis by 12-lead ECG and pathology.

This case demonstrates the use of QRS scoring to quantify myocardial scar in a patient with cardiac sarcoidosis and left bundle branch block who progressively received an implantable defibrillator, cardiac resynchronization therapy (CRT), left ventricular assist device and cardiac transplantation. QRS scoring has been shown to correlate with magnetic resonance imaging measurements of scar, identify arrhythmogenic substrate and predict response to CRT, but had not previously been compared to pathology-documented scar in nonischemic cardiomyopathies. Further study is warranted to assess the ability of QRS scoring to guide therapy for individual patients.

Reproducibility of left atrial ablation with high-intensity focused ultrasound energy in a calf model.

Objective: Achieving transmural tissue ablation might be necessary for successful treatment of atrial fibrillation. The purpose of this study was to evaluate the reproducibility of transmural left atrial ablation using a high-intensity focused ultrasound energy system in a calf model.

Methods: Nine heparinized bovines underwent a beating-heart left atrial ablation with a single application of the high-intensity focused ultrasound device.

Single-port cholecystectomy with the TransEnterix SPIDER: simple and safe.

Introduction: Single-port or single-incision cholecystectomy with current rigid laparoscopic devices is limited by in-line visualization, restricting the ability to approach the surgical site with proper angles and instrumentation. A single-port access system with articulating arms and strong instrumentation should minimize these issues. The TransEnterix system may facilitate safe and straightforward single-port surgery.

Histopathology and transmurality of acute microwave lesions on the beating human atrium.

Microwave energy allows thoracoscopic beating-heart ablation for the treatment of atrial fibrillation. However, there is a paucity of data on the histologic effects of microwave energy on the beating human heart. This study aims to histopathologically characterize microwave lesions on the beating human atrium.

Systolic contraction within aneurysmal rabbit myocardium following transplantation of autologous skeletal myoblasts.

Objective: Transplantation of autologous skeletal myoblasts (SKMB) into infarcted heart (or cellular cardiomyoplasty, CCM) augments myocardial performance in animal models of myocardial infarction. However, the effect of CCM in the setting of ventricular aneurysm has not been evaluated. This study analyzes the effects of transplanted SKMB on regional wall motion in a rabbit model of postinfarct ventricular aneurysm.