Anaesthesia concerns and perioperative management in a child with DiGeorge syndrome with corrected tetralogy of Fallot with pulmonary atresia posted for laparoscopic orchidopexy: Case report.

DiGeorge syndrome is afflicted with multiple congenital anomalies such as conotruncal and craniofacial anomaly, immune system dysfunction and hypoplasia/aplasia of parathyroid glands. Laparoscopy is a preferred surgical approach over open orchidopexy due to better visualisation of impalpable testis avoiding long incision, minimal tissue damage and a faster recovery. We report a case of DiGeorge syndrome with corrected tetralogy of Fallot with pulmonary atresia in a 1-year-old male child posted for laparoscopic orchidopexy.

Indian guidelines for indications and timing of intervention for common congenital heart diseases: Revised and updated consensus statement of the Working group on management of congenital heart diseases.

A number of guidelines are available for the management of congenital heart diseases (CHD) from infancy to adult life. However, these guidelines are for patients living in high-income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for CHD, as often these patients present late in the course of the disease and may have coexisting morbidities and malnutrition.

Key technical steps in dual-pathway repair of congenital pulmonary vein stenosis.

We present the case of a 2-year-old girl with congenital stenosis of the left inferior pulmonary vein associated with a large perimembranous ventricular septal defect. The child underwent repair of the left inferior pulmonary vein with autologous left atrial appendage as a pedicled tube, followed by closure of the ventricular septal defect. Important technical steps to minimize the restenosis rate are highlighted.

Intraoperative customized double-patch device with twin sutures for multiple muscular septal defects.

Objectives: Closure of multiple muscular ventricular septal defects (VSDs) remains a challenge because of anatomical complexity.

Methods: We mapped all the VSDs using en face reconstruction of the right ventricular septal surface through echocardiography and then performed an 'Intraoperative Customized Double-Patch Device' technique to surgically close them in 39 patients (male:female = 25:14). The median age of the patients was 6 months (2 months-10 years), and mean weight was 5.

Transposition of the Great Arteries With Total Anomalous Pulmonary Venous Connection.

Transposition of the great arteries (TGA) with total anomalous pulmonary venous connection (TAPVC) is a rare association. Very few such cases have been reported. Among them 1 patient underwent anatomic repair.

Right Posterior Thoracotomy for Open-Heart Surgery in a Rare Morphology.

The closure of atrial septal defects through right-sided limited posterior thoracotomy has been well established in selected subsets. We present a case of large ostium secundum atrial septal defect, pulmonary valvar stenosis, absent right superior vena cava, and isolated left superior vena cava draining to right atrium via coronary sinus. The child successfully underwent total correction through limited posterior thoracotomy with necessary modifications of intraoperative steps.

Modified pediatric Bentall procedure: A novel technique in a rare case.

Aneurysms of ascending aorta are rarely seen in pediatric age group. Only few cases with Marfans syndrome have been reported in the literature. Preferred treatment for these children has been the standard Bentall procedure (aortic root replacement with composite graft prosthesis).

An unusual fenestration in single-stage Fontan operation.

Fontan operation and importance of fenestration in the treatment of unusual and complex forms of double outlet right ventricle (DORV) are well established. Nonetheless, rarely, the creation of fenestration becomes challenging in complex morphologies. We present one such child with situs solitus, dextrocardia, DORV, hypoplastic right ventricle, large ventricular septal defect, severe pulmonic stenosis, extremely small right atrium and left juxtaposed atrial appendages, who underwent Fontan operation.

Complex aortopulmonary window in a single ventricle setting: Technical considerations for staged palliation.

We report a successful surgical management of a case presented with a combination of aortopulmonary window (APW) with large ventricular septal defect (VSD) amounting to a single ventricle, with a view to highlight technical considerations during staged single-ventricle palliation.

Closure of muscular ventricular septal defects guided by en face reconstruction and pictorial representation.

Background: A surface reconstruction of the location and dimensions of muscular ventricular septal defects (VSDs) on right ventricular (RV) septal surface could serve as a better guide to surgical closure amid different classifications and confusing terminologies.

Methods: We reconstructed muscular VSD requiring surgery on an en-face view of the RV septal surface from echocardiographic orthogonal views in 34 consecutive patients. The location, dimensions of the defects, and relation to various RV septal landmarks are illustrated as a diagram.