Recurrent Pediatric Respiratory Distress From a Challenging Vascular Anomaly: The Uncrossing Operation for Circumflex Aorta.

The authors report a case of circumflex aorta causing persistent respiratory distress in a 9-year-old boy who had previously undergone vascular ring division and multiple aortopexies. The uncrossing operation was performed, with symptomatic relief of both posterior and right-sided tracheal compression from the transverse aorta and right aortic arch, respectively.

Transcriptomes and metabolism define mouse and human MAIT cell populations.

Mucosal-associated invariant T (MAIT) cells are a subset of T lymphocytes that respond to microbial metabolites. We defined MAIT cell populations in different organs and characterized the developmental pathway of mouse and human MAIT cells in the thymus using single-cell RNA sequencing and phenotypic and metabolic analyses. We showed that the predominant mouse subset, which produced IL-17 (MAIT17), and the subset that produced IFN-γ (MAIT1) had not only greatly different transcriptomes but also different metabolic states.

Norwood Aneurysm: Report of a Rare Phenomenon.

An 18-year-old male with complex single-ventricle physiology status post-3-stage palliation developed a large Norwood aneurysm (77 × 67 mm). The patient underwent a successful surgical reconstruction. Care providers must be aware of this rare complication and provide appropriate surveillance.

The number of postoperative surgical or diagnostic procedures following congenital heart surgery correlates with both mortality and hospital length of stay.

Background: Outcomes for congenital heart disease have dramatically improved over the past several decades. However, there are patients who encounter intraoperative or postoperative complications and ultimately do not survive. It was our hypothesis that the number of postoperative procedures (including surgical and unplanned diagnostic procedures) would correlate with hospital length of stay and operative mortality.

Tetralogy of Fallot and Aortic Dissection: Implications in Management.

We present the case of a 61-year-old man with tetralogy of Fallot postrepair and mechanical aortic valve replacement with an aortic root/ascending/arch aneurysm with chronic type A aortic dissection. He underwent uncomplicated aortic root and total arch replacement. Continued surveillance for aortic aneurysm is necessary in the tetralogy of Fallot population.

Pseudoaneurysm of Innominate Artery Graft Stump in an Infant.

Innominate artery grafts are often utilized in pediatric cardiac surgery and very rarely lead to complications, including infection. Here, we present a unique case of an infant who underwent repair of coarctation of the aorta and hypoplastic arch using a GORE-TEX graft (W. L.

Long-term Results Using Glutaraldehyde-treated Homograft Pericardium in Congenital Heart Surgery.

Background: This study reports the long-term outcomes using glutaraldehyde-treated cryopreserved homograft pericardium (CPH) in neonates, infants, children, and young adults undergoing congenital cardiac surgery.

Methods: A retrospective review was performed of all patients at a single institution (Rady Children's Hospital, San Diego, CA) who had undergone surgical implantation with CPH between 2006 and 2016. The study identified 134 consecutive patients who underwent implantation of a total of 276 patches.

Prevalence of Complications Following Unifocalization and Pulmonary Artery Reconstruction Procedures.

Background: Unifocalization and pulmonary artery reconstructions have been developed to treat complex disorders of pulmonary artery development. These procedures require extremely long periods of cardiopulmonary bypass (CPB) to facilitate surgical repair. The objective of this study was to document the prevalence of complications in patients undergoing unifocalization or pulmonary artery reconstructions associated with prolonged periods of CPB.

Aortopulmonary Window With Pulmonary Atresia and Interrupted Aortic Arch: A Very Rare Triad.

Aortopulmonary window (APW) is a rare lesion, accounting for 0.2% to 0.6% of all congenital heart diseases.

Successful Management of a Patient With Jacobsen Syndrome and Hypoplastic Left Heart Syndrome.

Jacobsen syndrome (JS) is a rare genetic condition characterized by intellectual disability, hematologic abnormalities, and congenital heart defects. A male infant presented at birth with phenotypic findings of JS and echocardiographic findings of hypoplastic left heart syndrome (HLHS). Array comparative genomic hybridization was performed at age three days and revealed an 8.

Fontan Revision: Presurgical Planning Using Four-Dimensional (4D) Flow and Three-Dimensional (3D) Printing.

Pulmonary arteriovenous malformations (AVMs) can be a complication of certain postoperative Fontan patients whose hepatic venous blood return is not distributed evenly to both lungs. A ten-year-old female, who had previously undergone staged single ventricle palliation for complex congenital heart disease, underwent a Fontan revision due to significant left-sided pulmonary AVMs and increasing arterial oxygen desaturation. The combination of four-dimensional flow cardiac magnetic resonance imaging and three-dimensional printing enabled presurgical planning for a Fontan takedown and diversion of hepatic venous flow to the azygous vein that resulted in significant clinical improvement.

Arterial Switch Operation in a Patient With Ehlers-Danlos Syndrome Type IV.

Ehlers-Danlos syndrome vascular type IV is characterized by translucent skin, easy bruising, and fragility of arteries. A full-term female infant presented at four weeks of age with a diagnosis of d-transposition of the great arteries with restrictive atrial septal defect. She successfully underwent emergent balloon atrial septostomy and placement of patent ductus arteriosus (PDA) stent.

Non-anastomotic failure of woven Dacron tube grafts in the thoracic aorta in young adults.

Background: Dacron tube grafts have been used in the surgical management of cardiovascular disease since the 1970s. Complications at the site of the anastomosis have been well described. Non-anastomotic failure is far less common.

Collateral Deception: A Unique Presentation of an Anomalous Coronary Artery.

• ARCAPA is a rare congenital anomaly. • Anomalous coronary artery origins are often isolated and diagnosed incidentally. • Echocardiography is the initial modality for diagnosing coronary artery anomalies.

Epicardial Intraoperative Three-Dimensional Mapping of Wolff-Parkinson-White Syndrome in a Child With Ebstein's Anomaly.

Catheter ablation of arrhythmias in small patients has inherent significant risk because of the patient, catheter, and lesion size. Heart block, perforation, and coronary damage may occur. This report shows adaptation of a three-dimensional electroanatomic mapping system for intraoperative use in a small patient with Ebstein's anomaly who was undergoing a cone procedure and a bidirectional Glenn procedure.

Per-Ventricular Insertion of Melody Valve-in-Valve in the Neoaortic Position in a Single-Ventricle Patient.

Percutaneous therapies for congenital heart disease have been evolving rapidly despite limited investment from industry. The Melody transcatheter pulmonary valve (Medtronic, Inc, Minneapolis, MN USA) replacement therapy represents an important advancement in this arena. It has been approved in the United States for use in the pulmonary position, on a Humanitarian Device Exemption status.

Esophageal Bronchus.

Communicating bronchopulmonary foregut malformations are uncommon anomalies. This report describes a case of "esophageal bronchus" managed successfully in a newborn with a nearly total pneumonectomy.

Intraoperative Three-Dimensional Mapping of Supraventricular Tachycardia in a Young Fontan Patient.

Preoperative tachycardia is uncommon before an initial Fontan procedure. Catheter intervention can risk complications in small patients. A patient with fetal tachycardia and double inlet left ventricle underwent Norwood and bidirectional Glenn procedures.